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Congenital absence of the pulmonary valve leaflets associated with complete transposition of the great arteries
263
International Journal of Cardiologv, 1 (1982) 263-211 Elsevier Biomedical Press
Congenital absence of the pulmonary valve leaflets associated with complete transposition of the great arteries Jacob0
Ovseyevitz,
Fause
Attie
and Alfonso
Buendia
Department of Pediatric Cardiology, Institute National de Cardiologia ‘Ignacio Chavez: Mexrco (Received
Ovseyevitz associated 263-271.
15 July 198 1; second revised version received 30 November
198 1; accepted
5 December
198 1)
J, Attie F, Buendia A. Congenital absence of the pulmonary valve leaflets with complete transposition of the great arteries. Int J Cardiol 1982; 1:
We describe the clinical and surgical findings of a patient with absence of the pulmonary valve leaflets and complete transposition of the great arteries. The main clinical features were a loud, single second heart sound, a loud pansystolic murmur over the entire precordiaf area, but no diastolic murmurs were heard. Tbe electrocardiogram suggested right ventricular hypertrophy and right bundle branch block. Tbe roentgenographic study showed cardiomegaiy with dilatation of both branches of the pulmonary artery. Cardiac catheterization and angiocardiography showed systemic arterial desaturation, discordant vent&do-arterial connections and absence of the pulmonary valve leaflets. The patient underwent surgical correction by the Rastelli technique with a favorable clinical course.
Congenital absence of the pulmonary valve is a rare abnormality that is usually associated with concordant atrio-ventricular and ventriculo-arterial connections, obstruction of the right ventricular outflow tract and ventricular septal defect [ 1- 131. The prognosis of untreated patients is poor; in more than half of the reported cases the patients died in childhood [ 121. The clinical features of this syndrome have now
Reprint requests to: Fause Attie, M.D. Instituto I. Mexico, D.F. 14080, Mexico.
0167-5273/82/0000-0000/$02.75
National
de Cardiologia
0 1982 Elsevier Biomedical
Press
‘Ignacio
Chavez’, Juan Badiano
264
been recognized, allowing precise preoperative diagnosis [2]. The object of this report is to present a case of congenital absence of the pulmonary valve with transposition of the great arteries [ 14,151, subpulmonic ventricular septal defect and obstruction of the outflow tract of the left ventricle. The patient underwent total correction by the Rastelli technique [ 161.
Case report A lo-year-old boy was admitted to the National Institute of Cardiology in June 1978. He had had cyanosis since birth, and a cardiac murmur was detected at the age of 1 month. He complained of tiredness and shortness of breath on moderate exercise. He was considerably underdeveloped for his age. Clubbing and cyanosis were present. The pulse was of normal character and the blood pressure was 1 lo/70 mm Hg. The cardiac impulse suggested moderately severe right ventricular enlargement. A systolic thrill was felt at the suprasternal notch, and there was a loud, single second sound at the second left intercostal space. A loud systolic murmur was heard
Fig. 1. Plain chest roentgenogram. The cardiac shadow is moderately enlarged. Both pulmonary arteries are dilated with poorly visualized peripheral circulation. The aorta forms the upper left border of the cardiac shadow in a case of complete transposition with L-position of the aorta.
265
over the precordial area but no diastolic murmurs were detected. No signs of congestive heart failure were observed. The electrocardiogram showed sinus rhythm with a mean frontal QRS vector of + 150’ and qR pattern with inverted T waves in lead Vl, RS in V5 and qrS in V6, suggesting right ventricular hypertrophy associated with right bundle branch block. The chest X-ray (Fig. 1) showed moderate enlargement of the cardiac shadow (cardiothoracic ratio = 0.57), with marked dilatation of the right and left pulmonary branches. The left superior border suggested an L-position of the aorta. Cardiac catheterization showed right ventricular pressure of 108/14 mm Hg, aortic pressure of 105/50 mm Hg and pulmonary artery pressure of 20/9 mm Hg. The systemic arterial saturation was 88% and the Qp/Qs ratio was 1.3 : 1 with an effective pulmonary blood flow of 4liters/minute per m2. The atrio-ventricular connections were concordant. Angiograms performed in the right and left ventricles showed discordant ventriculo-arterial connections. There was impressive dilatation of the main and right pulmonary arteries, with a sharp transition in caliber from the
Fig. 2A. For legend see p. 267
Fig. 2B. For legend see opposite page.
