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Aortopulmonary Septal Defect Coexisting with Ventricular Septal Defect and Pulmonary Atresia
Aortopulmonary Septal Defect Coexisting with Ventricular Septal Defect and Pulmonary Atresia Darryl F. Shore, F.R.C.S. ,Siew Yen Ho, Ph.D. , Robert H. Anderson, M.D. , Marc de Leval, M.D., and Christopher Lincoln, F.R.C.S. ABSTRACT Three patients are described in whom an aortopulmonary septal defect (aortopulmonary window) coexisted with a ventricular septal defect and pulmonary atresia. One patient had mild and another, moderate aortic regurgitation. In addition, one patient had a sinus of Valsalva aortic aneurysm, while another had a single coronary artery arising from the pulmonary trunk. One patient underwent surgical correction in infancy; the other two, in early adult life. In all three patients, surgical correction involved closure of the aortopulmonary window, closure of the ventricular septal defect, and placement of a valved conduit between the right ventricle and the distal pulmonary trunk. One patient died 3 weeks postoperatively due to secondary hemorrhage where the conduit had been sutured to the right ventricle. The other two patients are alive and well 3yz years and 6 months after surgery, respectively. The presence of aortopulmonary window permits normal development of the pulmonary arteries in the presence of the coexisting pulmonary atresia; in the three patients described, the absence of pulmonary vascular disease made total surgical repair feasible for this combination of defects.
Aortopulmonary window is a rare congenital cardiac anomaly. When encountered, it is frequently associated with other congenital heart defects, the most common of which are persistent ductus arteriosus, ventricular septal defect, and anomalies of the coronary arteries. To the best of our knowledge, only one patient has been described in whom aortopulmonary septal defect coexisted with piilmonary atresia and
From the Department of Surgery and Paediatrics, Brompton Hospital, Fulham Rd, and the Thoracic Unit, Hospital for Sick Children, Great Ormond St, London, United Kingdom. Accepted for publication Dec 2, 1981. Address reprint requests to Mr. Lincoln, Department of Surgery, Brompton Hospital, Fulham Rd, London SW3 6HP, United Kingdom.
ventricular septal defect [ll. This patient did not undergo surgical correction. In this article, we describe three further examples of the combination. Surgical correction was performed in one patient in infancy and in the other two in early adult life. Case Reports
This patient was seen at the age of 3 months with a history of marked tachypnea, difficulty with feeding, and failure to thrive. Examination showed a female infant with no dysmorphic features. Her birth weight was 3.4 kg. She was miserable and cachectic. The femoral pulses were bounding. There was a grade 314 ejection systolic murmur in the second and fourth intercostal spaces. The second heart sound was single, and there was an audible third heart sound. The patient was dyspneic at rest. Chest roentgenograms revealed cardiomegaly with increased pulmonary vascular markings. PATIENT 2. This 18-year-old man was known since birth to suffer from congenital heart disease and had been experiencing increasing cyanosis and dyspnea on exertion. He had not been previously investigated. On examination, he was seen to be peripherally and centrally cyanosed. There was marked clubbing of fingers. The pulse was regular and collapsing in nature; pulse rate was 70 beats per minute. The heart was clinically enlarged, with a normal first sound and a loud single second sound with an ejection click. Systolic thrills were palpable at both the apex and the left sternal edge. A loud systolic murmur and a moderate diastolic murmur were audible at the apex and the left sternal edge. Chest roentgenograms revealed an enlarged heart, moderately plethoric lung fields, and an empty pulmonary artery bay. PATIENT 3. This patient was a 23-year-old man who was known to have had a heart murmur since the age of 2 months. He had suffered PATIENT 1.
132 0003-4975/83/020132-06$01.50 0 1982 by The Society of Thoracic Surgeons
133 Shore et al: Aortopulmonary Septa1 Defect with VSD and Pulmonary Atresia
from increasing shortness of breath throughout his life; this breathlessness was associated with chronic cough and palpitations. On examination, he was seen to have both peripheral and central cyanosis, as well as finger clubbing. The pulse was regular and collapsing in nature; the pulse rate was 72 beats per minute. The heart was clinically enlarged, with both left and right ventricular heaves. A continuous murmur was audible over the precordium and was associated with a palpable thrill. Chest roentgenograms showed cardiac enlargement with a prominent pulmonary artery and ascending aorta. There was mild pulmonary plethora.
