Pediatric Radiology
Pediat. Radiol. 6, 132-140 (1977)
9 by Springer-Verlag1977
Total Anomalous Pulmonary Venous Connection Report of Ten Cases and Review of the Literature
S. B. Paster1, R. E. Swensson2, and S. M. Yabek2 Departments of 1Radiology and 2pediatrics, University of New Mexico, School of Medicine, Albuquerque, New Mexico, USA
Abstract. Ten cases of total anomalous pulmonary venous connection, all presenting at or below the age of eight weeks, are presented: three below the diaphragm, two to the coronary sinus, one to the right atrium, three to the left vertical vein, and one to the right superior vena cava. Six patients had other cardiac anomalies, and surgical mortality represented 75 %. A review of the subject is presented. Key words: Veins, pulmonary heart - abnormalities - Lungs, blood supply
After complete history and physical examination each patient had an EKG, chest radiograph, and complete cardiac catheterization with angiography. Table 1 summarizes the pertinent clinical, echocardiographic, roentgenologic, catheterization and angiographic, and follow-up data. Associated cardiac anomalies occurred in six of the ten patients in this series (60 %) and include patent ductus arteriosus in three patients, hypoplastic left ventricle in two patients, and complex congenital heart disease associated with the asplenia syndrome in one patient.
Discussion
Total anomalous pulmonary venous connection (TAPVC) is an uncommon abnormality comprising 1 to 2 % of all congenital heart lesions [9, 21, 22]. Despite its relative infrequency and high mortality, reports of increasing surgical success are now appearing. A review of our ten patients under two months of age is presented along with a review of the subject. Clinical Material The present clinical series consists of ten patients, two months of age and under, who presented to the University of New Mexico School of Medicine during a forty-three month period from September 1973 through April 1976. They ranged in age from one day to two months. Three patients presented within the first week of life, six patients presented from one to six weeks, and one patient presented at eight weeks. There were seven males and three females. All patients presented with cyanosis and cardiac murmurs; three patients also had feeding difficulties.
Emb~o~ The pulmonary venous system has a dual origin [1, 2]. The lungs develop as an outpouching of the ventral wall of the foregut and the splanchnic plexus covering the foregut becomes the anlage of the venous plexus covering the lung bud [3, 4]. The common pulmonary vein develops as an outpouching from the dorsum of the sinus venosus [1, 3, 4, 5]. The splanchnic venous plexus covering the lung bud develops a communication with the common pulmonary vein and this communication shifts leftward with the development of the interatrial septum to finally drain into the left atrium. Differential growth results in all four tributary veins emptying into the left atrium separately [3, 4]. During early stages of development of the lungs, the splanchnic venous plexus covering the foregut does not directly communicate with the heart but normally Communicates with the surrounding systemic and visceral veins. The
S. B. Paster et al.: Total Anomalous Pulmonary Venous Connection
133
Table 1. Clinical Data Case
Age/Sex
Clinical presentation
EKG
Chest x-ray
1
2 day/F
Cyanotic, murmur
Small heart, pulmonary venous congestion
2
7 day/M
Cyanotic, murmur
RAD, RVH, no posterior forces RAD, RVH
3
5 week/M
Cyanotic, murmur
RAD, RVH, RAE
Normal heart, pulmonary venous congestion
4
2 month/M
Cyanotic, pneumonia, congestive failure, murmur
RAD, RVH, RAE
Large heart, overcirculation, pulmonary venous congestion
5
5 week/F
Cyanotic, congestive failure, RDS, difficulty feeding, murmur
RAD
Large heart, pulmonary venous congestion
6
4 week/M
Cyanotic, difficulty feeding, murmur
RAD, RVH
Large heart, pulmonary venous congestion
7
12 day/M
Cyanotic, murmur
Small heart, diminished blood flow
8
6 week/M
Cyanotic, pneumonia, difficulty feeding, murmur
RAD, RVH, poor posterior forces RVH
9
1 day/F
Cyanotic, murmur
RVH
Large heart, pulmonary venous congestion, snowman
10
51/2 week/M
Cyanotic, murmur
RVH
Small heart initially -~ large heart, overcirculation, pulmonary venous congestion
Case
Cath/Angio
Follow-up (Surg or post)
1
TAPVC below diaphragm (to IVC), PDA, (right ~ left)
Surgery ~ death
2
TAPVC below diaphragm
Surgery ~ death
3
TAPVC below diaphragm, PDA (right ~ left)
Surgery -~ death
4
TAPVC to right atrium (right side also drained separately into the RSVC
Surgery -* death
TAPVC to coronary sinus
Successful surgical correction
TAPVC to coronary sinus
Surgery -~ death, associated hypoplastic left ventricle found at surgery
TAPVC to RSVC, transposition of great arteries, common ventricle, hypoplastic pulmonary artery, asplenia
Palliative surgery (Waterston) -~ death, postmortem revealed bilateral SVC, one atrioventricular valve, absent coronary sinus and right aortic arch with aberrant right subclavian artery
TAPVC to left vertical vein
Successful surgical correction
TAPVC to left vertical vein, hypoplastic left ventricle and aortic atresia
No surgery, died
TAPVC to left vertical vein, PDA (right ~ left)
No surgery, cardiac arrest --- died, post documented TAPVC to left vertical vein along with PDA
10
Small heart, pulmonary venous congestion
Borderline heart size, overcirculation, pulmonary venous congestion
Abbreviations: RAD = Right axis deviation RVH = Right ventricular hypertrophy RAE = Right atrial enlargement s y s t e m i c v e i n s are f o r e r u n n e r s o f t h e pre- a n d post-cardial veins which eventually form the s u p e r i o r v e n a cava, i n n o m i n a t e v e i n , a z y g o s v e i n s , a n d c o r o n a r y sinus. T h e v i s c e r a l v e i n s o r u m b i l i c o vitelline s y s t e m are f o r e r u n n e r s f o r t h e p o r t a l v e i n , d u c t u s v e n o s u s , a n d i n f e r i o r v e n a c a v a [3, 4]. I f
d u r i n g t h e n o r m a l stages o f e m b r y o n i c d e v e l o p m e n t , failure o f c o n n e c t i o n o c c u r s o r if obliteration of a normally developed connection ensues, t h e e m b r y o n i c c o n n e c t i o n s p e r s i s t to c r e a t e t h e s p e c t r u m o f total a n o m a l o u s p u l m o n a r y v e n o u s c o n n e c t i o n . T A P V C to t h e r i g h t a t r i u m o c c u r s
S. B. Paster et al.: Total Anomalous Pulmonary Venous Connection
134 Table 2. Classification of TAPVC by frequency Burroughs & Edwards Supracardiac 56 Left vertical vein (LSVC) RSVC 31 Cardiac 30 Right atrium Coronar 18 Infracardiac 28 Mixed 16 Total 179
Carter & Capriles
Jensen & Blount
W u k a s c h Gathman & Cooley & Nadas
Delisle& Van Praagh
Nakib Total & Edwards No. %
34
12
60
26
26
12
226
37 %
7 3
2 6
15 15
8 3
16 7
6 6
85 70
14% 11%
20 8 3 75
5 2 0 27
18 8 9 125
14 16 8 75
17 22 5 93
8 9 3 44
100 93 44 618
16 % 15% 7% 100%
if the atrial septum has grown abnormally far to the left; in this particular instance, no abnormal embryologic communication exists [3, 4].
