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It has been largely acknowledged that pulmonary vascular disease (PVD) and congenital heart disease (CHD) are closely related. This link has been established for more than a century now, and is based on careful observations and hard scienti ic work of many outstanding investigators. Much of the current knowledge on the pathobiology of pulmonary hypertension (the clinical and hemodynamic expression of PVD) is based on detailed analysis of lung biopsy specimens collected over decades during surgical repair of congenital cardiac anomalies (or lung tissue obtained at autopsy).[1-4] From isolated medial hypertrophy of pulmonary arteries to complex dilated lesions, necrotizing arteritis, and pulmonary capillary hemangiomatosis, all types of vascular abnormalities known to be present in pulmonary arterial hypertension in general (PAH) and idiopathic pulmonary arterial hypertension (IPAH) have been extensively described in PAH-CHD.

Is Surgical Treatment the Cure for Patients with Congenital Heart Disease?