Diagnostic Studies Electrocardiography showed that all three patients were in sinus rhythm with evidence of biventricular hypertrophy and right axis deviation. Patient 2 also showed right bundle-branch block and right atrial hypertrophy. The diagnosis for all three patients was truncus arteriosus type I, although the alternative diagnosis of aortopulmonary window and pulmonary atresia was entertained for Patient 3. With hindsight, however, a diagnosis of aortopulmonary window and pulmonary atresia could have been made from the angiograms for Patients 2 and 3, as the pulmonary trunk proximal to the aortopulmonary window was outlined (Figs 1, 2). Right and left ventricular pressure was equal in all three patients. Pulmonary artery pressure in Patient 1 was at systemic level. There was a gradient of 100 mm Hg between aorta and pulmonary artery in Patient 3, and in Patient 2, the pulmonary artery was not entered. Patient 2 also had an aneurysm of the right coronary sinus of Valsalva, and moderate aortic incom-
Fig I . Aortogram of Patient 2 . The area of the aortopulmonary window (bracket) is shown, with the proximal and distal pulmonary artery trunk faintly outlined.
petence was present.
Results Operative Findings and Procedure The Operative findings are represented in Figure 3 . In Patient 1, the pulmonary trunk was connected to the aorta just above the aortic valve and extended from the window toward the right ventricle, where it ended blindly. The right ventricle had a short infundibulum with a ventricular septa1 defect reaching back to the
Fig 2 . Angiocardiogram of Patient 3 , showing aortopulmonary window with stenosis of the pulmonary trunk (amow) distal to this defect (see also Fig 3 A ) .
134 The Annals of Thoracic Surgery Vol 35 No 2 February 1983
A
B
A\
C
Fig 3 . Anatomical findings: ( A ) Patient 3 , (B)Patient 2 , (C) Patient I .
tricuspid valve, as is usually found in tetralogy of Fallot. The pulmonary trunk in this patient was detached from the aorta, and the defect in the aorta was closed with a double running suture. A longitudinal ventriculotomy was performed, along with infundibular resection of the right ventricle mainly on the parietal side. Then, patch closure of the ventricular septal defect was performed, and a No. 20 Hancock prosthesis was sutured proximally to the right ventricle and distally to the pulmonary trunk. In Patient 2, the ascending aorta was found to be enlarged, and there was a 5 x 2 cm aneurysm of the right sinus of Valsalva. In addition, there were three aortic valve leaflets and a 1 cm aortopulmonary window 4 cm distal to the aortic valve. An extremely large ventricular septal defect was present, together with pulmonary atresia. The pulmonary trunk ended in a blind sac at the most distal part of the right ventricular infundibulum. The aortopulmonary window in Patient 2 was closed with interrupted buttressed sutures through the pulmonary trunk. Hypertrophic muscle bundles of the right ventricular outflow tract were excised. Patch closure of the ventricular septal defect through the aortic valve was performed. The aneurysm of the sinus of Valsalva was excluded with interrupted horizontal mattress sutures, and 2.6 cm homograft
Fig 4 . Surgical procedure performed in Patient 3 .
