REVIEWS

Cyanotic Congenital

MARY New

ALLEN

ENGLE,

MD,

Heart Disease

FACC

York, New York

From the Division of Pediatric Cardiology, The New York Hospital-Cornell University Medical Center, New York, N. Y. This work was supported in part by Training Grant 5 TO 1 HL 05989-03 and Research Grant 5 RO 1 HL 16246-02 from the National Institutes of Health, Bethesda, Md. Manuscript accepted April 23, 1975. Address for reprints: Mary Allen Engle, MD, Division of Pediatric Cardiology, The New York Hospital, 525 E. 68th St., New York, N.Y. 10021.

This review describes the evolving concepts of diagnosis and management of patients with cyanotic congenital heart disease. Early palliative surgical procedures were followed by reparative operations and are now to a large extent replaced by these operations which are designed to relieve the problem. Collaboration of the team of cardiologists, surgeons, radiologists, anesthesiologists and nurses has made the many developments possible. The teamwork has not only widened the scope of what can be accomplished but has also extended the opportunities for beneficial reparative surgery down to the first weeks and months of life. Precise diagnosis and meticulous operative and perioperative care by the team are essential elements of success. Long-term follow-up and regular analyses of results have led to continuing improvements. Although these patients were born to be blue, their color and their outlook have been changed during these last 3 decades to something close to rosy.

Physical disability and premature death are the lot of the cyanotic person unless his condition can be relieved by surgery and complications prevented or ameliorated by medical measures. Breathlessness and easy fatigue on exertion limit activities. Growth rate is usually impaired. Cyanosis and clubbing are cosmetic handicaps. So is scoliosis, which by adolescence is common and is sometimes rapidly progressive. In addition to the risk of infectious endocarditis there is a special hazard of cerebral complication in the form of brain abscess or cerebrovascular thrombosis or embolism. Poor dental structure with defective enamel and dental caries contributes to the risk of brain abscess. Polycythemia is associated with headache and with problems of bleeding or clotting. Some men with red cell breakdown and hyperuricemia have gout. If the hypoxic, polycythemic woman is able to become pregnant, she has a greater than average risk that the pregnancy will result in abortion or in a baby with intrauterine growth retardation who may also have congenital heart disease. Cardiac failure or renal impairment are late consequences of the unrelieved cardiac burden and hypoxia. Sudden death is not an uncommon event in cyanotic patients with Ebstein’s anomaly or with pulmonary vascular obstructive disease, or in patients with unstable cardiac rhythm or conduction occurring naturally or postoperatively. Progress

Report

The year 1974 marked the 30th anniversary of a new way of life for most blue babies. It was November 1944 when Eileen Saxon’s color changed from blue to pink and she stopped suffering from the attacks of paroxysmal dyspnea that had characterized her few months of life. That miracle transpired as a result of teamwork that combined the diagnostic acumen of Helen Taussig and the surgical skill of Alfred Bla1ock.i These two Hopkins’ doctors had the courage to apply

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Taussig’s ideas about increasing deficient pulmonary blood flow and Blalock’s experimentally tested technique of vascular anastomoses. Their collaboration led to the development of two interdependent new specialties: pediatric cardiology and cardiovascular surgery. Improvements in diagnostic techniques and both medical and surgical capabilities leapfrogged over the next decades. Teams expanded to include pediatric and medical cardiologists, cardiovascular anesthesiologists, surgeons, radiologists, physiologists, pathologists, pharmacologists, nurse specialists, respiratory therapists, technicians, social workers and experts in rehabilitation. Centers of excellence were erected where the concentration of human skills and physical facilities made optimal care available 24 hours a day. The patients with cyanotic congenital heart disease who have benefited range from the high risk newborn with a critically severe malformation to the older patient who survived into adult life either because of the relatively mild nature of his malformation or as the result of previous surgical intervention. Some of the medical and surgical highlights of the challenge and conquest in this field are shown in Tables I and II. The state of the art in rendering care to the cyanotic patient has developed to a high level. Palliative procedures to increaseLJL7,125J32 or to decrease’s2 pulmonary blood flow, as the case required, or to reroute venous return120J2g,133 were followed by reparative surgery designed to correct or to relieve the malformation 118,119,124,126,127,130,131,134,136-139,141,142 Only a few anomalies are still beyond such help. Skills in diagnosis and in operative and perioperative management now allow open heart surgery to be carried out safely even in the neonate.140J43-146 As accomplishments have advanced, so too has evaluation of early and long-term results of management. Attention has been directed not only to the expected good results of operation but also to recognition and treatment or prevention of undesirable sequelae or complications.147-175 Alth ough the great majority of patients enjoy considerable benefit from surgery, only in the rare instance is “total” correction of the malformation a reality. As we review the status of cyanotic congenital heart disease, let us first consider diagnosis and then management, for these go hand in hand. Diagnostic

Categories

Cyanotic congenital heart disease can be divided into categories based on the degree of pulmonary blood flow, excessive or deficient, and on the pressure in the pulmonary circuit. In all anomalies in which there is cyanosis, some abnormal communication exists that permits right to left shunting at one or more of these levels: atrial, ventricular or vascular (venous return or arterial outlet). But it is the status of pulmonary blood flow that to a great extent determines the ability of the individual patient to survive, function and undergo cardiac surgery.

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When such a large communication exists and the capacity of the pulmonary bed and the left heart is unrestricted and the pulmonary vascular resistance is unusually low, the pulmonary flow may be torrential, increased to four- or five-fold greater than normal. The lungs are literally flooded. Such a state is not a steady one. Either death occurs or some adjustments take place that limit the excessive pulmonary flow to a near-normal amount or even restrict it so that it is less than normal. These adjustments are in the form of pulmonary stenosis, pulmonary arterial obstruction or spontaneous decrease in size or closure of the defect. One or the other of these limiting factors may be present at birth or may develop during the childhood years. Once established, that adaptation may continue stable or may progress, so that pulmonary stenosis may develop,176 or pulmonary stenosis may convert to pulmonary atresia,150 or increased tone of small pulmonary arteries may proceed to fibrosis and obliteration.177-L80 A noncyanotic condition at birth may convert to a cyanotic one, or a mildly cyanotic condition may become more deeply so through the mechanism of these adaptations. In the overall consideration of cyanotic heart disease in general and of any one patient in particular, this spectrum of changes in the pulmonary flow, pressure and resistance should be borne in mind. Although there is a spectrum, the points along the way are recognizable. Excessive

Pulmonary

Blood Flow

In the cyanotic person, excessive pulmonary blood flow implies both right to left and left to right shunting. This shunting occurs when the great arteries or the pulmonary veins are transposed or malposed, when there is common mixing because of a single atrium, ventricle or aorticopulmonary trunk or when there is a hypoplastic left heart syndrome. The ease of bidirectional mixing and the resistance to pulmonary flow (pre- and postcapillary) determine the volume of pulmonary blood flow, and this in turn affects the degree of cyanosis. When there is no obstruction to flow or to mixing, then the lower the pulmonary resistance, the greater the pulmonary flow in relation to the systemic flow and the greater the oxyhemoglobin saturation of the admixed blood reaching the aorta. A patient with this condition is apt to present with cardiac failure and to be only minimally cyanotic. He has hyperkinetic pulmonary hypertension. A baby with complete transposition of the great arteries and a large ventricular septal defect typically presents in this way. In contrast, the most deeply cyanotic patients in this group have obstruction to mixing or to pulmonary venous return, or both. Examples are the newborn infant with transposed great arteries and an otherwise normal heart or the baby with obstructed anomalously draining pulmonary veins and a small patent foramen ovale. In these anomalies with increased pulmonary flow, the pulmonary arterial pressure is slightly to moderately increased. The presence of pulmonary stenosis

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TABLE

TABLE

I

Medical Progress in Cyanotic A Continuum, 1945-1975

Congenital

Development for Cyanotic

Heart Disease:

Team approach to diagnosis and treatment’-5 Antibiotics for control of infection6-* Prevention of endocarditis9’r0 Treatment of endocarditisrr-I3 Cardiac failure, recognition and management’4-‘9 Natural history of congenital heart diseasezo-23 Infancy, the risk period241a Early detection, diagnosis and continuing care5729-3’ Fetal, neonatal circulatory adjustments3z-34 Cardiopulmonary interrelationships and function35,36 Anesthetic, perioperative management3’ Hematologic adaptations3*‘39 Anatomy and function of: Cardiac structures40,41 Conduction system42-48 Embryology of cardiovascular malformations49--56 Cardiac catheterization: sophistication, miniaturization, trols, monitoring57-68 Contrast visualization Intravenous angiocardiography69-71 Selective angiocardiography61~7*74 Electrovectorcardiography75-8’ Phonocardiography82-84 Electrophysrologyss-88 His bundle electrograms89-91 Pacema kers92-94 Echocardiography95-‘00 Radiology’0’-‘03 Dysrhythmias: detection and treatment’04-‘09 Cardiopulmonary resuscitation”“,“’ Intensive care cardiorespiratory units5’“*-‘r4 Transport system5~‘14~“5 Stratified system of optimal cardiac carelI Cardiovascular center’r6

II of Surgical Procedures Congenital Heart Disease Reparative Procedure

Palliative Procedure

Year 1945

Blalock-Taussig’; subclavianRPA, LPA anastomosis

1946 1948

Potts:“’

aorta-LPA

anastomosis

1949 1950 1951

Blalock-Hanlon:‘20 tectomy Muller-Damman:‘22 arterial band

atrial seppulmonary

Gibbon:rz3 pump oxygenator Lewis-Taufic:lZ4 ASD closure under hypothermia

1952 safety, con-

1953 1954 1955

Glenn: I” SVC-RPA

1956

Boffes:“*partial venous detour for CTGA (RP veins-RA; IVC-LA)

anastomosis Lillehei:‘26 controlled cross circulation for open heart surgery Kirklin:lz7 Gibbon pumpoxygenator for open heart surgery

1959

1961 1962

. Waterston:r3’ ascending aortaRPA anastomosis

1964

pulmonary vascular obstruction of slight to moderate degree cuts down somewhat the excessive pulmonary flow and alters the pulmonary pressure. Increasing amounts of either pulmonary stenosis or vascular obstruction convert this anomaly into a situation with deficient pulmonary flow. or

Deficient

Pulmonary

1965

1966

1967

Flow

In the cyanotic patient deficient pulmonary flow signifies impairment of flow to or through the lungs. Without pulmonary hypertension: When flow into the lungs is inferfered with, the precapillary pulmonary arterial pressure is normal or less than normal. There is usually obstruction to pulmonary flow in the form of pulmonary stenosis or atresia but sometimes the obstruction is more proximal in the heart in the form of tricuspid stenosis, atresia or insufficiency. Rarely, the pulmonary circuit is bypassed by anomalous drainage of vena caval blood into the left atrium.1s1J82 The most common malformation in this group, at all ages, is the tetralogy of Fallot in which moderate or severe pulmonary stenosis coexists with a large high ventricular septal defect. Some conditions with shunting at ventricular level mimic a tetralogy (Table III). In these, severe pulmonary stenosis or atresia is

1968 1970

1971 1972

Brock:“’ pulmonary valvotomy Sellors:1’9 pulmonary valvotomy Bigelow:“’ hypothermia

Rashkind:r3s tostomy

balloon atrial sep-

Senning: I’9 intraatrial rerouting of venous return for CTGA Kirklin:“” VSD closure, infancy Sloan:‘3’ VSD closure, infancy Mustard:‘33 intraatrial baffle for rerouting venous return in CTGA Lillehei:‘34 tricuspid valve prosthesis, Ebstein’s anomaly Ross-Somerville: ‘X homograft aortic valve, pulmonary atresia Dillard:‘37 deep hypothermia, circulatory arres for total anomalous pulmonary venous return Rastelli:r3’ homograft aorta and valve for truncus arteriosus Rastelli:‘39 atrioventricular canal, repair Barratt-Boyes:r40 deep hypothermia and cardiopulmonary bypass for intracardiac surgery, infancy Fontan:14’ tricuspid atresia operation Sakakibara:“” repair single ventricle

ASD = atrial septal defect; CTGA = complete transposition of the great arteries; IVC = inferior vena cava; LA = left atrium; LPA = left pulmonary artery; RA = right atrium; RP = right pulmonary; RPA = right pulmonary artery; SVC = superior vena cava; VSD = ventricular septal defect.

