Magnification pulmonary wedge angiography in the evaluation of children with congenital heart disease and pulmonary hypertension.

In order to determine the presence and extent of obstructive pulmonary vascular disease in patients with congenital heart disease, magnified cineangiograms were obtained with a catheter in the pulmonary artery wedge position in 155 infants and children undergoing cardiac catheterization. The wedge angiograms (WA) were analyzed in groups according to the pulmonary hemodynamics: group A normal pulmonary blood flow (PBF) and pulmonary artery pressure (PAP) (31 patients; 53 WA); group B increased PBF and normal PAP (46 patients; 69 WA); group C: increased PBF and PAP but a pulmonary vascular resistance (PVR) < 6 u/m2 (19 patients; 33 WA); group D increased PAP and PVR > 6 u/M2 (30 patients; 66 WA); group E pulmonary venous hypertension (15 patients; 27 WA); and group F: infants < 3 months old with a variety of congenital heart defects (14 patients; 19 WA). WA from group A patients defined the microcirculation of the secondary pulmonary lobule with an evenly tapering, orderly arborization of muscular pulmonary arteries and numerous supernumerary vessels as small as 100 ,u in diameter with a full, even granular capillary blush surrounding each small, muscular artery. Increased PBF produced dilation of the elastic and muscular vessels with engorgement of the lobule, while increased PAP produced tortuosity of the vessels. An elevated PVR was manifest by dilation and tortuosity of the elastic vessels and a more abrupt tapering of the muscular arteries. Patients with a PVR > 12 u/M2 and those with obstructive vascular disease by histological examination (eight patients) showed marked reduction