[Pulmonary valve aplasia with ventricular septal defect. Hemodynamics and embryology]

The combination of absent pulmonary valve with ventricular septal defect, subvalvular pulmonic stenosis and aneurysm of the pulmonary artery presents a characteristic heamodynamic and clinical profile. It should be differentiated from other morphologically related cardiovascular malformations. According to the degree of right ventricular outflow tract obstruction, the circulatory pattern and the clinical picture is varying widely. We are differentiating an obstructive and a congestive form of the disease. An own case is demonstrated. After a short review of the literature the clinical and haemodynamic findings are discussed. The important role of the ductus arteriosus in fetal circulation is emphasized. The development of a huge pulmonary aneurysm and the premature closing of the ductus arteriosus are considered to be secondary to the serious disturbance of fetal haemodynamics.