Dextrocardia with pulmonary hypoplasia: an unusual cause of unilateral lung white-out

Abstract

Pulmonary agenesis is a rare developmental disorder with many syndromic associations. Type III agenesis presents with variable amounts of bronchial tree, pulmonary parenchyma and supporting vasculature. It is also reported to be associated with dextrocardia due to shifting of the heart to occupy the space left by the underdeveloped lung, which occurs as early as the fourth week of gestation. Right lung agenesis is considered rarer than its left-sided counterpart and also has a poorer prognosis due to increased chances of respiratory infections. We report a case of isolated right-sided lung hypoplasia with dextrocardia without situs inversus or heterotaxy.

Keywords: genetics; respiratory medicine.