Classification and Incidence A c o m m o n l y accepted classification of TAPVC into four types is based on the works of Darling and Snellen [2, 6]. Type I TAPVC occurs at the supracardiac level with drainage into a r e m n a n t of the left vertical vein (or left superior vena cava 1) or directly into the right superior vena cava. Type II TAPVC occurs at the cardiac level with drainage either into the coronary sinus or directly into the fight atrium. Type III TAPVC occurs at the infracardiac level i. e. below the diaphragm and drains into the portal vein, ductus venosus, or rarely into the inferior vena cava. Type IV TAPVC is a mixed pattern and occurs w h e n connection is to more than one of the above levels [2]. The frequency with which each type occurs is shown in Table 2 in which six h u n d r e d eighteen cases have been compiled from seven large series [4, 8-13]. This table includes TAPVC in both isolated form and also in association with other forms of congenital heart disease. TAPVC to the left vertical vein or left superior vena cava comprises the largest percentage of this series approximately 37 % with decreasing order of frequencies including TAPVC: to the coronary sinus (16%), to the infracardiac veins (15%), to the right superior vena cava (14%), to the right atrium (11%), and the mixed type (7%). The incidence of associated cardiac defects occurring with TAPVC approximates 34 % [3, 4, 8, 1 A true persistent left superior vena cavamust receive a hemiazygos vein, connect with a coronary sinus, or connect with a left atrium; if not, the more appropriate designation is anomalous vertical vein [7]
9, 10, 12]. The occurrence with abdominal heterotaxia (asplenia or polysplenia) is well recognized, but most often results in a great complexity of lesions which makes the presence o f TAPVC inapparent.
Anatomy and Pathophysiology TAPVC with and without pulmonary venous obstruction (PVO) differs clinically and prognostically. In TAPVC with PVO, the obstruction may occur at any level and the degree of obstruction may depend upon several factors including: the size of the interatrial communication, the length of the anomalous pathway, and the anatomic areas of narrowing due either to intrinsic stenosis or extrinsic areas of compression. Controversy exists regarding the size o.f the interatrial communication and its importance as an area of obstruction [7, 11]. Balloon septostomy may produce dramatic clinical and h e m o d y n a m i c improvement with small interatrial communications along with the presence of an atrial gradient [4, 15, 16]. Venous communications of long length and small diameter offer greater resistance to pulmonary venous flow than do shorter and wider channels [8, 171. Patients with shorter pathways and larger interatfial communications are less symptomatic, present later in life, and have a greater longevity than those with longer pathways and smaller interatrial communications [8, 17]. Intrinsic stenosis or extrinsic pressure may result in narrowing of a venous structure. A m o n g the various types, TAPVC below the diaphragm has the highest incidence of venous obstruction. Reasons appear to be several: the c o m m o n channel is very long; constriction may occur as the venous channel traverses the diaphragm; the hepatic sinusoids offer increased resistance to flow
S. B. Paster et al.: Total Anomalous Pulmonary Venous Connection
135
Fig. 1. TAPVC to the coronary sinus. The heart is enlarged. The lungs show a pattern of overcirculation and pulmonary venous congestion, A similar pattern may be seen with TAPVC to the right atrium
when the communication is to the portal system; and thoraco-abdominal pressure differentials may also be an obstructive factor with variations in respiration. Areas of anatomic narrowing or stenosis may occur in all types of TAPVC [8-12, 18, 19,20]. In TAPVC type I, venous obstruction of the left vertical vein may be caused by a vise-like compression between the anterior left pulmonary artery and the posterior left mainstem bronchus [10, 121. Stenosis or hypoplasia occurs commonly at the junction of the innominate vein with the right superior vena cava, and occasionally at the junction of the anomalous pulmonary vein and azygos vein [10]. The size of the left atrium and left ventricle are also important factors in TAPVC, having been described as being small to moderately small by various authors [19, 21]. The small left atrium is exceedingly important to the surgeon who must anastomose it to the c o m m o n pulmonary vein for complete repair. Goor has recently described the operative problems of the small left atrium in TAPVC. The c o m m o n pulmonary vein is anastomosed to the right atrium, the floor of the fossa ovalis is excised, and a pericardial conduit is constructed [22].
