A Radiographic Study of Congenital Pulmonary Atresia with Ventricular

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1 A Radiographic Study of Congenital Pulmonary Atresia with Ventricular Septal Defect BENIGNO SOTO,1 ALBERT D. PACIFICO,2 RODRIGO F. LUNA,1 AND L. M. BARGERON, JR.3 A radiographic analysis of 66 patients with congenital pulmonary atresia and ventricular septal defect was made to determine the frequency and variability of the (1) atrioventricular and ventriculoarterial relation, (2) source of pulmonary blood flow, and (3) pulmonary arterial anatomy. Of the 66 patients, 63 had situs solitus and 62 had atrioventricular concordance; the aorta arose from the right ventricle In six, left ventricle in six, and biventricularly in 54. Pulmonary arteries were present and confluent in 41 patients, nonconfluent in five, absent in 15, and In five patients a mixed distribution to selected areas of each lung existed. In almost half of those in whom pulmonary arteries were present, the source of pulmonary blood flow was from systemic arteries (bronchials) originating from the upper descending thoracic aorta. In some, these collaterals obscured detection of pulmonary arteries when conventional anteroposterior views of the aortogram were made. The use of semiaxial craniocaudal projections and/or selective angiography were required for proper definition. Chest radlographs were correlated with angiographic details and did not permit differentiation of the various subsets of congenital pulmonary atresia from each other nor from classical tetralogy of Fallot. Abnormal central vessels were identifled in 31 patients suggesting this diagnosis. The right or left pulmonary artery identified by angiography was not detected on the plain chest film in about half of the group. Angiograms of the intra- and extracardiac details of this group of malformations are presented, correlated with plain chest films, and their surgical implications discussed. The phrase congenital pulmonary atresia with ventricular septal defect describes a group of cardiac malformations which are characterized by (1) absence of direct anatomic continuity of the pulmonary arteries with the heart, (2) varying degrees of atresia or agenesis of the main and distal pulmonary arteries, and (3) varying sources of pulmonary blood flow [1-6]. We present a radiographic analysis of 66 patients with this malformation each haying two atria, and two atrioventnicular valves emptying into two respective and well developed ventricles. Patients with truncus arteriosus types I-Ill were excluded [7]. This report describes the angiographically determined frequency of the following anatomic variations: (1) visceroatrial situs, (2) atrioventricular relation, (3) origin of aorta, (4) presence and distribution of the pulmonary arteries, and (5) sources of pulmonary blood flow. A correlative analysis of features identified on the plain chest films is presented. Definition of Terms Patients with these congenital cardiac defects may be classified, using embryologic and pathologic considerations, into a variety of categories: tetrabogy of Falbot with congenital pulmonary atresia, end-stage tetrabogy of Fallot, transposition of the great arteries, double outlet right or left ventricle, and corrected transpositon-each with pulmonary atresia. However, the separation into these subsets often cannot be made by usual angiographiti and surgical methods, because it is not always possible to determine the ventricle from which the main pulmonary artery would have arisen were it not atretic. In addition, it is usually of no clinical importance to make this differentiation. The surgical problem in each of these malformations is similar and consists of reestablishing ventricubopulmonary artery continuity with a valved external conduit. This usually involves closing the source of pulmonary blood flow and closing the ventricular septal defect so that the aorta arises from the systemic ventricle. When there is valvular pulmonary atresia, a main pulmonary artery segment, and normally related great arteries, an outflow tract patch may be used in place of a valved external conduit. The cardiac radiologist must define the presence and distribution of pulmonary arteries, the source of pulmonary flow, the location of the aorta with respect to each ventricle, and the ventricular septal defect. The origin of the atretic pulmonary artery bears little clinical significance. Therefore, we prefer to classify these malformations under the heading of congenital pulmonary atresia with ventricular septal defect. The three major divisions are (1) bilateral presence of pulmonary arteries, further delineated as confluence or nonconfluence of left and right pulmonary arteries; (2) bilateral absence of pulmonary arteries (truncus arteriosus type IV); and (3) mixed types. Further definition is made of the situs of the viscerae and atria, atnioventricular relation, position and origin of the aorta, and position and size of the ventricular septal defect. This classification is similar to that of Edwards and McGoon [1]. When the pulmonary arteries are present and confluent, the entity has been called pseudotruncus arteniosus. The majority of these cases have a biventricular origin of the aorta, and the morphology of the right Received February 3, 1977; accepted after revision August 23, Department of Diagnostic Radiology, University of Alabama School of Medicine, 619 South 19th Street. Birmingham. Alabama Address reprint requests to B. Soto. Department of Surgery, University of Alabama School of Medicine, Birmingham, Alabama Department of Pediatrics. University of Alabama School of Medicine. Birmingham, Alabama Are J Roentgenol 129: , December

