REVIEW

ARTICLE SURVEY OF OPERATIVE CONGENITAL HEART DISEASE

Survey of Operative Congenital Heart Disease A Review Jesse E. Edwards, MD

ANION-G THE LARGE V-ARIETY of congenital malformations of the heart and great vessels there are certain forms that have proved amenable to surgical therapy. Beyond these, there are other conditions with varying levels of incidence that have not yet been overcome by any standard procedure. It is the purpose of this review to consider those anomalies for which predictable surgical therapy now exists. Among the items to be considered are the nature of the anomaly and its variations, the tendency for other anomalies to be associated with it, and any details in pathologic anatomy and/or function that represent practical problems to the undertaking of therapy. For the purpose of this review, congenital cardiovascular anomalies will be divided into three categories based on dominant clinical features as follows: a) left-to-right shunts, b) right-to-left shunts, and c) obstructions. Left-tRW Shunt Delivery of highly oxygenated blood from the left side of the heart into the right side of the pulmonary arterial system may be termed left-to-right shunt. Two general conditions underlie such a process, one being a septal defect and the other an abnormal communication of vessels, either between a vessel and a cardiac chamber or between two vessels. Sept Defect Of the septal defects, there are those involving the ventricular septum and those involving the atrial septum. Ventricular Septal Defect

The most common cardiac anomaly is ventricular septal defect (VSD). This condition, termed isolated VSD, may occur alone or be part of a From the Departments of Pathology of United Hospitals-Miller Division, St. Paul, and the University of NMinnesota, Minneapolis, Minnesota. Supported by Grant 5 ROl HL-05694 from the US Public Health Service and Research Training Grant a TOl HL-05570 from the National Heart and Lung Institute. Address reprint requests to Dr. Jesse E_ Edwards, Department of Pathology, United Hospitals-Miller Division, 125 West College Avenue, St. Paul, MN 55102. 408

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complex of anomalies, as in the tetralogy of Fallot. This discussion will concern itself with isolated VSD but with the realization that it may be associated with other anomalies that do not form with it a recognized developmental complex. Regarding location of VSDs, it is recognized that the right ventricle possesses an inflow portion, or sinus, and an outflow portion, or infundibulum. Within the infundibulum is an oblique column of muscle, the parietal limb of the crista supraventricularis, that runs from the base of the pulmonary valve, above, to the anterior aspect of the tricuspid ring, below. Most VSDs allow communication between the subaortic area of the left ventricle and the infundibulum of the right ventricle. Of these, the majority lie below the parietal limb of the crista, and are termed infracristal VSDs. In this location the defect usually involves the membranous portion of the ventricular septum. For this reason, such defects have often been called membranous VSDs, but it should be emphasized that often more of the septum is involved than its membranous portion. 1-2 Less common than the infracristal VSD is that which lies above the crista, the supracristal VSD. Classically, the latter borders on the pulmonarv valve, and through the defect elements of the pulmonary and aortic valve are continuous. Defects involving the inflow portion of the ventricular septum are commonly called muscular VSDs. These may be either single or, more commonly, multiple. The latter type represent tortuous channels through the muscular part of the septum and may be difficult to follow because of this characteristic. The major conduction tissue of the heart is closely related to the infracristal type of VSD. The bundle of His, as it proceeds from the AV node, follows closely the posterior-inferior edge of such defects 3.4 and is therefore subject to injury by the surgical steps involved in closure of the defect. 5.6 From a functional point of view, VSDs may be divided into the obstructive (small) and the nonobstructive (large). VSDs that are large allow free communication between the ventricles with equalization of pressures between them. Pulmonary hypertension is part of the functional state of large VSD. A left-to-right shunt occurs in the uncomplicated state, and this, in turn, may be responsible for left ventricular failure. If the affected individual survives, obstructive pulmonary vascular lesions may occur with corresponding decrease in the volume of the left-to-right shunt and wvith the ultimate appearance of a right-to-left shunt. At this stage, surgical closure of the VSD is contraindicated.7

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Isolated XVSD may be associated with independent anomalies, some of which mav complicate the operation. Girod and associates 8 studied 46 cases of V'SD in which autopsy showed additional anomalies. The latter were classified as obstructive malformations of the aorta (12 cases), positional anomalies of the great vessels (13 cases; 5 were corrected transposition of the great vessels and 8 double outlet right ventricle), additional shunts (12 cases; 4 atrial septal defect, 3 patent ductus arteriosus, and 5 left ventricular-right atrial communications), intracardiac obstruction (5 cases; 3 subaortic stenosis and 2 anomalous muscle bundle right ventricle) and aortic insufficiency (3 cases). In the material from which Girod obtained 46 cases of VSD with associated anomalies, there were 28 specimens of VSD without associated anomalies. The rather high incidence of associated anomalies may relate to the fact that an autopsy series was studied and that the chance of death is greater w,hen an associated condition is present than when no associated condition exists. Regarding the association of atrial septal defect with VSD, it should be mentioned that some of the atrial septal defects may not be true anomalies but rather secondary openings. The latter result from left atrial enlargement from the left-to-right shunt of the VSD with secondary distortion of the foramen ovale mechanism, as pointed out by Tandon and Edwards.9 Aortic insufficiency complicating VSD may result from inadequate support of the aorta by virtue of the presence of the VSD. It is more likely to occur with the supracristal than the infracristal type of VSD. Spontaneous closure of VSDs is probably a common phenomenon during the first year of life.'0 If the defect is present beyond that period, it is more likely to stay open than to close naturally. There appear to be two mechanisms by which VSDs close spontaneously." In the infracristal type, the septal leaflet of the tricuspid valve overhangs the defect. This may become adherent to the edges of the defect either through healing of bacterial endocarditis or through fibrosis in that area responding to the traumatic effects of flow through the defect. The latter would be more likely in the case of small rather than large defects. The second mechanism involves the muscular type of VSD in which, by growth of the septum, interventricular communications become pinched off and finally occluded by fibrous tissue. Left Ventricular-Right Atrial Communications

A variant of V'SD is the condition in which a defect occurs in that small part of the membranous septum that separates the floor of the right atrium

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from the left ventricular outflow tract. The left ventricular blood shunts directlv into the right atrium. A similar functional effect occurs when, in the presence of an infracristal VSD, there is also a cleft in the overlying septal leaflet of the tricuspid valve.12 Atrial Septal Defect

Atrial septal defects (ASDs) may appear as isolated conditions or be part of a complex involving the ventricular septum and the atrioventricular valves. Classicallv, ASD is associated with a large left-to-right shunt, while the pulmonary arterial pressure remains normal. In some cases, by the time adulthood is reached pulmonary hypertension appears, based on the presence of occlusive lesions in the pulmonary arterioles.'314 In considering the various locations in which ASDs may occur, an important landmark is the fossa ovalis. Most often, isolated ASDs occur at the fossa ovalis, less commonly superior to the fossa, and least commonly posteroinferior of the fossa."5 Defect at the Fossa Ovalis. Characteristically, defects at the fossa ovalis are large single openings, although some may show a fenestrated character. While the author prefers to call defects in this location atrial septal defect of fossa ovalis type, others refer to it as secundum type of atrial septal defect.'6 This type of defect, while relatively remote from the AV node, is closely allied with the entrance of the inferior vena cava into the right atrium. If the valve of the inferior vena cava is large, its free edge may be misinterpreted as the posterior edge of the ASD. Under this circumstance during attempted surgical closure of the ASD, the edge of the valve of the inferior vena cava may be sutured to the anterior edge of the defect. This results in the opening of the inferior vena cava into the left atrium and the postoperative appearance of cyanosis (Text-figure 1). ASD at the fossa ovalis may be associated with mitral stenosis (Lutembacher svndrome). It may be the case that the defect is secondary to the mitral stenosis (Text-figure 2). The practical point is that signs of the ASD mav be dominant, while those of the mitral stenosis may be occult. There is obvious danger in closing the ASD while leaving the mitral stenosis unattended. Defect Superior to Fossa Ovalis. Less common than a defect at the fossa ovalis is one which occurs superior to the fossa. This defect, sometimes called a sinus venosus type of atrial septal defect, lies subjacent to the entrance of the superior vena cava into the right atrium. The vein may overhang the defect and be directed partially into the left atrium. The sinus venosus type of defect is constantly associated with anomalous