Chest Radiographic Findings Radiologic evaluation of heart size is important in distinguishing between TAPVC with or without PVO. Chest radiographs in TAPVC without PVO
demonstrate cardiac enlargement with dilatation of the right atrium, right ventricle, and main pulmonary artery. The lungs may reflect changes of pulmonary overcirculation and occasionally of congestive heart failure. Left sided chambers are not usually enlarged (Figs. 1, 2). Depending on the pulmonary vascular resistance, chest radiography may initially show a normal or slightly increased heart size which then progresses to marked dilatation as decrease in pulmonary vascular resistance ensues and pulmonary flow increases [9]. Snellen first reported the so-called figure-ofeight or snowman configuration with TAPVC to the left vertical vein [17]. (Fig. 3). This consists of a dilated left vertical vein, a dilated left innominate vein superiorly, and a dilated right superior vena cava. This sign is seen in the older child and adult but rarely below six to eight months of age [7]. Only one of our three patients with TAPVC to the left vertical vein presented with a snowman configuration. Gasul noted dilatation of the right atrial appendage in some patients with TAPVC to the coronary sinus [23]. This sign was not present in the two patients in our series. Weaver recently pointed out a new sign in the diagnosis of TAPVC to the left vertical vein. The lateral chest x-ray demonstrates a soft tissue density anterior to the trachea which when correlated with lateral angiography, has been shown to be caused by a distended pretracheal vertical vein and superior vena cava [24]. Eisen described an anterior indentation of the barium filled esophagus, due to the confluence
136
S.B. Paster et al.: Total Anomalous Pulmonary Venous Connection
Fig. 2. TAPVC to the left vertical vein. The heart is markedly en/arged but does not show a snowman configuration.The lung findings are consistent with pulmonary venous congestion and overcirculation of the pulmonary veins, that appears just above the diaphragm but below the level of the left atrium [25]. Harris reported chest radiographs showing a normal sized heart and changes of passive congestion with pulmonary edema in patients with TAPVC with PVO. The perihilar shadows were obscured and air bronchograms were seen as a reticular pattern fanning out from both hilar regions. Septal lines may occassionally be noted in the periphery along with demonstration of fluid within the fissures [18]. All three of our cases of TAPVC below the diaphragm presented with this findings (Fig. 4). These radiographic findings may be seen in TAPVC with obstruction at any level or with other anatomic causes of PVO eg. cot triatriatum, mitral atresia, hypoplastic left ventricle, and aortic atresia [7, 11]. Shadravan has reported an x-ray sign of TAPVC which is characteristic of superior vena cava obstruction occurring below the entrance of the azygos vein [20]. Blood flow proceeds in a reverse direction from the superior vena cava to the left innominate vein and hemi-azygos vein to communicate with the azygos vein and inferior vena cava below the level of the diaphragms. This results in dilatation of the hemi-azygos vein, producing an irregular left paravertebral shadow. The chest radiograph is important also in the evaluation of abdominal situs as careful examination of the subdiaphragmatic area will reveal a centrally located liver (enlarged left lobe) in cases
of asplenia that may be associated with TAPVC [26, 27, 28]. Cardiac Catheterization Catheterization data in TAPVC is helpful in substantiating the diagnosis. Complete study is essential and care must be taken to delineate all sites of anomalous drainage by attempting to catheterize each vein, thus avoiding the possibility of overlooking a mixed type. Comparatively high systemic venous oxygen saturations at the supracardiac (superior vena cava), cardiac (right atrium), or infracardiac (inferior vena cava) level lead one to the general site of anomalous drainage. The similarity of oxygen saturations in the right atrium, pulmonary artery and systemic artery is a c o m m o n triad. Nadas notes that if the systematic artery is 3 % more highly saturated than the pulmonary artery, the drainage is most likely subdiaphragmatic [29]. Systemic saturation varies from 20 % in obstructed types to normal values in unobstructed types with high pulmonary arterial flow. This is reflected by the frequent absence of significant clinical cyanosis in this latter group. Pressures are usually slightly elevated in the right atrium. Systemic or suprasystemic right ventricular and pulmonary arterial pressures generally imply pulmonary venous obstruction. In two of our nonobstructed cases, however, near-
S. B. Paster et al.: Total Anomalous Pulmonary Venous Connection
137
4
Fig. 3. TAPVC to the left vertical vein. The heart is enlarged and shows a snowman configuration. The lung findings are consistent with pulmonary venous congestion. In addition, this patient had a hypoplastic left ventricle and aortic atresia Fig. 4. TAPVC below the diaphragm. The heart size appears normal. There is a stringy reticular pattern to the pulmonary vascular markings indicative of pulmonary venous congestion. Fluid in the horizontal fissure is also present systemic right-sided p r e s s u r e s were present, possibly reflecting associated p r i m a r y p u l m o n a r y h y p e r t e n s i o n . T h e relatively high altitude o f o u r patient p o p u l a t i o n (greater t h a n 5000 feet) m a y be i m p o r t a n t in the genesis o f this potentially confusing phenomenon.
Angiography T h e diagnosis o f T A P V C can be m a d e c o n f i d e n t l y with a n g i o g r a p h y a l t h o u g h T A P V C with P V O m a y p r e s e n t with s o m e difficulty. In those cases with an associated right to left d u c t u s arteriosus shunt,
138
S.B. Paster et al.: Total Anomalous Pulmonary Venous Connection
Fig. 5. TAPVC below the diaphragm. The levophase portion of a pulmonary artery injection reveals faint opacification of a common pulmonary vein superimposed over the spine and draining below the diaphragm. The lateral projection (not shown) demonstrated the common pulmonary vein partly projecting over the posterior border of the heart and draining below the diaphragm
a main pulmonary artery injection may result in considerable loss of contrast through the ductus into the descending thoracic aorta. This results in a poorly visualized levophase and poorly visualized anomalous pathway. It is therefore important to make selective right and/or left pulmonary artery injections with high volumes of contrast material [10]. TAPVC below the diaphragm must be looked for carefully since the common pulmonary vein returning below the diaphragm frequently is superimposed over the spine and is difficult to visualize. On the lateral view, this can be seen behind the heart and is often a helpful ancillary finding (Fig. 5). When the development of an anomalous vertical vein below the diaphragm occurs late embryologically, return may be to blind sinusoids and, consequently, this is very difficult to identify angiographically. Tynan described a technique in which the umbilical vein is catheterized during the neonatal period and direct portal venous angiography is performed, clearly demonstrating the anomalous pulmonary venous drainage [24]. Supracardiac types of TAPVC without obstruction reveal a characteristic angiogram (Fig. 6). The levophase demonstrates drainage directly into the right superior vena cava or azygos vein, or drainage
into a prominent left vertical vein with subsequent opacification of the innominate vein and right superior vena cava. Not infrequently the catheter will pass directly into the anomalous pulmonary vein from the right atrium by coursing up the superior vena cava into the innominate vein and then subsequently into the anomalous left vertical vein. The pullback pressure tracing across the site of communication is important in documenting areas of stenoses. Occasionally, TAPVC to the coronary sinus may be confused with return to the right atrium or to a common atrium. A sign we should like to stress is the so-called "goltball" sign (Fig. 7). This has previously been described by Rowe and others [30, 31]. With anomalous retum to the coronary sinus, this structure becomes markedly enlarged due to increased flow. It appears as a vertical, ovoid structure lying over the left side of the vertebral column and resembles an egg or big golfball within the right atrium. This can be distinguished from the more superiorly positioned left atrium. The left atrium is usually adjacent to the left mainstem bronchus whereas the dilated coronary sinus is more inferior and lateral in position. When the pulmonary veins drain directly into the right atrium, a circular opacity also is seen but this is usually more to the right and over the spine.
