We report a case of pulmonary atresia with intact ventricular septum and aortic valve stenosis in a neonate. This combination is extremely rare. Cardiac catheterization was performed at 2 days of age, which confirmed the prenatal echocardiographic diagnosis and identified associated moderate aortic valve stenosis. Soon after the examination, an urgent surgical intervention was required due to uncontrollable circulatory instability. The baby underwent a successful Fontan-type repair achieved by surgical management only.
Peraira MoralJRBurguens ValeroMGarcia-Guereta SilvaL. Pulmonary valve atresia with intact ventricular septum and severe aortic stenosis. Pediatr Cardiol2005; 26: 117–118.
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KobayashiTMomoiNFukudaYSuzukiH. Percutaneous balloon valvuloplasty of both pulmonary and aortic valves in a neonate with pulmonary atresia and critical aortic stenosis. Pediatr Cardiol2005; 26: 839–842.
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HerbergUGoltzDWeissHGembruchUBreuerJ. Combined pulmonary and aortic valve stenosis—prenatal diagnosis and postnatal intervention therapy. Neonatology2009; 96: 244–247.
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da CruzEBillieuxMHBeghettiM. A neonate with isolated combined aortic and pulmonary valvar stenosis. Int J Cardiol2007; 116: e13–e14.
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YoshimuraNYamaguchiMOshimaY. Pulmonary atresia with intact ventricular septum: strategy based on right ventricular morphology. J Thorac Cardiovasc Surg2003; 126: 1417–1426.