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1T_\-i -Hi(. BF 1-ASD of fossa RA ovalis type %vith prominent salse of inferior %ena casva A-Right atrium RA and right sentricleect RV The XSD is at the fossa V V 'F osalis The valse of the inferior senra casa 11-C is prominent '. C5 and obscures the posterior portion of the AS.D IV-C = inferior sena ostium of coronarv CaVa. CS sinus B-The relationship of the ASD to the salse of the infeR rior vena cava is sho.-n. C/ The entrance of the imferior sena cav a into the atrial portion of the f heart and in relation to the ASD is shos-sn in its natural state. LA = left atrium. D-Proper closure of XSD. w-herein infenror sena casa is directed into the right atritum. E-Faulty closure of XSD %-herein free edge of valve of inLA RA LA RA fen'or Xvena eava is sew-n to the A O A /V anitenror edve of the XSD. Inferior s-ena casa is noss- directed into the D C left atrin'um. Ic IV

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connection of right pulmonary veins, either to the superior vena cava near the right atrium or directlv to the right atrium. The right upper pulmonary vein is alw ays involved, while in the minority of cases the right lower pulmonary vein is involved as well. That anomalous connection of right pulmonary veins occurs in this condition is important to realize in the surgical treatment of the type of ASD involved. Ideallv, the defect is closed in such a wXav to allow direction of the anomalous pulmonarv veins into the left atrium. Otherwise, following closure of the ASD, anomalous connection of pulmonary veins remains. Defect Posteroinferior to Fossa OQalis. An uncommon ASD lies in the posteroinferior angle of the atrial septum. From the right side, it is at the anticipated location of the ostium of the coronary sinus. This defect is part of a developmental complex in which the coronary sinus is absent. Also, there is usually a persistent left superior vena cava which joins the left atrium directly at the base of the left atrial appendage.17 When this happens, the left superior vena cava should be interrupted; otherwise, a right-to-left shunt persists after the ASD is closed.

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secundum. Below- are shown stages of progression w-herein IAO 11 is pulled backsward as the left atrium dilates as a consequence of the mitral stenosis. U1ltimatelv. an ASD is deseloped bh- this process. Xs a left-to-right shunt develops, the foramen ov ale enlarges as part of right atrial enlargement. This compounds the basic effects. enlarging the XSD. (Illustration deseloped bh Dr. R. Tandon and the

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Atrial Septal Defect Inferior to Fossa Ovalis. Second only in incidence to ASD at the fossa ovalis is a defect below the fossa and involving the lowermost part of the atrial septum. Classically, this type of defect is part of a complex anomaly known variously as persistent common atrioventricular canal or endocardial cushion defect (AVC). The other components of the anomalv include clefts in the anterior mitral and/ or septal tricuspid leaflet as well as deficiency of the ventricular septum. The deficiency of the ventricular septum is most prominently evident in the subaortic area, but the height of the ventricular septum in its posterior aspect and the lateral wall of the left ventricle are also deficient."8 These changes account for the "goose neck" deformity of the left ventricle as seen in angiocardiograms. 1921 Though there is a deficiency in the amount of ventricular septal tissue, there need not be an interventricular communication. If the anterior mitral leaflet is not adherent to the posterior edge of the deficient ventricular septum, an interventricular communication occurs. In this

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state the dvnamics are basically like those in large V'SD. In some cases the anterior mitral leaflet is adherent to the ventricular septum, precluding an interventricular communication. The dynamics are then like those in ASD (mitral insufficiency may be associated as a consequence of the cleft of the anterior leaflet). Between these two mentioned extremes are cases in which chordae of the anterior mitral leaflet are adherent to the ventricular septum with spaces present between these. If the aggregate of the openings is restricted, the interventricular relationships are like those in small V7SD (in addition to the ASD). Two anatomic features of AVC are particularly pertinent with regard to surgical therapy, one concerning the ventricular septum and the other chordae tendineae of the mitral component of the atrioventricular valvular apparatus. The deficiency of the ventricular septum in the subaortic area dictates that some material be inserted to enlarge the ventricular septum.22 If the anterior mitral leaflet is sutured directly to the posterior edge of the ventricular septum, it is likely that a subaortic stenosis will be created. Regarding mitral chordae tendineae, it is the case that chordae extend into the edges of the cleft anterior leaflet. If the cleft is sutured closed, a single anterior leaflet results, and this has normal chordal connections with the papillarv muscles. Those chordae that extend into the edges of the cleft leaflet attach to the ventricular septum; if these attach at a relativelv low level of the septum, they may unduly restrain the surgically created single anterior mitral leaflet, resulting in mitral insufficiency. In order to prevent this, it is proper to cut the chordae attaching to the edges of the cleft if the cleft is to be closed.23 Regarding associated conditions, AVC is the most common type of congenital cardiac anomaly associated with Down's syndrome (mongolism.) 24 With respect to other cardiac anomalies associated with AVC, ASD at the fossa ovalis is perhaps the most common. Double orifice of the mitral valve is also fairly common. Such an association was noted in five of 28 specimens reviewed by Wakai and Edwards.25 A relatively uncommon yet interesting and complicated situation results from the association of the tetralogy of Fallot with AVC.26 Surgical treatment requires enlarging the deficient ventricular septum while closing the communication between the right ventricle and aorta that pertains in the tetralogy of Fallot. Abnormal Connections of Vessels

When vessels are involved in an abnormal connection so that they underlie a left-to-right shunt, the connection may be between two vessels

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or between a vessel and a right-sided cardiac chamber. The most common type is patent ductus arteriosus. Anomalous connections of pulmonary veins and ruptured congenital aortic aneurysm, anomalous origin or termination of a coronary artery, and aorticopulmonary window are less common types. Patent Ductus Arteriosus

Persistent patency of the normal fetal connection between the pulmonary arterial system and the aorta is usually referred to as persistent patent ductus arteriosus or, simply, patent ductus arteriosus (PDA). There are two forms of this condition from a functional viewpoint: a) the classic and b) the wide. Classic PDA offers sufficient resistance to flow so that aortic pressure is not fully transmitted to the pulmonary arterial system. During the entire cardiac cycle the aortic pressure is higher than the pulmonary arterial pressure. Therefore, a left-to-right shunt along with the classic machinery murmur are common expressions of this condition. In classic PDA, the pulmonary arterial pressure is near normal and the pulmonary vascular bed does not exhibit secondary obstructive lesions. The left ventricle is the only ventricle that carries the blood involved in the shunt. Its integrity is maintained except in the rare cases wherein the shunt is unusually large. The major danger in classic PDA is that infection may occur. This usually arises on the wall of the pulmonary artery opposite the pulmonary ostium of the ductus. The wide PDA represents an unobstructed opening between the aorta and pulmonary arterial system. Pulmonary hypertension is associated, and in the early years of life the large left-to-right shunt may become complicated with left ventricular failure. As a result of the latter, pulmonary venous and capillary pressures become elevated. In tum, there follows pulmonary congestion and edema. Pneumonia may be the ultimate complication. The pulmonary hypertension in its uncomplicated state is associated with medial hypertrophy of the muscular precapillary arterial vessels of the lungs. With time, the pulmonary hypertension associated with a wide PDA mav become complicated by occlusive lesions involving the large and small muscular arteries of the lung, as may occur in large VSD. These variously take the form of nonspecific intimal thickening and plexiform and "dilatation" lesions. Arteritis with thrombosis occurs, but uncommonly. 4 When the stage is reached in which widespread occlusive lesions appear in the pulmonary arterial bed, the pulmonary vascular resistance rises.