conduit was sutured proximally to the right ventricle and distally to the pulmonary trunk. The incompetent aortic valve was repaired. In Patient 3 (Figs 3A, 4A, 4B), an aortopulmonary window approximately 1 cm in diameter was found in the posterolateral wall of the aorta. The aortic root was inspected through a longitudinal aortotomy (not shown). The aortic valve was tricuspid, and no coronary ostia could be identified in the aortic root. There appeared to be a single coronary artery emerging from the posterior wall of the main pulmonary artery. One centimeter cephalad to the point where the coronary artery arose, there was a discrete stenosis of the pulmonary artery. The main pulmonary artery in this patient
135 Shore et al: Aortopulmonary Septa1 Defect with VSD and Pulmonary Atresia
was opened by a longitudinal incision from a point just distal to the stenosis and extending to the bifurcation. A Dacron patch was sutured circumferentially inside the pulmonary artery at the point of stenosis, effectively isolating the proximal portion that gave rise to the aortopulmonary window and the coronary ostia; therefore, the coronary artery continued to be supplied with systemic arterial blood. Patch closure of the ventricular septal defect was performed through a right infundibular ventriculotomy. A 2.7 cm external homograft conduit was sutured proximally to the right ventricular infundibulum and distally to the pulmonary artery. Hospital M o r t a l i t y
There was one hospital death. Patient 2 died on the twenty-first postoperative day of sudden hemorrhage from the site of anastomosis between the homograft and the right ventricular outflow tract. Up to this time, postoperative progress had been uneventful. The hemorrhage was later proved to be due to infection. Postmortem examination confirmed the presence of an atretic pulmonary outflow tract with a membrane separating the pulmonary trunk from the right ventricular infundibulum. The aortopulmonary window was 2 cm above the atretic segment (Fig 5). The ventricular septal defect was similar to the one seen in tetralogy of Fallot (Fig 6). Patients 1and 3 are alive and well 3% years and 6 months after surgery, respectively.
Comment Aortopulmonary window has features in common with truncus arteriosus-so much so that some authors include cases of window within the general grouping of truncus arteriosus [2]. However, in the majority of patients, there is an unequivocal distinction to be made. In truncus arteriosus, a common arterial trunk is guarded by a single arterial valve, whereas in aortopulmonary window, there is a communication of varying dimensions between two separate arterial trunks, each guarded by its own arterial valve. But as the patients described here demonstrate, the presence of two separate arterial valves is not a pathognomonic criterion for an aortopul-
Fig 5 . Specimen of the heart from Patient 2 . The aortopulmonary window is 2 cm above the atretic segment of the pulmonary artery. Right ventricular (RV)infundibulum is seen proximal to a diaphragm separating the right ventricle from the pulmonary artery.
Fig 6 . lnterior surface of the right ventricle of the heart from Patient 2 . The ventricular septal defect (VSD)is similar to that seen in tetralogy of Fallot.
136 The Annals of Thoracic Surgery Vol 35 No 2 February 1983
monary window. This is because there are patients in whom two arterial trunks, one of which is atretic, leave the base of the heart, with a window between their ascending portions. Thus, aortopulmonary window can be found in the presence of either a single aortic trunk with pulmonary atresia, as reported in this article, or in the presence of a single pulmonary trunk with aortic atresia. The distinguishing feature of aortopulmonary window is the presence of two arterial trunks attached to the base of the heart, although not always in potential communication with the ventricular cavities. It must be pointed out that the association of aortopulmonary window with pulmonary atresia is rare; to our knowledge, only one case has been described previously [l].This was an infant weighing 2.9 kg who had severe symptoms of heart failure when first seen. Following catheterization, the patient received optimal medical therapy but died 1month later, before undergoing surgical correction. The diagnosis of aortopulmonary window with pulmonary atresia was made at postmortem examination, catheter diagnosis having been type I truncus arteriosus. A similar difficulty was encountered in making an accurate preoperative diagnosis in our patients, all three of whom were thought to have truncus arteriosus type I, although in Patient 3 the alternative diagnosis of aortopulmonary window and pulmonary atresia was entertained. Like the previously reported patient, Patient l was first seen in infancy with severe heart failure. Our other two patients had increasing dyspnea on exertion and cyanosis throughout childhood, and came to us early in adult life when the severity of symptoms began to restrict their activities. The likely explanation for their delay in seeking treatment was that pulmonary blood flow was limited in both cases. In Patient 2, the aortopulmonary window measured only l cm at its widest point in adult life. This contrasts with the relative size of the aortopulmonary windows in the patient who presented to us in infancy and the one reported by Faulkner and associates [ll; these windows were 6 mm at their widest points. In Patient 3, pulmonary blood flow was limited by stenosis of the pulmonary trunk beyond the point at which it gave rise to the single coronary artery.