2 1028 SOTO ET AL. ventricle is similar to that found in the tetralogy of Fallot. This has been described as tetralogy with congenital pulomonary atresia or as end-stage tetralogy of Fallot. When the aorta is further dextroposed, originating entirely from the right ventricle, the entity has been called transposition of the great arteries with congenital pulmonary atresia [8]. However, the angiographer, and sometimes the surgeon, cannot be certain that had the main pulmonary artery been connected with the heart it would have arisen from the left ventricle. It may have arisen from the right ventricle, in which case the entity might be called double-outlet right ventricle with pulmonary atresia [9]. Similarly, if the aorta arises entirely from the left ventricle, the entity may be described as double-outlet left ventricle with pulmonary atresia or simply as ventnicular septal defect with pulmonary atresia. These considerations and the surgical limitations make us believe it reasonable to group these cardiac malformations under the heading of congenital pulmonary atresia with ventnicular septal defect, although we recognize the variety of terms indicated above are preferred by others. Patients with congenital pulmonary atresia with yentricular septal defect may have positional anomalies of the heart, abnormal visceroatrial situs, and abnormal atrioventricular connections. When atrioventricular discordance is present, the phrase corrected transposition with pulmonary atresia is sometimes preferred, since corrected transposition is a malformation characterized by atrioventricular discordance and a discordant yentriculoarterial relation. We prefer to describe this malformation as congenital pulmonary atresia with ventricular septal defect, and atrioventricular discordance as origin of the aorta from the right ventricle. The pulmonary arterial system is the usual anatomic arterial tree which supplies blood to the alveolar capillares within the lungs. It is formed by main pulmonary artery, right and left pulmonary arteries, and their lobar, segmental, and subsegmental branches. In some patients part of this usual anatomic sequence may be absent. Right and left pulmonary arteries are seen angiographically as gently curved structures, concave toward the midline, extending downward and outward from each hilum. The descriptive terms palm leaf or comma have been used for these configurations [10, 11]. The left and right pulmonary arteries may not join together (nonconfluent) or they may be connected by a confluence. This confluence may or may not join a partially developed main pulmonary trunk. The source of pulmonary blood flow may be a ductus arteriosus, well developed bronchial collateral arteries, a coronary-pulmonary fistula, a collateral artery from the innominate artery, a surgically created systemic pulmonary arterial shunt, or large bronchial collaterals. In some patients well developed bronchial collateral arteries join the pulmonary arteries in the hilum, while in others these extend within each lung to form a second and separate well developed intrapulmonary arterial tree. These vessels, which originate from the upper descending thoracic aorta, usually have a spiral coiled pattern and may have areas of stenosis along their course. Angiographic TABLE 1 Findings Absent Noncon Finding Confluent Pulmonany Arteries Pulmonary Antenies fluent Pul- monany Mixed Arteries Situs: Solitus 39 (95.1) 15 (100.0) 5 4 Inversus 2 (4.9) Atnioventnicular connections: Concordant 40 (97.0) 14 (93.3) 4 4 Discordant 1 (2.4) 1 (6.7) 1 1 Origin of the aorta: Biventnicular 31 (75.6) 13 (87.0) 4 3 Right ventricle 4 (9.8) 2 (13.0) 1 0 Left ventricle 6 (14.6) Systemic-pulmonary connections: Patent ductus arteriosus 13(32.0) Large systemic antenies 19 (46.0) 14 (93.0) 3 1 Small systemic antenies 1 (2.0) 1 (6.7) 0 0 Surgically created shunts 8 (20.0) Note-Numbers in parentheses are percentages. - Tnuncus arteniosus type IV. TABLE 2 Radiographic Findings Non- Confluent Absent con-.. Pulmo- Pulmo- fluent Finding nary Ar nary An- Puimotenies tenies nary Artenies. Mixed Heart size: Normal Enlarged Aortic arch: Right Left Pulmonary artery: Right Left Lobar and segmental branches Collateral circulation Abnormal central vessels: Right Left Truncus anteniosus type IV. Whether these arteries are truly enlarged bronchial arteries or other systemic arteries is a matter of controversy [12, 13]. When there is absence of the true anatomic pulmonary arterial tree, the arterial supply to the lung is solely from these bronchial collateral arteries and the condition is called truncus arteniosus type IV, as described by Collett and Edwards [7]. Some patients have varying combinations of pulmonary arterial pattern called the mixed type. This usually consists of a pulmonary arterial system to one or more lobes and a separate systemic arterial supply to the remaining lobes.

