Aplasia de las válvulas pulmonares

Abstract

The clinical, phonocardiographic, hemodynamic and angiocaidiographic findings in 8 children with aplastic pulmonary valve are presented. All had a ventricular septal defect and in 6 there was aiso an infundibular pulmonic stenosis. Clinically, the most striking feature was late, low frecuency diastolic murmur of crecendo decrescendo quality and maximal intensity in the second and third left intercostal space, asociated with single, normal intensity second sound and, radiologically, abnormal parahiliar shadows, specially on the right side, or aneurysmatic dilatation of the pulmonary artery trunk. The angiocardiographic work-up usually showed aneurysmatic dilatation of the pulmonary artery trunk or his main branches and sudden peripheric reduction of their lumen. These findings are not seen in any other heart disease. The ethiology and results of surgical treatment of this abnormality are analized.