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The volume of the left-to-right shunt falls and may be followed by the appearance of a right-to-left shunt. At this stage, the condition has been called "reversing ductus." When it is reached, it is inadvisable to interrupt the ductus, since in its patent state it acts as an escape valve for blood from the obstructed pulmonary arterial system to escape into the aorta. The state of reversing ductus is usually not reached until adult life. There are infants in whom a right-to-left shunt occurs through the ductus. If this is of significant volume, it usually means that there is an obstructive lesion such as mitral stenosis or mitral atresia downstream from the pulmonarv arterial bed. Aorticopulmonary Window

A relatively unusual condition is that in which there is a perforation, termed AP window, between the pulmonary trunk and the ascending aorta. The basic dynamics are similar to those of wide PDA. When present, this defect usually occurs as an isolated entity, but in some cases other anomalies are associated with it. Neufeld and associates 27 reviewed 66 cases (60 from the literature) and found PDA to be the most commonly associated condition (8 cases; 12% ). Other conditions included coarctation of the aorta (3 cases), membranous subaortic stenosis (1 case), and origin of the right pulmonary artery from the aorta (1 case). A case of AP window associated with the tetralogy of Fallot was reported by Cooley and associates.28 A similar case was described by Tandon and associates among 3 cases of AP window associated with VSD.2 Anomalous Connection of Pulmonary Veins

The term anomalous connection of pulmonary veins is usually synonymous with anomalous pulmonary venous drainage. In most situations it means that part or all of the pulmonary venous system connects either with a systemic vein or with the right atrium. The condition may be partial, involving only some of the pulmonary veins, or total, in which the entire pulmonary venous system makes one or several abnormal communications, as indicated. Partial Forms. In some cases, partial anomalous pulmonary venous connection (APVC) is an isolated condition, while in other cases it is part of a syndrome with other anomalies. As an isolated anomaly, one pulmonary vein is usually involved, such as the left upper pulmonary vein joining the left innominate vein or the right upper pulmonary vein joining

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the superior vena cava. In this condition, the atrial septum may either be intact or show a defect at the fossa ovalis. In specific syndromes in which partial APVC is present along with other anomalies, there may be APVC of right veins to the superior vena cava or right atrium in association with an ASD superior to the fossa ovalis. Partial APVC of left pulmonary veins to the right atrium may be part of the syndrome of polysplenia.30 Total Forms. In total APVC, none of the pulmonary veins join the left atrium. The usual veins leave the pulmonary hila to join a venous confluence which lies outside of the pericardium and superior to the left atrium. From this confluence, an anomalous vein runs to a systemic vein. Systemic venous termination of the anomalous veins may be either below (infradiaphragmatic APVC) or above (supradiaphragmatic APVC) the diaphragm. In the infradiaphragmatic type, the anomalous vein originating at the pulmonary venous confluence descends into the abdominal cavity through the esophageal hiatus of the diaphragm in company with the esophagus. The vein terminates in an element of the portal venous system such as the ductus venosus, the portal vein, or the left gastric vein. Infradiaphragmatic types of APVC are usually associated with major degrees of pulmonary venous obstruction.31-3' This process results from one of several factors such as a) compression of anomalous vein at hiatus of diaphragm, b) stenosis of receiving systemic veins (especially if it is the ductus venosus), c) obligatory passage of the pulmonary venous blood through the sinusoidal system of the liver, and d) through stenotic lesions in the anomalous vein. Total supradiaphragmatic termination of pulmonary veins is usually into a systemic vein and most commonly into the left innominate vein, followed in order of decreasing frequency by the coronary sinus, the superior vena cava, and the azygos vein. Termination of the pulmonary veins directly into the right atrium is uncommon. When present, it is usually part of the polysplenic syndrome When the pulmonary venous system terminates in a supradiaphragmatic systemic vein, there is usually one large vein which ascends from the confluence of the pulmonary veins. In the commonest type wherein the anomalous vein joins the left innominate vein, the vein ascends usually anterior to the left pulmonary hilus (Text-figure 3). Usually, in this situation, pulmonary venous obstruction does not occur. Less commonly, the vein ascends between the left pulmonary artery, in front, and the left main bronchus, behind. In this situation, the vein is obstructed by a

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TEXT-FIGURE :3-Total APVC to the left innominate vein without pulmonarv venous obstruction. The ascending anomalous vein is wide and ascends anterior to the left pulmonarv hilus (from Elliott and Edwards,M4 reproduced with permission of the American Heart Association, Inc.).

"hemodvnamic vise"' (Text-figure 4). Additional causes of obstruction, which are uncommon, include a narrow state of or intrinsic stenotic lesions in the anomalous vein.3 37 Classically, total APVC is not associated with other cardiac anomalies unless asplenia is associated. L-4,., ..'

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TEXT-FIGURE 4-Total APVC to the left innominate vein with pulmonary venous obstruction. The ascending anomalous vein ascends between the left pulmonary artery, in front, and the left main bronchus, behind. The vein is caught in a "hemodvnamic vise" (from Elliott and Edwards,* reproduced with permission of the American Heart Association, Inc.).

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When all of the pulmonary venous blood enters the right atrium, either directlv or by union with a systemic vein, the only route through which the left side of the heart receives blood is a patent foramen ovale. Classically, the left-sided chambers are smaller than normal. Experience has shown, however, that these usually are of adequate size to handle a normal volume of blood after surgical correction of the anomaly.'" The latter consists of ligating the anomalous vein while connecting the pulmonarv venous confluence to the left atrium. Aortic Sinus Aneurysms

Communications on congenital bases may occur between the aorta, on one hand, and a cardiac chamber, on the other. There are two major tvpes, namely, ruptured aortic sinus aneurysm and aortico-left ventricular tunnel. Congenital aortic sinus aneurysm is a congenital anomaly in the sense that an aneurysm forms at a site of congenital weakness. The essential lesion is an avulsion of the aortic media from the aortic annulus. As the aorta loses its natural attachment, it retracts upward, leaving for its wall that structure which normally lies against the involved sinus.39 Two of the three aortic sinuses show this tendency, namely, the posterior (noncoronary) and the right. Aneurysms of the posterior aortic sinus classically present into the right atrium from its septal wall. If rupture occurs, an aortic-right atrial communication develops. In this location, aortic sinus aneurysms are usually not associated with other anomalies. Those aneurysms which arise from the right aortic sinus present into the infundibulum of the right ventricle. In this position, aortic sinus aneurysms tend to be associated with a VSD lying immediately subjacent to the right aortic cusp. A ruptured congenital aneurysm of an aortic sinus may become infected. In such a circumstance it may be difficult to distinguish this process from a sinus aneurysm which results from bacterial infection of the aortic wall. Aortico-Left Ventricular Tunnel

The aortico-left ventricular tunnel is a rare condition.40 It is characterized by both coronary arteries being present and, in addition, a vessel-like structure leaves the ascending aorta. It then enters the epicardium, penetrates the ventricular septum and opens into the left ventricle. The condition yields a functional state like that of aortic insufficiency. Congenital aortic stenosis may be present, and the epicardial part of the anomalous structure may become complicated by a

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saccular aneurysm. Treatment consists of closing the aortic ostium of the tunnel. Abnormal Origin or Termination of a Coronary Artery