3 CONGENITAL PULMONARY ATRESIA WITH VENTRICULAR SEPTAL DEFECT 1029 TI. #{149}1 Fig. 1.-Origin of aorta. A, Right ventricular angiogram, lateral view, showing aorta originating equally from both ventricles. Note large ventricular septal defect (arrow). B, Right ventnicularorigin ofaorta. C, Rightventnicular origin of aorta in patient with situs inversus and discordant atrioventnicular relation. D, Left ventricular origin of aorta. Note ventricular septal defect (arrow).

4 1030 SOTO ET AL. Surgery was performed on 48 of the 66 patients and consisted of corrective procedures in 29, systemic-pulmonaryarteryshunts in 12, and exploratory thoracotomy alone in seven. Surgically correlatable information on intracardiac and pulmonary artery anatomy was therefore available in only 29 of the 66 patients. Fig. 2.-Aortogram showing pulmonary arterial system in patient with confluent pulmonary arteries and right Blalock-Taussig shunt. Tip of catheter is in aortic arch (AO). Right (RPA) and left (LPA) pulmonary arteries are opacified through Blalock anastomosis (B). Pulmonary arteries have usual palm leaf configuration. Subjects and Methods A total of 66 patients with congenital pulmonary atresia and ventricular septal defect who had complete radiographic studies made at the University of Alabama in Birmingham were selected for this study. Patients with truncus arteniosus types I-Ill were arbitrarily excluded. Patients ranged in age from 2 days to 47 years; there were 34 males and 32 females. Of the 66, 41 had confluent pulmonary arteries, 15 truncus arteniosus type IV, five had nonconfluent pulmonary arteries, and five had the mixed type. The angiognaphic study consisted of a right ventricular angiogram, aortogram after injection of contrast media into the upper descending thonacic aorta, and, in some cases, films made from selective injection of vessels originating from the upper descending thoracic aorta. More recently, the craniocaudal semiaxial projection has been used to improve identification of the pulmonary arteries. These angiognams were obtained on large film series (roll film, Elema-Schonanden changer) and 35 mm cine. The segmental approach for the diagnosis of congenital heart disease was followed [4]. Definition of the (1) situs of the viscerae and atnia, (2) atnioventnicular connection, and (3) ventniculan-great artery relation were made for each patient based upon angiography. Chest films were examined with particular attention to heart size, position of the aortic arch, and identification of the night and left pulmonary arteries and their branches. Abnormal blood vessels in the hilum of the lungs were identified and later correlated with angiographic studies. Indentation in the opacified esophagus has been found useful as an indicator of large bronchial arteries, but was not evaluated in this study. Results The angiographic details are listed in table 1. The findings recognized from analysis of the chest radiographs are described in table 2. Heart size was considered enlarged ifthe cardiothoracic ratio was greater than Confluent Pulmonary Arteries Confluent pulmonary arteries was the most frequent type of congenital pulmonary atresia in this series (41 patients). There were 25 females and 16 males, ranging in age from 2 days to 34 years. The visceroatrial situs was solitus in all but two patients, and the atrioventricular relation was concordant in all but one. Angiographic studies. The origin of the aorta in the 41 patients was biventricular in 31, from the left ventricle in six, and from the right ventricle in four (fig. 1). The source of pulmonary blood flow was the bronchial collateral arteries arising from the descending thoracic aorta in 20 patients, a patent ductus arteriosus in 13, and solely a surgically created