S. B. Paster et al,: Total Anomalous Pulmonary Venous Connection
139
Fig. 6. TAPVC to the left vertical vein. The levophase portion of a main pulmonary artery injection reveals opacification of the left vertical vein, innominate vein, and right superior vena cava
Fig. 7. TAPVC to the coronary sinus. The levophase portion of a selective left pulmonary artery injection reveals a large "golfball" like density within the cardiac silhouette but in a position lower than the normally expected left atrium. This represents an enlarged dilated coronary sinus
140
S.B. Paster et al.: Total Anomalous Pulmonary Venous Connection
References 1. Auer, J.: The development of the human pulmonary veins and its major variations. Anat. Rec. 101, 581 (1948) 2. Darling, R. C., Rothney, W. B., Craig, J. M.: Total pulmonary venous drainage into the right side of the heart. Report of 17 autopsied cases not associated with other major cardiovascular anomaly. Lab. Invest. 6, 44 (1957) 3. Gott, V. L., Lester, R. G., Lillihei, C. W., Varco, R. L.: Total anomalous pulmonary venous return. Analysis of 30 cases. Circulation 13, 543 (1956) 4. Jensen, J. B., Blount, S. G., Jr.: Total anomalous pulmonary venous return. A review and report of the oldest surviving patient. Am. Heart J. 82, 387 (1971) 5. Buell, C.: The origin of the pulmonary vessels in the chick. Contrib. Embryol. 14, 13 (1922) 6. Snellen, H. A., Van lngen, H. C., Hoefsmit, E. C. H. M.: Patterns of anomalous pulmonary venous drainage. Circulation 38, 45 (1968) 7. Lucas, R. V., Jr., Schmidt, R. E.: Anomalous venous connections, pulmonary and systemic. In: A. J. Moss, F. A. Adams: Heart Disease in infants, Children, and Adolescents, Chapter 32, pp. 672-727. Baltimore: Williams and Wilkens 1968 8. Burroughs, J. T., Edwards, J. E.: Total anomalous pulmonary venous connection. Am. Heart J. 59, 913 (1960) 9. Carter, R. E. B., Capriles, M., Noe, Y.: Total anomalous pulmonary venous drainage. A clinical and anastomical study of 75 children. Br. Heart J. 31, 45 (1969) 10. Delisle, G., Ando, M., Calder, A. L., Zuberbuhler, J. R., Rochenmacher, S., Alday, L. E., Mangini, O., VanPraagh, S., VanPraagh, R.: Total anomalous pulmonary venous connection: report of 93 autopsied cases with emphasis on diagnostic and surgical considerations. Am. Heart J. 91, 99 (1976) 11. Gathman, G. E., Nadas, A. S.: Total anomalous pulmonary venous connection. The clinical and physiologic observation in 75 pediatric patients. Circulation 42, 143 (1970) 12. Nakib, A., Moller, J. H., Kanjuh, V. I., Edwards, J. E.: Anomalies of the pulmonary veins. Am. J. Cardiol. 20, 77 (1967) 13. Wukasch, D. C., Deutsch, M., Reul, G. J., I-Iallman, G. L., Cooley, D. A.: Total anomalous pulmonary venous return. Review of 125 patients treated surgically. Ann. Thorac. Surg. 19, 622 (1975) 14. Friedli, B., Davignon, A., Stanely, P.: Infradiaphragmatic anomalous pulmonary venous return. Surgical correction in a newborn infant. J. Thorac. Cardiovasc. Surg. 62, 301 (1971) 15. Berman, M. A., Fishbone, G., Stansel, H. C., Jr.: Total anomalous pulmonary venous return with obstruction at the foramen ovale. Successful palliation by open atrial septectomy. Am. J. Surg. 124, 679 (1972) 16. el-Said, G., Mullins, C. E., McNamara, D. G.: Management of total anomalous pulmonary venous return. Circulation 45, 1240 (1972) 17. SneUen, H. A., Albers, F. H.: The clinical diagnosis of anomalous pulmonary venous drainage. Circulation 6, 801 (1952)
18. Harris, G. B. C., Neuhauser, E. B. D., Giedion, A.: Total anomalous pulmonary venous return below the diaphragm. Am. J. Roentgenol. Radium Ther. NucL Med. 84, 436 (1960) 19. Hastreiter, A. R., Paul, M. H., Molthan, M. E., Miller, R. A.: Total anomalous pulmonary venous connection with severe pulmonary venous obstruction. Circulation 25, 916 (1962) 20. Shadravan, I., Baucum, R., Fowler, R. L., Villadiego, R., Puyau, F. A.: Obstructed anomalous pulmonary venous return. Am. Heart J. 82, 232 (1971) 21. Bove, K. E., Geiser, E. A., Meyer, R. A.: The left ventricle in anomalous pulmonary venous return. Morphometric analysis of 36 fatal cases in infancy. Arch. Pathol. 99, 522 (1975) 22. Goor, D. A., Yellin, A., Frand, M., Smolinsky, A., Neufeld, H. N.: The operative problem of small left atrium in total anomalous pulmonary venous connection: report of five patients. Ann. Thorac. Surg. 22, 245 (1976) 23. Gasul, B. M., Arcella, R. A., Lev, M.: Heart Disease in Children, page 147. Philadelphia: J. B. Lippincott and Company 1966 24. Tynan, M., Behrendt, D., Urquhart, W., Graham, G. R.: Portal vein catheterization and selective angiography in diagnosis of total anomalous pulmonary venous connection. Br. Heart J. 36, 1155 (1974) 25. Eisen, S., Elliot, L. P.: A plain film study of total anomalous pulmonary venous connection below the diaphragm. Am. J. Roentgenol. Radium Ther. Nucl. Med. 102, 372 (1966) 26. Rabinowitz, J. G., Liu, L., Lindner, A.: Asplenia associated with infradiaphragmatic total anomalous pulmonary venous return and esophageal varices. Radiology 93, 350 (1969) 27. Rose, V., Izukawa, T., Moes, C. A. E.: Syndromes of asplenia and polysplenia. Review of cardiac and noncardiac malformations in 60 cases with specific reference to diagnosis and prognosis. Br. Heart J. 37, 840 (1975) 28. Ruttenberg, H. D., Neufeld, H. N., Lucas, R. V., Jr., Cary, L. S., Adams, P., Jr., Anderson, R. C., Edwards, J. E.: Syndrome of congenital cardiac disease with asplenia. Am. J. Cardiol. 