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TABLE

III

Tetralogy-Like and Pulmonary

_

Anomalies With Ventricular Stenosis or Atresia

Shunting

1. 2.

Pentalogy of Fallot ftetralogy plus atrial septal defect) Restrictive ventricular septal defect and severe pulmonary stenosis 3. Pseudotruncus arteriosus (large ventricular septal defect with pulmonary atresra and systemic arterial supply to one or both pulmonary arteries) 4. Type IV truncus arteriosus (as above except for absence of demonstrable pulmonary arteries) 5. Tetralogy with absent pulmonary valve, pulmonary insuffiency and aneurysmal dilatation of pulmonary arteries 6. Large ventricular septal defect and severe pulmonary stenoscs with: a. d-transposition of great arteries b. /-transposition of great arteries c. Origin of both great arteries from the right or left ventricle (double outlet right or left ventricle) 7. Atrioventricular canal with severe pulmonary stenosis 8. Single ventricle and severe pulmonary stenosis with: a. Normally related great arteries b. d-transposition of great arteries c. /-transposition of great arteries

present together with a single ventricle, atrioventricular canal, ventricular septal defect with a double outlet right ventricle or with transposed great arteries. Other conditions with cyanosis and decreased pulmonary flow are less likely to be confused with tetralogy. These include pulmonary stenosis or atresia with intact ventricular septum and patent foramen ovale or atria1 septal defect; rudimentary right ventricle with tricuspid and pulmonary stenosis or atresia; and Ebstein’s anomaly of the tricuspid valve with patent foramen ovale or atria1 septal defect. With pulmonary hypertension at systemic level: There is obstruction to flow through the lungs with pulmonary hypertension due to pulmonary vascular obstruction that is usually in the precapillary bed but may be distal to the capillaries in the form of stenosis of pulmonary veins or obstruction to left atria1 flow. Pathologically, changes of graded severity

are seen in the intima, media and adventitia of small pulmonary arteries and, as the obstructive-obliterative process worsens, in the larger pulmonary vessels as we11.177-1soThe term Eisenmenger syndrome encompasses this group of malformations when there is an intracardiac or extracardiac communication between the two circulations and, in addition, resistance to pulmonary blood flow that is equal to or in excess of systemic vascular resistance. Within the heart, the shunt site in Eisenmenger syndrome traditionally is a ventricular septal defect, but right to left shunting in the presence of high pulmonary vascular resistance can also occur with absence of the ventricular septum or through connections at the atria1 level (patent foramen ovale, atria1 septal defect, common atrium). At the aorticopulmonary level, the shunt sites include truncus arteriosus, aorticopulmonary window and patent ductus arteriosus. In all of these situations except the last, cyanosis is distributed equally over the body. However, when shunt reversal occurs through a patent ductus arterious distal to the left subclavian artery, venous blood preferentially enters the descending aorta and there is differential cyanosis of the toes and lower part of the body in comparison with the face and fingers. Methods of Diagnosis Accurate diagnosis is based on a synthesis of details from informative noninvasive techniques and from cardiac catheterization with contrast visualization. This full diagnostic evaluation is needed in anticipation of surgery and at least once in the analysis of the results. Usually the catheterization is repeated 1 year after operation, whereas the noninvasive diagnostic studies are used for lifelong follow-up. Noninvasive Techniques To the time-honored noninvasive techniques of history, physical examination, phonocardiography,

AORTIC ROOT

SEPTUM MITRAL VALVE

FIGURE 1. Tetralogy of Fallot and marked dextroposition of the aorta. Echocardiogram detnonstrates septal-aortic discontinuity (arrow) on sweep from the septum to the aortic root.

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TABLE

IV

Noninvasive

_----~

Techniques

in Differential Diagnosis of Common Cyanotic Congenital Anomalies* Roentgenogram

Anomaly

S*P

A.

Excessive Pulmonary

CTG A

t

RAD,

RVH

TAPVR

t

RAD,

RAE,

RVH, RAD or superior axis Variable CVH. RVH or LVH

Common

atrium

t

Common Common

ventricle truncus

t t

B. Deficient Tetralogy Tetralogy-like

anomaly*

PS with intact ventricular septum and PFO, ASD Tricuspid atresia or stenosis Ebstein’s anomaly

-1

RAD, RVH RAD, RVH, in lead V, RVS

-1 J-

LAD, RAD,

J 1

C. Deficient Eisenmenger syndrome (PVO, VSD and ASD or A-P connection)

t

RAD,

RVCD

Pulmonary

qR

LVD low voltage RVCD

Concave

t

t

Convex

t

t

Convex

t

f t

Variable Concave; variable

z t

Pulmonary Concave Concave

1 J-

N or t

Convex unless pure infundibular PS Concave Concave

1

N N or t

N or t

Other

Narrow base, frontal; wide base, RAO Vascular collar if drains to SVC. R or L

. .

.

Hypertension

N N

Flow With Pulmonary

RVH

Vast

Flow

t

Flow Without

sometimes

Pulmonary

MPA

CIT

ECG

Hypertension

at Systemic

Convex

31

Boot-shaped

RAE

heart

“’

Level 1

In outer lung fields

* Echocardiographic confirmation of the anomaly was obtained in all cases. + See Table III. f = increased; & = decreased. A-P = aorticopulmonary; ASD = atrial septal defect; C/T = cardiothoracic ratio; CTGA = complete transposition of great arteries; CVH = comvined ventricular hypertrophy; ECG = electrocardiogram; LAD = left axis deviation; LVD = left ventricular dominance; LVH = left ventricular hypertrophy; MPA = main pulmonary artery; N = normal; PFO = patent foramen ovale; PS = pulmonary stenosis; P Vast = pulmonary vascularity; PVO = pulmonary vascular obstruction; RAD = right axis deviation; RAE = right atrial enlargement; RAO = right anterior oblique view; RVCD = right ventricular conduction delay; RVH = right ventricular hypertrophy; RVS = right ventricular strain; S,P = second heart sound, pulmonary component; SVC = superior vena cava; VSD = ventricular septal defect.

electrovectorcardiography and cardiac series of chest roentgenograms (Table IV), three additional methods have recently been added. One especially valuable new tool is echocardiography.41,g5-100 Recording of the ultrasonic beam as it is reflected from cardiac chambers, valves, arterial roots and the ventricular septum has provided a new dimension and refinement to judging cardiac structure and function. Just as the correlation of phonocardiographic data with findings at cardiac catheterization and angiocardiography strengthened the accuracy of the physician’s interpretation of heart sounds and murmurs, so does the correlation of echocardiographic with angiocardiographic results enhance the accuracy of auscultatory, electrocardiographic and roentgenographic interpretation. Specific questions, such as that of aorticmitral valve continuity,g5 presence or absence of the ventricular septumls3 or septal-aortic continuity (Fig. 1) can often be answered with use of ultrasound. Two other diagnostic techniques in the evaluation of the cyanotic patient are continuous electrocardiographic monitoring for disturbances of rhythm and conduction and standardized tests of exercise function to assess physical performance. Invasive Techniques

Cardiac catheterization with contrast visualization utilizing cineangiocardiograms or cut film, or both, in simultaneous biplane projections affords detailed

analysis of shunt site and size as well as the other abnormalities that are associated with or a consequence of a particular cyanotic patient’s anomaly. Ventricular performance is evaluated at the same study. Recording of His bundle electrograms and electrical pacingsg-gl permit analysis of cardiac rhythm and conduction, which may be abnormal as part of the anomaly (for instance, in “corrected” transposition with ventricular inversion) or as a result of surgery. In the operating room electrophysiologic mapping of the conduction system can aid in prevention of heart block 184,185

Steps in Diagnosis:

Preliminary

Classification

Now let us apply these techniques in a systematic approach to diagnosis and differential diagnosis of the cyanotic patient, beginning with findings on physical examination and adding to these clues the information from other studies until certain possibilities are eliminated and a most likely diagnosis is reached and then precisely defined. Cardiac and visceral situs: The first step is to identify cardiac and visceral situs by physical examination and roentgenogram. In the normal situation (situs solitus) the cardiac apex and stomach are on the left and the liver, inferior vena cava and right atrium are on the right. The reverse situation is situs inversus with dextrocardia. Cyanotic anomalies in

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FIGURE 2. Levocardia with situs inversus and visceral heterotaxia in a child with common atrium and anomalous systemic and pulmonary venous drainage. Note cardiomegaly with prominent main pulmonary artery and branches. The increased markings extend to the outer lung fields. The right diaphragm is higher than the left. The stomach bubble is on the right.

these two situations tend to be more straightforward and are also easier to analyze than when there is discordance of cardiac position and abdominal viscera: levocardia with situs inversus (Fig. 2), mesocardia with situs solitus or inversus, dextrocardia with situs solitus or malposition with visceral heterotaxy (scrambled relation of viscera).is6 In those situations, anomalies of systemic and pulmonary venous return, defective or absent cardiac septa and transposed arteries are common. Associated conditions of splenic agenesis or polysplenia should be suspected.ls7Js8 If the heart is in the right side of the chest (dextrocardia), one must determine whether that position is due to cardiac displacement, to rotation (dextrover-

sion) or to inversion of cardiac chambers (mirrorimage dextrocardia). The form of the P wave in lead I of the electrocardiogram is of help here: If there is a normal sinus mechanism, an upright P wave indicates that the right atrium is on the right side; the heart is dextrorotated. An inverted P wave indicates inversion of the atria with the anatomic right atrium on the left. If there is a changing P wave pattern or if the P wave is biphasic or of other unusual contour, then the pacemaker is ectopic and further analysis of atria1 situs cannot be made from the electrocardiogram. Electrocardiographic evaluation of cardiac chamber hypertrophy and conduction: The electrocardiogram can provide other information (Table IV). When visceroatrial situs has been determined, the electrocardiogram assumes additional diagnostic value in evaluation of cardiac chambers affected by the anomaly. Most cyanotic malformations are associated with hypertrophy of the right ventricle. When this chamber is concentrically hypertrophied without dilatation, and right ventricular systolic pressure is at a systemic level (as in tetralogy of Fallot), the electrocardiogram shows right axis deviation and R-S reversal in leads chest. When right ventricular pressure is greater than systemic, S-T segments become depressed and T waves are inverted in the right precordial leads (right ventricular “strain”).18g Pure severe pulmonary stenosis with intact ventricular septum and patent foramen ovale is the leading diagnostic possibility when right ventricular “strain” is found. Conduction delay over the right ventricle (incomplete or complete right bundle branch block) suggests in the patient not operated on that the volume of the right ventricle is increased, as in Ebstein’s anomaly of the tricuspid valve or in total anomalous pulmonary venous return. In the newborn, absence of the expected right ventricular forces implies that the right ventricle is rudimentary. Left ventricular hy-

FIGURE 3. Pseudotruncus arteriosus (tetralogy of Fallot with pulmonary atresia and systemic arterial supply to the lungs) in a teenaged girl. Frontal (A) and lateral (B) views. A, note the boot-shaped heart with concave main pulmonary arterial segment and the scanty pulmonary arterial markings in the lung fields. Scoliosis began around the age of 12 years. B, note the irregular areas of anterior displacement of the barium-filled esophagus (arrow) due to collateral vessels from the descending aorta (shown in aortogram, Fig. 4).