Coronarv arteries may be involved in left-to-right shunts, either because of abnormal origin or by abnormal termination of one of its branches. Abnormal Origin. Coronary arterial origin may be from the pulmonarv trunk. While both arteries may originate abnormally, usually abnormal origin involves only one of the two vessels. It is much more common that the left artery originate from the pulmonary trunk than for the right to do so. After birth, there is a tendency for rich collaterals to develop between branches of the two coronary arteries. As these become established, an arteriovenous fistula, in effect, occurs through which blood from the aortic-arising coronary artery is diverted into that arising from the pulmonary trunk. Aortic blood is delivered into the pulmonary trunk through this system. The shunt has an adverse effect upon the myocardium through diversion of blood away from it and through an inadequate perfusion pressure. The myocardial effect results in infarction and/or sudden death. The myocardial infarction may be so located as to cause mitral insufficiency. In some cases, the latter condition may play a dominant role in the pattern of illness and may remain as a problem after correction of the coronary arterial anomaly.41 The subject of anomalous origin of the left coronary artery from the pulmonary trunk has been covered widely in the literature.42" Origin of the right coronary artery from the pulmonary trunk has received less attention, since it is much less common than anomalous origin of the left. There has been a myth that anomalous origin of the right is a benign condition when, in fact, it may give rise to clinical coronary disease. From published accounts, it does appear to be a fact that this condition tends not to be troublesome until adult life.45-47 Two forms of definitive therapy have been employed for anomalous origin of a coronary artery from the pulmonary trunk. One consists of ligating the anomalous artery, thereby converting the coronary system into that of a single coronary artery.47 The other, preferable method is to transplant the anomalous pulmonary origin into the ascending aorta " or to insert a graft between the aorta and the ligated anomalous artery." A particular problem arises when anomalous origin of a coronary artery from the pulmonary trunk is associated with a large VSD. When a defect is present the pulmonary arterial pressure is high and serves to perfuse the

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anomalous coronary artery. If the defect is closed, the pulmonary arterial pressure falls and mvocardial ischemia results.4950 Abniormlal Terminiationi. Anomalous termination of a coronarx artern usuallv involves a branch of either the right or left coronary artery, the branch terminating into the coronary sinus, the pulmonary trunk or any one of the cardiac chambers. 1.51 53 Classically, the parent vessel and its anomalously inserting branch are dilated; these areas of dilation may be the sites of complicating saccular aneurysms.

The treatment consists of interrupting the anomalous connection.48 During the operation, electrocardiographic monitoring is necessary. If temporary occlusion is associated with ischemic changes, it means that a normal feeding vessel has been obstructed. Dissection should be carried more distally to a point at which temporary occlusion does not vield electrocardiographic abnormalities. Right-toLeft Shunts Three major anomalies have received surgical attention for the treatment of right-to-left shunts. These consist of pulmonary valvular obstruction associated with intact ventricular septum, the tetralogv of Fallot, and complete transposition of the great vessels. A less common condition is pulmonary arteriovenous fistula. Puknonary Vahvular Obstuction With Intact Ventricular Septum

Under this category are included pulmonary stenosis and pulmonary atresia. In the latter, a right-to-left shunt at the atrial level is universal, w-hile among cases of pulmonary stenosis a right-to-left shunt is not always present. If present, the shunt occurs across the atrial septum. The subjects of pulmonary stenosis and of pulmonary atresia are each considered in a subsequent section, that dealing with obstructions.

Tetraom of Fallot The tetralogy of Fallot is the most common cause of a right-to-left shunt in individuals who survive infancy. The tetrad involves the presence of right ventricular hypertrophv, a subaortic XVSD, biventricular origin of the aorta (dextroposition of the aorta) and obstruction to pulmonary flow. The latter may varv from no channel being present between the right xentricle and the pulmonary trunk (pseudotruncus arteriosus) through severe, moderate, or mild pulmonary stenosis. Those cases with mild degrees of pulmonary stenosis tend not to yield cyanosis since the shunt is dominantly in a left-to-right direction. Pulmonary hypertension and other

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features of VSD are usually present. Such cases are commonly referred to as either acvanotic or pink tetralogies. In the classic example of the tetralogy of Fallot and in the pseudotruncus arteriosus there is sufficient obstruction to pulmonary flow to be responsible for a right-to-left shunt from the right ventricle into the aorta. The area of obstruction to pulmonary flow is usually dominant in the outflow tract of the right ventricle leading to the pulmonary trunk.55.56 While the pulmonary valve is commonly malformed (either bicuspid or unicuspid), and may contribute to pulmonary stenosis, this factor is usually of lesser degree than the associated infundibular stenosis. In isolated cases either the structure of the right ventricular infundibulum or the pulmonary valve alone may be the basis for pulmonary stenosis. A right aortic arch is present in about one-quarter of the cases. V'arious types of surgical techniques have been emploved in the treatment of the tetralogy of Fallot. The oldest is a shunt procedure introduced by Blalock and Taussig57 in which a subclavian artery is anastomosed to a pulmonarv artery. This was followed by Pott's procedure, in which the aortic arch is anastomosed to the left pulmonarv artery. 8 Each was attended by its own problem. Especially in the treatment of infants, a Blalock-Taussig anastomosis may be of inadequate caliber to increase pulmonary blood flow significantly in infants, while, unless controlled, the lumen of the anastomosis of the Pott's procedure mav allow excessive flow and so be responsible for left ventricular failure. Currently, a popular type of anastomosis consists of anastomosing the right pulmonary artery to the ascending aorta (Waterston procedure).59 In cases of excess flow through an anastomosis that is in place for months or years, there may appear organic obstructive changes in the pulmonary vascular bed. 62 The most common type of procedure currently practiced is the definitive correction of the anomaly. This involves cardiopulmonary bvpass as a basis for opening the right ventricle. Through this chamber the communication between the right ventricle and aorta is closed (termed closing the VSD), thus effecting relief of pulmonary stenosis. The latter procedure usually involves resecting right ventricular tissue to increase the caliber of the subpulmonary tract and performing a pulmonary valvotomy. Depending on details, a cloth "patch" may additionally be inserted in the anterior wall of the right ventricle and carried for varying distances across the pulmonary valve and into the pulmonary trunk. The most common problem, especially in the past, has been inadequate relief of pulmonary stenosis. Less common problems include a residual shunt from inadequate closure of the communication between the right

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ventricle and aorta and creation of a new shunt by over-resecting ventricular septal tissue, thus creating an unwanted VSD. As with VSD, injury to the major conduction tissue may occur, resulting in complete heart block.63 Another problem in the definitive treatment of the tetralogy of Fallot is that in performing the right ventriculotomy there may be injurv to a major coronary artery. This results from the phenomenon that in this condition major branches may cross the right ventricular infundibulum. Some of these are unusually prominent conal arteries, while others represent variation in origin of a vessel, as origin of the anterior descending artery from the right coronary artery. Other operations pertain when there is either the pseudotruncus arteriosus or when one of the two pulmonary arteries fails to arise from the pulmonarv trunk and, instead, has an aortic origin. For pseudotruncus arteriosus, common practice involves placing a valved graft between the right ventricle and the pulmonary trunk in addition to closing the VSD.&' For origin of a pulmonary artery from the aorta, attempts have been made to transplant the abnormally arising pulmonary artery to the pulmonary trunk as an additional step in the standard operation. General problems conceming the tetralogy of Fallot are that other anatomic states may simulate the condition. When unsuspected, these may yield surprises to the surgeon who embarks upon treatment of what he believes to be a classic condition. Simulating conditions include double outlet right ventricle with pulmonary stenosis, VSD and anomalous muscle bundle of the right ventricle, corrected transposition and/or single ventricle, each with pulmonary stenosis.65 Complte Trpion

The condition of complete transposition is characterized by a fundamentally normally constructed heart but one in which the great arteries are transposed and the aorta arises from the right ventricle and the pulmonary trunk from the left ventricle. This condition, which has a male dominance (4:1) classically causes death in infancy, although in exceptional cases the patients live to childhood or even adult life. In just under half of the cases, a VSD is present, while in the other the ventricular septum is intact. In less than a quarter, pulmonary stenosis is present."67 The Mustard procedure has evolved from many techniques attempting to direct pulmonarv venous blood into the aorta." In essence, this consists of replacing the natural atrial septum with an artificial septum in such a way as to direct pulmonary venous blood to the tricuspid valve and systemic venous blood to the mitral valve.