13, 387 (1964) 29. Nadas, A. S., Fyler, D. C.: Pediatric Cardiology, 3rd edition, pp 640-655. Philadelphia: W. B. Saunders 1972 30. Fellows, K.: Personal communication 31. Rowe, R. D., Glass, I. H., Keith, J. D.: Total anomalous pulmonary venous drainage at cardiac level. Angiographic differentiation. Circulation 23, 77 (1961)
Date of final acceptance: June 28, 1977
S. B. Paster, M. D. Department of Radiology University of New Mexico School of Medicine Albuquerque, NM 87131 USA
Pediat. Radiol. 6, 132-140 (1977)
9 by Springer-Verlag1977
Total Anomalous Pulmonary Venous Connection Report of Ten Cases and Review of the Literature
S. B. Paster1, R. E. Swensson2, and S. M. Yabek2 Departments of 1Radiology and 2pediatrics, University of New Mexico, School of Medicine, Albuquerque, New Mexico, USA
Abstract. Ten cases of total anomalous pulmonary venous connection, all presenting at or below the age of eight weeks, are presented: three below the diaphragm, two to the coronary sinus, one to the right atrium, three to the left vertical vein, and one to the right superior vena cava. Six patients had other cardiac anomalies, and surgical mortality represented 75 %. A review of the subject is presented. Key words: Veins, pulmonary heart - abnormalities - Lungs, blood supply
After complete history and physical examination each patient had an EKG, chest radiograph, and complete cardiac catheterization with angiography. Table 1 summarizes the pertinent clinical, echocardiographic, roentgenologic, catheterization and angiographic, and follow-up data. Associated cardiac anomalies occurred in six of the ten patients in this series (60 %) and include patent ductus arteriosus in three patients, hypoplastic left ventricle in two patients, and complex congenital heart disease associated with the asplenia syndrome in one patient.
Discussion
Total anomalous pulmonary venous connection (TAPVC) is an uncommon abnormality comprising 1 to 2 % of all congenital heart lesions [9, 21, 22]. Despite its relative infrequency and high mortality, reports of increasing surgical success are now appearing. A review of our ten patients under two months of age is presented along with a review of the subject. Clinical Material The present clinical series consists of ten patients, two months of age and under, who presented to the University of New Mexico School of Medicine during a forty-three month period from September 1973 through April 1976. They ranged in age from one day to two months. Three patients presented within the first week of life, six patients presented from one to six weeks, and one patient presented at eight weeks. There were seven males and three females. All patients presented with cyanosis and cardiac murmurs; three patients also had feeding difficulties.
Emb~o~ The pulmonary venous system has a dual origin [1, 2]. The lungs develop as an outpouching of the ventral wall of the foregut and the splanchnic plexus covering the foregut becomes the anlage of the venous plexus covering the lung bud [3, 4]. The common pulmonary vein develops as an outpouching from the dorsum of the sinus venosus [1, 3, 4, 5]. The splanchnic venous plexus covering the lung bud develops a communication with the common pulmonary vein and this communication shifts leftward with the development of the interatrial septum to finally drain into the left atrium. Differential growth results in all four tributary veins emptying into the left atrium separately [3, 4]. During early stages of development of the lungs, the splanchnic venous plexus covering the foregut does not directly communicate with the heart but normally Communicates with the surrounding systemic and visceral veins. The
S. B. Paster et al.: Total Anomalous Pulmonary Venous Connection
133
Table 1. Clinical Data Case
Age/Sex
Clinical presentation
EKG
Chest x-ray
1
2 day/F
Cyanotic, murmur
Small heart, pulmonary venous congestion
2
7 day/M
Cyanotic, murmur
RAD, RVH, no posterior forces RAD, RVH
3
5 week/M
Cyanotic, murmur
RAD, RVH, RAE
Normal heart, pulmonary venous congestion
4
2 month/M
Cyanotic, pneumonia, congestive failure, murmur
RAD, RVH, RAE
Large heart, overcirculation, pulmonary venous congestion
5
5 week/F
Cyanotic, congestive failure, RDS, difficulty feeding, murmur
RAD
Large heart, pulmonary venous congestion
6
4 week/M
Cyanotic, difficulty feeding, murmur
RAD, RVH
Large heart, pulmonary venous congestion
7
12 day/M
Cyanotic, murmur