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pertrophy indicates that the left ventricle has a greater than normal pressure or volume load. Wide notched P waves signify left atria1 abnormality whereas high peaked P waves indicate right atria1 hypertrophy. Acceleration or prolongation of atrioventricular (A-V) conduction has specific significance. For instance, Wolff-Parkinson-White syndrome commonly occurs in Ebstein’s anomaly. High grade or complete heart block is rare unless the patient has “corrected” transposition of the great arteries. The mean electrical axis is to the right (90“ to 150°) with right ventricular hypertrophy. Left axis deviation (0’ to 45O) is associated with lack of development of the right ventricle. A superior axis (around -90’) suggests an A-V canal defect or eccentric ventricular hypertrophy of the Noonan syndrome.lgO Evaluation of pulmonary blood flow and pressure: With the questions of visceroatrial situs and of cardiac conduction and hypertrophy settled, it is time to classify the patient according to pulmonary blood flow and pressure. The intensity and timing of the pulmonary closure component of the second heart sound offer the best way to judge presence or absence of pulmonary hypertension, whereas the best guide to status of pulmonary blood flow is a well penetrated chest roentgenogram. Attention is paid to the contour of the main pulmonary artery as well as to the main branches in the hilar area and to the degree of vascularity in the mid- and outer thirds of the lung fields. Concavity in the region of the main pulmonary

artery means that the artery is hypoplastic (as in severe pulmonary stenosis), absent (as in pulmonary atresia, Fig. 3 and 4) or else is misplaced (as in transposition of the great arteries, Fig. 5 and 6, or truncus arteriosus, Fig. 7). Prominent convexity of the main pulmonary artery may be due either to poststenotic dilatation distal to valvular pulmonary stenosis (Fig. 8) or to the opposite situation wherein there is increased flow or pressure in the main pulmonary artery (Fig. 2 and 9). Perusal of the hilar regions and the rest of the lung fields helps to decide which of these situations prevails. Enlarged pulmonary arterial markings in the hilar areas and mid-lung fields that extend to the outer third are seen with increased pulmonary arterial flow (Fig. 2 and 9), whereas with pulmonary vascular obstruction, the right and left pulmonary arteries appear cut off in mid-lung field, and the periphery is clear. Stippled punctate markings radiating from the hilar regions suggest that pulmonary venous return is obstructed, whereas a hazy perihilar appearance of fluffy areas suggests pulmonary edema. In the infant, such edema may be localized to the right upper lobe. Kerley B lines above the diaphragm signifying lymphatic and pulmonary venous congestion are less often present in the infant or child with pulmonary venous hypertension than in the adolescent and adult. Small pulmonary arterial branches in the hilar regions and unusually radiolucent lung fields are evidence of impairment to blood flow into the lungs, and hence indicate pulmonary stenosis or atresia (Fig. 3

FIGURE 4. Selective aortogram of same patientwith pseudotruncus arteriosus. A, the systemic arteries are opacified as they leave the aorta and anastomose with the pulmonary arteries (B), which are confluent with one another and supply the right and left lungs. The confluence terminates in a main pulmonary artery, which ends blindly as it approaches the heart (arrow).

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FIGURE 5. Cardiac series of chest roentgenograms of an 8 hour old baby with complete &transposition of the great arteries. A, frontal and lateral views are at left and right. B, right anterior oblique view is on left, and the left anterior oblique view on right. Note the average-sized heart with concavity in region of main pulmonaiy artery and the dense hilar vascularity. The base of the heart is narrow in the frontal view and doubles in width in the right anterior oblique view, directly below. This is a characteristic finding when the aorta is transposed and is to the right of the pulmonary artery.

FIGURE 6. Angiocardiogram of same newborn with complete or dtransposition of great arteries. A, shows right ventricular injection in simultaneous frontal (left) and lateral views with opacification of the transposed aorta. This vessel is anterior and to the right of the pulmonary artery, which is opacified in B after left ventricular injection. There is no opacification of the other ventricle on either injection, indicating that the ventricular septum is intact. There is no opacification of the ductus, and there is no subpulmonary stenosis. The coarsely trabeculated, hypertrophied right ventricle and finely trabeculated left ventricle are of average size and normal contour.

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and 4). When hilar pulmonary arterial markings are scant or absent but a lacy appearance of small vessels is seen in the lung fields, one thinks of pulmonary atresia and collateral circulation to the lungs from the systemic circulation (see Fig. 12). Barium swallow often confirms the presence of these vessels (Fig. 3 and 4) as they leave the anterior part of the descending aorta and indent the esophagus forward. On the cardiac series of chest roentgenograms one also obtains information concerning overall heart size, specific cardiac chamber enlargement and aortic size. With barium swallow in each view, one can tell whether. the aorta arches to the right (as it does in about 25 percent of patients with tetralogy of Fallot or truncus arteriosus, Fig. 7) or to the left (Fig. 2 and 3), where it descends and whether there are retroesophageal vessels (Fig. 3). Displacement of the bariumfilled esophagus by an enlarged left atrium does not often occur in the cyanotic patient. This finding suggests abnormality of the left A-V valve or excessive pulmonary blood flow and venous return, just as in the noncyanotic person. Correlating this information, we can place the cyanotic patient into one of the main diagnostic categories of pulmonary flow-high flow, low pressure and flow, or high resistance-and then proceed further to refine the diagnosis. Differential and Definitive Diagnosis Table IV summarizes some of the data from noninvasive techniques that help in differential diagnosis of the cyanotic anomalies.

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FIGURE 7. Common truncus arteriosus. type I, with common pulmonary trunk emerging near base of truncus on anterolateral aspect. A, preoperative angiograms in simultaneous frontal (left) and lateral views. Selective injection into the left venticle shows the truncus arteriosus, which continues as a left aortic arch. In the lateral view, the large ventricular septal defect and both ventricles are opacified. The unopacified crescent below the defect represents the ventricular septum. The truncus arises chiefly from the right ventricle. This infant had cardiac failure and was operated on under ‘profound hypothermia. 236 The ventricular septal defect was closed with a patch. The pulmonary trunk was dissected off the common trunk and the edges of the trunk (now the aorta) were oversewn. An aortic homograft with aortic valve was inserted between the outflow tract of the right ventricle and the end of the pulmonary trunk. B, right atrial angiocardiogram in frontal and lateral views before discharge shows the physiologic course of venous blood flow to the pulmonary arteries.

1. Excessive Pulmonary Blood Flow Physical examination of the cyanotic baby with increased pulmonary blood flow reveals a taehypneic infant who may be in respiratory distress and cardiac failure. The volume-overloaded heart is enlarged and overly active. Children who survive this period usually show grooving of the rib cage at the level of the diaphragm as a sequel of chronic respiratory effort. A precordial bulge is due to underlying ventricular enlargement. It is rare to find a cyanotic adult who still has a large left to right shunt. In most, conversion to the Eisenmenger syndrome has occurred by adulthood. Anomalous unobstructed pulmonary venous return is the most likely diagnosis in such an adult with increased pulmonary blood flow. Since some pulmonary hypertension is present with excessive pulmonary flow, the pulmonary component of the second heart sound (Ss) is accentuated and often occurs early so that Ss is narrowly split. When the great arteries are transposed or there is truncus arteriosus, the loud second sound at the second left interspace is the aortic or truneus closure sound. An early diastolic murmur at the base is heard when a semilunar valve is incompetent. In these anomalies, the systolic cardiac murmur is most often due to an associated ventricular septal defect or to excess flow across the pulmonary outflow tract. The electrocardiogram usually reflects both pressure and volume overload of the right ventricle and

shows right axis deviation with right ventricular hypertrophy and conduction delay. The roentgenograms show pulmonary arterial congestion and cardiomegaly. Particular attention is paid to the base of the heart, which is deformed by the abnormal arterial or venous trunks. For instance, in the newborn, a narrow basal shadow in frontal view that widens to double that size in the right anterior oblique view is almost pathognomonic of complete (d) transposition of the great arteries (Fig. 5 and 6). A big vascular collar at the base of the heart on both sides is caused by anomalous drainage of the pulmonary veins to a left vertical vein and so around to the dilated right superior vena cava and right atrium (“figure of eight,” “ snowman” or “cottage loop’ sign, Fig. 9). If the dilatation involves only the superior vena cava on the right, one suspects that as the point of entry of the pulmonary veins. An abnormally wide aortic shadow suggests truncus arteriosus (Fig. 7). Echocardiographic analysis,g5-gg together with cardiac catheterization and selective injection of contrast medium, serves to define the often complex anatomy and hemodynamics of these cyanotic patients with bidirectional shunting. Types of Malformations Transposition complexes: In the newborn, the most common anomaly in this category is complete

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FIGURE 8. Selective right ventricular angiocardiagrams of an 8 year old boy with severe valvular pulmonary stenosis, intact ventricular septum and patent foramen ovale. Right ventricular systolic pressure was greater than systemic at 186 mm Hg. A, frontal view. Note cardiomegaly due to right ventricular dilatation and hypet-trophy. The Swiss cheese appearance of the apical and septal portions of the right ventricle is due to enormously hypertrophied right ventricular musculature. The main pulmonary artery is dilated distal to the obstruction, so there is an abnormally convex main pulmonary artery shadow. B and C, lateral views in systole and diastole, respectively. The contrast medium (arrow) jets through the central portion of the thickened pulmonary valve into the dilated pulmonary artery beyond. The subvalvular area of the right ventricle narrows in systole (B) as a result of cristai hypertrophy, but widens in diastale. (C).