systemic-pulmonary artery shunt in eight. In some cases the presence of the pulmonary arterial system was easily demonstrated soon after injection of contrast material into the aorta (fig. 2). However, when the source of pulmonary blood flow is bronchial collateral arteries arising from the descending thoracic aorta, demonstration of the pulmonary arterial system may be difficult and may require selective catheterization of each ofthese vessels [14, 15] (fig. 3). Simple aortography in this patient (fig. 3) did not demonstrate a pulmonary arterial system; without the use of selective arteriography of each collateral vessel, a diagnosis of truncus arteriosus type IV would have been incorrectly made. More recently we have found improved radiographic separation of the pulmonary confluence from the systemic collateral arteries with the use of semiaxial craniocaudal projections (fig. 4.). The angiographic details of the intra- and extracardiac anatomy were verified at the time of corrective surgery in 29 of these 41 patients. Chest films. The heart size was normal on chest films in 35 patients (85%) and the main pulmonary artery segment was concave or absent in 31 (75%). The right and left pulmonary arteries were identified only in about half of the cases (table 2). The peripheral vessels were decreased and/or of normal caliber in the same proportion of cases, but no evidence of increased vascularity was found in this series. On lateral views, the shadow of the right and left pulmonary arteries was visualized in 30 of 36 patients (83%) (fig. 5). The majority of patients in whom pulmonary arteries were not identified on the plain chest film, but were identified angiographically, were infants. Peripheral collateral circulation commonly observed in cyanotic patients with reduced pulmonary blood flow was identified in 24 patients of this group. This term was used by Campbell and Gardner [11] to describe vascular markings which do not appear related to the comma shape of the normal pulmonary artery. These markings are dense vascular shadows high in the mediastinum and nodular hilar structures with abnormal branching in the

5 CONGENITAL PULMONARY ATRESIA WITH VENTRICULAR SEPTAL DEFECT 1031 lung field, and are radiographic evidence of collateral circulation, most probably bronchial arteries. The aortic arch was on the right side in 13 patients (32%), a frequency similar to that previously reported for patients with this malformation [6]. Unusual vascular markings were identified in the hilum in 15 of the 41 patients in this group. The angiograms indicated that these vessels were abnormal systemic connections arising from the upper descending thoracic aorta (fig. 6). They were identified more frequently in the right (37%) than left hilum (27%), which differs from the results of Jefferson et al. [13], who reported an equal distribution. Our angiographic studies identified these abnormal hilar vessels in four additional patients, although this was not suspected from the plain chest radiograph. The association of normal heart size, right aortic arch, concave pulmonary segment, and decreased pulmonary vascularity are the most important chest film findings in these patients and are not significantly different from classical tetralogy of Fallot. Absence of Pulmonary Arteries (Truncus Arteriosus Type IV) The absence of pulmonary arteries was the second most common subset in this series, present in 15 of the 66 patients (22%). There were nine males and six females, ranging in age from 2 to 26 years. Angiographic studies. Situs solitus with a concordant atrioventricular relation was found in 14 patients; one patient had a discordant relation. The origin of the aorta was biventricular in 13 patients and from the right ventricle in two (fig.7).