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Among the problems of this operation is the tendency for postoperative superior vena caval obstruction to occur. Other problems include the inordinately early occurrence of obstructive pulmonary vascular disease. Still another problem concems the presence of associated disease of the tricuspid and/or the mitral valve which may introduce such factors as tricuspid or mitral insufficiency.'970 A particular problem concerns overcoming pulmonary stenosis when present. While this problem may be caused by isolated pulmonary valvular stenosis, it is often more complex. In most cases the pulmonary stenosis results from the subpulmonary part of the left ventricle being formed by a distinct stenotic infundibulum. Iess commonly, subpulmonary stenosis results from anomalous attachment of the anterior mitral leaflet to the ventricular septum. Puknonary Atwervu Fitua

Pulmonary arteriovenous fistula is responsible for a right-to-left shunt as pulmonary arterial blood flows directly into the pulmonary venous system without passing through the pulmonary capillary bed. While excision of the fistula is the basic treatment, problems arise from the tendency for this condition to be manifested by multiple lesions. A variant of pulmonary arteriovenous fistula is that in which a pulmonary arterial branch makes apparent direct connection with the left atrium by joining one of the major pulmonary veins 71 (Text-figure 5). Obstuctions Congenital obstructions may occur at or in relation to any of the cardiac valves, in the aorta or in the pulmonary arteries. Aob

Of the locations at which aortic obstruction may occur, the most common is at the junction of the arch and descending portions. Uncommonly, localized stenosis may occur in the lower thoracic or abdominal portions. Somewhat more common than the latter two sites is the ascending aorta, yielding supravalvular aortic stenosis. Interruption, Tubular Hypoplasia, and Coarctation of the Aorta

Of the obstructions that occur at the junction of the arch and descending portions, there are three types-namely, interruption, tubular hypoplasia, and classic coarctation. In rare cases of interruption of the arch, an atretic strand represents the "interrupted" segment. Usually, there is no continuity between the arch and descending portions of the aorta. In about half of the cases, the

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nection of the right lo^.er pulmonarv arterv to the right los.er pulmonary vein. This variant of pulmonarv arteriovenous fistula is responsible for a right-to-left shunt (from Lucas and associates., reprR A ducrd svith permission of the Xkmeric-an Heart Association.

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interruption occurs just distal to the origin of the left subclavian artery, while in the other half the interruption occurs between the origins of the left common carotid and left subclavian arteries, the latter vessel arising from the descending aorta.72 Classically, the descending aorta is fed through a PDA. In only the exceptional case is there a normal heart, the most common malformation being a VSD. It is also common for the outflow portion of the left ventricle to be obstructed by a spur of muscle Iving distal to the position of the V'SD.73 Two problems pertain in attempted surgical treatment of interruption of the aortic arch. One is the common association of intracardiac malformations. The other relates to the fact that the usual patient is symptomatic in infancy. There remains the problem of aortic obstruction following survival of establishing continuitv between the initially two separated portions of the aorta. Tubular hypoplasia of the aorta is characterized by a histologically normal but grossly narrow segment of the aortic arch. This segment may lie bet-een the origins of the left carotid and subelavian arteries or, more commonly, just distal to the latter. In this position it may be difficult to distinguish the condition from the normally narrow state of the aortic isthmus, as observed in young infants. Tubular hypoplasia may appear as

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an isolated condition but more commonly some degree of it is associated with classic coarctation. Intracardiac malformations are not uncommon. Classic coarctation is characterized as a localized lesion of the aortic media, usuallv at the junction of the arch and descending portions. The medial tissue is focally thickened and protrudes as a curtain into the aortic lumen causing it to be narrowed in varied degree.74 The curtain involves the superior, anterior, and posterior walls of the aorta but not the lower. The narrowed lumen is, therefore, eccentric, lying near the lower wall. Externally, corresponding with the position of the medial curtain, there is often a sharp identation at the adventitial aspect of the vessel. Survival of individuals with coarctation appears to depend on two factors, namelv, associated conditions and the location of the coarctation with respect to the position of the aortic entrance of the ductus arteriosus. Regardless of the question of associated conditions, preductal coarctation has a less favorable outlook than does the postductal type. Survival with coarctation requires a collateral circulation to carry blood from the proximal to the distal segment. This depends on branches arising from branches of the subclavian arteries.75 Three potential problems face the patient treated for coarctation of the aorta. The first concerns those operated during infancy and childhood. In these, there is the chance of "restenosis" as the caliber of the aorta at the site of resection and anastomosis fails to grow in concert with the rest of the aorta. The second concerns the problem of associated cardiovascular conditions which, along with the coarctation, adversely influence the circulation. Becker and associates studied this problem in 100 autopsied cases.76 In addition to the occurrence of bicuspid aortic valve in almost half of the cases, they observed that associated anomalies were more common (91 %) among the 77 cases in which the patient was under 1 year of age at the time of death than in those who survived 2 years or longer. In the latter group, associated anomalies were found in 74% of 23 cases. The tvpes of associated anomalies included septal defects, PDA, tubular hypoplasia of the aortic arch and either left ventricular outflow or inflow obstruction. The third problem concerns the bicuspid aortic valve that is commonly associated with coarctation. Even after resection of the coarctation, this state has the potential for intrinsic aortic valvular insufficiency, bacterial endocarditis and, most commonlv over a long span of years, calcific aortic stenosis. Supravalvular Aortic Stenosis

Supravalvular aortic stenosis involves the ascending aorta. Three forms have been recognized, the membranous, the hour-glass, and the

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hvpoplastic tvpe. The membranous type is characterized by a localized fibrous encirclement of the aorta, a lesion somewhat comparable to that of membranous subaortic stenosis. The hour-glass type is self explanatory. In the hvpoplastic type, the entire length of the aorta is thickened. Supravalvular aortic stenosis is, in effect, one aspect of a systemic condition. Associated cardiovascular lesions include stenosis of the pulmonary arteries, branches of the aortic arch, and the coronary ostia. Surgical attempts at relieving the obstruction involves widening the aorta bv placement of a patch graft at the site of stenosis." Postoperative problems include inadequate relief of the obstruction, especially in the hvpoplastic type, and residual lesions in other parts of the vascular system. Subaortic Stenosis

Obstruction at the left ventricular outflow tract is commonly called subaortic stenosis. Subaortic stenosis may result from intrinsic disease of the left ventricular outflow tract, or it may be secondary to abnormalities of the endocardium or of the mitral valve.78 The intrinsic types are of two forms, namely, the membranous and hypertrophic muscular types. Membranous subaortic stenosis is characterized by a fibrous encirclement of the left ventricular outflow tract with some of the fibrous tissue attached to the ventricular aspect of the anterior mitral leaflet. The membranous type often shows jet lesions on the contact surfaces of the aortic cusps and these may be complicated by bacterial endocarditis.79 Resection of the fibrous ring is the essential method of treating membranous subaortic stenosis. As part of the ring involves the anterior mitral leaflet, there is danger of injury to the latter structure. Also, resection of ventricular septal tissue may cause injury to the bundle of His. The hvpertrophic muscular type of subaortic stenosis, also known as asvmmetric septal hypertrophy, is characterized by prominence of the base of the ventricular septum. The prominence is made up of atypical mvocardial fibers which form interlacing bundles.* Considerable speculation on the basis for left ventricular outflow obstruction in this condition has occurred. As mitral insufficiency is commonly associated with it, some have postulated that the basal attachment of the anterior mitral leaflet is abnormallv situated. The author has not been able to confirm such a claim. Through echocardiography, a firm understanding has been gained of the basis for subaortic stenosis in this condition. Such studies have shown that during ventricular systole, the anterior mitral leaflet flutters in

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proximity to the septal mass. At one time this causes left ventricular outflow obstruction and mitral insufficiency. Subaortic stenosis may result from mitral lesions. These may take the form of anomalous chordal attachment of the anterior mitral leaflet to the ventricular septum or accessory clusters of fibrous tissue attached to the anterior mitral leaflet that obstruct the left ventricular outflow tract. Accessorv endocardial tissue of the mitral valve's anterior leaflet or the left ventricle may produce polypoid masses that may obstruct the aortic orifice. The latter condition is important, as such masses are usually attached to the point of origin by distinct pedicles, making removal potentially simple. The Valves