(d) transposition of the great arteries,lgl wherein the systemic and pulmonary circuits are independent. Survival depends on some shunt mechanism for mixing of oxygenated and desaturated blood. Blood that is directed to the lungs tends to be “trapped” in the left heart-pulmonary circuit so that pulmonary flow comes to exceed systemic flow, and both pulmonary arterial and venous pressures are elevated. If the newborn’s heart is otherwise normal, with only a patent foramen ovale and patent ductus, so little mixing of the circuits occurs that the infant is deeply cyanotic and unlikely to survive beyond the first few hours or days (Fig. 6). However, if there is a ventricular septal defect large enough to equalize the systolic pressure in the right and left ventricles (Fig. 10) and to permit unobstructed mixing of venous and arterial blood, the effect of the disproportionately large vol-

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ume that circulates through the lungs and is oxygenated is to minimize or even obscure the cyanosis. In such a baby the condition is often unrecognized and he leaves the newborn nursery only to return in a few weeks with cardiac failure. Other transposition complexes’g2 such as “corrected” or l-transposition (when the aorta is anterior and to the left of the pulmonary artery [Fig. 111 and the ventricles are usually inverted) and types of double outlet right ventriclelg3 and double outlet left ventriclelg4 fit into this cyanotic group of conotruncal anomalies with increased flow if the relation of the ventricular septal defect and the great arteries is such as to permit streaming of some unoxygenated blood into the aorta. The Taussig-Bing1g5,1g6 anomaly is a form of double outlet right ventricle in which the ventricular septal defect is in relation to the pul-

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FIGURE 9. Total anomalous pulmonary venous drainage to the left

vertical vein and around to superior vena cava on the right, in a 1 month old infant in respiratory distress and cardiac failure. A, frontal roentgenogram. Note the right-sided cardiomegaly and the dilated main pulmonary artery and branches. A diagnostic finding is the vascular collar around base of heart due to dilatation of the systemic veins that receive the pulmonary veins. B and C, angiocardiograms, show opacification of these structures and of the dilated pulmonary artery and engorged pulmonary arteries. The catheter is in the main pulmonary artery, where injection was made. B, contrast medium leaving the convex main pulmonary artery to fill the enlarged pulmonary arteries and the capillaries. C, confluence of opacified pulmonary viens and the large left vertical vein as it courses to the dilated right superior vena and the enlarged right atrium. The opacification of these vessels and chambers explains the diagnostic radiologic findings in A.

monary artery, which then has a higher oxygen concentration than the aorta. With double outlet right ventricle, the ventricular septal defect may be infra-, intra- or supracristal, may be muscular, or may be multiple whereas the great arteries may have their normal anteroposterior relation, be side to side or in a position of d- or l-transposition. The functional result of these different arrangements may be similar, but the defined anatomy makes a considerable difference in expectation of favorable result of surgery. No one of these unusual transposition complexes, nor the conditions to be discussed later (truncus arteriosus, single ventricle), is common. But they are of uncommon interest because of the challenge they pose for understanding the anatomy and function and for planning surgical repair. In each of them, the pulmonary artery and the aorta have the same pressure. Unless some degree of pulmonary stenosis exists, pulmonary vascular resistance is likely to be 70 percent or more of systemic resistance by early or middle childhood. This is an unfavorable situation

for any surgical undertaking since such elevation of pulmonary vascular resistance is unlikely to decline and is apt to continue to rise. Truncus arteriosus: The classification of truncus arteriosus into four types, based on point of origin of the pulmonary arteries, lg7 has assumed practical significance now that it is possible in some carefully selected patients to remove the pulmonary arteries from the truncus and anastomose them to the distal end of a graft containing a valve. The proximal end is sewn into the outflow tract of the right ventricle so that a new pulmonary outflow tract is created.138 The types are identified by selective injections of contrast medium.lg8 Type I truncus arteriosus, in which the common pulmonary trunk exits from the lateral aspect of the trunk near the truncus valve (Fig. 7), lends itself to repair more easily and is more common than the other types. In type II, there is separate takeoff of right and left pulmonary arteries from the posterior aspect of the ascending trunk; they are not in conti-

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FIGURE 11. Selective ventricular injection of child with single ventricle and “corrected” or /-transposition of the great arteries with valvular pulmonary stenosis. The catheter was introduced from the inferior vena cava and right atrium into the ventricle, which has the contour of an anatomic left ventricle. The aorta on the left and the main pulmonary artery on the right opacify simultaneously. Arrow points to radiolucency of the deformed and stenotic pulmonary valve. Lateral view (not shown) shows the aorta anterior to the pulmonary artery.

FIGURE 10. Selective

right ventricular angiocardiogram in lateral view of child with complete transposition of the great arteries and large ventricular septal defect. Note spread of contrast medium through the defect into the posterior (left) ventricle and the unopacified area of thick ventricular septum beneath the ventricular septal defect. The aorta is anterior and the level of the aortic valve is high because of the subaortic conus. The transposed posteriorly placed pulmonary artery is as large as the aorta. There is no subplumonary conus, so the pulmonary valve is at a lower level than the aortic valve. There is no subpulmonary stenosis, either angiocardiographitally or by pressure measurements.

nuity. Type III is more complicated, since the pulmonary arteries arise from the lateral aspects of the ascending aorta or from brachiocephalic arteries or a ductus-like structure from the transverse arch. Type IV truncus resembles the condition of pseudotruncus arteriosusigs (Fig. 4 and 12), a variant of tetralogy of Fallot. In both, the lung vessels derive from systemic arteries leaving the descending aorta, but in type IV truncus, no remnant of pulmonary arteries is found. Some patients have unusual vascular arrangements that do not fit neatly into this classification but are atypical or are hybrids. Pulmonary vascular supply to the two lungs may be different; there may be only a single pulmonary artery. These variations were considered in Edwards and McGoon’s reclassification1gg of the condition in which there is absence of anatomic origin from the heart of the pulmonary arterial supply. That reclassification has clinical significance for repair. Of 262 patients with this finding, 64 percent had confluence of right and left pulmonary arteries, 3 percent had nonconfluence, 24 percent had no pulmonary arteries and 9 percent had unilateral absence of a pulmonary artery.200

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At the time of study it is important for consideration of surgery to determine not only pulmonary resistance and flow, and the points of origin of the pulmonary arteries and their continuity or discontinuity with one another, but also to identify where the truncus arteriosus arises in relation to the right or left ventricle, the presence of a ventricular septum and size of the defect, and the presence of truncus valve insufficiency201p202 or stenosis. Coronary arterial anatomy can usually be determined from the selective injection just above the truncus valve. Single ventricle: Definition of the type of single ventricle203-205 has also assumed practical significance with the present early success of placing a synthetic septum in selected cases.142,206,207The most common type of single ventricle is that with two atria and with inversion of the A-V valves that communicate with one ventricle that is an inverted left ventricle with an outlet chamber that represents the right ventricular infundibulum or conus (Fig. 11). The great arteries are usually in an Z-transposition relation (Fig. 11) but may be d-transposed, or side by side, or in the normal relation.208 Obstruction to flow may occur at an A-V valve, within the ventricle at the bulboventricular foramen leading to the outlet chamber, or in the valves (Fig. 11) or great arteries themselves. There may be a common truncus arteriosus. Each of these possibilities must be carefully evaluated in diagnosis of the patient with a single ventricle.20g Common atrium: This defect, (Fig. 2) is even more unusual. It is sometimes found in patients with Ellis van Creveld syndrome.21o Determination of sys-

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FIGURE 12. Aortogram of infant with pseudotruncus arteriosus with right aortic arch and descending aorta. A, large systemic arteries leaving the descending aorta. In B, anastomosis of these arteries with small left and right pulmonary arteries that become confluent and terminate in a small blind main pulmonary artery (arrow) that does not connect with the right ventricle.

temic venous return by catheterization from the left arm as well as from the leg and of pulmonary venous drainage by selective injection into the pulmonary artery is needed in planning repair. Total

anomalous

pulmonary

venous

drainage:

This is another uncommon condition that is amenable to surgery.137,211,212Its rarity is indicated by its reported occurrence once in 6,825 live births surveyed in Auckland, New Zealand.212 Mitchell et a1.213 did not list it among the malformations found in the 457 youngsters with congenital heart disease from 12 centers in the United States during a recent cooperative prospective study of the outcome of 56,109 births. The incidence rate of congenital heart disease in that large series was 8.14 percent. The anomaly constituted 1 percent of the autopsy specimens from newborns with congenital heart disease at Johns Hopkins. 212 Fyler et a1.214reported an incidence rate of 2 percent in the critically ill infants in the 1st month of life in the New England Regional Infant Cardiac Program, in which the emphasis is on detection and treatment of the sick newborn with heart disease. It is estimated that 10 percent or fewer with this anomaly seek medical attention after the age of 1 year. Then the condition is much more benign and resembles a simple atria1 septal defect in its manifestations and suitability for surgery.215 The most generally accepted classification is that of Darling et a1.216 who classified the anomalies of drainage of all the pulmonary veins according to their point of juncture with the right atrium or a tributary thereof. In decreasing order of frequency, there are four groups: supracardiac, cardiac, infracardiac and mixed. The condition is recognized by the identity of oxyhemoglobin saturation in the right and left sides of the heart. The type of drainage is determined by selectively injecting contrast medium into the main

pulmonary artery and observing the confluence of pulmonary veins and their course above or below the diaphragm to the point of abnormal entry (Fig. 9). Any narrowing in the path of drainage or obstruction to flow from right atrium into the left atrium and systemic circulation is analyzed by this visualization and by measurement of pressures and oxygen in the pulmonary artery, venae cavae and right heart chambers. When the right side of the heart is greatly dilated, the volume of the left atrium and ventricle by comparison appears small but is usually adequate. Selective left ventricular injection helps to evaluate size and whether the ventricular septum is intact. Hypoplastic left heart syndrome: This syndrome217T218 functionally mimics the condition of total anomalous pulmonary venous return and is about eight times as common. Echocardiography has become quite useful in distinguishing the inoperable situation of hypoplastic left heart syndrome from the potentially operable condition of total anomalous pulmonary venous return. In the former, one might not wish to perform cardiac catheterization, whereas in the other situation, the distressed newborn requires cardiac catheterization with contrast visualization as an emergency procedure in anticipation of open heart surgery. The distinguishing features on ultrasonic study of the hypoplastic left heart syndrome are the absence of echoes or small amplitude of echoes from the mitral valve and the absence or unusually small size of the left ventricle and aortic root 96,183,219 When babies with the hypoplastic left heart syndrome are studied, selective injection of contrast medium into the main pulmonary artery often gives the essential information about the left side of the heart: the patent ductus arteriosus that supplies blood to the descending aorta and some reflux up the arch,

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the normal venous return to the left atrium and then the hypoplasia or atresia of the mitral and aortic valves, left ventricle and ascending aorta.21s 2. Deficient Pulmonary Hypertension

