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Pulmonary atresia and aortic valve stenosis
103
IJC 0555D
Pulmonary atresia and aortic valve stenosis R.P. Martin, R. Radley-Smith, M.H. Yacoub Department
of Paediatric Cardiology and Cardiac Surgev,
Harefield Hospital, Harefield
Middlesex,
U.K.
(Received 12 January 1987; revision accepted 30 January 1987)
A case is described of pulmonary atresia with ventricular septal defect and severe aortic valve stenosis. The aortic valve gradient measured at the time of insertion of an aortic to pulmonary arterial shunt was only 5 mm Hg and aortic valvotomy was deferred. Post-operative low output cardiac failure resulted in the death of the patient. The severity of the aortic stenosis was confiied by post-mortem examination. We suggest consideration of aortic valvotomy in similar cases as the intraoperative pressure gradient may be unreliable.
Key words: Aortic valve stenosis; Pulmonary atresia
Introduction
Aortic valve stenosis in association with pulmonary atresia and ventricular septal defect is a rare disorder. There is little information available regarding the optimal form of treatment. This report describes a case and discusses the most appropriate therapy in this situation. Case Report
A full-term infant was noted to be cyanosed with a cardiac murmur shortly after a normal delivery. Following transfer to our care, the infant was centrally cyanosed with an arterial oxygen tension of 3.2 Kpa in 100% oxygen. There was a prominent apical impulse and, on auscultation, there was a grade 3/6 ejection murmur at the left sternal edge radiating to the carotid arteries. There was a single second sound and the liver edge was palpable 2 cm below the costal margin. The electrocardiogram demonstrated sinus rhythm with voltage evidence of left ventricular hypertrophy. Chest X-ray showed a cardiothoracic ratio of 0.63 with prominence of the left upper mediastinal shadow, and pulmonary oligaemia. A cross-sectional echocardiogram revealed pulmonary atresia with ventricular septal defect. There was conspicuous biventricular hypertrophy, particularly of the interventricular septum. The aortic valve was thickened and stenosed with post-stenotic dilation of the ascending aorta and a right sided aortic arch. The pulmonary trunk and its branches appeared hypoplastic but of adequate size for a shunt operation. Cardiac catheterisation confirmed the echocardiographic findings and a right ventriculogram demonstrated a normally related atretic right ventricular sub-pulmonary outflow tract Correspondence to: Dr. R.P. Martin, Department of Paediatric Cardiology, Harefield Hospital, Harefield, Middlesex UB9 6JH, U.K. International Journal of Cardiology, 16 (1987) 103-105 0 Elsevier Science Publishers B.V. (Biomedical Division)
104
Fig. 1. Right ventriculogram in the left lateral projection demonstrating an anteriorly positioned right ventricular outflow tract (RVOT) with filling of the puimonary trunk (MPA) via an arterial
(Fig. 1). The pulmonary arteries filled via a persistent arterial duct. crossed but the angiographic features suggested significant stenosis pressure of 130 mm Hg with a non simultaneous systemic pressure this. A shunt was inserted between the ascending aorta and the left been planned to perform aortic valvoplasty intra-operatively, but
atretic duct.
The aortic valve was not (Fig. 2). A left ventricular of 80 mm Hg supported pulmonary artery. It had the gradient measured at
Fig. 2. Right ventriculogram. A. Anteroposterior projection showing opacification of the right ventricle (RV) and filling of the aorta (AO) via a large ventricular septal defect. Note the hypertrophy of the interventricular septum (IVS). B. Left lateral projection showing thickened aortic valve leaflets (AV) that dome in systole. There is post-stenotic dilation of the ascending aorta.