branches to the peripheral vessels. No sigmoid cusps were visualized. An obstructive annulus was identified in the site of the pulmonary ring (Fig. 2A, B). At operation, the presence of transposition of the great arteries, ventricular septal defect and congenital absence of the pulmonary valve and obstructive pulmonary valvular ring were confirmed. During surgery the main pulmonary artery was divided from the left ventricle, and the cardiac end was oversewn. The ventricular septal defect was repaired in such a manner as to establish a connection between the left ventricle and the aorta. The right ventricle was connected to the distal end of the divided pulmonary artery by a 22 mm composite woven Dacron and bovine pericardium valved external conduit. The patient made a good recovery after surgery with no major complications. A postoperative M-mode echocardiogram with parasternal and suprasternal approaches and contrast injection demonstrated that anatomical continuity had been established between the right ventricle and the pulmonary artery (Fig. 3). Cardiac
267
Fig. 2. A. Anteroposterior right ventriculogram. The catheter is passing through the tricuspid valve into the right ventricle, the aorta is in L-position and the dilated pulmonary artery is contrasted via a ventricular septal defect. B. Lateral left ventriculogram. The catheter is passing through the mitral valve. The left ventricle is posterior and the pulmonary artery is connected to this cavity. Stenosis of the annular region can be recognized. C. Postoperative lateral right ventriculogram. The right ventricle is connected with the pulmonary artery via the valved external conduit. Ao=aorta; C=valved conduit: LV= left ventricle; PA =pulmonary artery; RPA = right pulmonary artery; RV = right ventricle.
catheterization performed 2 months after surgery showed a right ventricular systolic pressure of 50/9 mm Hg and the pulmonary artery pressure of 43/ 18 mm Hg. There was no evidence of a right-to-left or left-to-right shunt, and the arterial oxygen The right ventricular angiocardiogram showed the reconsaturation was 94%. structed pulmonary outflow tract (Fig. 2C). He has been followed for 18 months thereafter and has led a very active life without symptoms.
Discussion After the first description of isolated congenital absence of the pulmonary valve cusps [ 171, 266 cases have been reported [2,18,19], including 13 cases with intact
268
Fig. 3. Postoperative M-mode echocardiogram with contrast injection. Suprasternal view. Contrast material can be seen in the right pulmonary branch after its passage thropgh the right ventricle as was evidenced with the parasternal approach. Ao= aorta; RPA = right pulmonary artery.
ventricular septum [1,20]. This malformation has also been described in association with tetralogy of Fallot [2,5,6,13,23], atria1 septal defect [ 19,211, double-outlet right ventricle [22,23], patent ductus arteriosus [5,24,25], endocardial cushion defects [20], and Marfan’s syndrome [26]. We found no reports of cases with transposition of the great arteries. In the present case the pulmonary valvular ring was small and the cusps were absent. The subpulmonic ventricular septal defect was large and situated in the membranous area of the interventricular septum [6,7]. The only difference in comparison with the usual cases of absence of the pulmonary valve was the type of ventriculo-arterial connection and the imposition of the outflow obstruction over the left ventricle. Patients with absent pulmonary valve and ventricular septal defect may develop
269
heart failure during fetal life [5,24,25] due to the high pulmonary arterial resistance, or in infancy due to poorly tolerated pulmonary regurgitation. This syndrome frequently presents in infancy with severe respiratory distress [5,6,12] due to compression of the major bronchi by the dilated main pulmonary artery. Those patients surviving beyond infancy may appear to have variants of tetralogy of Fallot, with a bidirectional shunt or a predominant left-to-right shunt. In our case the discordant ventriculo-arterial connection could have changed the hemodynamic behavior of this malformation as the pulmonary regurgitation produced diastolic overload of the left ventricle. Some authors believe that the diastolic gradient from the pulmonary trunk to the recipient ventricle protects this ventricle from failure because of a diminished volume of regurgitation [6]. However, the combination of volume and pressure overload places that ventricle under two handicaps so that it does not function as it would under normal conditions or with a same degree of obstruction but without regurgitation. Despite these facts our patient had no signs of left ventricular failure, and we cannot find any suitable explanation for this unexpected feature. Diagnosis The diagnosis is suspected clinically by the presence of a loud systolic murmur heard maximally at the left lower sternal border and a less intense diastolic murmur usually in the second and third left intercostal space along the sternal border [8,20]. In our case the absence of the typical murmur observed in this malformation can be explained by the posterior, right-sided pulmonary artery. The systolic murmur heard over the precordial area was probably produced by obstruction of the left ventricular outflow tract. As in cases with concordant ventriculo-arterial connection, the roentgenographic image of a moderately enlarged heart with massively dilated right and left pulmonary arteries suggests absence of pulmonary leaflets associated with tetralogy of Fallot [ 111. In our patient the L-position of the aorta permited good visualization of the right pulmonary artery. Surgical treatment
Surgical treatment may be required to relieve bronchial obstruction, pulmonary stenosis, and regurgitation, and for closure of the ventricular septal defect [7,11,13]. Homo- and heterograft valves have been used for the reconstruction of the right ventricular outflow tract in patients with pulmonary atresia, persistent truncus arteriosus and other forms of hypoplasia of the outflow tract. The long-term function of the prostheses may depend largely on the preservation of the tissue before surgery.