Blood Flow Without Pulmonary

It was for many of this group of cyanotic patients with reduced pulmonary flow and low pressure that shunt procedures to increase pulmonary blood flow proved to be so helpful and opened up the field of surgery on blue babies. Physical examination of the patient with right to left shunting and severe valvular or subvalvular pulmonary stenosis reveals diminution and delay of the pulmonary component of the second heart sound (Table IV). It may even be inaudible so that only the sound of aortic closure is heard. The systolic murmur in these cyanotic patients is due to pulmonary stenosis, not to a septal defect. It is a crescendo-decrescendo ejection type systolic murmur, maximal in the second left interspace, that radiates along the course of pulmonary blood flow so that it is audible over the lung fields posteriorly. In phonocardiograms this murmur appears diamond-shaped. An early systolic ejection sound signifies valvular pulmonary stenosis, although this helpful sign may be absent when the stenosis is severe. With pulmonary atresia, no cardiac murmur is heard, but over the lung fields anteriorly or posteriorly, on one or both sides, one may hear a continuous murmur of collateral circulation due to

systemic arteries anastomosing with intrapulmonary arteries (pseudotruncus arteriosus).lg8 Electrovectorcardiography depicts the hypertrophy or dilatation, or both, of the right ventricle and shows “strain” if the pressure is greater than the systemic pressure. If the right ventricle is rudimentary or underdeveloped, there is absence of the expected right ventricular forces. Radiologic confirmation of decreased pulmonary blood flow is provided by the hypovascularity of the lung fields, which appear unusually radiolucent. If there is infundibular stenosis with hypoplasia of the main pulmonary artery, the heart appears bootshaped with a concave outflow tract area (Fig. 3). On the other hand, if the stenosis is purely valvular, there is poststenotic dilatation of the main pulmonary artery and often of the left main branch (Fig. 8). The cardiothoracic ratio is average unless the ventricular septum is intact. in the patient with tetralogy Echocardiographyg8 of Fallot reveals right and left ventricular chambers of equal size, a ventricular septum and, when there is overriding of the aorta, discontinuity between the septum and the aortic root (Fig. 1). Sweep of the transducer from mitral valve to aorta shows these structures to be in continuity, thus excluding a double outlet right ventricle.g5 In general, the larger the aortic root, the smaller is the pulmonary anulus in tetralogy; thus, determination of the size of the aortic root helps in this assessment of severity of the anom-

FIGURE 13. Selective right ventricular injection in a child with tetralogy of Fallot. Simultaneous frontal (left) and lateral views. A, opacified right ventricle and pulmonary artery of average size. Arrow points to discrete subpulmonary stenosis, which widens in diastole (6). The pulmonary valve area appears normal. In 6, faint opacification of minimally overriding aorta is seen in the frontal view; in the lateral view, contrast medium is seen passing through the high ventricular septal defect into the left ventricle.

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aly. With pulmonary stenosis and intact septum, there is septal-aortic continuity. Tricuspid atresia with rudimentary right ventricle is characterized by the absence of tricuspid echoes and the presence of a small right ventricular chamber; in Ebstein’s anomaly abnormal movement and delay of tricuspid closure are recorded.220 These preliminary determinations help to plan the cardiac catheterization with contrast visualization. When the pressure in the right ventricle is measured to be at a systemic level, the patient is positioned and injections are made selectively to distinguish tetralogy of Fallot (Fig. 13 and 14) from the conditions that mimic it (Table III). Types of Malformations Tetralogy of Fallot: This defect consists of a high ventricular septal defect, large enough to equaliz;! systolic pressure in the two ventricles at a systemic level, in conjunction with pulmonary stenosis of such severity that the pressure in the pulmonary artery is normal or less than normal. When the diagnosis of tetralogy of Fallot is established, then analysis is made of the many features that are important in planning surgery: the presence and the severity of subvalvular, valvular and supravalvular pulmonary stenosis; proximity of the infundibular stenosis to the pulmonary valve; size of the pulmonary anulus; size of the main pulmonary artery and the two branches and whether

there is normal continuity; the location of the ventricular septal defect, which is almost always infracristal but may be intracristal and is rarely multiple; and the degree of overriding of the aorta. In an unusual variant of tetralogy, there is absence of the pulmonary valve and aneurysmal dilatation of the main pulmonary artery and branches.“l The obstruction is at the anulus. Important too is the determination of any systemic blood supply to the lungs. Sometimes one pulmonary artery arises from the aorta rather than from the right ventricle like its mate. If a shunt procedure has previously been performed, that too is analyzed for patency and for pressure in the pulmonary artery beyond as well as for any distortion or complications such as kinking157 or aneurysmal dilatation. Tricuspid atresia: If this defect is present, one analyzes the adequacy of right atria1 outlet to the left atrium and the outflow from the left ventricle; presence of obstruction to aortic flow at cardiac level or beyond; presence of a large or a small and restrictive ventricular septal defect for communication with the rudimentary right ventricle; the size of the right ventricular chamber, pulmonary anulus and vessels; and any systemic blood supply to the pulmonary artery. Pulmonary stenosis: With an intact septum (Fig. 8) or a small ventricular septal defect, the systolic pressure in the right ventricle may soar to over 200 mm Hg and the wall and cristae may be greatly hypertrophied. The right ventricle usually is of adequate size but may be rudimentary or, conversely, greatly dilated. There may be secondary tricuspid regurgitation. In the newborn, the pulmonary valve may be atretic. Ebstein’s anomaly of the tricuspid valve: The degree of downward displacement of the tricuspid valve and the portion of the right ventricle that is atrialized23,222,223 are analyzed because these features determine severity. Intracardiac recording of the electrocardiogram aids in distinguishing the point of change of the atrialized right ventricle and atrium.224 Anomalous systemic venous return to the left atrium: This conditionzz5 may occur alone or be part of a more complex anomaly. As an isolated event it is associated with cyanosis but no heart murmur, electrocardiographic abnormality, nor cardiomegaly. A straight, vertical density at the base of the heart on the left suggests a left superior vena cava. Usually that structure drains into the coronary sinus, but it may enter the left atrium. If one suspects anomalous systemic venous drainage, the anomaly can be identified by selective injection from the arms and from below the diaphragm. 3. Deficient Pulmonary Flow With Pulmonary Hypertension at Systemic Level

FIGURE 14. Selective right ventricular angiocardiogram in lateral view of boy with tetralogy of Fallot with subvalvular, valvular and supulmonary stenosis. Contrast medium opacifies pravalvular (arrow) the region of the ventricular septal defect and enters the large and moderately overriding aorta.

The characteristic finding tion of right to left shunting

on physical

examina-

and increased pulmonary pressure and vascular resistance is accentuation of the pulmonary component of the second heart sound. The pulmonary component occurs earlier than normal so that the second sound is narrowly

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split or single. Sometimes there is a murmur of pulmonary insufficiency: a high-pitched, decrescendo diastolic murmur occurring in early and mid-diastole along the left sternal border and heard better with the patient supine than upright. There may be no systolic murmur at all or a short early systolic at the upper or lower sternal border. Occasionally an early systolic ejection sound is heard. There is a right ventricular tap. The heart is not much enlarged until it decompensates. The right ventricular hypertrophy is reflected in the electrocardiogram. Radiographically there is enlargement of the pulmonary arterial tree up to the point of precapillary obstruction. This gives the effect of abrupt “pruning” of the pulmonary vascularity in the outer third of the lung fields, whereas the hilar region appears full and the main pulmonary artery is usually convex. Echocardiography shows hypertrophy and dilatation of the right ventricle and the relation of the ventricular septum, aortic root and mitral valve. When the cyanotic patient is shown by cardiac catheterization and angiocardiography to have the Eisenmenger reaction, he is not a candidate for cardiac surgery. Although surgery has little to offer, under medical management the patient may be remarkably free from symptoms and lead an active life as an adult.226 Management Today’s population of patients born with a form of cyanotic heart disease consists of a mix of patients not operated on whose conditions range from severe to mild, as well as a variety of surgically treated children and adults with a wide spread of functional and anatomic results. Management is individualized and is based on accurate diagnosis and regular reassessment of that patient and of the changing trends in therapy. In the cyanotic patient’s lifetime of care,5,31,226 his primary physician, cardiologist and cardiovascular surgeon provide a cooperating continuum with different responsibilities at different times. In consultation with the patient’s physician, the cardiologist provides definitive diagnosis, treatment of complications and systematic long-term follow-up. Reminders of the importance of antibiotic agents in therapeutic doses at the time of oral or genitourinary surgery help prevent infectious endocarditis. Counseling for schooling, job selection, recreation, marriage and family begins early and continues, with the goals being to encourage the patient to perform near the peak of his capacity and to be a well-adjusted, effective member of society. To be competitive in the job market, the person born with a cardiac defect has to overcome a certain reluctance of employers to hire the “handicapped.” Therefore, we should not only help the adolescent to be as nearly normal as modern medicine and surgery permit, but we should also encourage his being as well prepared for his job as possible. Consummate skill and continuing commitment to 296

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improvement of results are required of the cardiovascular surgeon, who must understand and strive to repair the great variety of abnormalities of structure and function that constitute cyanotic congenital heart disease. Consideration and planning for cardiac surgery and the perioperative management of the patient are a shared medical and surgical responsibility, wherein skills and experience are joined to ensure a favorable outcome. Meticulous attention to detail is just as important in the intensive care unit after surgery as it is in the operating room. The younger and smaller the subject, the less room there is for small error or for deviation from the guidelines for care previously agreed upon and regularly in use by the hospital team. Our present attitude about the indications and the timing of surgery for patients with the most common cyanotic malformations follows. For the unusual anomalies, similar principles apply. Each patient is judged individually but in general, if the anatomic features are suitable for open primary repair at low risk, we believe that this approach is preferable to early palliation followed by open heart surgery. Short-term results thus far support this concept. 1. Excessive Pulmonary Flow Palliative

Procedures

Palliation for this group consists of two types of procedures, creation of an atria1 septal defect and pulmonary arterial banding. Creation of an atria1 septal defect: This can be done either medically by the Rashkind technique of balloon atria1 septostomy135 in the newborn or surgically by the Blalock-Hanlon procedure of atria1 septectomy.lzO The purposes are to improve interatrial mixing and relieve obstruction to pulmonary venous return in patients with complete transposition of the great arteries or pulmonary veins. For the newborn infant under the age of 2 weeks who has complete transposition of the great arteries, the Rashkind procedure is life-saving. 15g-163 Benefit usually lasts for several months but rarely in our experience for as long as a year. 227,22s When the baby becomes more cyanotic and tachypneic, he should be operated on before he is severely ill with cardiac failure, pulmonary edema or a cerebrovascular accident. Should he then undergo surgical septectomy and defer physiologic rerouting of the venous return until he is 3 to 5 years old, or should the Mustard procedure of intraatria1 venous rerouting be undertaken as the next step?22g~230 Experience at our institutionZ31 and at others over the past few years indicates that these sick infants can undergo open repair at the same low risk and with the same good expectation of improvement as older children.16g173,231-234 Therefore we no longer recommend the Blalock-Hanlon repair as an intermediate step. For sick infants with total anomalous pulmonary venous return, we believe early open repair is preferable to palliation by balloon septostomy or atria1 septectomy. Pulmonary arterial banding: The other pallia-