Successful closure of aortopulmonary window with intracardiac repair of tetralogy of Fallot was previously described by Cooley and his co-workers in 1957 [3] and by Blieden and Moller in 1974 141, but we believe that this is the first case of surgical repair of aortopulmonary window associated with pulmonary atresia. In this combination of defects, the left-to-right shunt at the level of the great arteries allows normal development of the pulmonary artery despite the presence of pulmonary atresia. The absence of a raised pulmonary vascular resistance can be attributed to the early discovery of the defect in Patient 1, the restrictive size of the aortopulmonary window in Patient 2, and the discrete stenosis of the pulmonary trunk in Patient 3. The combination of normally developed pulmonary arteries and the absence of pulmonary vascular disease made these patients highly suitable for total surgical repair. In each of our patients and in the patient reported by Faulkner and associates [l], the aortopulmonary septa1 defect was situated in the posterolateral wall on the aorta. This defect would be categorized as type I according to the classification of Richardson and colleagues [5]. Aberrant origin of the right or left coronary artery is a frequent association with aortopulmonary windows [3, 5-7, 8-13]. Eight patients with absent right coronary arteries, two patients with aberrant left coronary arteries, and one with an aberrant single coronary artery are reported in the literature. In our third patient, a single coronary artery originated from the posterior aspect of the pulmonary trunk proximal to its stenotic region. It was successfully included on the aortic side of a patch, which was placed within the pulmonary trunk and sutured to the superior margin of the aortopulmonary defect. The importance of being aware of the frequent association of aberrant coronary arteries and aortopulmonary window, and the advisability of demonstrating their origin angiographically, has been pointed out by Richardson and colleagues [5]. One patient in their series had a single coronary artery arising from the pulmonary trunk. This condition was not diagnosed preoperatively, and the artery was initially closed into the pulmonary trunk. When
137 Shore et al: Aortopulmonary Septa1 Defect with VSD and Pulmonary Atresia
correct repair was finally achieved, cardiac performance proved inadequate, and the patient died.
7. Burroughs JT,Schmutzer KJ, Linder F, et al: Anomalous origin of the right coronary artery with aortico-pulmonary window and ventricular septal defect: report of a case with complete operative correction. J Cardiovasc Surg (Torino) 3:142, 1962 References 8. Deverall PB, Lincoln JCR, Aberdeen E, et al: 1. Faulkner SL, Oldam RR, Atwood GF: AortoAorto-pulmonary window. J Thorac Cardiovasc pulmonary window ventricular septal defect and Surg 57479, 1968 membranous pulmonary atresia with a diagnosis 9. Luisi SV, Ashraf MH, Gula G, et al: Anomalous of truncus arteriosus. Chest 65:351,1974 origin of the right coronary artery with aorto2. Calder L, Van Praagh R, Van Praagh S, et al: Trunpulmonary window. Thorax 35:446,1980 cus arteriosus communis. Am Heart J %:B, 1976 3. Cooley DA, McNamara DG, Latson JR: Aortico- 10. Meisner H, Schmidt-Habelman F, Sebening F, et al: Surgical correction of aorto-pulmonary septal pulmonary septal defect: diagnosis and surgical defects. Dis Chest 53:750, 1968 treatment. Surgery 42120, 1957 4. Blieden LC, Moller JH: Aortico-pulmonary septal 11. Morrow AG, Greenfield LJ, Braunwald E: Condefect. Br Heart J 36530, 1974 genital aorto-pulmonary septal defect: clinicaland 5. Richardson JV,Doty DB, Ross NP, et al: The hemodynamic findings, surgical technique and spectrum of anomalies of aorto-pulmonary sepresults of operative correction. Circulation 25: tation. J Thorac Cardiovasc Surg 78:21, 1977 463, 1962 6. Agius V, Rushworth A, Connolly N: Anomalous 12. Neufield HN, Lester RG, Adams P, et al: origin of the left coronary artery from pulmonary Aortico-pulmonary septal defect. Am J Cardiol 9:12, 1962 artery associated with an aorto-pulmonary defect. Br Heart J 32700, 1970 13. Schumaker HB: Discussion [31
Notice from the Southern Thoracic Surgical Association The Thirtieth Annual Meeting of the Southern Thoracic Surgical Association will be held at the Mariott's Marco Island Beach Hotel in Marco Island, FL, Nov 3-5, 1983. There will be a $100 registration fee for nonmember physicians except for guest speakers, authors and coauthors on the program, and residents. There will be a $25 registration fee for attendees of the Postgraduate Course on Thursday, Nov 3. Members wishing to participate in the scientific program should submit abstracts in tiplicate by May 15, 1983, to Raymond C. Read, M.D., 300 E Roosevelt Rd, Little Rock, AR 72206. Abstracts should be double-spaced on
one side of one sheet of paper, with a l-inch left margin, and limited to 200 words. All slides used during the presentation must be 35 mm. Manuscripts of accepted papers must be submitted to The Annals of Thoracic Surgery prior to the 1983 meeting or to the Secretary-Treasurer at the opening of the Scientific Session. Applications for membership should be completed by Sept 1, 1983, and forwarded to John H. Selby, M.D., 3801 19th St, Lubbock, TX 79410.