270
Acknowledgments The authors express their sincere who operated on this patient.
appreciation
to Dr. Fernando
Lbpez-Soriano
References 1 Buendia A, Attie F, Canale J, Ovseyevitz J, Acosta C, Zamora C, Kuri J, Mufioz L. Congenital absence of the pulmonary valve (abstract). London: World Congress of Paediatric Cardiology, 1980. 2 Calder AL, Brandt RWT, Barratt-Boyes BG, Neutze JM. Variant of tetralogy of Fallot with absent pulmonary valve leaflets and origin of one pulmonary artery from the ascending aorta. Am J Cardiol 1980; 46: 106. 3 Campeau L, Gilbert G, Aerichide N. Absence of pulmonary valve: Report of two cases associated with other congenital lesions. Am J Cardiol 1961; 8: 113. 4 D’Cruz IA, Lendrum BL, Novak G. Congenital absence of the pulmonary valve. Am Heart J 1964; 68: 1728. 5 Emmanouilides GC. Thanopoulos B, Siassi B. Fishbein M. ‘Agenesis’ of ductus arteriosus associated with the syndrome of tetralogy of Fallot and absent pulmonary valve. Am J Cardiol 1976; 37: 403. 6 Lakier JB, Stanger P, Heymann MA, Hoffman JIE, Rudolph AM. Tetralogy of Fallot with absent pulmonary valve. Natural history and hemodynamic considerations, Circulation 1974; 50: 167. 7 Layton CA, McDonald A, McDonald L, Towers M, Weaver J. Yacoub M. The syndrome of absent pulmonary valve. Total correction with aortic valvular homografts. J Thorac Cardiovasc Surg 1972; 63: 800. 8 Macartney FJ, Miller GAH. Congenital absence of the pulmonary valve. Br Heart J 1970; 32: 483. 9 Osman MZ, Meng CCL, Girdany BR. Congenital absence of the pulmonary valve: report of eight cases with review of the literature. Am J Roentgen01 1969; 106: 58. 10 Perez-Trevilio C, Wabi DC. Agenesia de las valvulas pulmonares. Presentation de once cases y revision de la literatura. Arch Inst Cardiol Mex 1972; 42: 33. 11 Pernot C, Hoeffel JC. Henry M, Worns AM, Stehlin H. Louis JP. Radiological patterns of congenital absence of the pulmonary valve in infants. Radiology 1972; 102: 619. 12 Pinsky WW, Nihill MR. Mullins CE, Harrison G. McNamara DG. The absent pulmonary valve syndrome. Considerations of management. Circulation 1978; 57: 159. 13 Stafford EG, Mair DD, McGoon DC, Danielson GK. Tetralogy of Fallot with absent pulmonary valve: Surgical considerations and results. Circulation 1973: 47-48 (Suppl. III): 111-24. I4 Van Praagh R, Perez-Treviiio C. Lopez-Cuellar M. Baker FW, Zuberbuhler JR. Quero M. Perez VM, Moreno F, Van Praagh S. Transposition of the great arteries with posterior aorta. anterior pulmonary artery, subpulmonary conus and fibrous continuity between aortic and atrioventricular valves. Am J Cardiol 1971; 28: 621. 15 Shinebourne EA. Anderson RH. Current paediatric cardiology. Oxford-New York-Toronto: Oxford University Press, 1980: 174. 16 Rastelli GC, McGoon DC, Wallace RB. Anatomic correction of transposition of the great arteries with ventricular septal defect and subpulmonary stenosis. J Thorac Cardiovasc Surg 1969; 58: 545. 17 Chevers N. Recherches sur les maladies de l’artere pulmonaire. Arch Gen Med 1847; 15: 488. 18 Hueb WA, Mazzieri R, Oliveira HA, Souza JM, Palacios E, Oliveira SA. Agenesia da valva pulmonar associada a comunicacao interventricular. Arq Bras Cardiol 1981; 36: 53. 19 Ilbawi MN, Idriss FS. Muster AJ. Wessel HV, Paul MH, De Leon SY. Tetralogy of Fallot with absent pulmonary valve. Should valve insertion be part of intracardiac repair? J Thorac Cardiovasc Surg 1981; 81: 906. 20 Moss AJ, Adams FH, Emmanouilides GC. Heart disease in infants. children and adolescents. Baltimore: The Williams & Wilkins Co.. 1977: 258. 21 Chiemmongkoltip P, Replogle RL, Arcilla RA. Congenital absence of the pulmonary vaalve with atria1 septal defect surgically corrected with aortic homograft. Chest 1972: 62: 100.