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tive operation for cyanotic infants with increased pulmonary flow is pulmonary arterial banding.122 This technique was utilized in babies with excessive pulmonary flow and uncontrollable cardiac failure in an effort to gain a better balance of systemic and pulmonary flow. It was also employed, although infrequently, in some babies with early evidence of elevated pulmonary vascular resistance in an attempt to protect the pulmonary bed. Subjects who were candidates for pulmonary arterial banding included those with any of the transposition complexes in association with a large ventricular septal defect, those with a single ventricle without pulmonary stenosis and those with common truncus arteriosus. In these patients with cyanotic heart disease, it was especially difficult not only to place the pulmonary arterial band, if there was an anomalous or posterior location of the pulmonary artery, but it was also more difficult than in patients with a simple ventricular septal defect to know how tight the band should be. Too loose a band meant an ineffective operation with the high risk that accompanies any operation on a sick patient that does not help. Too tight a band caused deepening of cyanosis and an increase in polycythemia. Pericardial adhesions made later surgery difficult. Additional problems encountered when later repair was attempted concerned not only the tightness but also the width of the band and its location on the artery. Often it was necessary to repair and to enlarge the constricted artery. The position of the band too close to the pulmonary valve caused damage to the leaflets as they opened. Migration of the band to the bifurcation obstructed flow to one or both pulmonary arterial branches, which then did not enlarge with growth of the patient. Open Corrective Procedures Against these problems of banding the pulmonary artery must be weighed the risks and rewards of early open heart surgery in the cyanotic individual with the anomalies mentioned. If the risks seem equal or better with the direct approach, we favor repair over surgical palliation and subsequent open operation. There is a distinct benefit to growth and development that comes with early disappearance of a right to left shunt. Complete transposition of the great arteriesthe Mustard procedure: This open reparative operation is recommended for the infant with palliation as soon as he begins to lose the benefit of the created atria1 septal defect. Over the past several years the lower age limit for this repair has gradually been reduced from the previous “ideal” age of 2 years or more, down into the 1st year of life and even to the age of a few weeks, with no increase in mortality above the previously experienced rate of 5 to 10 percent.22gJ31-235 The youngest survivor in our series was a 13 day old baby.231p236r237 The technique of profound hypothermia has been particularly valuable in this lowering of the age limit.140~233~234 The Mustard procedure physiologically corrects the condition of transposition of the great arteries by

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rerouting the systemic and pulmonary venous return within the atrium. The atria1 septum is excised and a pericardial patch is emplaced so that the blood in the venae cavae and coronary sinus drain into the new physiologic right atrium, mitral valve and left ventricle, and so to the pulmonary artery. Pulmonary venous blood enters the new physiologic left atrium (previous anatomic right atrium) and crosses the tricuspid valve to be pumped by the right ventricle into the aorta.133,22g Early problems following this procedure consisted of damage to the conduction system, superior vena caval obstruction, pulmonary venous obstruction, inadequacy of size of the new right or left atrium, shriveling of the patch, leaks about the patch and tricuspid insufficiency.168-173,235 Each problem was analyzed and is now to a large extent preventable. Particularly troublesome in early series was damage to the conduction system that resulted in heart block or in the “sick sinus syndrome” with episodes of bradyor tachyarrhythmia. 168In our experience this has not been a problem in the past 4 years after a modification was made in the suturing of the patch about the coronary sinus, in the manner of opening the right atrium and in the avoidance of trauma to the area near the superior vena cava where the sinus node is located.231 Superior uena caval obstruction has been minimized by complete excision of the upper atria1 septum. Enlarging the new pulmonary venous atrium with a piece of pericardium has prevented pulmonary venous obstruction.22g Care is taken not to damage the tricuspid leaflets during the repair. These and other technical details of tailoring and sewing in the pericardial patch have overcome some of the early problems that were part of the “learning curve” in dealing with this anomaly. Questions still to be answered concern what will happen to the new atria and to the venous return when the patient grows to adulthood, and whether the right ventricle and tricuspid valve can function efficiently for decades at a systemic pressure level. Complete transposition of the great arteriescorrection of additional anomalies: Additional anomalies pose other problems. Ventricular septal defect with complete transposition can be small and restrictive and of little physiologic consequence, or it can be large and of considerable significance. The large defect permits mixing so that the baby is not deeply cyanotic, but it is also associated with the early onset of severe pulmonary vascular obstruction.17g In a recent study of lung specimens of 200 patients with transposition of the great arteries, only 9 of 107 (8.4 percent) with an intact septum or a small ventricular septal defect demonstrated greater than grade 2 (Heath-Edwards classification177) pulmonary vascular disease whereas among patients with a large defect, 37 of 93 (40 percent) infants and 26 of 35 (75 percent) over the age of 1 year had greater than grade 2 changes. A large patent ductus arteriosus was similarly harmful in this regard, for all five infants under 1 year who had grade 3 or 4 changes had a large pat-

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ent ductus arteriosus. The conclusions drawn were that the large ventricular septal defect or patent ductus arteriosus should be closed before age 6 months to prevent progressive damage.238 This can be accomplished at the same time as the Mustard procedure. The ventricular septal defect can usually be closed through the tricuspid anulus with a patch. 239,240 High pulmonary vascular resistance is usually a contraindication to repair, unless there is an element of pulmonary venous hypertension from inadequate atria1 mixing that can be relieved by the Mustard procedure. One of our most dramatic results of surgery was in an intensely cyanotic 18 month old boy with a hematocrit of 80 percent, probe-patent foramen ovale and a moderate ventricular septal defect. The pressure in the pulmonary artery was only 10 mm Hg less than in the aorta preoperatively, but 1 year after the Mustard procedure and closure of the ventricular septal defect, he had no shunts and had normal pulmonary arterial pressure and resistance. A small amount of pulmonary stenosis with transposition of the great arteries can sometimes be relieved at the same time, although the obstruction, which is usually subpulmonary in the left ventricular outflow tract, is difficult to reach through the posterior pulmonary artery or ventricular septal defect. Other complicating anomalies such as coarctation of the aorta, rudimentary right ventricle and tricuspid atresia are rare.241 Although significant coarctation may be separately repaired, the other two situations call for palliation if atria1 mixing is inadequate, and if the pulmonary flow is really excessive, pulmonary banding may help. Other malformations in the transposition complexes, or conotruncal positional anomalies with double outlet right or left ventricle, with and without ventricular inversion: Open repair of these lesions has produced some gratifying results as ingenious techniques have been utilized to interrupt the shunt at ventricular level and to direct the blood flow in physiologic direction.192~242 Repair depends on the location of the ventricular septal defect in relation to the great arteries and on the degree of rotation of the great arteries in relation to one another and the ventricular septum. The surgeon may use, in addition to the Mustard procedure, the Rastelli procedure of an external valved conduit, or an internal interventricular rerouting by a hammock-like [email protected] felt prosthesis. For example, the cyanotic child with Taussig-Bing anomaly (double outlet right ventricle and subpulmonary ventricular septal defect could undergo open heart surgery with a hammocklike Teflon patch to interrupt the ventricular shunt and to connect the overriding pulmonary artery to the left ventricle, leaving the aorta arising from the right ventricle. Thus the condition would be functionally a complete transposition. But a Mustard procedure could then be carried out at the same operation to reroute the venous return so as to correct the flow of blood physiologically. Truncus arteriosus-Rastelli procedure: This lesion can be repaired by the Rastelli procedureL38*174

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even in early infancy236 (Fig. 7) when the baby’s heart failure has been treated and the anatomy defined. Later operation in childhood to place a larger external valved conduit would be a necessary sequel to early surgical success. Surgical planning requires preoperative delineation of the relation of the truncus to the right or left ventricle, the takeoff of the pulmonary arteries and the question of truncus insufficiency or stenosis. 243,244 Type I truncus with the pulmonary arteries emerging from the left basal aspect of the trunk in a nearly normal position is fortunately the most common type, since it presents less difficulties in placing a graft with a valve to create an outflow from the right ventricle to the pulmonary artery (Rastelli procedure13s) than do types II and III with the pulmonary arteries arising separately and more remotely. Type IV truncus without any pulmonary arteries but with systemic arterial supply from the descending aorta appears inoperable at this time. High pulmonary vascular resistance, greater than 70 percent of systemic, renders the other types inoperable. The ventricular septal defect is closed through the right ventriculotomy. Severe truncus insufficiency can be relieved by replacing the truncus valve.202 Single ventricle-prosthetic septum: Just recently, selected patients with single ventricle without pulmonary vascular obstruction have been operated upon.1g4,206 The surgical candidates have had two A-V valves with separate attachment of papillary muscles so that a prosthetic septum could be constructed to allow for blood flow in the physiologic direction to the great arteries. The cardiac conduction system is anomalous and tends to run, not posteroinferiorly distal to the tricuspid valve but anterosuperiorly and in relation to the bulboventricular foramen. Intraoperative mapping of conduction pathways has added to the safety of this operation by avoidance of injury and heart block. Since no growth of the prosthetic septum can be anticipated, it would seem advisable at this time to defer such surgery until after age 5 years. Until then, banding of the pulmonary artery might be considered as a temporary measure to cut down excessive flow. Common atrium: This defect can be successfully repaired (Fig. 2) with a large Teflon patch after precise definition of the points of entry of each pulmonary vein and vena cava and identification of the coronary sinus.210 Often there are bilateral superior venae cavae; the anomalous left vena cava then may drain into the coronary sinus as it usually does, or may drain into the left side of the common atrium as it did in the patient with levocardia and visceral heterotaxia whose heart is shown in Figure 2. She also had anomalous pulmonary venous drainage into the right as well as left side of the common atrium. Therefore, it is essential that drainage of systemic and of pulmonary veins be accurately identified in the operating room before the large patch is sewed in to create two atria appropriately related to the atrioventricular valves. Total anomalous pulmonary venous return: Patients with this lesion present as symptomatic infants

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in two different ways: (1) as a sick, slightly cyanotic distressed baby with heart failure, volume-overloaded right heart chambers, flooded lungs, some pulmonary hypertension and slight or no obstruction to pulmonary venous drainage (Fig. 9), or (2) as a deeply cyanotic dyspneic baby with a small heart and obstructed pulmonary venous outlet.245,246 Both presentations are true cardiac emergencies that call for prompt recognition and immediate diagnostic studies followed by open heart surgery to reunite the pulmonary veins with the left atrium.137J40J46,211,216,247 There is then about a 75 percent chance for survival and improvement. When the suture line in the left atrium is extended maximally to include the left atrial appendage, the risk that the anastomosis will become obstructive as the baby grows into childhood is minimized. Consistently successful surgery in these babies is too recent for long-term follow-up of the effects of growth. About 10 percent of patients with total anomalous pulmonary venous return do not get into such difficulty early in life, probably because there is no obstruction to the anomalously draining veins and because the pulmonary vascular bed is compliant enough to accept a large volume without pulmonary hypertension. For this group of patients, open repair can be scheduled electively. We prefer to do this after the age of 5 or 6 years to ensure as large an anastomosis q possible. Risk of surgery then is small. Hypoplastic left heart syndrome: Infants with this condition have little chance for salvage unless there is some extra malformation that permits a sufficiently large ascending aorta to supply adequate coronary and cerebral arterial circulation.217,21s Then creation of an atria1 septal defect can relieve some of the obstruction to pulmonary venous outflow, and banding the pulmonary artery can bring pulmonary flow into better balance with systemic flow. At The New York Hospital, two children with mitral atresia and a single ventricle giving rise to both great arteries were so treated and are living relatively symptomfree lives at the ages of 10 l/2 and 7 years. 2. Deficient Pulmonary Blood Flow With Normal or Low Pulmonary Pressures Palliative