6 1032 SOTO ET AL. The diagnosis of truncus arteriosus type IV in these patients rests upon the absence of an identifiable pulmonary artery. The source of pulmonary blood flow is solely bronchial arteries originating from the descending thoracic aorta. This must be differentiated from patients who have the same source of pulmonary blood flow, but who in addition have a true and separate pulmonary arterial system, as discussed above. The importance of this differentiation is clear, since surgical correction is possible only when a true pulmonary arterial system is present. Four of these 14 patients underwent exploratory thoracotomy, but a pulmonary artery could not be found in any, verifying the angiographic diagnosis. Chest films. Ten patients had normal sized hearts and five had cardiomegaly on chest radiography (fig. 8). The main pulmonary artery segment was absent in 13 patients, reduced from normal in one, and normal in another. Enlarged systemic arteries in the latter two patients were shown by angiography to account for this misinterpretation of the plain films. Peripheral vessels were of small caliber in each case, and peripheral collateral circulation was identified in 10 patients. The posteroanterior film chest more frequently demonstrated abnormal hilar vessels in patients with absent pulmonary arteries, but these films were not significantly different from those of patients with confluent pulmonary arteries when the whole group is considered. However, absence of pulmonary artery branches was more clearly demonstrated in the lateral projection (fig. 8), as emphasized by Vix and Klatte [16]. Nonconfluence of Pulmonary Arteries There were five patients who had nonconfluence of pulmonary arteries. All were male, ranging in age from 2 months to 13 years. Fig. 4.-Aortogram in patient with confluent pulmonary arteries. A, Anteropostenor view showing systemic arteries (SA) originating from upper descending aorta (Ao) and supplying both lungs. Pulmonary arterial system not evident because of superimposition of systemic branches. B, Semiaxial craniocaudal view of same patient showing separation of systemic arteries (SA) above, and right (RPA) and left (LPA) pulmonary arteries below. Angiographic studies. Situs solitus was present in all five patients; the atrioventricular relation was concordant in four patients and discordant in one. The aorta had a biventricular origin in four patients and arose from the right ventricle in one. The source of pulmonary blood flow was bronchial collateral arteries in each patient (fig. 9). Corrective surgery performed in one patient verified the angiographically determined anatomy. Systemicpulmonary artery shunts were made in three patients, confirming the presence of a nonconfluent pulmonary artery. Chest films. The heart size was normal in all five (fig. 10). Although the main pulmonary artery was absent in each patient, large bronchial collateral arteries were responsible for the incorrect identification of a pulmonary artery segment, reported as normal in two and diminshed in three patients. The right pulmonary artery was identified in three patients and the left pulmonary artery in one. The peripheral vessels were of small caliber in four cases and normal in one. Peripheral collateral circulation was uniformly present. Abnormal hilar vessels were identified on the right in three patients and on the left in two. These findings were similar to those found by analysis of the plain chest films of patients with confluent pulmonary arteries and also those with absent pulmonary arteries; thus they were not helpful in differentiating these entities. Mixed Type Five patients had the mixed type of congenital pulmonary atresia with ventricular septal defect. There were four males and one female, ranging in age from 3 days to 47 years. Angiographic studies. Situs solitus was present in four patients and situs inversus totalis in one. Four patients

7 CONGENITAL PULMONARY ATRESIA WITH VENTRICULAR SEPTAL DEFECT 1033 Fig. 5.-Chest radiographs in posteroantenion (A) and lateral (B) views showing congenital pulmonary atresia, ventricular septal defect, and confluent pulmonary arteries. Normal-sized heart and diminished pulmonary vasculanity are prominent features. Note right (arrowheads) and left (arrows) pulmonary arteries. Signs of peripheral collateral circulation are seen in lower segments. Fig. 6.-A, Postenoantenion chest film of patient with confluent pulmonary arteries showing diminished peripheral pulmonary vascularity and large abnormal vascular shadows at left hilum (arrows) and more penipherally (arrowheads). Abnormal vessel is in higher position than pulmonary artery, indicating collateral vessel. B, Angiogram of same patient showing that abnormal vessel (arrow) is systemic artery supplying left lung and originating from descending thoracic aorta. Right and left pulmonary arteries were demonstrated in later frames. had a concordant atrioventricular relation and one, a discordant connection. The source of pulmonary blood flow was in part a patent ductus arteriosus in two patients, a surgically created systemic-pulmonary artery shunt in two, and solely large bronchial arteries in one. Each patient had some segments of either lung supplied by a pulmonary arterial system and other segments supplied solely by bronchial arteries (fig. 11). Chest films. Heart size on the chest radiographs was normal in all five cases. The pulmonary artery segment

8 1034 SOTO ET AL. Fig. 7.- Aortogram, anteropostenior projection, showing systemic circulation of lungs in patient with absent pulmonary arteries (truncus arteniosus type lv). Tip of catheter is at upper descending aorta. Two systemic arteries supply right and left lungs, respectively. Notice tortuosity of these channels and areas of stenosis (arrows) following classical spiral coiled course. Fig. 8.-Posteroantenior (A) and lateral (B) chest films of patient with absent pulmonary arteries (truncus arteniosus type IV). Right and left branches of main pulmonary artery are not visualized.