Congenital obstruction of the cardiac valves may take the form either of atresia or stenosis. Atresia is an absolute term meaning that no opening is present, while the term stenosis indicates that there is an opening. XVarying degrees of stenosis may exist. In general, surgical techniques have been limited in instances of valvular stenosis, with the exception of atresia of the pulmonarv valve. Recent attempts at correction of tricuspid atresia have been forthcoming, while no definitive therapy has been proposed for aortic or mitral atresia. In the following section, particular attention will be focused on those valvular obstructive conditions for which predictable results may be obtained by surgical techniques. Aortic Stenosis

Left ventricular outflow obstruction may result from supravalvular aortic stenosis or subaortic stenosis, as described. The most common basis is stenosis of the aortic valve itself. Congenital aortic stenosis is characterized by the presence of a unicommissural unicuspid valve, yielding a modified dome. The degree of stenosis varies from case to case. Cases present either with a small or normal-sized left ventricle. When the left ventricle is small, its endocardium is also markedly thickened, the total picture being one of restricted capacity of the left ventricle for which definitive operation is not available. When, on the contrary, the left ventricle is of normal size, there is a potential for relief of the aortic valvular obstruction by performance of aortotomv. There is, however, danger of postoperative aortic insufficiency since elements of the deformed valve may become flail as a result of the surgical incisions into the valve.81 Another problem in aortic stenosis with normal-sized left ventricle is that there may be infarction of the left ventricular wall, including the

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papillary muscles. This process, in turn, maxy lead to mitral insufficiency of significant degree.82 In those cases of congenital aortic stenosis in which the obstructive factor is mild, no evidence of disease max appear until adult life. Byr this time the valve may become heavily calcified and evidences stenosis. The developmental sequence may be as follows: In early years, the deformed valve may not be regurgitant on the basis of flutter-valve action of the dome-like structure. As the valve becomes calcified and rigid, the fluttervalve action disappears and aortic regurgitation makes its appearance. In this state, the valve may first show signs of being obstructive (Text-figure 6). Pulmonary Stenosis

The pulmonary valve may be stenotic or atretic, while the ventricular septum is intact. In congenital pulmonary valvular stenosis with intact ventricular septum, the valve is dome-shaped, having the configuration of a truncated cone. The opening in the dome is the only channel of outlet for the right ventricle. This opening is frequently only a few millimeters

TEXT-FI(.tRE 6-Congenital unicuspid. unicommissural aortic stenosis. ADuring sentricular sy stole. the opened valve has the configuration of a modified stenotic dome. B-In the unaltered stage the valve may close during sentricular diastole bv a flutter valve effect. C-Beginning fibrosis and calcification of the salve interferes with flutter salse action. D-When salve becomes calcified, it cannot close properly. thereby introducing aortic insufficiencv. This serves to compound the effects of the basicallv stenotic salve

A

B

c.

D

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wide, and the pulmonary trunk shows unusual degrees of dilatation (poststenotic dilatation). As the right ventricular pressure is abnormally high, right ventricular hypertrophy is present and may be of such a degree as to encroach upon the cavity. In the infundibular area this process may add a feature of stenosis over that in the valve. This process may be responsible for residual right ventricular outflow obstruction after relief of the valvular stenosis.i In pulmonary stenosis with intact ventricular septum, the foramen ovale may either be sealed or maintain the fetal state of valvular patency. In the former circumstance, no shunt is possible while, in the latter, a right-to-left shunt may occur at the atrial level. An uncommon form of congenital pulmonary stenosis is characterized by fibrous dvsplasia of the three cusps of the pulmonarn' valve.4 Stenosis is caused by rigidity of the cusps and, in some cases, there max be an additional element of intrinsic narrowness of the pulmonary orifice. Pulmonary Atresia

Congenital pulmonary valvular atresia with intact ventricular septum is characterized by an imperforate fibrous membrane at the valve area. As there is no normal outlet for the right ventricle, an obligatory right-to-left shunt occurs at the atrial level through which returning systemic venous blood is carried into the left atrium. Pulmonary arterial supply depends on patency of the ductus arteriosus. Tw o types of right ventricle are seen in this condition.8586 One type is characterized by a small chamber associated with a markedly hypertrophied wall. The other type is characterized bv either a normal-sized or enlarged right ventricular chamber. This type of right ventricle depends on tricuspid insufficiencv. When present, the latter mav be a consequence of associated Ebstein malformation of the tricuspid valve. Limited numbers of successful results have been obtained from pulmonary valvotomy in cases having the latter type of right ventricle.87 W7hen the right ventricle is small, the size of the right ventricle causes severe degree of inflox obstruction so that performance of a pulmonary valvotomv falls short of correcting the hemodynamic abnormality. The ventricular musculature approaches the bases of the pulmonary cusps more closely than the aortic cusps. When a ventriculotomr' is done through the pulmonary trunk, it is possible for a dissecting tract to occur through right ventricular muscle rather than directly' into the right ventricular cavity.

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Akrmetrir Vales

Congenital stenosis mav involve either atrioventricular valve, but tricuspid stenosis is a rare condition. Mitral stenosis may occur for one of several anatomic reasons, the most common being the parachute deformity of this valve. The parachute mitral valve is characterized by the two usual cusps but only one papillary muscle being present." The chordae of both leaflets converge to extend into this structure so that the leaflets are restricted in movement, one away from the other. To reach the left ventricle, blood must flow through the spaces between the chordae. If the interchordal spaces are narrow, mitral stenosis occurs. The parachute mitral valve is part of a developmental complex which includes supravalvular ring of the left atrium, subaortic stenosis, and coarctation. In a given case, only one of several of these elements may be present. When present, each may be of varying severity. One significant lesion may obscure the evidence for another. For example, in a given case coarctation may appear to be the sole anomaly, but after treatment, mitral stenosis may become evident." The author is aware of 1 case wherein relief of mitral stenosis of a parachute mitral valve was accomplished. This was done by Dr. Frank Johnson of Minneapolis. The procedure was to resect sufficient chordae to increase the caliber of the interchordal spaces while, at the same time, preserving sufficient chordal support for the mitral leaflets. Less common causes of congenital mitral stenosis include the presence of large papillary muscles in association with short chordae 89 and the condition termed anomalous mitral arcade.90 Cor triatriatum, while not a condition involving a valve, is properly mentioned here as it may simulate mitral stenosis. The condition appears to result from faulty absorption of the pulmonary veins into the definitive left atrium. Grossly, it is characterized by division of the left atrium into upper and lower segments. The two are separated by a perforated muscular diaphragm. The pulmonary veins join the upper chamber and the atrial appendage communicates with the lower. If the perforation in the diaphragm is narrow, a point of stenosis exists.9' Therapy consists of resection of the diaphragm which separates the two components of the left atrium. References 1.

Edwards JE: The pathology of ventricular septal defect. Semin Roentgenol 1:2, 1966

