Pulmonary Agenesis with Dextrocardia and Hypertrophic Cardiomyopathy: First Case Report

Pulmonary agenesis is a rare condition with complete absence of bronchus, lung tissue and vessels. A variety of cardiovascular defects are present in upto 1/3 rd cases of pulmonary agenesis. However, a combination of dextrocardia and hypertrophic cardiomyopathy in association with pulmonary agenesis is not known. Here we report the first case of a neonate presenting with respiratory distress since birth, diagnosed to have hypertrophic cardiomyopathy in association with dextrocardia, multiple cardiac defects and right lung agenesis. Association of heart disease with lung agenesis adversely affects the course and outcome making them a highly lethal association

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