9 CONGENITAL PULMONARY ATRESIA WITH VENTRICULAR SEPTAL DEFECT 1035 Fig. 9.-Aortogram of patient with nonconfluent right and left pulmonary arteries showing several small bronchial arteries supplying right and left lungs. In addition, large bronchial artery supplies right upper lobe (vertical arrow). Vessels outlined by horizontal arrows have angiographic appearance of pulmonary arteries. These two vessels are not confluent and their proximal parts are within hilum of each lung. was absent in three patients and indeterminate in two. Peripheral pulmonary vascularity was diminished in four patients and normal in one. Peripheral collateral circulation was absent in each patient, and abnormal hilar yessels were identified in only one patient. Analysis of the chest films in this group does not demonstrate any unique characteristics which permit differentiation from other subsets of these malformations. Discussion The major challenge to the cardiac radiologist studying patients with congenital pulmonary atresia is to provide precise knowledge of the absence or presence, size, and distribution (confluent central, nonconfluent central, on peripheral) of the left and right pulmonary arteries. This information allows the surgeon to decide whether palliative or corrective surgery is possible and/ or advisable. In those cases without pulmonary arteries, surgery is not possible. Pulmonary arteries were present and confluent in 41 of the 66 patients (62%), nonconfluent in five (8%), absent in 15 (23%); in five (8%) a mixed distribution existed. Aortography in the conventional anteroposterior view showed systemic arteries (bronchial) originating from the upper descending thoracic aorta supplying both lungs in 24 of 51 patients (47%) in whom pulmonary arteries were identified. It is this subset of patients with congenital pulmonary atresia with ventricular septal defect who require special attention by the cardiac radiologist. The presence of pulmonary arteries may not be identified without either selective angiograms of the bronchial arteries or a semiaxial craniocaudal view to separate the overlapping pulmonary artery from the bronchial collateral arteries (figs. 3 and 4). Since lack of identification of pulmonary arteries deems the patient inoperable, either of these specialized angiographic methods should be used when pulmonary arteries are not readily identified by conventional aortography. The decision for or against surgery also requires the following details which must be defined angiographically: (1) atrioventricular relations; (2) presence, position, and anatomy of both a right and left ventricle and their respective atrioventricular valves; (3) position and origin of the aorta; (4) location, size, and number of ventricular septal defects; (5) absence or presence, size, and distribution (confluent central, nonconfluent central, or peripheral) of the left and right pulmonary arteries; and (6) source of pulmonary blood flow. Situs solitus of the viscera and atria occurred in 95% of this series, and the atrioventricular relation was concordant in 94%. There was biventricular origin of the aorta in 72%; it arose completely from the right ventricle in 9% and from the left ventricle in 9%. Corrective surgery was performed in 29 of the 66 patients; in each case the angiographic definition of right ventricular and central pulmonary artery anatomy was verified. The right ventnicle had an underdeveloped or absent infundibulum, with either atresia of the pulmonary valve or main pulmonary artery. The ventricular septal defect was single, large, and located either in the typical position found in classic tetralogy of Fallot or slightly more anteriorly, as often found in truncus arteriosus types I and II. When the aorta originates both from the left and right ventricle so that it overrides the ventricular septal defect, the anatomy of the right ventricle is similar to that of tetralogy of Fallot. The infundibulum is absent or severely underdeveloped and the cephalad margin of the ventricular septal defect is formed by the aortic valve leaflets. The aorta is large and aorticmitral valve continuity is ususally present (fig. 1A). When the aorta originates entirely from the right ventricle, the appearance of the right ventricle is similar to normal. The infundibulum is usually well developed and there is discontinuity between the aortic valve and the atrioventricular values. The ventricular septal defect is usually located more anteriorly than in classical tetralogy of Fallot. The left ventricle is a posterior chamber with no outlet except for the ventricular septal defect (fig. 1 B). When the aorta originates from the morphologic left ventricle, the angiographic appearance of the left ventricle is similar to that of the normal heart. The ventricular septal defect is almost always located in the anterior interventricular septum in the left ventricular outflow tract (fig. 1D). When atrioventricular discordance is associated with pulmonary atresia, the intracardiac angiographic anatomy is similar to that of corrected transposition if the aorta arises from the right ventricle (fig. 1C). When the aorta arises from the left ventricle and there is atnioventricular discordance, the angiographic anatomy is similar to that in isolated ventricular inversion.