105
the time of operation was only 5 mm Hg so aortic valvotomy was deferred. Low output cardiac failure developed six hours after the operation and, despite treatment with positive inotropic agents, the patient died. Post-mortem examination revealed the presence of severe aortic valve stenosis with pulmonary atresia and ventricular septal defect. Discussion The occurrence of abnormalities of the aortic valve in tetralogy of Fallot or pulmonary atresia with ventricular septal defect is uncommon. Haemodynamically significant stenosis has been recorded by Pate1 et al. [l] in two cases of pulmonary afresia with ventricuiar septal defect and in one case of pulmonary atresia with intact interventricular septum. The aortic valve gradients demonstrated were between 20 mm Hg and 28 mm Hg. Systemic to pulmonary arterial shunts alone were performed in two patients and, in the third patient, this was combined with aortic valvotomy. The latter patient died. The other situation where stenosis of a functionally single arterial trunk has been described is in common arterial trunk with truncal valve stenosis. Calder et al. [2] described an incidence of truncal valve stenosis of one-third. It is well recognized that the presence of significant truncal valve stenosis or incompetence is associated with a high mortality rate [1,3-51. The purpose of this communication is to highlight this rare condition and also to discuss appropriate management. The transvalvar gradient measured on the operating table was only 5 mm Hg and, therefore, conservative management of the aortic valve stenosis was employed. it is probable that the intra-operative pressure gradient underestimated the true severity of stenosis as there was evidence of left ventricular hypertrophy and the left ventricular pressure was 130 mm Hg at the time of cardiac catheterisation. Death resulted from ventricular failure. The aortic valve stenosis was a likely contributing factor as post-mortem examination confirmed the severity of the aortic stenosis. We believe that either balloon aortic valvotomy or open valvotomy should be considered in similar cases as the intra-operative pressure gradient measured may be unreliable.
References 1 Pate1 RG, Freedom RM, Bloom KR, Rowe RD. Truncal or aortic valve stenosis in functionally single arterial trunk: a clinical, hemodynamic and pathologic study of six cases. Am J Cardiol 1978;42:800-808. 2 Calder L, Van Praagb R, Van Praagb S, et al. Truncus arteriosus com~~~unis:Clinical, angiographic, and pathologic findings in 100 patients. Am Heart J 1976;92:23-38. 3 Gelband H, Van Meter S, Gersony W. Truncal valve abnormalities in infants with persistent truncus arteriosus: a clinicopatbologic study. Circulation 1972;45:397-403. 4 Lee MH, Bellon EM, Liebman J, Perrin EV. Truncal valve stenosis. Am Heart J 1973;85:398-400. 5 Bumell RH, McEnery G, Miller GAH. Tnmcal valve stenosis. Br Heart J 1971;33:423-424.
IJC 0555D
Pulmonary atresia and aortic valve stenosis R.P. Martin, R. Radley-Smith, M.H. Yacoub Department
of Paediatric Cardiology and Cardiac Surgev,
Harefield Hospital, Harefield
Middlesex,
U.K.
(Received 12 January 1987; revision accepted 30 January 1987)
A case is described of pulmonary atresia with ventricular septal defect and severe aortic valve stenosis. The aortic valve gradient measured at the time of insertion of an aortic to pulmonary arterial shunt was only 5 mm Hg and aortic valvotomy was deferred. Post-operative low output cardiac failure resulted in the death of the patient. The severity of the aortic stenosis was confiied by post-mortem examination. We suggest consideration of aortic valvotomy in similar cases as the intraoperative pressure gradient may be unreliable.
Key words: Aortic valve stenosis; Pulmonary atresia
Introduction
Aortic valve stenosis in association with pulmonary atresia and ventricular septal defect is a rare disorder. There is little information available regarding the optimal form of treatment. This report describes a case and discusses the most appropriate therapy in this situation. Case Report
A full-term infant was noted to be cyanosed with a cardiac murmur shortly after a normal delivery. Following transfer to our care, the infant was centrally cyanosed with an arterial oxygen tension of 3.2 Kpa in 100% oxygen. There was a prominent apical impulse and, on auscultation, there was a grade 3/6 ejection murmur at the left sternal edge radiating to the carotid arteries. There was a single second sound and the liver edge was palpable 2 cm below the costal margin. The electrocardiogram demonstrated sinus rhythm with voltage evidence of left ventricular hypertrophy. Chest X-ray showed a cardiothoracic ratio of 0.63 with prominence of the left upper mediastinal shadow, and pulmonary oligaemia. A cross-sectional echocardiogram revealed pulmonary atresia with ventricular septal defect. There was conspicuous biventricular hypertrophy, particularly of the interventricular septum. The aortic valve was thickened and stenosed with post-stenotic dilation of the ascending aorta and a right sided aortic arch. The pulmonary trunk and its branches appeared hypoplastic but of adequate size for a shunt operation. Cardiac catheterisation confirmed the echocardiographic findings and a right ventriculogram demonstrated a normally related atretic right ventricular sub-pulmonary outflow tract Correspondence to: Dr. R.P. Martin, Department of Paediatric Cardiology, Harefield Hospital, Harefield, Middlesex UB9 6JH, U.K. International Journal of Cardiology, 16 (1987) 103-105 0 Elsevier Science Publishers B.V. (Biomedical Division)
104
Fig. 1. Right ventriculogram in the left lateral projection demonstrating an anteriorly positioned right ventricular outflow tract (RVOT) with filling of the puimonary trunk (MPA) via an arterial
(Fig. 1). The pulmonary arteries filled via a persistent arterial duct. crossed but the angiographic features suggested significant stenosis pressure of 130 mm Hg with a non simultaneous systemic pressure this. A shunt was inserted between the ascending aorta and the left been planned to perform aortic valvoplasty intra-operatively, but
atretic duct.
