[Hypertensive pulmonary vascular disease in complete transposition of the great arteries (author's transl)].

Pulmonary vascular disease and heart failure are the two major problems in complete transposition of the great arteries (TGA) with increased pulmonary flow. Hypertensive pulmonary vascular disease was observed in patients with complete TGA and increased flow (3.6%) as well as in those without increased pulmonary flow (3.3%). An intact ventricular septum or pulmonic stenosis did not appear to prevent the occurrence of progressive pulmonary vascular disease in all patients. The pulmonary lesion observed in these subjects cannot be explained by chronic severe volume overload alone. Clinically unrecognized pulmonary microthrombi are an additional cause for the development of pulmonary vascular disease in patients with complete TGA. Therefore cyanosis and its complications can be a major factor beside increased pulmonary flow in causing pulmonary vascular changes. Early corrective surgery performed after the age of 3 months is the therapy of choice to avoid progressive pulmonary vascular damage and other thrombo-embolic accidents.