Pediatr Cardiol 11:41-43, 1990
Pediatric Cardiology 9 Springer-Verlag New York Inc. 1990
Case Reports A o r t o p u l m o n a r y W i n d o w C o e x i s t i n g with T e t r a l o g y o f Fallot: Echocardiographic Diagnosis Mario Carminati,1 Adele Borghi,l Orazio Valsecchi,l Maria Quattrociocchi,l Abele Balduzzi, l Paolo Rusconi, 1 Maria Giovanna Russo, 1 Pierluigi Festa, l Laura Preda, l and Roberto Tiraboschi 2 Departments of ~Cardiology and 2Cardiac Surgery, Ospedali Riuniti, Bergamo, Italy
SUMMARY. Aortopulmonary window coexisting with tetralogy of Fallot (TF) was prospectively diagnosed by two-dimensional (2D) echocardiography and Doppler in an 18-month-old boy; the diagnosis was confirmed by cardiac catheterization and angiocardiography. Surgical correction was performed, but the patient died in the operating room from right ventricular dysfunction. The autopsy showed an adequate surgical repair, but the histologic examination of the lungs demonstrated severe pulmonary vascular disease, which was presumed to be the cause of death. KEY WORDS: Aortopulmonary window - - Tetralogy of Fallot - - Echocardiography
Aortopulmonary window associated with tetralogy o f F a l l o t ( T F ) is v e r y r a r e ; to o u r k n o w l e d g e o n l y 12 c a s e s h a v e b e e n r e p o r t e d [ 1 - 4 , 6]. W e d e s c r i b e a n a d d i t i o n a l c a s e , in w h i c h , p e r h a p s f o r t h e first t i m e , the diagnosis was prospectively achieved by echocardiography.
view showed a subaortic ventricular septal defect with overriding aorta, as typically found in TF (Fig. 1). The right anterior oblique view from the subcostal approach [5] showed a moderately severe subpulmonary obstruction; a large communication between the ascending aorta and the pulmonary artery, at the
Case Report An 18-month-old boy was referred to our institution because of failure to thrive. He was born in another hospital after an uncomplicated pregnancy; a heart murmur was heard at 15 days of age; neither cyanosis nor cardiac failure was present. An echocardiogram performed at that time was reported as typical for TF; the patient was subsequently followed clinically, and considered as having an acyanotic form of TF. At the age of 18 months he was referred to us for further evaluation. On admission the child was mildly underdeveloped, and noncyanotic. The first heart sound was normal, the second sound was single and slightly accentuated; an ejection systolic murmur was heard all over the precordium. The ECG showed a normal sinus rhythm, with right ventricular hypertrophy. The chest x-ray showed mild cardiomegaly with normal pulmonary vascular markings. Two-dimensional (2D) echocardiographic examination demonstrated a usual arrangement of atrial chambers and concordant atrioventricular connections. The long-axis parasternal
Fig. 1. Long-axis parasternal view: subaortic ventricular septal Address offprint requests to: Dr. Mario Carminati, Via Fogazzaro 8, 24100 Bergamo, Italy.
defect with overriding aorta. AO, aorta; LA, left atrium; LV, left ventricle; RV, right ventricle; A, anterior; I, inferior; P, posterior; S, superior.
42
junctional point between the main trunk and the right pulmonary branch, consistent with aortopulmonary window, was also clearly displayed in the same cut (Fig. 2). A suprasternal view demonstrated a right-sided aortic arch. Pulsed Doppler interrogation, with the sample volume placed in the pulmonary artery, showed a systolic flow directed from the aorta to the pulmonary artery; the diastolic flow was
Pediatric Cardiology Vol. 11, No. 1, 1989
nearly completely absent, suggesting a left-to-right shunt occurring almost exclusively during systole, probably because of pulmonary hypertension (Fig. 3). The characteristics of the Doppler tracings (short duration to peak velocity with spectral broadening, and low velocity flow in spite of right ventricular outflow tract obstruction) also suggested the presence of high pressure in the pulmonary artery. The diagnosis of TF with aortopulmonary window and pulmonary hypertension was fully confirmed by cardiac catheterization and angiocardiography. Surgical correction was performed under extracorporeal circulation. Unfortunately, at the end of the procedure, the pressure in the pulmonary artery remained at systemic level, and the right ventricle was dilated and poorly contracted; the patient eventually died from irreversible cardiac arrest. At autopsy the surgical repair looked adequate; the histologic examination of the lungs demonstrated unequivocal signs of severe pulmonary vascular disease.
Discussion
Fig. 2. Right anterior oblique view from a subcostal approach: the open arrow indicates the subpulmonary obstruction; the small arrows indicate the large " w i n d o w " between the ascending aorta and the pulmonary artery, at the junction between the main trunk and the right pulmonary artery. AO, aorta; PT, pulmonary trunk; RA, right atrium; RPA, right pulmonary artery; RV, right ventricle; I, inferior; L, left; R, right; S, superior.
