Hypoplastic intrapulmonary vascular system with multiple peripheral stenosis of the pulmonary artery

A case history is reported of a nine-years-old girl with a hypoplastic intrapulmonary vascular system and with multiple peripheral stenoses of the pulmonary artery. The clinical, laboratory, and X-ray symptoms of peripheral stenoses of the pulmonary artery are described. The haemodynamic effects of peripheral stenoses of the pulmonary artery on the pressure tracing recorded from the pulmonary artery trunk are analyzed. For an exact diagnosis, angiocardiographic examination is the best informative method.