2. Edwards JE: Ventricular septal defect: Unresolved problems. Am J Cardiol 19:832-849, 1967

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3. Lev M: The anatomic basis for disturbances in conduction and cardiac arrhvthmias. Prog Cardiovasc Dis 2:360-369, 1960 4. Titus JL Daugherty GW, Kirklin JW, Edwards JE: Lesions of the atrioventricular conduction system after repair of ventricular septal defect: Relation to heart block. Circulation 28:824-8, 1963 5. Reemtsma K, Delgado JP, Creech 0 Jr: Heart block following intracardiac surgery: Localization of conduction tissue injury. J Thor Surg 39:688-693, 1960 6. Kirklin JW, McGoon DC, DuShane JW: Surgical treatment of ventricular septal defect. J Thor Surg 40:763-775, 1960 7. Heath D, Helmholz HF Jr, Burchell HB, DuShane JW, Kirklin JW, Edwards JE: Relation between structural changes in the small pulmonary arteries and the immediate reversibility of pulmonary hypertension following closure of ventricular and atrial septal defects. Circulation 18:1167-1174, 1958 8. Girod DA, Raghib G, Adams P Jr, Anderson RC, Wang Y, Edwards JE: Cardiac malformations associated with ventricular septal defect. Am J Cardiol 17:73-82, 1966 9. Tandon R, Edwards JE: Atrial septal defect in infancy: Common association with other anomalies. Circulation 49:1005-1010, 1974 10. Nadas AS, Scott LP, Hauck AJ, Rudolph AM: Spontaneous functional closing of ventricular septal defects. N Engl J Med 264:309-316, 1961 11. Simmons RL, Moller JH, Edwards JE: Anatomic evidence for spontaneous closure of ventricular septal defect. Circulation 34:38-45, 1966 12. Gerbode F, Hultgren H, Melrose D, Osborn J: Syndrome of left ventricular-right atrial shunt: Successful surgical repair of defect in five cases, with observation of bradyeardia on closure. Ann Surg 148:433-446, 1958 13. Edwards JE: The Lewis A. Conner memorial lecture. Functional pathology of the pulmonary vascular tree in congenital cardiac disease. Circulation 15:164-196, 1957 14. Heath D, Edwards JE: The pathology of hypertensive pulmonary vascular disease: A description of six grades of structural changes in the pulmonary arteries with special reference to congenital cardiac septal defects. Circulation 18:533-547, 1958 13. Edwards JE: The pathology of atrial septal defect. Semin Roentgenol 1:24, 1966 16. Lewis FJ, Winchell P, Bashour FA: Open repair of atrial septal defects: Results in sixty-three patients. JAMA 165:922-927, 195;7 17. Raghib G, Ruttenberg HD, Anderson RC, Amplatz K, Adams P Jr, Edwards JE: Termination of left superior vena cava in left atrium, atrial septal defect, and absence of coronary sinus: A developmental complex. Circulation 31:906-918, 1965 18. Goor D, Lillehei CW, Edwards JE: Further observations on the pathology of the atrioventricular canal malformation. Arch Surg 97:954-962, 1968 19. Baron MG: Endocardial cushion defects. Radiol Clin N Am 6:343-360, 1968 20. Rastelli G, Kirklin JW, Kincaid OW: Angiocardiography of persistent common atrioventricular canal. Proc Mayo Clin 42:200-209, 1967 21. Blieden LC, Randall PA, Castaneda AR, Lucas RV Jr, Edwards JE: The "goose neck" of the endocardial cushion defect: Anatomic basis. Chest 65:13-17, 1974 22. Rastelli GC, Ongley PA, Kirklin JW, McCoon DC: Surgical repair of the complete form of persistent common atrioventricular canal. J Thor Cardiov Surg 55:299-308, 1968 23. Edwards JE: The problem of mitral insufficiency caused by accessory chordae tendineae in persistent common atrioventricular canal. Proc Mayo Clin 35:299-305, 1960

24. Tandon R, Edwards JE: Cardiac malformations associated with Down's syndrome. Circulation 47:1349-1355, 1973 25. Wakai CS, Edwards JE: Pathologic study of persistent common atrioventricular canal: Review. Am Heart J 56:779-794, 1958 26. Tandon R, Moller JH, Edwards JE: Tetralogy of Fallot associated with persistent

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37. 38. 39. 40. 41. 42.

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common atrioventricular canal (endocardial cushion defect). Br Heart J 36:197-206, 1974 Neufeld HN. Lester RG, Adams P Jr, Anderson RC, Lillehei CNN', Edwards JE: Aorticopulmonary septal defect. Arm J Cardiol 9:12-25, 1962 Coolev DA, McNamara DG, Latson JR: Aorticopulmonan- septal defect: Diagnosis and surgical treatment. Surgery 42:101-120, 1957 Tandon R, da Silva CL, Moller JH, Edwards JE: Aorticopulmonary septal defect coexisting with ventricular septal defect. Circulation 30:188-191, 1974 Moller JH, Nakib A, Anderson RC, Edwards JE: Congenital cardiac disease associated with polysplenia: A developmental complex of bilateral "left-sidedness." Circulation :36:789-799, 1967 Keith JD, Rowe RD, Xlad P, O'Hanlev JH: Complete anomalous pulmonary venous drainage. Am J Med 16:23-38, 1954 Johnson AL., Wiglesworth FXV, Dunbar JS, Siddoo S, Grajo NI: Infradiaphragmatic total anomalous pulmonary venous connection. Circulation 17:340-347, 1938 Harris GBC, Neuhauser EBD, Giedion A: Total anomalous pulmonary venous return below the diaphragm: The roentgen appearances in three patients diagnosed during life. Am J Roentgenol 84:436-441, 1960 Lucas RN Jr, Adams P Jr, Anderson RC, V'arco RL, Edwards JE, Lester RG: Total anomalous pulmonary venous connection to the portal venous system: A cause of pulmonanr venous obstruction. Am J Roentgenol 86:361-573, 1961 Elliott LP, Edwards JE: The problem of pulmonary venous obstruction in total anomalous pulmonary venous connection to the left innominate vein (Editorial). Circulation 25:913-913, 1962 Kauffman SL, Ores CN, Andersen DH: Two cases of total anomalous pulmonary venous return of the supracardiac type with stenosis simulating infradiaphragmatic drainage. Circulation 25:376--382, 1962 Carey LS, Edwards JE: Severe pulmonarv venous obstruction in total anomalous pulmonary venous connection to the left innominate vein: Report of case. Am J Roentgenol 90:593-598, 1963 Gersony W\'NM, Bowman FO Jr, Steeg CN, Haves CJ, Jesse MJ, NMalm JR: Management of total anomalous pulmonary venous drainage in early infancy. Circulation 4:3 & 44 (suppl I):119-I124, 1971 Edwards JE, Burchell HB: The pathological anatomy of deficiencies between the aortic root and the heart, including aortic sinus aneurysms. Thorax 12:125-139, 1937 Lev. MJ, Lillehei CNN, Anderson RC, Amplatz K, Edwards JE: Aortico-left ventricular tunnel. Circulation 27:841-853, 1963 Noren GR, Raghib G, NMoller JH, Amplatz K, Adams P Jr, Edwards JE: Anomalous origin of the left coronary artery from the pulmonanr trunk with special reference to the occurrence of mitral insufficiencv. Circulation 30:171-179, 1964 Keith JD: The anomalous origin of the left coronary artery from the pulmonary artery. Br Heart J 21:149-161, 1959 Agustsson NIH, Gasul BNI, Lundquist R: Anomalous origin of left coronary artery from the pulmonary artery (adult type): A case report. Pediatrics 29:274-282, 1962 Rudolph AN\, Gootman NL, Kaplan N, Rohman NI: Anomalous left coronary artery arising from the pulmonary artery with large left-to-right shunt in infancy. J Pediatr 63:543-549, 196:3 Wald S, Stonecipher K, Baldwin BJ, Nutter DO: Anomalous origin of the right coronary artery from the pulmonary artery. Am J Cardiol 27:677-681, 1971 Tingelstad JB, Lower RR, Eldredge WVJ: Anomalous origin of the right coronary artery from the main pulmonarv artenr. Am J Cardiol 30:670-673, 1972 Rowe GG, Young WVP: Anomalous origin of the coronary arteries w%ith special reference to surgical treatment. J Thor Cardiov Surg 39:7 7-780, 1960