22 Baker WP, Kelminson LL, Turner WM, Blount SG. Absence double outlet right ventricle. Circulation 1967: 36: 452.
of pulmonic
valve associated
with
23 Litwin SB, Rosenthal A, Fellows K. Surgical management of young infants with tetralogy of Fallot, absence of pulmonary valve, and respiratory distress. J Thorac Cardiovasc Surg 1972: 65: 552. 24 Smith RD. DuShane JW, Edwards JE. Congenital insufficiency of the pulmonary valve, including a case of fetal cardiac failure. Circulation 1959; 20: 554. 25 Ito T, Engle MA, Holswade GR. Congenital insufficiency of the pulmonic valve. A rare cause of neonatal heart failure. Pediatrics 1961; 28: 712. 26 Childers RW, McCrea PC. Absence of the pulmonary valve. A case occurring in the Marfan Syndrome. Circulation 1964; 29: 598.
International Journal of Cardiologv, 1 (1982) 263-211 Elsevier Biomedical Press
Congenital absence of the pulmonary valve leaflets associated with complete transposition of the great arteries Jacob0
Ovseyevitz,
Fause
Attie
and Alfonso
Buendia
Department of Pediatric Cardiology, Institute National de Cardiologia ‘Ignacio Chavez: Mexrco (Received
Ovseyevitz associated 263-271.
15 July 198 1; second revised version received 30 November
198 1; accepted
5 December
198 1)
J, Attie F, Buendia A. Congenital absence of the pulmonary valve leaflets with complete transposition of the great arteries. Int J Cardiol 1982; 1:
We describe the clinical and surgical findings of a patient with absence of the pulmonary valve leaflets and complete transposition of the great arteries. The main clinical features were a loud, single second heart sound, a loud pansystolic murmur over the entire precordiaf area, but no diastolic murmurs were heard. Tbe electrocardiogram suggested right ventricular hypertrophy and right bundle branch block. Tbe roentgenographic study showed cardiomegaiy with dilatation of both branches of the pulmonary artery. Cardiac catheterization and angiocardiography showed systemic arterial desaturation, discordant vent&do-arterial connections and absence of the pulmonary valve leaflets. The patient underwent surgical correction by the Rastelli technique with a favorable clinical course.
Congenital absence of the pulmonary valve is a rare abnormality that is usually associated with concordant atrio-ventricular and ventriculo-arterial connections, obstruction of the right ventricular outflow tract and ventricular septal defect [ 1- 131. The prognosis of untreated patients is poor; in more than half of the reported cases the patients died in childhood [ 121. The clinical features of this syndrome have now
Reprint requests to: Fause Attie, M.D. Instituto I. Mexico, D.F. 14080, Mexico.
0167-5273/82/0000-0000/$02.75
National
de Cardiologia
0 1982 Elsevier Biomedical
Press
‘Ignacio
Chavez’, Juan Badiano
264
been recognized, allowing precise preoperative diagnosis [2]. The object of this report is to present a case of congenital absence of the pulmonary valve with transposition of the great arteries [ 14,151, subpulmonic ventricular septal defect and obstruction of the outflow tract of the left ventricle. The patient underwent total correction by the Rastelli technique [ 161.