Procedures

Palliation by a shunt to increase pulmonary flow is indicated for the symptomatic baby with tetralogy of Fallot who has hypercyanotic spells or is polycythemic but who has unfavorable anatomy for open repair, due to marked hypoplasia of the right ventricular outflow tract and pulmonary anulus and arteries. Palliation is also indicated for infants with severe pulmonary stenosis together with rudimentary right ventricle or with single ventricle and for those with complete transposition of the great arteries and ventricular septal defect. For some of these infants who are helped by a shunt to increase pulmonary flow, there is hope for reparative operation a few years later. If that is not possible, it is comforting to know that benefit from a first palliative operation can last

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for many years and that there are other shunt procedures available if the early one is outgrown. Blalock-Taussig anastomosis: Our choice of the shunt has been modified by experience and by longterm follow-up. The Blalock-Taussig end to side anastomosis of subclavian to pulmonary artery1 has provided the most satisfactory long-term result~~*~~~*~ with only a small risk of pulmonary hypertension and with relative ease of takedown at later open heart surgery. It is our first choice for children and for large babies. However, in the 1st month of life, there is still difficulty with patency of the anastomosis when a small subclavian artery is turned down to a hypoplastic pulmonary artery. Glenn anastomosis: The Glenn anastomosis of superior vena cava to right pulmonary artery125J54-156 is also ineffective in the 1st month and at any age when there is great disproportion between the size of the vena cava and the artery. Furthermore, this shunt trends to “shrivel” as time passes. Therefore, we reserve it for specially selected cases, such as tricuspid atresia when a Fontan procedure is a possibility in the future.i*’ Waterston shunt: Because of the problem of kinking and even of interruption of continuity of the right pulmonary artery when the ascending aorta is anastomosed to it (Waterston shunt157,248,24g),we do not recommend this procedure unless there is no other alternative. Potts anastomosis: If a, shunt procedure is needed in the 1st month of life, we currently prefer a Potts anastomosis of measured diameter between the left descending aorta and adjacent left pulmonary artery. If this shunt is not too large initially and does not grow too large, it provides relatively equal blood flow to both lungs without pulmonary hypertension, cardiac failure or interruption of continuity of pulmonary arteries. Surgical techniques have been devised that make the takedown at later repair less difficult than it was originally250 and, in our opinion, less difficult than takedown of the Waterston shunt when the right pulmonary artery must be patched to enlarge it or restore continuity.248,24g Medical palliative procedures: Thus far we have considered surgical palliation for these symptomatic babies. Medical palliation sometimes buys time until the infant grows a bit. If he is anemic, iron therapy may be given and then discontinued when the hemoglobin reaches 15 to 17 g and the hematocrit 48 to 50 percent. To persist in iron supplementation beyond this mildly polycythemic level is to run the risk of excessive polycythemia and its consequences. Another medical measure that may temporarily relieve the hypercyanotic spells that are attributed to infundibular spasm is use of the beta blocking drug propranolol. 251,252It can be administered orally or intravenously for an acute episode, and can be given four times daily beginning at a dose of 1 mg/kg per day and doubling or trebling that amount if necessary. There has been concern that in such patients who come to open repair, myocardial function may be impaired. One then has the problem of stopping the 1976

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medication for a period of time before the operation and running the risk of severe spells, or of continuing the therapy and perhaps compromising postoperative success. If babies are receiving propranolol, we have stopped the medication while they were in the hospital for a few days before surgery. Isoproterenol or catecholamines as pharmacologic antagonists may be helpful, but. we have not needed to use them. Open Corrective

Procedures

Tetralogy of Fallot: Open repair is now our preference over palliation for the patient with tetralogy of Fallot (Fig. 13 and 14) who as an infant beyond the 1st month of life has severe symptoms and favorable anatomy, with the pulmonary anulus and pulmonary artery at least one third the size of the aorta.22,165,253m 255 For the minimally symptomatic baby or toddler, operation can usually be deferred until age 2 or 3 years. Some patients are older than that when first referred to a cardiac center. Some have already had a shunt procedure and may range in age from preschoolers to adults. They are candidates for open repair unless the anatomy of the pulmonary tree is unsuitable.256 Open repair through a right ventriculotomy consists first of relief of the obstruction by resection of infundibular stenosis and incision along the lines of fusion of pulmonary valve cusps, and then of closure of the ventricular septal defect with a Teflon felt patch, taking care to avoid the conduction system.255 The hypoplastic outflow tract may need to be enlarged by a pericardial patch, which sometimes extends through the pulmonary anulus and out onto the main pulmonary artery to enlarge these areas too if they appear restrictive to pulmonary flow. Pressure measurements in the operating room help to judge this point. It is desirable if the right ventricular systolic pressure is less than 70 percent of the systemic pressure and pressure in the main pulmbnary artery is normal. In exceptional cases, pulmonary hypertension is measured that is due to peripheral pulmonary stenosis or hypertensive vascular changes related to a previous large systemic to pulmonary arterial shunt.257 Clinical results of open repair of tetralogy of Fallot usually are excellent from the patient’s point of

view, but the preexisting anatomy determines how “corrective” the operation is.164-167 The child whose anatomy is illustrated in Figure 13 has the least derangement from the normal (discrete infundibular pulmonary stenosis only, a normal-sized pulmonary anulus and pulmonary arteries and minimal overriding of the aorta). The repair should result in complete closure of the ventricular septal defect by the patch, without heart block, and in complete resection of the infundibular stenosis without damage to the valve and with no systolic pressure gradient between right ventricle and pulmonary artery. This is the uncommon tetralogy. Much more often there are multiple areas of stenosis with hypoplasia from the outflow tract through the pulmonary arterial branches, as in Figure 14. The 302

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pulmonary valve may be incompetent after incision. It is incompetent when the outflow tract of the right ventricle must be enlarged by a pericardial patch that extends through the valve ring and out onto the main pulmonary artery. Even then, the systolic pressure gradient between the right ventricle and pulmonary arterial branches often is not abolished. The pulmonary insufficiency that is a necessary consequence of this enlargement is usually well tolerated if there is no large residual left to right shunt through an incompletely closed ventricular septal defect. Postoperative complete heart block that was a problem in the early days of open heart surgery for tetralogy of Fallot has been prevented by proper placement of stitches to avoid the conduction bundle in the danger zone in the posteroinferior rim of the defect.255 Another potential problem is the postoperative pattern in the electrocardiogram of left anterior hemiblock (left axis deviation and right bundle branch block). Especially if tachycardia or ventricular premature beats occur spontaneously or on exercise, there is a risk of sudden death.258,25g It is our observation in short-term follow-up that early open heart surgery can be carried out in very young children with as much safety and with as good results as those *achieved in children over age 6 years. The mortality rate should be no more than 5 to 10 percent. These children have the benefit early of being acyanotic and of having no impairment to growth that can be attributed to cyanosis or to a surgically created left to right shunt. Early repair also forestalls problems that are acquired and make the anatomy worse as the patient grows older, problems such as progressive subvalvular pulmonary stenosis or even atresia. Initial reparative surgery avoids the problems mentioned earlier that may occur in longterm follow-up of systemic to pulmonary arterial shunts.14g-15g Pseudotruncus arteriosus: Patients with pseudotruncus arteriosus (Fig. 3, 4 and 12), that form of tetralogy with pulmonary atresia and systemic blood supply to the pulmonary arterial branches, usually get along moderately well through infancy and childhood. Open repair by the Rastelli procedure138 is best deferred in these patients until later childhood or adolescence, since part of the repair concerns the introduction of a homograft vessel and valve or of a [email protected] conduit with porcine valve to establish a route of blood flow to the pulmonary artery. Since there will be no growth of the graft, it is preferable to wait, if the patient’s condition permits, until a graft can be inserted that is large enough so that it will not later restrict pulmonary flow. The large vessels of collateral circulation are ligated near the aorta and the ventricular septal defect is closed at the repair. Longterm results are not yet available; however, one report that extended almost 5 years after the operation noted some residual or recurrent pulmonary stenosis but clinical well-being.260 Other complicated tetralogy-like lesions: Patients with tetralogy-like lesions are cdndidates for open heart surgery if the team is fully cognizant of Volume

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the anatomy and thoroughly experienced with techniques of repair of these unusual situations. Open repair may be complex if reconstruction of continuity of the right pulmonary branch is required2487249 or if an external conduit or a homograft or porcine valve is needed because there is absence of the pulmonary valve with pulmonary insufficiency,221p261 aortic origin of one pulmonary artery2(j2 or atresia of the pulmonary valve,lg8 main pulmonary artery or a main branch.26” The combination of transposition of the great arteries with ventricular septal defect and subvalvular pulmonary stenosis poses special problems that may be overcome by a combination of procedures such as a hammock-like patch closure of the ventricular septal defect to direct left ventricular blood to the aorta and use of an external valved conduit from the distal end of oversewn pulmonary artery to the right ventricle.264 Pulmonary stenosis with atria1 septal defect: Patients with pulmonary stenosis, intact ventricular septum and defective atria1 septum should undergo open repair when the diagnosis is made, since the stenosis must be severe in order for the right to left shunt at atria1 level to be established. Commonly the stenosis is purely valvular (Fig. 8) and is relieved through a pulmonary arteriotomy by incising out to the pulmonary anulus along the lines of fusion. UnIess the right ventricular systolic pressure measured in the operating room after the valvotomy is still elevated to near systemic levels, it is not necessary to do an infundibular resection since that secondary form of obstruction in the outflow tract of the ventricle (Fig. 8) subsides spontaneously as hypertrophy regresses after an effective valvotomy.265 Long-term results have shown relief of obstruction without recurrence.266 A murmur of pulmonary insufficiency is audible in about 25 percent of patients postoperatively, but the insufficiency appears of little physiologic consequence. Pure pulmonary atresia with intact ventricular septum: This is a difficult lesion to treat.267 It poses a different problem depending on whether the right ventricle is of adequate size or not. Sometimes it is considerably enlarged; at other times it is greatly underdeveloped. If the judgment is that the ventricle is too small to recieve and discharge a full complement of systemic venous return, we believe that an attempt to create an opening into the pulmonary artery has little chance of success and that a shunt procedure should be undertaken. Tricuspid atresia-Fontan operation: Some children with tricuspid atresia268 and patent pulmo-