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48. El-Said GM, Dawson JT Jr, Sandiford FM, Mullins CE, Hallman GL, Cooley DA, McNamara DG: Coronary artery anomalies: Diagnosis, indications and results of surgical management. Eur J Cardiol 1:63, 1973 49. Feldt RH, Ongley PA, Titus JL: Total coronary arterial circulation from pulmonary artery with survival to age seven: Report of a case. Proc Mayo Clin 40:539-543, 1965 .50. Monselise MB, Vlodaver Z, Neufeld HN: Single coronary artery: Origin from the pulmonary trunk in association with ventricular septal defect. Chest 58:613-616, 1970 .31. Neufeld HN, Lester RG, Adams P Jr, Anderson RC, Lillehei CW, Edwards JE: Congenital communication of a coronary artery with a cardiac chamber or the pulmonary trunk ("coronary artery fistula"). Circulation 24:171-179, 1961 52. Noonan JA, Spencer FC: Single coronary artery with coronary arteriovenous fistula communicating with the right ventricle. Am J Cardiol 15:848-852, 1965 .5:3. Oldham HN Jr, Ebert PA, Young WG, Sabiston DC Jr: Surgical management of congenital coronarv artery fistula. Ann Thor Surg 12:5034513, 1971 34. Edwards JE, Gladding TC, Weir AB Jr: Congenital communication between the right coronarv arterv and the right atrium. J Thor Surg 35.-662-673, 1958 .z). Lev NM, Eckner FAQ: The pathologic anatomy of tetralogy of Fallot and its variations. Dis Chest 43:251-261, 1964 ,56. Rao BNS, Anderson RC, Edwards JE: Anatomic variations in the tetralogy of Fallot. Arn Heart J 81:3614-371, 1971 57. Blalock A, Taussig HB: The surgical treatment of malformations of the heart in which there is pulmonary stenosis or pulmonary atresia. JAMA 128:189-20'2, 1945 58. Potts WJ, Smith S, Gibson S: Anastomosis of the aorta to a pulmonary artery; certain types in congenital heart disease. JAMA 132:627-631, 1946 59. Waterston DJ: [The treatment of Fallot's tetralogy in infants under the age of one year. ] Rozhl Chir 41:181-183, 1962 60. Daoud G, Kaplan S, Helmsworth JA: Tetralogy of Fallot and pulmonary hypertension. Complication after systemic-to-pulmonary anastomosis. Am J Dis Child 111:166-177, 1966 61. Hancock EW, Hultgren HN, March HW: Pulmonary hypertension after BlalockTaussig anastomosis. Am Heart J 67:817-823, 1964 62. Roberts WVC, Friesinger GC, Cohen LS, Mason DT, Ross RS: Acquired pulmonic atresia: Total obstruction to right ventricular outflow after systemic to pulmonary arterial anastomoses for cyanotic congenital cardiac disease. Am J Cardiol 24:&335-345, 1969 6:3. Titus JL, Daughertv GW, Edwards JE: Anatomy of the atrioventricular conduction system in ventricular septal defect. Circulation 28:72-81, 1963 64. Rastelli GC, Ongley PA, Davis GD, Kirklin JW: Surgical repair for pulmonary valve atresia with coronary-pulmonary artery fistula: Report of a case. Proc Mayo Clin 40:521-527, 1965 65. Rao BNS, Edwards JE: Conditions simulating the tetralogy of Fallot. Circulation 49:173-178, 1974 66. Lev MI, Alcalde VMI, Baffes TG: Pathologic anatomy of complete transposition of the arterial trunks. Pediatrics 28:293-430, 1961 67. Elliott LP, Neufeld HN, Anderson RC, Adams P Jr, Edwards JE: Complete transposition of the great vessels. I. An anatomic study of sixty cases. Circulation 27:1105-1117, 1963

68. Mlustard WT: Successful two-stage correction of transposition of the great vessels. Surgerv 5:469-472, 1964 69. Layman TE, Edwards JE: Anomalies of the cardiac valves associated with complete transposition of the great vessels. Am J Cardiol 19:247-255, 1967 70. Tynan NI, Aberdeen E, Stark J: Tricuspid incompetence after the Mustard oper-

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72. 73.

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ation for transposition of the great arteries. Circulation 45 & 46 (suppl I):I111-1115, 1972 Lucas RV Jr, Lund GW, Edwards JE: Direct communication of a pulmonary artery with the left atrium: An unusual variant of pulmonary arteriovenous fistula. Circulation 24:1409-1414, 1961 Moller JH, Edwards JE: Interruption of aortic arch: Anatomic patterns and associated cardiac malformations. Am J Roentgenol 95:557-572, 1965 Becu LM, Tauxe WN, DuShane JW, Edwards JE: A complex of congenital cardiac anomalies: Ventricular septal defect, biventricular origin of the pulmonary trunk, and subaortic stenosis. Am Heart J 50:901-911, 1955 Edwards JE, Christensen NA, Clagett OT, McDonald JR: Pathologic considerations in coarctation of the aorta. Proc Mayo Clin 23:324-332, 1948 Edwards JE, Clagett OT, Drake RL, Christensen NA: The collateral circulation in

coarctation of the aorta. Proc Mayo Clin 23:333-39, 1948 76. Becker AE, Becker MJ, Edwards JE: Anomalies associated with coarctation of aorta. Particular reference to infancy. Circulation 41:1067-1075, 1970 77. Rastelli GC, McGoon DC, Ongley PA, Mankin HT, Kirklin JW: Surgical treatment of supravalvular aortic stenosis: Report of 16 cases and review of literature. J Thor Cardiov Surg 51:8734-82, 1966 78. Sellers RD, Lillehei CW, Edwards JE: Subaortic stenosis caused by anomalies of the atrioventricular valves. J Thor Cardiov Surg 48:289-302, 1964 79. Morrison RW, Edwards JE: Subaortic stenosis: Report of two cases, one associated with patent ductus arteriosus, the other complicated by bacterial endocarditis. Bull Int Assoc Med Museums 31:734-3, 1950 80. Maron BJ, Edwards JE, Henry WL, Clark CE, Bingle GJ, Epstein SE: Asymmetric septal hypertrophy (ASH) in infancy. Circulation 50:809-820, 1974 81. Ellis FH Jr., Kirklin JW: Congenital valvular aortic stenosis: Anatomic findings and surgical technique. J Thor Surg 43:199-202, 1962 82. Moller JH, Nakib A, Edwards JE: Infarction of papillary muscles and mitral insufficiency associated with congenital aortic stenosis. Circulation 34:87-91, 1966 83. Kirklin JW, Connolly DC, Ellis FH Jr, Burchell HB, Edwards JE, Wood EH: Problems in the diagnosis and surgical treatment of pulmonic stenosis with intact ventricular septum. Circulation 8:849-8, 1953 84. Koretzkv ED, Moller JH, Korns ME, Schwartz CJ, Edwards JE: Congenital pulmonary stenosis resulting from dysplasia of valve. Circulation 40:43-53, 1969 85. Davignon AL, Greenwold WE, DuShane JW, Edwards JE: Congenital pulmonary atresia with intact ventricular septum: Clinicopathologic correlation of two anatomic types. Am Heart J 62:591-602, 1961 86. Elliott LP, Adams P Jr, Edwards JE: Pulmonary atresia with intact ventricular septum. Br Heart J 25:489-SO1, 1963 87. Bowman FO Jr, Malm JR, Hayes CJ, Gersony WM, Ellis K: Pulmonary atresia with intact ventricular septum. J Thor Cardiov Surg 61:85-95, 1971 88. Shone JD, Sellers RD, Anderson RC, Adams P Jr, Lillehei CW, Edwards JE: The developmental complex of "parachute mitral valve," supravalvular ring of left atrium, subaortic stenosis, and coarctation of aorta. Am J Cardiol 11:714-725, 1963 89. Castaneda AR, Anderson RC, Edwards JE: Congenital mitral stenosis resulting from anomalous arcade and obstructing papillary muscles: Report of correction by use of ball valve prosthesis. Am J Cardiol 24:237-240, 1969 90. Lavman TE, Edwards JE: Anomalous mitral arcade: A type of congenital mitral insufficiency. Circulation 35:389-395, 1967 91. Marin-Garcia J, Tandon R, Lucas RV Jr, Edwards JE: Cor triatriatum: Study of 20 cases. Am J Cardiol 35:59-66, 1975

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American Journal of

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Pathology

Survey of operative congenital heart disease. A review.

REVIEW ARTICLE SURVEY OF OPERATIVE CONGENITAL HEART DISEASE Survey of Operative Congenital Heart Disease A Review Jesse E. Edwards, MD ANION-G THE...
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