Small heart, diminished blood flow
8
6 week/M
Cyanotic, pneumonia, difficulty feeding, murmur
RAD, RVH, poor posterior forces RVH
9
1 day/F
Cyanotic, murmur
RVH
Large heart, pulmonary venous congestion, snowman
10
51/2 week/M
Cyanotic, murmur
RVH
Small heart initially -~ large heart, overcirculation, pulmonary venous congestion
Case
Cath/Angio
Follow-up (Surg or post)
1
TAPVC below diaphragm (to IVC), PDA, (right ~ left)
Surgery ~ death
2
TAPVC below diaphragm
Surgery ~ death
3
TAPVC below diaphragm, PDA (right ~ left)
Surgery -~ death
4
TAPVC to right atrium (right side also drained separately into the RSVC
Surgery -* death
TAPVC to coronary sinus
Successful surgical correction
TAPVC to coronary sinus
Surgery -~ death, associated hypoplastic left ventricle found at surgery
TAPVC to RSVC, transposition of great arteries, common ventricle, hypoplastic pulmonary artery, asplenia
Palliative surgery (Waterston) -~ death, postmortem revealed bilateral SVC, one atrioventricular valve, absent coronary sinus and right aortic arch with aberrant right subclavian artery
TAPVC to left vertical vein
Successful surgical correction
TAPVC to left vertical vein, hypoplastic left ventricle and aortic atresia
No surgery, died
TAPVC to left vertical vein, PDA (right ~ left)
No surgery, cardiac arrest --- died, post documented TAPVC to left vertical vein along with PDA
10
Small heart, pulmonary venous congestion
Borderline heart size, overcirculation, pulmonary venous congestion
Abbreviations: RAD = Right axis deviation RVH = Right ventricular hypertrophy RAE = Right atrial enlargement s y s t e m i c v e i n s are f o r e r u n n e r s o f t h e pre- a n d post-cardial veins which eventually form the s u p e r i o r v e n a cava, i n n o m i n a t e v e i n , a z y g o s v e i n s , a n d c o r o n a r y sinus. T h e v i s c e r a l v e i n s o r u m b i l i c o vitelline s y s t e m are f o r e r u n n e r s f o r t h e p o r t a l v e i n , d u c t u s v e n o s u s , a n d i n f e r i o r v e n a c a v a [3, 4]. I f
d u r i n g t h e n o r m a l stages o f e m b r y o n i c d e v e l o p m e n t , failure o f c o n n e c t i o n o c c u r s o r if obliteration of a normally developed connection ensues, t h e e m b r y o n i c c o n n e c t i o n s p e r s i s t to c r e a t e t h e s p e c t r u m o f total a n o m a l o u s p u l m o n a r y v e n o u s c o n n e c t i o n . T A P V C to t h e r i g h t a t r i u m o c c u r s
S. B. Paster et al.: Total Anomalous Pulmonary Venous Connection
134 Table 2. Classification of TAPVC by frequency Burroughs & Edwards Supracardiac 56 Left vertical vein (LSVC) RSVC 31 Cardiac 30 Right atrium Coronar 18 Infracardiac 28 Mixed 16 Total 179
Carter & Capriles
Jensen & Blount
W u k a s c h Gathman & Cooley & Nadas
Delisle& Van Praagh
Nakib Total & Edwards No. %
34
12
60
26
26
12
226
37 %
7 3
2 6
15 15
8 3
16 7
6 6
85 70
14% 11%
20 8 3 75
5 2 0 27
18 8 9 125
14 16 8 75
17 22 5 93
8 9 3 44
100 93 44 618
16 % 15% 7% 100%
if the atrial septum has grown abnormally far to the left; in this particular instance, no abnormal embryologic communication exists [3, 4].
Classification and Incidence A c o m m o n l y accepted classification of TAPVC into four types is based on the works of Darling and Snellen [2, 6]. Type I TAPVC occurs at the supracardiac level with drainage into a r e m n a n t of the left vertical vein (or left superior vena cava 1) or directly into the right superior vena cava. Type II TAPVC occurs at the cardiac level with drainage either into the coronary sinus or directly into the fight atrium. Type III TAPVC occurs at the infracardiac level i. e. below the diaphragm and drains into the portal vein, ductus venosus, or rarely into the inferior vena cava. Type IV TAPVC is a mixed pattern and occurs w h e n connection is to more than one of the above levels [2]. The frequency with which each type occurs is shown in Table 2 in which six h u n d r e d eighteen cases have been compiled from seven large series [4, 8-13]. This table includes TAPVC in both isolated form and also in association with other forms of congenital heart disease. TAPVC to the left vertical vein or left superior vena cava comprises the largest percentage of this series approximately 37 % with decreasing order of frequencies including TAPVC: to the coronary sinus (16%), to the infracardiac veins (15%), to the right superior vena cava (14%), to the right atrium (11%), and the mixed type (7%). The incidence of associated cardiac defects occurring with TAPVC approximates 34 % [3, 4, 8, 1 A true persistent left superior vena cavamust receive a hemiazygos vein, connect with a coronary sinus, or connect with a left atrium; if not, the more appropriate designation is anomalous vertical vein [7]
9, 10, 12]. The occurrence with abdominal heterotaxia (asplenia or polysplenia) is well recognized, but most often results in a great complexity of lesions which makes the presence o f TAPVC inapparent.