Harwey W . Bender, jr., M . D . Secretay-Treasurer
Aortopulmonary window is a rare congenital cardiac anomaly. When encountered, it is frequently associated with other congenital heart defects, the most common of which are persistent ductus arteriosus, ventricular septal defect, and anomalies of the coronary arteries. To the best of our knowledge, only one patient has been described in whom aortopulmonary septal defect coexisted with piilmonary atresia and
From the Department of Surgery and Paediatrics, Brompton Hospital, Fulham Rd, and the Thoracic Unit, Hospital for Sick Children, Great Ormond St, London, United Kingdom. Accepted for publication Dec 2, 1981. Address reprint requests to Mr. Lincoln, Department of Surgery, Brompton Hospital, Fulham Rd, London SW3 6HP, United Kingdom.
ventricular septal defect [ll. This patient did not undergo surgical correction. In this article, we describe three further examples of the combination. Surgical correction was performed in one patient in infancy and in the other two in early adult life. Case Reports
This patient was seen at the age of 3 months with a history of marked tachypnea, difficulty with feeding, and failure to thrive. Examination showed a female infant with no dysmorphic features. Her birth weight was 3.4 kg. She was miserable and cachectic. The femoral pulses were bounding. There was a grade 314 ejection systolic murmur in the second and fourth intercostal spaces. The second heart sound was single, and there was an audible third heart sound. The patient was dyspneic at rest. Chest roentgenograms revealed cardiomegaly with increased pulmonary vascular markings. PATIENT 2. This 18-year-old man was known since birth to suffer from congenital heart disease and had been experiencing increasing cyanosis and dyspnea on exertion. He had not been previously investigated. On examination, he was seen to be peripherally and centrally cyanosed. There was marked clubbing of fingers. The pulse was regular and collapsing in nature; pulse rate was 70 beats per minute. The heart was clinically enlarged, with a normal first sound and a loud single second sound with an ejection click. Systolic thrills were palpable at both the apex and the left sternal edge. A loud systolic murmur and a moderate diastolic murmur were audible at the apex and the left sternal edge. Chest roentgenograms revealed an enlarged heart, moderately plethoric lung fields, and an empty pulmonary artery bay. PATIENT 3. This patient was a 23-year-old man who was known to have had a heart murmur since the age of 2 months. He had suffered PATIENT 1.
132 0003-4975/83/020132-06$01.50 0 1982 by The Society of Thoracic Surgeons
133 Shore et al: Aortopulmonary Septa1 Defect with VSD and Pulmonary Atresia
from increasing shortness of breath throughout his life; this breathlessness was associated with chronic cough and palpitations. On examination, he was seen to have both peripheral and central cyanosis, as well as finger clubbing. The pulse was regular and collapsing in nature; the pulse rate was 72 beats per minute. The heart was clinically enlarged, with both left and right ventricular heaves. A continuous murmur was audible over the precordium and was associated with a palpable thrill. Chest roentgenograms showed cardiac enlargement with a prominent pulmonary artery and ascending aorta. There was mild pulmonary plethora.