Aortopuimonary window associated with TF is very rare, and presents a diagnostic challenge [3-6]. In the cases reported in literature, the diagnosis was achieved by angiographic means. However, diagnosis can be missed even by angiography, unless an aortogram is performed properly [4-6]. In our patient a prospective and complete diagnosis was achieved by 2D echocardiography and Doppler. The clinical implications in this rare association of defects are very important: the effects of pulmonary stenosis are masked by a left-to-right shunt through the window, and this can, in part, be beneficial by reducing the cyanosis. On the other hand, if the aortopulmonary shunt is very large (as it was in
Fig. 3. A Pulsed Doppler examination (subcostal approach). AO, aorta; PT, pulmonary trunk; RA, right atrium; RPA, right pulmonary artery; RV, right ventricle; 1, inferior; L, left; R, right; S, superior. B A Doppler tracing shows systolic flow directed away from the transducer (from aorta to pulmonary artery); the duration to peak velocity is short, with spectral broadening, and a low velocity flow (0.9 m/s) suggests pulmonary hypertension.
M. Carminati eta[.: Aortopulmonary Window with Tetralogy of Fatlot
this particular patient) pulmonary hypertension due to an increased vascular resistance is likely to develop early in infancy. Successful repair has been reported, even at an older age, but no irreversible changes in the pulmonary vessels are present [1-3]. On the other hand, the patient described by PdrezMartinez and coworkers [6] died a few hours after operation, and the cause of death was thought to be pulmonary hypertension and right ventricular failure. References 1. Castaneda AR, Kirklin JW (1977) Tetralogy of Fallot with aorticopulmonaty window. Report of two surgical cases. J Thorac Cardiooasc Surg 74:467-468
43
2. Clarke CP, Richardson JP (1976) The management of aortopulmonary window. Advantages of transaortic closure with a Dacron patch. J Thorac Cardiovasc Surg 72:48-51 3. Cooley DA, McNamara DG, Latson JR (1957) Aorticopulmonary septal defect: diagnosis and surgical treatment. Surgeo, 42:101 4. Crawford FA, Watson DG, Joransen JA (1981) Tetralogy of Fallot with coexisting type II aortopulmonary window. Surgery 31:78-81 5. Marino B, Ballerini L, Marcelletti C, Piva R, Pasquini L, Zacch~ C, Giannico S, De Simone G (1984) Right oblique subxifoid view for two-dimensional echocardiographic visualization of the right ventricle in congenital heart disease. Am J Cardio154:1064-1068 6. Perez-Martinez VM, Burgueros M, Quero M~ Perez Leon J, Haler G (1976) Aortico-pulmonary window associated with Tetralogy of Fallot. Report of one case and review of the literature. AngioIogy 27:526-534
Pediatric Cardiology 9 Springer-Verlag New York Inc. 1990
Case Reports A o r t o p u l m o n a r y W i n d o w C o e x i s t i n g with T e t r a l o g y o f Fallot: Echocardiographic Diagnosis Mario Carminati,1 Adele Borghi,l Orazio Valsecchi,l Maria Quattrociocchi,l Abele Balduzzi, l Paolo Rusconi, 1 Maria Giovanna Russo, 1 Pierluigi Festa, l Laura Preda, l and Roberto Tiraboschi 2 Departments of ~Cardiology and 2Cardiac Surgery, Ospedali Riuniti, Bergamo, Italy
SUMMARY. Aortopulmonary window coexisting with tetralogy of Fallot (TF) was prospectively diagnosed by two-dimensional (2D) echocardiography and Doppler in an 18-month-old boy; the diagnosis was confirmed by cardiac catheterization and angiocardiography. Surgical correction was performed, but the patient died in the operating room from right ventricular dysfunction. The autopsy showed an adequate surgical repair, but the histologic examination of the lungs demonstrated severe pulmonary vascular disease, which was presumed to be the cause of death. KEY WORDS: Aortopulmonary window - - Tetralogy of Fallot - - Echocardiography
Aortopulmonary window associated with tetralogy o f F a l l o t ( T F ) is v e r y r a r e ; to o u r k n o w l e d g e o n l y 12 c a s e s h a v e b e e n r e p o r t e d [ 1 - 4 , 6]. W e d e s c r i b e a n a d d i t i o n a l c a s e , in w h i c h , p e r h a p s f o r t h e first t i m e , the diagnosis was prospectively achieved by echocardiography.
view showed a subaortic ventricular septal defect with overriding aorta, as typically found in TF (Fig. 1). The right anterior oblique view from the subcostal approach [5] showed a moderately severe subpulmonary obstruction; a large communication between the ascending aorta and the pulmonary artery, at the
Case Report An 18-month-old boy was referred to our institution because of failure to thrive. He was born in another hospital after an uncomplicated pregnancy; a heart murmur was heard at 15 days of age; neither cyanosis nor cardiac failure was present. An echocardiogram performed at that time was reported as typical for TF; the patient was subsequently followed clinically, and considered as having an acyanotic form of TF. At the age of 18 months he was referred to us for further evaluation. On admission the child was mildly underdeveloped, and noncyanotic. The first heart sound was normal, the second sound was single and slightly accentuated; an ejection systolic murmur was heard all over the precordium. The ECG showed a normal sinus rhythm, with right ventricular hypertrophy. The chest x-ray showed mild cardiomegaly with normal pulmonary vascular markings. Two-dimensional (2D) echocardiographic examination demonstrated a usual arrangement of atrial chambers and concordant atrioventricular connections. The long-axis parasternal
Fig. 1. Long-axis parasternal view: subaortic ventricular septal Address offprint requests to: Dr. Mario Carminati, Via Fogazzaro 8, 24100 Bergamo, Italy.
defect with overriding aorta. AO, aorta; LA, left atrium; LV, left ventricle; RV, right ventricle; A, anterior; I, inferior; P, posterior; S, superior.