Case report A lo-year-old boy was admitted to the National Institute of Cardiology in June 1978. He had had cyanosis since birth, and a cardiac murmur was detected at the age of 1 month. He complained of tiredness and shortness of breath on moderate exercise. He was considerably underdeveloped for his age. Clubbing and cyanosis were present. The pulse was of normal character and the blood pressure was 1 lo/70 mm Hg. The cardiac impulse suggested moderately severe right ventricular enlargement. A systolic thrill was felt at the suprasternal notch, and there was a loud, single second sound at the second left intercostal space. A loud systolic murmur was heard
Fig. 1. Plain chest roentgenogram. The cardiac shadow is moderately enlarged. Both pulmonary arteries are dilated with poorly visualized peripheral circulation. The aorta forms the upper left border of the cardiac shadow in a case of complete transposition with L-position of the aorta.
265
over the precordial area but no diastolic murmurs were detected. No signs of congestive heart failure were observed. The electrocardiogram showed sinus rhythm with a mean frontal QRS vector of + 150’ and qR pattern with inverted T waves in lead Vl, RS in V5 and qrS in V6, suggesting right ventricular hypertrophy associated with right bundle branch block. The chest X-ray (Fig. 1) showed moderate enlargement of the cardiac shadow (cardiothoracic ratio = 0.57), with marked dilatation of the right and left pulmonary branches. The left superior border suggested an L-position of the aorta. Cardiac catheterization showed right ventricular pressure of 108/14 mm Hg, aortic pressure of 105/50 mm Hg and pulmonary artery pressure of 20/9 mm Hg. The systemic arterial saturation was 88% and the Qp/Qs ratio was 1.3 : 1 with an effective pulmonary blood flow of 4liters/minute per m2. The atrio-ventricular connections were concordant. Angiograms performed in the right and left ventricles showed discordant ventriculo-arterial connections. There was impressive dilatation of the main and right pulmonary arteries, with a sharp transition in caliber from the
Fig. 2A. For legend see p. 267
Fig. 2B. For legend see opposite page.
branches to the peripheral vessels. No sigmoid cusps were visualized. An obstructive annulus was identified in the site of the pulmonary ring (Fig. 2A, B). At operation, the presence of transposition of the great arteries, ventricular septal defect and congenital absence of the pulmonary valve and obstructive pulmonary valvular ring were confirmed. During surgery the main pulmonary artery was divided from the left ventricle, and the cardiac end was oversewn. The ventricular septal defect was repaired in such a manner as to establish a connection between the left ventricle and the aorta. The right ventricle was connected to the distal end of the divided pulmonary artery by a 22 mm composite woven Dacron and bovine pericardium valved external conduit. The patient made a good recovery after surgery with no major complications. A postoperative M-mode echocardiogram with parasternal and suprasternal approaches and contrast injection demonstrated that anatomical continuity had been established between the right ventricle and the pulmonary artery (Fig. 3). Cardiac
267
Fig. 2. A. Anteroposterior right ventriculogram. The catheter is passing through the tricuspid valve into the right ventricle, the aorta is in L-position and the dilated pulmonary artery is contrasted via a ventricular septal defect. B. Lateral left ventriculogram. The catheter is passing through the mitral valve. The left ventricle is posterior and the pulmonary artery is connected to this cavity. Stenosis of the annular region can be recognized. C. Postoperative lateral right ventriculogram. The right ventricle is connected with the pulmonary artery via the valved external conduit. Ao=aorta; C=valved conduit: LV= left ventricle; PA =pulmonary artery; RPA = right pulmonary artery; RV = right ventricle.
catheterization performed 2 months after surgery showed a right ventricular systolic pressure of 50/9 mm Hg and the pulmonary artery pressure of 43/ 18 mm Hg. There was no evidence of a right-to-left or left-to-right shunt, and the arterial oxygen The right ventricular angiocardiogram showed the reconsaturation was 94%. structed pulmonary outflow tract (Fig. 2C). He has been followed for 18 months thereafter and has led a very active life without symptoms.