Diagnostic Studies Electrocardiography showed that all three patients were in sinus rhythm with evidence of biventricular hypertrophy and right axis deviation. Patient 2 also showed right bundle-branch block and right atrial hypertrophy. The diagnosis for all three patients was truncus arteriosus type I, although the alternative diagnosis of aortopulmonary window and pulmonary atresia was entertained for Patient 3. With hindsight, however, a diagnosis of aortopulmonary window and pulmonary atresia could have been made from the angiograms for Patients 2 and 3, as the pulmonary trunk proximal to the aortopulmonary window was outlined (Figs 1, 2). Right and left ventricular pressure was equal in all three patients. Pulmonary artery pressure in Patient 1 was at systemic level. There was a gradient of 100 mm Hg between aorta and pulmonary artery in Patient 3, and in Patient 2, the pulmonary artery was not entered. Patient 2 also had an aneurysm of the right coronary sinus of Valsalva, and moderate aortic incom-
Fig I . Aortogram of Patient 2 . The area of the aortopulmonary window (bracket) is shown, with the proximal and distal pulmonary artery trunk faintly outlined.
petence was present.
Results Operative Findings and Procedure The Operative findings are represented in Figure 3 . In Patient 1, the pulmonary trunk was connected to the aorta just above the aortic valve and extended from the window toward the right ventricle, where it ended blindly. The right ventricle had a short infundibulum with a ventricular septa1 defect reaching back to the
Fig 2 . Angiocardiogram of Patient 3 , showing aortopulmonary window with stenosis of the pulmonary trunk (amow) distal to this defect (see also Fig 3 A ) .
134 The Annals of Thoracic Surgery Vol 35 No 2 February 1983
A
B
A\
C
Fig 3 . Anatomical findings: ( A ) Patient 3 , (B)Patient 2 , (C) Patient I .
tricuspid valve, as is usually found in tetralogy of Fallot. The pulmonary trunk in this patient was detached from the aorta, and the defect in the aorta was closed with a double running suture. A longitudinal ventriculotomy was performed, along with infundibular resection of the right ventricle mainly on the parietal side. Then, patch closure of the ventricular septal defect was performed, and a No. 20 Hancock prosthesis was sutured proximally to the right ventricle and distally to the pulmonary trunk. In Patient 2, the ascending aorta was found to be enlarged, and there was a 5 x 2 cm aneurysm of the right sinus of Valsalva. In addition, there were three aortic valve leaflets and a 1 cm aortopulmonary window 4 cm distal to the aortic valve. An extremely large ventricular septal defect was present, together with pulmonary atresia. The pulmonary trunk ended in a blind sac at the most distal part of the right ventricular infundibulum. The aortopulmonary window in Patient 2 was closed with interrupted buttressed sutures through the pulmonary trunk. Hypertrophic muscle bundles of the right ventricular outflow tract were excised. Patch closure of the ventricular septal defect through the aortic valve was performed. The aneurysm of the sinus of Valsalva was excluded with interrupted horizontal mattress sutures, and 2.6 cm homograft
Fig 4 . Surgical procedure performed in Patient 3 .
conduit was sutured proximally to the right ventricle and distally to the pulmonary trunk. The incompetent aortic valve was repaired. In Patient 3 (Figs 3A, 4A, 4B), an aortopulmonary window approximately 1 cm in diameter was found in the posterolateral wall of the aorta. The aortic root was inspected through a longitudinal aortotomy (not shown). The aortic valve was tricuspid, and no coronary ostia could be identified in the aortic root. There appeared to be a single coronary artery emerging from the posterior wall of the main pulmonary artery. One centimeter cephalad to the point where the coronary artery arose, there was a discrete stenosis of the pulmonary artery. The main pulmonary artery in this patient
135 Shore et al: Aortopulmonary Septa1 Defect with VSD and Pulmonary Atresia
was opened by a longitudinal incision from a point just distal to the stenosis and extending to the bifurcation. A Dacron patch was sutured circumferentially inside the pulmonary artery at the point of stenosis, effectively isolating the proximal portion that gave rise to the aortopulmonary window and the coronary ostia; therefore, the coronary artery continued to be supplied with systemic arterial blood. Patch closure of the ventricular septal defect was performed through a right infundibular ventriculotomy. A 2.7 cm external homograft conduit was sutured proximally to the right ventricular infundibulum and distally to the pulmonary artery. Hospital M o r t a l i t y
There was one hospital death. Patient 2 died on the twenty-first postoperative day of sudden hemorrhage from the site of anastomosis between the homograft and the right ventricular outflow tract. Up to this time, postoperative progress had been uneventful. The hemorrhage was later proved to be due to infection. Postmortem examination confirmed the presence of an atretic pulmonary outflow tract with a membrane separating the pulmonary trunk from the right ventricular infundibulum. The aortopulmonary window was 2 cm above the atretic segment (Fig 5). The ventricular septal defect was similar to the one seen in tetralogy of Fallot (Fig 6). Patients 1and 3 are alive and well 3% years and 6 months after surgery, respectively.
