Combined pulmonary and aortic valvar stenosis is an extremely rare congenital anomaly. Fetuses and neonates with critical pulmonary valve stenosis or atresia and intact ventricular septum may present with varying degrees of abnormalities of the right ventricle, tricuspid valve, and coronary arteries, but combination of right and left ventricular outflow tract obstruction is hardly ever seen. Until bilateral balloon valvuloplasty had been established in the neonate, the only therapeutic option was the surgical relief of right and left ventricular obstruction. We highlight the essentials of perinatal management in cases with severe bilateral valvar obstruction and provide a guideline for sequential interventional therapy of this rare lesion.

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