Discussion After the first description of isolated congenital absence of the pulmonary valve cusps [ 171, 266 cases have been reported [2,18,19], including 13 cases with intact
268
Fig. 3. Postoperative M-mode echocardiogram with contrast injection. Suprasternal view. Contrast material can be seen in the right pulmonary branch after its passage thropgh the right ventricle as was evidenced with the parasternal approach. Ao= aorta; RPA = right pulmonary artery.
ventricular septum [1,20]. This malformation has also been described in association with tetralogy of Fallot [2,5,6,13,23], atria1 septal defect [ 19,211, double-outlet right ventricle [22,23], patent ductus arteriosus [5,24,25], endocardial cushion defects [20], and Marfan’s syndrome [26]. We found no reports of cases with transposition of the great arteries. In the present case the pulmonary valvular ring was small and the cusps were absent. The subpulmonic ventricular septal defect was large and situated in the membranous area of the interventricular septum [6,7]. The only difference in comparison with the usual cases of absence of the pulmonary valve was the type of ventriculo-arterial connection and the imposition of the outflow obstruction over the left ventricle. Patients with absent pulmonary valve and ventricular septal defect may develop
269
heart failure during fetal life [5,24,25] due to the high pulmonary arterial resistance, or in infancy due to poorly tolerated pulmonary regurgitation. This syndrome frequently presents in infancy with severe respiratory distress [5,6,12] due to compression of the major bronchi by the dilated main pulmonary artery. Those patients surviving beyond infancy may appear to have variants of tetralogy of Fallot, with a bidirectional shunt or a predominant left-to-right shunt. In our case the discordant ventriculo-arterial connection could have changed the hemodynamic behavior of this malformation as the pulmonary regurgitation produced diastolic overload of the left ventricle. Some authors believe that the diastolic gradient from the pulmonary trunk to the recipient ventricle protects this ventricle from failure because of a diminished volume of regurgitation [6]. However, the combination of volume and pressure overload places that ventricle under two handicaps so that it does not function as it would under normal conditions or with a same degree of obstruction but without regurgitation. Despite these facts our patient had no signs of left ventricular failure, and we cannot find any suitable explanation for this unexpected feature. Diagnosis The diagnosis is suspected clinically by the presence of a loud systolic murmur heard maximally at the left lower sternal border and a less intense diastolic murmur usually in the second and third left intercostal space along the sternal border [8,20]. In our case the absence of the typical murmur observed in this malformation can be explained by the posterior, right-sided pulmonary artery. The systolic murmur heard over the precordial area was probably produced by obstruction of the left ventricular outflow tract. As in cases with concordant ventriculo-arterial connection, the roentgenographic image of a moderately enlarged heart with massively dilated right and left pulmonary arteries suggests absence of pulmonary leaflets associated with tetralogy of Fallot [ 111. In our patient the L-position of the aorta permited good visualization of the right pulmonary artery. Surgical treatment
Surgical treatment may be required to relieve bronchial obstruction, pulmonary stenosis, and regurgitation, and for closure of the ventricular septal defect [7,11,13]. Homo- and heterograft valves have been used for the reconstruction of the right ventricular outflow tract in patients with pulmonary atresia, persistent truncus arteriosus and other forms of hypoplasia of the outflow tract. The long-term function of the prostheses may depend largely on the preservation of the tissue before surgery.
270
Acknowledgments The authors express their sincere who operated on this patient.