42
junctional point between the main trunk and the right pulmonary branch, consistent with aortopulmonary window, was also clearly displayed in the same cut (Fig. 2). A suprasternal view demonstrated a right-sided aortic arch. Pulsed Doppler interrogation, with the sample volume placed in the pulmonary artery, showed a systolic flow directed from the aorta to the pulmonary artery; the diastolic flow was
Pediatric Cardiology Vol. 11, No. 1, 1989
nearly completely absent, suggesting a left-to-right shunt occurring almost exclusively during systole, probably because of pulmonary hypertension (Fig. 3). The characteristics of the Doppler tracings (short duration to peak velocity with spectral broadening, and low velocity flow in spite of right ventricular outflow tract obstruction) also suggested the presence of high pressure in the pulmonary artery. The diagnosis of TF with aortopulmonary window and pulmonary hypertension was fully confirmed by cardiac catheterization and angiocardiography. Surgical correction was performed under extracorporeal circulation. Unfortunately, at the end of the procedure, the pressure in the pulmonary artery remained at systemic level, and the right ventricle was dilated and poorly contracted; the patient eventually died from irreversible cardiac arrest. At autopsy the surgical repair looked adequate; the histologic examination of the lungs demonstrated unequivocal signs of severe pulmonary vascular disease.
Discussion
Fig. 2. Right anterior oblique view from a subcostal approach: the open arrow indicates the subpulmonary obstruction; the small arrows indicate the large " w i n d o w " between the ascending aorta and the pulmonary artery, at the junction between the main trunk and the right pulmonary artery. AO, aorta; PT, pulmonary trunk; RA, right atrium; RPA, right pulmonary artery; RV, right ventricle; I, inferior; L, left; R, right; S, superior.
Aortopuimonary window associated with TF is very rare, and presents a diagnostic challenge [3-6]. In the cases reported in literature, the diagnosis was achieved by angiographic means. However, diagnosis can be missed even by angiography, unless an aortogram is performed properly [4-6]. In our patient a prospective and complete diagnosis was achieved by 2D echocardiography and Doppler. The clinical implications in this rare association of defects are very important: the effects of pulmonary stenosis are masked by a left-to-right shunt through the window, and this can, in part, be beneficial by reducing the cyanosis. On the other hand, if the aortopulmonary shunt is very large (as it was in
Fig. 3. A Pulsed Doppler examination (subcostal approach). AO, aorta; PT, pulmonary trunk; RA, right atrium; RPA, right pulmonary artery; RV, right ventricle; 1, inferior; L, left; R, right; S, superior. B A Doppler tracing shows systolic flow directed away from the transducer (from aorta to pulmonary artery); the duration to peak velocity is short, with spectral broadening, and a low velocity flow (0.9 m/s) suggests pulmonary hypertension.
M. Carminati eta[.: Aortopulmonary Window with Tetralogy of Fatlot
this particular patient) pulmonary hypertension due to an increased vascular resistance is likely to develop early in infancy. Successful repair has been reported, even at an older age, but no irreversible changes in the pulmonary vessels are present [1-3]. On the other hand, the patient described by PdrezMartinez and coworkers [6] died a few hours after operation, and the cause of death was thought to be pulmonary hypertension and right ventricular failure. References 1. Castaneda AR, Kirklin JW (1977) Tetralogy of Fallot with aorticopulmonaty window. Report of two surgical cases. J Thorac Cardiooasc Surg 74:467-468
43
2. Clarke CP, Richardson JP (1976) The management of aortopulmonary window. Advantages of transaortic closure with a Dacron patch. J Thorac Cardiovasc Surg 72:48-51 3. Cooley DA, McNamara DG, Latson JR (1957) Aorticopulmonary septal defect: diagnosis and surgical treatment. Surgeo, 42:101 4. Crawford FA, Watson DG, Joransen JA (1981) Tetralogy of Fallot with coexisting type II aortopulmonary window. Surgery 31:78-81 5. Marino B, Ballerini L, Marcelletti C, Piva R, Pasquini L, Zacch~ C, Giannico S, De Simone G (1984) Right oblique subxifoid view for two-dimensional echocardiographic visualization of the right ventricle in congenital heart disease. Am J Cardio154:1064-1068 6. Perez-Martinez VM, Burgueros M, Quero M~ Perez Leon J, Haler G (1976) Aortico-pulmonary window associated with Tetralogy of Fallot. Report of one case and review of the literature. AngioIogy 27:526-534