Anatomy and Pathophysiology TAPVC with and without pulmonary venous obstruction (PVO) differs clinically and prognostically. In TAPVC with PVO, the obstruction may occur at any level and the degree of obstruction may depend upon several factors including: the size of the interatrial communication, the length of the anomalous pathway, and the anatomic areas of narrowing due either to intrinsic stenosis or extrinsic areas of compression. Controversy exists regarding the size o.f the interatrial communication and its importance as an area of obstruction [7, 11]. Balloon septostomy may produce dramatic clinical and h e m o d y n a m i c improvement with small interatrial communications along with the presence of an atrial gradient [4, 15, 16]. Venous communications of long length and small diameter offer greater resistance to pulmonary venous flow than do shorter and wider channels [8, 171. Patients with shorter pathways and larger interatfial communications are less symptomatic, present later in life, and have a greater longevity than those with longer pathways and smaller interatrial communications [8, 17]. Intrinsic stenosis or extrinsic pressure may result in narrowing of a venous structure. A m o n g the various types, TAPVC below the diaphragm has the highest incidence of venous obstruction. Reasons appear to be several: the c o m m o n channel is very long; constriction may occur as the venous channel traverses the diaphragm; the hepatic sinusoids offer increased resistance to flow
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Fig. 1. TAPVC to the coronary sinus. The heart is enlarged. The lungs show a pattern of overcirculation and pulmonary venous congestion, A similar pattern may be seen with TAPVC to the right atrium
when the communication is to the portal system; and thoraco-abdominal pressure differentials may also be an obstructive factor with variations in respiration. Areas of anatomic narrowing or stenosis may occur in all types of TAPVC [8-12, 18, 19,20]. In TAPVC type I, venous obstruction of the left vertical vein may be caused by a vise-like compression between the anterior left pulmonary artery and the posterior left mainstem bronchus [10, 121. Stenosis or hypoplasia occurs commonly at the junction of the innominate vein with the right superior vena cava, and occasionally at the junction of the anomalous pulmonary vein and azygos vein [10]. The size of the left atrium and left ventricle are also important factors in TAPVC, having been described as being small to moderately small by various authors [19, 21]. The small left atrium is exceedingly important to the surgeon who must anastomose it to the c o m m o n pulmonary vein for complete repair. Goor has recently described the operative problems of the small left atrium in TAPVC. The c o m m o n pulmonary vein is anastomosed to the right atrium, the floor of the fossa ovalis is excised, and a pericardial conduit is constructed [22].
Chest Radiographic Findings Radiologic evaluation of heart size is important in distinguishing between TAPVC with or without PVO. Chest radiographs in TAPVC without PVO
demonstrate cardiac enlargement with dilatation of the right atrium, right ventricle, and main pulmonary artery. The lungs may reflect changes of pulmonary overcirculation and occasionally of congestive heart failure. Left sided chambers are not usually enlarged (Figs. 1, 2). Depending on the pulmonary vascular resistance, chest radiography may initially show a normal or slightly increased heart size which then progresses to marked dilatation as decrease in pulmonary vascular resistance ensues and pulmonary flow increases [9]. Snellen first reported the so-called figure-ofeight or snowman configuration with TAPVC to the left vertical vein [17]. (Fig. 3). This consists of a dilated left vertical vein, a dilated left innominate vein superiorly, and a dilated right superior vena cava. This sign is seen in the older child and adult but rarely below six to eight months of age [7]. Only one of our three patients with TAPVC to the left vertical vein presented with a snowman configuration. Gasul noted dilatation of the right atrial appendage in some patients with TAPVC to the coronary sinus [23]. This sign was not present in the two patients in our series. Weaver recently pointed out a new sign in the diagnosis of TAPVC to the left vertical vein. The lateral chest x-ray demonstrates a soft tissue density anterior to the trachea which when correlated with lateral angiography, has been shown to be caused by a distended pretracheal vertical vein and superior vena cava [24]. Eisen described an anterior indentation of the barium filled esophagus, due to the confluence
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Fig. 2. TAPVC to the left vertical vein. The heart is markedly en/arged but does not show a snowman configuration.The lung findings are consistent with pulmonary venous congestion and overcirculation of the pulmonary veins, that appears just above the diaphragm but below the level of the left atrium [25]. Harris reported chest radiographs showing a normal sized heart and changes of passive congestion with pulmonary edema in patients with TAPVC with PVO. The perihilar shadows were obscured and air bronchograms were seen as a reticular pattern fanning out from both hilar regions. Septal lines may occassionally be noted in the periphery along with demonstration of fluid within the fissures [18]. All three of our cases of TAPVC below the diaphragm presented with this findings (Fig. 4). These radiographic findings may be seen in TAPVC with obstruction at any level or with other anatomic causes of PVO eg. cot triatriatum, mitral atresia, hypoplastic left ventricle, and aortic atresia [7, 11]. Shadravan has reported an x-ray sign of TAPVC which is characteristic of superior vena cava obstruction occurring below the entrance of the azygos vein [20]. Blood flow proceeds in a reverse direction from the superior vena cava to the left innominate vein and hemi-azygos vein to communicate with the azygos vein and inferior vena cava below the level of the diaphragms. This results in dilatation of the hemi-azygos vein, producing an irregular left paravertebral shadow. The chest radiograph is important also in the evaluation of abdominal situs as careful examination of the subdiaphragmatic area will reveal a centrally located liver (enlarged left lobe) in cases
of asplenia that may be associated with TAPVC [26, 27, 28]. Cardiac Catheterization Catheterization data in TAPVC is helpful in substantiating the diagnosis. Complete study is essential and care must be taken to delineate all sites of anomalous drainage by attempting to catheterize each vein, thus avoiding the possibility of overlooking a mixed type. Comparatively high systemic venous oxygen saturations at the supracardiac (superior vena cava), cardiac (right atrium), or infracardiac (inferior vena cava) level lead one to the general site of anomalous drainage. The similarity of oxygen saturations in the right atrium, pulmonary artery and systemic artery is a c o m m o n triad. Nadas notes that if the systematic artery is 3 % more highly saturated than the pulmonary artery, the drainage is most likely subdiaphragmatic [29]. Systemic saturation varies from 20 % in obstructed types to normal values in unobstructed types with high pulmonary arterial flow. This is reflected by the frequent absence of significant clinical cyanosis in this latter group. Pressures are usually slightly elevated in the right atrium. Systemic or suprasystemic right ventricular and pulmonary arterial pressures generally imply pulmonary venous obstruction. In two of our nonobstructed cases, however, near-
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4
Fig. 3. TAPVC to the left vertical vein. The heart is enlarged and shows a snowman configuration. The lung findings are consistent with pulmonary venous congestion. In addition, this patient had a hypoplastic left ventricle and aortic atresia Fig. 4. TAPVC below the diaphragm. The heart size appears normal. There is a stringy reticular pattern to the pulmonary vascular markings indicative of pulmonary venous congestion. Fluid in the horizontal fissure is also present systemic right-sided p r e s s u r e s were present, possibly reflecting associated p r i m a r y p u l m o n a r y h y p e r t e n s i o n . T h e relatively high altitude o f o u r patient p o p u l a t i o n (greater t h a n 5000 feet) m a y be i m p o r t a n t in the genesis o f this potentially confusing phenomenon.