The aortic valve was not (Fig. 2). A left ventricular of 80 mm Hg supported pulmonary artery. It had the gradient measured at
Fig. 2. Right ventriculogram. A. Anteroposterior projection showing opacification of the right ventricle (RV) and filling of the aorta (AO) via a large ventricular septal defect. Note the hypertrophy of the interventricular septum (IVS). B. Left lateral projection showing thickened aortic valve leaflets (AV) that dome in systole. There is post-stenotic dilation of the ascending aorta.
105
the time of operation was only 5 mm Hg so aortic valvotomy was deferred. Low output cardiac failure developed six hours after the operation and, despite treatment with positive inotropic agents, the patient died. Post-mortem examination revealed the presence of severe aortic valve stenosis with pulmonary atresia and ventricular septal defect. Discussion The occurrence of abnormalities of the aortic valve in tetralogy of Fallot or pulmonary atresia with ventricular septal defect is uncommon. Haemodynamically significant stenosis has been recorded by Pate1 et al. [l] in two cases of pulmonary afresia with ventricuiar septal defect and in one case of pulmonary atresia with intact interventricular septum. The aortic valve gradients demonstrated were between 20 mm Hg and 28 mm Hg. Systemic to pulmonary arterial shunts alone were performed in two patients and, in the third patient, this was combined with aortic valvotomy. The latter patient died. The other situation where stenosis of a functionally single arterial trunk has been described is in common arterial trunk with truncal valve stenosis. Calder et al. [2] described an incidence of truncal valve stenosis of one-third. It is well recognized that the presence of significant truncal valve stenosis or incompetence is associated with a high mortality rate [1,3-51. The purpose of this communication is to highlight this rare condition and also to discuss appropriate management. The transvalvar gradient measured on the operating table was only 5 mm Hg and, therefore, conservative management of the aortic valve stenosis was employed. it is probable that the intra-operative pressure gradient underestimated the true severity of stenosis as there was evidence of left ventricular hypertrophy and the left ventricular pressure was 130 mm Hg at the time of cardiac catheterisation. Death resulted from ventricular failure. The aortic valve stenosis was a likely contributing factor as post-mortem examination confirmed the severity of the aortic stenosis. We believe that either balloon aortic valvotomy or open valvotomy should be considered in similar cases as the intra-operative pressure gradient measured may be unreliable.
References 1 Pate1 RG, Freedom RM, Bloom KR, Rowe RD. Truncal or aortic valve stenosis in functionally single arterial trunk: a clinical, hemodynamic and pathologic study of six cases. Am J Cardiol 1978;42:800-808. 2 Calder L, Van Praagb R, Van Praagb S, et al. Truncus arteriosus com~~~unis:Clinical, angiographic, and pathologic findings in 100 patients. Am Heart J 1976;92:23-38. 3 Gelband H, Van Meter S, Gersony W. Truncal valve abnormalities in infants with persistent truncus arteriosus: a clinicopatbologic study. Circulation 1972;45:397-403. 4 Lee MH, Bellon EM, Liebman J, Perrin EV. Truncal valve stenosis. Am Heart J 1973;85:398-400. 5 Bumell RH, McEnery G, Miller GAH. Tnmcal valve stenosis. Br Heart J 1971;33:423-424.