Zeitschrift für Neonatalbiologie

Abstrakt

Pulmonary agenesis with dextrocardia and hypertrophic cardiomyopathy: First case report

Sheetal Agarwal, Arti Maria, Dinesh Yadav and Narendra Bagri

Pulmonary agenesis is a rare condition in which bronchi, lung tissue and vessels are completely absent. Various cardiovascular defects are present in up to one-third of cases of pulmonary agenesis. However, a combination of dextrocardia and hypertrophic cardiomyopathy associated with pulmonary agenesis is not known. Here we report the first case of a newborn infant suffering from respiratory distress since birth who was diagnosed with hypertrophic cardiomyopathy associated with dextrocardia, multiple heart defects and right pulmonary agenesis. The association of cardiac disease with pulmonary agenesis negatively affects the course and outcome and is therefore a highly lethal association.