Comment Aortopulmonary window has features in common with truncus arteriosus-so much so that some authors include cases of window within the general grouping of truncus arteriosus [2]. However, in the majority of patients, there is an unequivocal distinction to be made. In truncus arteriosus, a common arterial trunk is guarded by a single arterial valve, whereas in aortopulmonary window, there is a communication of varying dimensions between two separate arterial trunks, each guarded by its own arterial valve. But as the patients described here demonstrate, the presence of two separate arterial valves is not a pathognomonic criterion for an aortopul-
Fig 5 . Specimen of the heart from Patient 2 . The aortopulmonary window is 2 cm above the atretic segment of the pulmonary artery. Right ventricular (RV)infundibulum is seen proximal to a diaphragm separating the right ventricle from the pulmonary artery.
Fig 6 . lnterior surface of the right ventricle of the heart from Patient 2 . The ventricular septal defect (VSD)is similar to that seen in tetralogy of Fallot.
136 The Annals of Thoracic Surgery Vol 35 No 2 February 1983
monary window. This is because there are patients in whom two arterial trunks, one of which is atretic, leave the base of the heart, with a window between their ascending portions. Thus, aortopulmonary window can be found in the presence of either a single aortic trunk with pulmonary atresia, as reported in this article, or in the presence of a single pulmonary trunk with aortic atresia. The distinguishing feature of aortopulmonary window is the presence of two arterial trunks attached to the base of the heart, although not always in potential communication with the ventricular cavities. It must be pointed out that the association of aortopulmonary window with pulmonary atresia is rare; to our knowledge, only one case has been described previously [l].This was an infant weighing 2.9 kg who had severe symptoms of heart failure when first seen. Following catheterization, the patient received optimal medical therapy but died 1month later, before undergoing surgical correction. The diagnosis of aortopulmonary window with pulmonary atresia was made at postmortem examination, catheter diagnosis having been type I truncus arteriosus. A similar difficulty was encountered in making an accurate preoperative diagnosis in our patients, all three of whom were thought to have truncus arteriosus type I, although in Patient 3 the alternative diagnosis of aortopulmonary window and pulmonary atresia was entertained. Like the previously reported patient, Patient l was first seen in infancy with severe heart failure. Our other two patients had increasing dyspnea on exertion and cyanosis throughout childhood, and came to us early in adult life when the severity of symptoms began to restrict their activities. The likely explanation for their delay in seeking treatment was that pulmonary blood flow was limited in both cases. In Patient 2, the aortopulmonary window measured only l cm at its widest point in adult life. This contrasts with the relative size of the aortopulmonary windows in the patient who presented to us in infancy and the one reported by Faulkner and associates [ll; these windows were 6 mm at their widest points. In Patient 3, pulmonary blood flow was limited by stenosis of the pulmonary trunk beyond the point at which it gave rise to the single coronary artery.
Successful closure of aortopulmonary window with intracardiac repair of tetralogy of Fallot was previously described by Cooley and his co-workers in 1957 [3] and by Blieden and Moller in 1974 141, but we believe that this is the first case of surgical repair of aortopulmonary window associated with pulmonary atresia. In this combination of defects, the left-to-right shunt at the level of the great arteries allows normal development of the pulmonary artery despite the presence of pulmonary atresia. The absence of a raised pulmonary vascular resistance can be attributed to the early discovery of the defect in Patient 1, the restrictive size of the aortopulmonary window in Patient 2, and the discrete stenosis of the pulmonary trunk in Patient 3. The combination of normally developed pulmonary arteries and the absence of pulmonary vascular disease made these patients highly suitable for total surgical repair. In each of our patients and in the patient reported by Faulkner and associates [l], the aortopulmonary septa1 defect was situated in the posterolateral wall on the aorta. This defect would be categorized as type I according to the classification of Richardson and colleagues [5]. Aberrant origin of the right or left coronary artery is a frequent association with aortopulmonary windows [3, 5-7, 8-13]. Eight patients with absent right coronary arteries, two patients with aberrant left coronary arteries, and one with an aberrant single coronary artery are reported in the literature. In our third patient, a single coronary artery originated from the posterior aspect of the pulmonary trunk proximal to its stenotic region. It was successfully included on the aortic side of a patch, which was placed within the pulmonary trunk and sutured to the superior margin of the aortopulmonary defect. The importance of being aware of the frequent association of aberrant coronary arteries and aortopulmonary window, and the advisability of demonstrating their origin angiographically, has been pointed out by Richardson and colleagues [5]. One patient in their series had a single coronary artery arising from the pulmonary trunk. This condition was not diagnosed preoperatively, and the artery was initially closed into the pulmonary trunk. When
137 Shore et al: Aortopulmonary Septa1 Defect with VSD and Pulmonary Atresia
correct repair was finally achieved, cardiac performance proved inadequate, and the patient died.