Angiography T h e diagnosis o f T A P V C can be m a d e c o n f i d e n t l y with a n g i o g r a p h y a l t h o u g h T A P V C with P V O m a y p r e s e n t with s o m e difficulty. In those cases with an associated right to left d u c t u s arteriosus shunt,
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Fig. 5. TAPVC below the diaphragm. The levophase portion of a pulmonary artery injection reveals faint opacification of a common pulmonary vein superimposed over the spine and draining below the diaphragm. The lateral projection (not shown) demonstrated the common pulmonary vein partly projecting over the posterior border of the heart and draining below the diaphragm
a main pulmonary artery injection may result in considerable loss of contrast through the ductus into the descending thoracic aorta. This results in a poorly visualized levophase and poorly visualized anomalous pathway. It is therefore important to make selective right and/or left pulmonary artery injections with high volumes of contrast material [10]. TAPVC below the diaphragm must be looked for carefully since the common pulmonary vein returning below the diaphragm frequently is superimposed over the spine and is difficult to visualize. On the lateral view, this can be seen behind the heart and is often a helpful ancillary finding (Fig. 5). When the development of an anomalous vertical vein below the diaphragm occurs late embryologically, return may be to blind sinusoids and, consequently, this is very difficult to identify angiographically. Tynan described a technique in which the umbilical vein is catheterized during the neonatal period and direct portal venous angiography is performed, clearly demonstrating the anomalous pulmonary venous drainage [24]. Supracardiac types of TAPVC without obstruction reveal a characteristic angiogram (Fig. 6). The levophase demonstrates drainage directly into the right superior vena cava or azygos vein, or drainage
into a prominent left vertical vein with subsequent opacification of the innominate vein and right superior vena cava. Not infrequently the catheter will pass directly into the anomalous pulmonary vein from the right atrium by coursing up the superior vena cava into the innominate vein and then subsequently into the anomalous left vertical vein. The pullback pressure tracing across the site of communication is important in documenting areas of stenoses. Occasionally, TAPVC to the coronary sinus may be confused with return to the right atrium or to a common atrium. A sign we should like to stress is the so-called "goltball" sign (Fig. 7). This has previously been described by Rowe and others [30, 31]. With anomalous retum to the coronary sinus, this structure becomes markedly enlarged due to increased flow. It appears as a vertical, ovoid structure lying over the left side of the vertebral column and resembles an egg or big golfball within the right atrium. This can be distinguished from the more superiorly positioned left atrium. The left atrium is usually adjacent to the left mainstem bronchus whereas the dilated coronary sinus is more inferior and lateral in position. When the pulmonary veins drain directly into the right atrium, a circular opacity also is seen but this is usually more to the right and over the spine.
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Fig. 6. TAPVC to the left vertical vein. The levophase portion of a main pulmonary artery injection reveals opacification of the left vertical vein, innominate vein, and right superior vena cava
Fig. 7. TAPVC to the coronary sinus. The levophase portion of a selective left pulmonary artery injection reveals a large "golfball" like density within the cardiac silhouette but in a position lower than the normally expected left atrium. This represents an enlarged dilated coronary sinus
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18. Harris, G. B. C., Neuhauser, E. B. D., Giedion, A.: Total anomalous pulmonary venous return below the diaphragm. Am. J. Roentgenol. Radium Ther. NucL Med. 84, 436 (1960) 19. Hastreiter, A. R., Paul, M. H., Molthan, M. E., Miller, R. A.: Total anomalous pulmonary venous connection with severe pulmonary venous obstruction. Circulation 25, 916 (1962) 20. Shadravan, I., Baucum, R., Fowler, R. L., Villadiego, R., Puyau, F. A.: Obstructed anomalous pulmonary venous return. Am. Heart J. 82, 232 (1971) 21. Bove, K. E., Geiser, E. A., Meyer, R. A.: The left ventricle in anomalous pulmonary venous return. Morphometric analysis of 36 fatal cases in infancy. Arch. Pathol. 99, 522 (1975) 22. Goor, D. A., Yellin, A., Frand, M., Smolinsky, A., Neufeld, H. N.: The operative problem of small left atrium in total anomalous pulmonary venous connection: report of five patients. Ann. Thorac. Surg. 22, 245 (1976) 23. Gasul, B. M., Arcella, R. A., Lev, M.: Heart Disease in Children, page 147. Philadelphia: J. B. Lippincott and Company 1966 24. Tynan, M., Behrendt, D., Urquhart, W., Graham, G. R.: Portal vein catheterization and selective angiography in diagnosis of total anomalous pulmonary venous connection. Br. Heart J. 36, 1155 (1974) 25. Eisen, S., Elliot, L. P.: A plain film study of total anomalous pulmonary venous connection below the diaphragm. Am. J. Roentgenol. Radium Ther. Nucl. Med. 102, 372 (1966) 26. Rabinowitz, J. G., Liu, L., Lindner, A.: Asplenia associated with infradiaphragmatic total anomalous pulmonary venous return and esophageal varices. Radiology 93, 350 (1969) 27. Rose, V., Izukawa, T., Moes, C. A. E.: Syndromes of asplenia and polysplenia. Review of cardiac and noncardiac malformations in 60 cases with specific reference to diagnosis and prognosis. Br. Heart J. 37, 840 (1975) 28. Ruttenberg, H. D., Neufeld, H. N., Lucas, R. V., Jr., Cary, L. S., Adams, P., Jr., Anderson, R. C., Edwards, J. E.: Syndrome of congenital cardiac disease with asplenia. Am. J. Cardiol. 13, 387 (1964) 29. Nadas, A. S., Fyler, D. C.: Pediatric Cardiology, 3rd edition, pp 640-655. Philadelphia: W. B. Saunders 1972 30. Fellows, K.: Personal communication 31. Rowe, R. D., Glass, I. H., Keith, J. D.: Total anomalous pulmonary venous drainage at cardiac level. Angiographic differentiation. Circulation 23, 77 (1961)
Date of final acceptance: June 28, 1977
S. B. Paster, M. D. Department of Radiology University of New Mexico School of Medicine Albuquerque, NM 87131 USA