appreciation
to Dr. Fernando
Lbpez-Soriano
References 1 Buendia A, Attie F, Canale J, Ovseyevitz J, Acosta C, Zamora C, Kuri J, Mufioz L. Congenital absence of the pulmonary valve (abstract). London: World Congress of Paediatric Cardiology, 1980. 2 Calder AL, Brandt RWT, Barratt-Boyes BG, Neutze JM. Variant of tetralogy of Fallot with absent pulmonary valve leaflets and origin of one pulmonary artery from the ascending aorta. Am J Cardiol 1980; 46: 106. 3 Campeau L, Gilbert G, Aerichide N. Absence of pulmonary valve: Report of two cases associated with other congenital lesions. Am J Cardiol 1961; 8: 113. 4 D’Cruz IA, Lendrum BL, Novak G. Congenital absence of the pulmonary valve. Am Heart J 1964; 68: 1728. 5 Emmanouilides GC. Thanopoulos B, Siassi B. Fishbein M. ‘Agenesis’ of ductus arteriosus associated with the syndrome of tetralogy of Fallot and absent pulmonary valve. Am J Cardiol 1976; 37: 403. 6 Lakier JB, Stanger P, Heymann MA, Hoffman JIE, Rudolph AM. Tetralogy of Fallot with absent pulmonary valve. Natural history and hemodynamic considerations, Circulation 1974; 50: 167. 7 Layton CA, McDonald A, McDonald L, Towers M, Weaver J. Yacoub M. The syndrome of absent pulmonary valve. Total correction with aortic valvular homografts. J Thorac Cardiovasc Surg 1972; 63: 800. 8 Macartney FJ, Miller GAH. Congenital absence of the pulmonary valve. Br Heart J 1970; 32: 483. 9 Osman MZ, Meng CCL, Girdany BR. Congenital absence of the pulmonary valve: report of eight cases with review of the literature. Am J Roentgen01 1969; 106: 58. 10 Perez-Trevilio C, Wabi DC. Agenesia de las valvulas pulmonares. Presentation de once cases y revision de la literatura. Arch Inst Cardiol Mex 1972; 42: 33. 11 Pernot C, Hoeffel JC. Henry M, Worns AM, Stehlin H. Louis JP. Radiological patterns of congenital absence of the pulmonary valve in infants. Radiology 1972; 102: 619. 12 Pinsky WW, Nihill MR. Mullins CE, Harrison G. McNamara DG. The absent pulmonary valve syndrome. Considerations of management. Circulation 1978; 57: 159. 13 Stafford EG, Mair DD, McGoon DC, Danielson GK. Tetralogy of Fallot with absent pulmonary valve: Surgical considerations and results. Circulation 1973: 47-48 (Suppl. III): 111-24. I4 Van Praagh R, Perez-Treviiio C. Lopez-Cuellar M. Baker FW, Zuberbuhler JR. Quero M. Perez VM, Moreno F, Van Praagh S. Transposition of the great arteries with posterior aorta. anterior pulmonary artery, subpulmonary conus and fibrous continuity between aortic and atrioventricular valves. Am J Cardiol 1971; 28: 621. 15 Shinebourne EA. Anderson RH. Current paediatric cardiology. Oxford-New York-Toronto: Oxford University Press, 1980: 174. 16 Rastelli GC, McGoon DC, Wallace RB. Anatomic correction of transposition of the great arteries with ventricular septal defect and subpulmonary stenosis. J Thorac Cardiovasc Surg 1969; 58: 545. 17 Chevers N. Recherches sur les maladies de l’artere pulmonaire. Arch Gen Med 1847; 15: 488. 18 Hueb WA, Mazzieri R, Oliveira HA, Souza JM, Palacios E, Oliveira SA. Agenesia da valva pulmonar associada a comunicacao interventricular. Arq Bras Cardiol 1981; 36: 53. 19 Ilbawi MN, Idriss FS. Muster AJ. Wessel HV, Paul MH, De Leon SY. Tetralogy of Fallot with absent pulmonary valve. Should valve insertion be part of intracardiac repair? J Thorac Cardiovasc Surg 1981; 81: 906. 20 Moss AJ, Adams FH, Emmanouilides GC. Heart disease in infants. children and adolescents. Baltimore: The Williams & Wilkins Co.. 1977: 258. 21 Chiemmongkoltip P, Replogle RL, Arcilla RA. Congenital absence of the pulmonary vaalve with atria1 septal defect surgically corrected with aortic homograft. Chest 1972: 62: 100.
22 Baker WP, Kelminson LL, Turner WM, Blount SG. Absence double outlet right ventricle. Circulation 1967: 36: 452.
of pulmonic
valve associated
with
23 Litwin SB, Rosenthal A, Fellows K. Surgical management of young infants with tetralogy of Fallot, absence of pulmonary valve, and respiratory distress. J Thorac Cardiovasc Surg 1972: 65: 552. 24 Smith RD. DuShane JW, Edwards JE. Congenital insufficiency of the pulmonary valve, including a case of fetal cardiac failure. Circulation 1959; 20: 554. 25 Ito T, Engle MA, Holswade GR. Congenital insufficiency of the pulmonic valve. A rare cause of neonatal heart failure. Pediatrics 1961; 28: 712. 26 Childers RW, McCrea PC. Absence of the pulmonary valve. A case occurring in the Marfan Syndrome. Circulation 1964; 29: 598.