7. Burroughs JT,Schmutzer KJ, Linder F, et al: Anomalous origin of the right coronary artery with aortico-pulmonary window and ventricular septal defect: report of a case with complete operative correction. J Cardiovasc Surg (Torino) 3:142, 1962 References 8. Deverall PB, Lincoln JCR, Aberdeen E, et al: 1. Faulkner SL, Oldam RR, Atwood GF: AortoAorto-pulmonary window. J Thorac Cardiovasc pulmonary window ventricular septal defect and Surg 57479, 1968 membranous pulmonary atresia with a diagnosis 9. Luisi SV, Ashraf MH, Gula G, et al: Anomalous of truncus arteriosus. Chest 65:351,1974 origin of the right coronary artery with aorto2. Calder L, Van Praagh R, Van Praagh S, et al: Trunpulmonary window. Thorax 35:446,1980 cus arteriosus communis. Am Heart J %:B, 1976 3. Cooley DA, McNamara DG, Latson JR: Aortico- 10. Meisner H, Schmidt-Habelman F, Sebening F, et al: Surgical correction of aorto-pulmonary septal pulmonary septal defect: diagnosis and surgical defects. Dis Chest 53:750, 1968 treatment. Surgery 42120, 1957 4. Blieden LC, Moller JH: Aortico-pulmonary septal 11. Morrow AG, Greenfield LJ, Braunwald E: Condefect. Br Heart J 36530, 1974 genital aorto-pulmonary septal defect: clinicaland 5. Richardson JV,Doty DB, Ross NP, et al: The hemodynamic findings, surgical technique and spectrum of anomalies of aorto-pulmonary sepresults of operative correction. Circulation 25: tation. J Thorac Cardiovasc Surg 78:21, 1977 463, 1962 6. Agius V, Rushworth A, Connolly N: Anomalous 12. Neufield HN, Lester RG, Adams P, et al: origin of the left coronary artery from pulmonary Aortico-pulmonary septal defect. Am J Cardiol 9:12, 1962 artery associated with an aorto-pulmonary defect. Br Heart J 32700, 1970 13. Schumaker HB: Discussion [31
Notice from the Southern Thoracic Surgical Association The Thirtieth Annual Meeting of the Southern Thoracic Surgical Association will be held at the Mariott's Marco Island Beach Hotel in Marco Island, FL, Nov 3-5, 1983. There will be a $100 registration fee for nonmember physicians except for guest speakers, authors and coauthors on the program, and residents. There will be a $25 registration fee for attendees of the Postgraduate Course on Thursday, Nov 3. Members wishing to participate in the scientific program should submit abstracts in tiplicate by May 15, 1983, to Raymond C. Read, M.D., 300 E Roosevelt Rd, Little Rock, AR 72206. Abstracts should be double-spaced on
one side of one sheet of paper, with a l-inch left margin, and limited to 200 words. All slides used during the presentation must be 35 mm. Manuscripts of accepted papers must be submitted to The Annals of Thoracic Surgery prior to the 1983 meeting or to the Secretary-Treasurer at the opening of the Scientific Session. Applications for membership should be completed by Sept 1, 1983, and forwarded to John H. Selby, M.D., 3801 19th St, Lubbock, TX 79410.
Harwey W . Bender, jr., M . D . Secretay-Treasurer