nary arteries may benefit from the Fontan operation141 which in effect utilizes the hypertrophied right atrium as the right heart chamber, both as receptacle and as pump, to send blood to the lungs, where the resistance is low. This is done by closure of the atria1 septal defect and connection of the right atrium to the main pulmonary artery through a valved conduit. Signs of systemic venous congestion with hepatomegaly, ascites and edema have been frequent postoperative problems. This technique requires more long-term, careful evaluation before it is generally recommended. Ebstein’s anomaly of the tricuspid valve: Certain patients who are severely symptomatic and have great cardiomegaly experience symptomatic improvement and a decrease in heart size by insertion of an artificial tricuspid valve near the tricuspid anulus.134 Care must be exercised to avoid damage to the conduction tissue near the coronary sinus and central fibrous body. The tradeoff for this relief is the presence of an artificial valve with its own problems of anticoagulation and possible pulmonary embolism and of infection. We believe the indications for surgery should be strong before this procedure is undertaken. Partial or total anomalous systemic venous drainage: This is perhaps the rarest of all the conditions discussed herein. It has been treated successfully by interrupting the abnormal connection into the left atrium and redirecting the flow to the right side of the heart.26g-271 3. Deficient Pulmonary Flow With Pulmonary Hypertension at Systemic Level There is no surgical treatment for patients with Eisenmenger syndrome. Medical measures to relieve symptoms and to prolong life include phlebotomy or erythropheresis for excessive polycythemia and treatment of cardiac failure when that occurs. Some children, adolescents and young adults remain remarkably symptom-free for many years.226 Acknowledgment I should like to pay my respects to the team of doctors with whom T have been working: Drs. Kathryn Ehlers and Aaron Levin in Pediatric Cardiology; Harry Baltaxe in Cardiovascular Radiology; Marjorie Topkins in Anesthesiology; Paul A. Ebert and William Gay in Cardiovascular Surgery; and Sonia Cruz, Ronald Garutti, Seymour Hepner, Arthur Klein, Arthur Raptoulis and Laurel Steinherz who are Fellows in Pediatric Cardiology; as well as to our kindly and expert assistants as technicians or secretaries: Ms. Ethel Longo, Linda Yagoda, Jeanne Macaluso and JoanEllen Macredis.

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Blalock A, Taussig HB: The surgical treatment of malformations of the heart in which there is pulmonary stenosis or pulmonary atresia. JAMA 128: 189-202, 1945 2. Taussig HB: Congenital Malformations of the Heart. New York, The Commonwealth Fund, 1947. p 1 i-13. 553-572 3. McNamara DG: The role of the pediatric cardiologist in the team approach to cardiac surgery. In. Cardiovascular Clinics, Volume 4. number 3 (Engle MA, ed). Philadelphia, FA Davis, 1972, p 319-324

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cardium. In Ref. 3, p 43-57 Sahn OJ, Friedman WF: Difficulties in distinguishing cardiac from pulmonary disease in the neonate. Pediatr Clin North Am 20:293-301,1973 Comroe JH Jr, Forster RE, OuBois AB, et al: Clinical Physiology and Pulmonary Function Tests. Chicago, Year Book Medical Publishers, 1962 Smith RM: Anesthesia for Infants and Children. Saint Louis, CV Mosby, 1968 Hartmann RC: A hemorrhagic disorder occurring in patients with cyanotic heart disease. Bull Johns Hopkins Hosp 91:4967, 1952 Ekert H, Sheers M: Preoperative and postoperative platelet function in cyanotic congenital heart disease. J Thorac Cardiovast Surg 67:184-190, 1974 Oische MR: Observations on the morphological changes of the developing heart. In Ref. 3, p 175-191 Gramiak R, Shah PM, Kramer OH: Ultrasound cardiography. Contrast studies in anatomy and function. Radiology 92:939948, 1969 Lev M: The architecture of the conduction system in congenital heart disease. I. Common atrioventricular orifice. Arch Pathol 65:174-191, 1958 Lev M: The pathology of complete atrio-ventricular block. Prog Cardiovasc Dis 6:3 17-326, 1964 Lev M, Cuadros H, Paul M: Interruption of the atrioventricular bundle with congenital atrioventricular block. Circulation 43: 703-710,197l James TN: Anatomy of the human sinus node. Anat Ret 141: 109-139, 1961 James TN: The connecting pathways between the sinus node and A-V node and between the right and left atrium in the human heart. Am Heart J 66:498-508, 1966 James TN: Morphology of the human atrioventricular node, with remarks pertinent to its electrophysiology. Am Heart J 62:756-771, 1961 Massing GK, Liebman J, James TN: Cardiac conduction pathways in the infant and child. In Ref. 3, p 27-42 Van Mierop LHS, Alley RO, Kausel HW, et al: The anatomy and embryology of endocardial cushion defects. J Thorac Cardiovasc Surg 43:71-83, 1962 Van Mierop LHS: Pathology and pathogenesis of the common cardiac malformations. Cardiovasc Clin 2:27-59, 1970 Grant RP: The embryology of the ventricular flow pathways in man. Circulation 25:756-779. 1962 Van Praagh R, Van Praagh S: The anatomy of common aortice-pulmonary trunk (truncus arteriosus communis) and its embryologic implications. Am J Cardiol 16:406-425, 1965 Van Praagh R, Van Praagh S: Isolated ventricular inversion: a consideration of the morphogenesis, definition and diagnosis of nontransposed and transposed great arteries. Am J Cardiol 17:395-406, 1966 Goor DA, Oische R, Lillehei CW: The conotruncus. I. Its normal reverse torsion and conus absorption. Circulation 46: 375-384, 1972 Goor DA, Edwards JE: The spectrum of transposition of the great arteries: with specific reference to developmental anatomy of the conus. Circulation 48:406-4 15, 1973 Goor DA: The development of isolated VSD. persistent truncus arteriosus, A-V canal malformation, and transposition complexes. In Ref. 3, p 149-174 Bing RJ, Vandam LO, Gray FO: Physiological studies in congenital heart disease. I. Procedures. Bull Johns Hopkins Hosp 80:107-120, 1947 Dexter L, Haynes FW, Burwell CS, et al: Studies on congenital heart disease I. Technique of venous catheterization as a diagnostic procedure. J Clin Invest 26:547, 1947 Cournand A, Baldwin JS, Himmelstein A: Cardiac Catheterization in Congenital Heart Disease. New York, The Commonwealth Fund, 1949 Sones FM: Heart catheterization in infancy. Physiological studies. Pediatrics 16:544-554, 1955 Sones FM: Cine-cardio-angiography. Pediatr Clin North Am 5: 945-979, 1958 Rudolph AM, Cayler GG: Cardiac catheterization in infants and

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92. Liu L, Grlffith S: Implanted pacemakers in congenital

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diovasc Surg 68:711-718, 1974 Engle MA: Total anomalous pulmonary venous drainage: success story at last. Circulation 46:209-211, 1972 Ebert PA, Gay WA Jr, Oldham HN: Management of aorta-right pulmonary artery anastomosis during total correction of tetralogy of Fallot. Surgery 71:231-240, 1972 Gay WA Jr, Ebert PA: Aorta-to-right pulmonary artery anastomosis causing obstruction of the right pulmonary artery. Ann Thorac Surg 16:402-4 IO, 1973 Kirklln JW, Devloo RA: Hypothermic perfusion and circulatory arrest for surgical correction of tetralogy of Fallot with previously constructed Potts’ anastomosis. Dis Chest 39:87-91, 1961 Eriksson BO, Thoren C, Zetterqvlst P: Long-term treatment with propranolol in selected cases of Fallot’s tetralogy. Br Heart J 3 1:37-44, 1969 Ponce FE, Williams LC, Webb HIM, et al: Propranolol palliation of tetralogy of Fallot. Pediatrics 52:100-108. 1973 Ebert PA: Surgical treatment for tetralogy of Fallot: a quarter of a century of progress. In Ref. 3, p 305-318 Ebert PA: Operative treatment of cyanotic congenital heart disease. Pediatr Ann 3:90-95, 1974 Klrklln JW, Karp RB, Barcla A, et al: The Tetralogy of Fallot From a Surgical Viewpoint. Philadelphia, WB Saunders, 1970 Hislop A, Reid L: Structural changes in the pulmonary arteries and veins in tetralogy of Fallot. Br Heart J 35:1178-l 183. 1973 Ktnsley RH, McGoon DC, Danielson GK, et al: Pulmonary arterial hypertension after repair of tetralogy of Fallot. J Thorac Cardiovasc Surg 67:110-120, 1974 Wolff GS, Rowland TW, Ellison RC: Surgically induced right bundle-branch block with left anterior hemiblock: an ominous sign in postoperative tetralogy of Fallot. Circulation 46:587594, 1972 Downing JW, Kaplan S, Bove KE: Postsurgical left anterior hemiblock and right bundle branch block. Br Heart J 34:263270, 1972 Kawashima Y, Nalto Y, Kitamura S, et al: Use of large homograft artery with semilunar valve for correction of tetralogy of Fallot. A follow-up study. J Thorac Cardiovasc Surg 67:685693, 1974 Stafford EG, Malr DD, McGoon DC, et al: Tetralogy of Fallot with absent pulmonary valve: surgical considerations and results Circulation 48: Suppl lll:lll-24-111-30, 1973 Robin E, Sllberberg B, Ganguly SN, et al: Aortic origin of the left pulmonary artery: variant of tetralogy of Fallot. Am J Cardiol 35:324-329, 1975 Goldsmith M, Farina MA, Shaher RM: Tetralogy of Fallot with atresia of the left pulmonary artery. Surgical repair using a homograft aortic valve. J Thorac Cardiovasc Surg 69:458-466, 1975 Rastelli GC, McGoon DC, Wallace RB: Anatomic correction of transposition of the great’vessels with ventricular septal defect and pulmonary stenosis. J Thorac Cardiovasc Surg 58:545552, 1969 Engle MA, Holswade GR, Goldberg HP, et al: Regression after open valvotomy of infundibular stenosis accompanying severe valvular pulmonic stenosis. Circulation 17:862-873, 1958 Stone FM, Bessinger FB Jr, Lucas RV Jr, et al: Pre- and postoperative rest and exercise hemodynamics in children with pulmonary stenosis. Circulation 49: 1102-l 106, 1974 Vlad P: Pulmonary atresia with intact ventricular septum. In Ref 233, p 245-249 Tandon d, Edwards JE: Tricuspid atresia: a re-evaluation and classification. J Thorac Cardiovasc Surg 67530-542, 1974 Miller GAN, Ongley PA, Rastelli GC, eF al: Surgical correction of total anomalous systemic venous connection: report of a case. Mayo Clin Proc 40:532-538, 1965 Roberts KD, Edwards JM, Astley R: Surgical correction of total anomalous systemic venous drainage. J Thorac Cardiovast Surg 64:803-810, 1972 Crenshaw R, Okles JE, Phillips SJ, et al: Partial anomalous systemic venous return. Report of surgical treatment in two cases. J Thorac Cardiovasc Surg 69:433-436, 1975

Cyanotic congenital heart disease.

REVIEWS Cyanotic Congenital MARY New ALLEN ENGLE, MD, Heart Disease FACC York, New York From the Division of Pediatric Cardiology, The New Yo...
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