Ann Thorac Surg 1998;66:935-936
© 1998 The Society of Thoracic Surgeons
Case Reports
Absent aortic valve: successful palliation in the neonate
Yorikazu Harada, MDa,
Takamasa Takeuchi, MDa,
Gengi Satomi, MDa,
Satoshi Yasukouchi, MDa
a Department of Cardiovascular Surgery and Pediatric Cardiology, Nagano Children’s Hospital, Nagano, Japan
Accepted for publication March 23, 1998.
Address reprint requests to Dr Harada, Department of Cardiovascular Surgery, Nagano Children’s Hospital, 3100 Toyoshina, Minami-Azumi, Nagano 399-82, Japan
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Abstract
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A successful palliation of a neonate with absent aortic valve in hypoplastic left heart syndrome is reported.
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Introduction
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Although aortic valve atresia in hypoplastic left heart syndrome is well recognized and can be successfully repaired, absent aortic valve in hypoplastic left heart syndrome is rare. This report describes a successful palliation of a neonate with absent aortic valve in hypoplastic left heart syndrome.
A 2.9-kg male infant, born after an uneventful full-term pregnancy, was admitted from his local hospital to Nagano Children’s Hospital on the second day of life because of mild cyanosis and respiratory distress. Prostaglandin E1 infusion was already started at the local hospital to maintain ductal patency. On admission, an echocardiogram showed an atretic mitral valve with an absent aortic valve (Fig 1). The left ventricle had a small cavity with thickened walls. No coarctation of the aorta could be identified. Doppler examination demonstrated both antegrade and retrograde flow at the root of the aorta. The duct was widely patent with right to left shunt. Cardiac catheterization at 8 days of age revealed a left ventricular pressure of 62/30 mm Hg; the wave form was identical to that of the ascending aorta. There was a hypoplastic left ventricle with markedly diminished compliance and severe aortic regurgitation (Fig 2).
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Fig 1. Parasternal long-axis view demonstrating the left ventricle (LV) and aortic root. No aortic valve is seen in the aortic annulus. (Ao = ascending aorta; LA = left atrium.)
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Fig 2. Aortogram in the posteroanterior view shows severe aortic regurgitation and reduced size of the left ventricle (LV). (AO = ascending aorta.)
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Operation was performed with the aid of cardiopulmonary bypass when the patient was 9 days of age. A 4-mm Gore Tex graft (W.L. Gore & Assoc, Naperville, IL) was anastomosed to the right subclavian artery and used for aortic root perfusion. Both cavae were cannulated for venous drainage. The duct was divided. The right atrium was entered through a vertical incision and the primum septum was resected. The pulmonary artery was transected just proximal to the bifurcation and the distal end of the stump was closed with xenopericardium. With the patient at a rectal temperature of 20°C, vascular clamps were applied on the bracheocephalic artery and the aortic arch between the bracheocephalic artery and the left carotid artery. Brain perfusion was maintained through the graft on the right subclavian artery at a flow rate of 15 mL/min, although the rest of the body was on circulatory arrest. The ascending aorta was incised along the minor curvature after administration of one dose of cold crystalloid cardioplegia. The proximal stump of the pulmonary artery was anastomosed to the ascending aorta in an end-to-side fashion. The aortic annulus was observed through the anastomosis site on the aorta. The aortic valve was completely absent, with a tiny fibrous ridge that formed a mild narrowing at the aortic annulus. The left ventricular cavity was small and the endocardial surface had an appearance of endocardial fibroelastosis. We did not close the aortic annulus because we believed the coronary circulation would be maintained owing to the markedly diminished compliance of the small left ventricle. After completion of the anastomosis, the proximal portion of the pulmonary artery was cannulated and perfused instead of the Gore-Tex graft and the vascular clamps were removed. Then, the patient was fully rewarmed; during rewarming the other end of the graft was anastomosed to the central pulmonary artery near the ductal insertion. Bypass was discontinued successfully.
The patient remained hemodynamically stable on mild inotropic support and nitrates. The chest was splinted to avoid compression of the heart, and was uneventfully closed 7 days later. The patient was extubated after 13 days and discharged in good condition. At the age of 6 months the patient underwent a bidirectional Glenn operation successfully and is now waiting for a Fontan operation.
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Comment
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Absence of the aortic valve is a rare congenital cardiac malformation. Eleven cases have been described in literature so far [1–8]. Toews and associates [1] reported the first case of this anomaly associated with double outlet of the right ventricle. Absent aortic valve in hypoplastic left heart syndrome also was reported by several authors [4, 6, 7], who did not perform surgical intervention.
Prenatal echocardiography in this defect was described by Bierman and colleagues [2]. Because rapid hemodynamic deterioration shortly after birth may occur, prenatal diagnosis would facilitate neonatal critical care. Parikh and colleagues [6] suggested in their report that cardiac transplantation should be considered in the neonatal period. Sudden and rapid deterioration may occur in this type of anomaly because of a severe regurgitation across the aortic annulus, which results in a coronary steal syndrome. In our case, however, the baby survived the palliative operation because "markedly diminished compliance" of the left ventricle existed, which made it possible to maintain coronary perfusion. Otherwise, the baby would have died of coronary steal soon after birth. Although the patient is now doing well as a 2-year-old boy without digoxin or diuretics after a successful bidirectional Glenn operation, it remains to be seen how long the stiffness of the left ventricle will continue.
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References
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- Toews W.H., Lortscher R.H., Kelmison L.L. Double outlet right ventricle with absent aortic valve. Chest 1975;68:381-382.[Abstract/Free Full Text]
- Bierman F.Z., Yeh M.N., Swersky S., Martin E., Wigger J.H., Fox H. Absence of the aortic valve: antenatal and postnatal two-dimensional and Doppler echocardiographic features. J Am Coll Cardiol 1984;3:833-837.[Abstract]
- Niwa K., Ikeda F., Miyamoto H., Nakajima H., Ando M. Absent aortic valve with normally related great arteries. Heart Vessels 1987;3:104-107.[Medline]
- Rossi M.B., Ho S.Y., Tasker R.C. Absent aortic valve leaflets. Int J Cardiol 1986;11:235-237.[Medline]
- Cabrera A., Galdeano J.M., Pastor E. Absence of the aortic valve cusps with mitiral atresia, normal left ventricle, and intact ventricular septum. Br Heart J 1990;63:187-188.[Abstract/Free Full Text]
- Parikh S.R., Hurwitz R.A., Caldwell R.L., Waller B. Absent aortic valve in hypoplastic left heart syndrome. Am Heart J 1990;119:977-978.[Medline]
- Lin A.E., Chin A.J. Absent aortic valve: a complex anomaly. Pediatr Cardiol 1990;11:195-198.[Medline]
- Nakamura S., Hatano T. A case of absent aortic valve with severe stenosis. Acta Cardiol Paediatr Jpn 1996;12:56-62.
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Abstract
Introduction