10 1036 SOTO ET AL. Fig. 10.-Posteroanterior (A) and lateral (B) chest films of child with nonconfluent right and left pulmonary arteries showing slightly enlarged heart with upturned apex and right aortic arch. Large vascular shadow is seen in right suprahilar region, indicating presence of abnormal vessel. Right and left pulmonary arteries not identified in B. The plain chest radiograph does not permit differentiation of the various subsets of congenital pulmonary atresia with ventricular septal defect from each other or from classical tetralogy of Fallot. These types of malformations may be suspected when a heart of normal size is associated with a concave or absent pulmonary artery segment, reduced peripheral pulmonary vascularity, and, in some cases, the presence of unilateral or bilateral abnormal hilar vessels. REFERENCES 1. Edwards JE, McGoon DC: Absence of anatomic origin from the heart of pulmonary arterial supply. Circulation 47: , Kinklin JW, Pacifico AD: Surgical treatment of congenital heart disease, in The Heart, edited by Hurst JW, New York, McGraw-Hill, 1974, pp Pacifico AD, Kirklin JW, Bargenon LM Jn, Soto B: Surgical treatment of common arterial trunk with pseudotruncus arteniosus. Circulation 49, suppl. 2:11 20-lI 26, Shinebounne EA, Macartney FJ, Anderson RH: Sequential chamber localization: logical approach to diagnosis in congenital heart disease. Br Heart J 38 : , 1976 S. Kinklin JW: Surgical treatment of patients with absence of direct anatomic continuity between pulmonary arterial systern and the heart and ventricular septal defects, in Heart Disease in Infancy, edited by Banratt-Boyes BG, Newtze JN, Harris EA, Baltimore, Williams & Wilkins, 1973, pp Somerville J: Management of pulmonary atnesia. Br Heart J 32: , Collett RW, Edwards JE: Persistent truncus arteniosus: a classification according to anatomic types. Surg Clin North Am 29: , 1949 Fig. 11.-Aortogram in patient with mixed type. Early (A) and late (B) phase showing systemic bronchial arteries (5) originating from descending aorta providing circulation to right and left lungs. Right (RPA) and left (LPA) pulmonary arteries, not seen in A, are well visualized in B. Right upper lobe and left lower lobe are supplied solely by bronchial arteries (arrows).

11 CONGENITAL PULMONARY ATRESIA WITH VENTRICULAR SEPTAL DEFECT Marcelletti C, Main DD, McGoon DC, Wallace RB, Danielson GK: Complete repair of transposition of the great arteries with pulmonary atresia. J Thorac Cardiovasc Surg 72 : , Baker WP, Kelminson LL, Turner WM Jr, Blount SG Jn: Absence of pulmonic valve associated with double-outlet right ventricle. Circulation 36:452, Proceedings of the annual meeting of the Netherlands Society of Radiology, April 1971.Radiol Clin Biol 42: , Campbell M, Gardner F: Radiographic features of enlarged bronchial arteries. Br Heart J 12: , Klinkharner AC: Angiography in candidates for total connection of persistent truncus arteniosus on pseudotnuncus anteniosus. Radio! Clin Biol 42: , Jeftenson K, Rees 5, Somerville J: Systemic arterial supply to the lungs in pulmonary atresia and its relation to pulmonary artery development. Br Heart J 34: , Cheslen E, Beck W, Schnire V: Selective catheterization of pulmonary on bronchial arteries in the preoperative assessment of pseudotruncus arteniosus and truncus arteniosus, type IV.Am J Cardiol 26:20-24, Levin DC, Baltaxe HA, Goldbert HP, Engle MA, Ebert PA, Sos TA, Levin AR: The importance of selective angiography of systemic arterial supply to the lungs in planning surgical correction of pseudotnuncus arteniosus. Am J Roentgeno! 121 : , Vix VA, Klatte EC: The lateral chest radiograph in diagnosis of hilar and mediastinal masses. Radiology 96: , 1970