ABSTRACT
Extracardiac congenital anomalies have long been recognised to be associated with congenital heart disease. Advances in molecular biology, genetic testing and epidemiologic studies have enhanced our understanding of the various associated genetic syndromes. A number of distinguishing long-term sequelae of treated and untreated congenital heart disease have been described. Bronchiolitis obliterans is the manifestation of chronic rejection following lung transplantation. Duodenal atresia may be associated with Down's syndrome, and as a result with ventricular septal defects or atrioventricular canal defects, although most often there is no associated congenital heart disease lesion. Pulmonary vascular occlusive disease is end-stage lung disease resulting from chronic pulmonary hypertension from unrepaired congenital heart disease. Double aortic arch is usually without other associated congenital heart disease lesions and has a left and a right aortic arch. Symptoms may be respiratory or oesophageal. Ventricular septal defects are common associations with other congenital heart lesions.
