Pulmonary wedge angiography for prediction of pulmonary vascular disease in Down syndrome.

We performed high resolution pulmonary wedge angiography (PWA) and conventional hemodynamics to predict the reversibility of structural pulmonary vascular disease. Sixty-one pulmonary wedge angiograms were performed on 41 patients with intracardiac shunts and Down syndrome (median age 8 months). Balloon occlusion wedge angiograms were analyzed for (1) monopedial branches from the distal 10 mm of muscular arteries, (2) capillary blush, (3) tapering indices, and (4) tortuosity. Twenty-five patients had open lung biopsy, graded by the Health Edwards classification, and analyzed morphometrically. Pulmonary vascular resistance of > or = 6 units was 100% sensitive and 94% specific for Heath Edwards Grade III-IV. A monopedial count < 3 vessels was 83% sensitive and 100% specific for Heath Edwards Grade III-IV. Abnormal capillary blush was 83% sensitive and 69% specific for Heath Edwards Grade III-IV. Tapering indices and tortuosity showed no significant correlation with lung biopsy. A combination of pulmonary vascular resistance < 6 units, monopedial count > or = 3, and normal capillary blush was 100% sensitive and specific for Heath Edwards Grade 0-II, and a combination of pulmonary vascular resistance > or = 6 units, monopedial count < 3, and abnormal capillary blush was 100% sensitive and specific for Heath Edwards Grade III-IV. Using the 3 criteria, Heath Edwards Grade was accurately predicted in 17 patients. In 4 patients, only 2 criteria were available. Morphometric analysis showed an inverse relationship between the lowest monopedial count and the number of occlusive vessels per cm of tissue, r = -0.74 p < 0.001. Arteries showing intimal and/or medial thickening causing > 90% luminal narrowing were scored as "occlusive." These results show that when the hemodynamic and pulmonary wedge angiography data are concordant, the structural changes of pulmonary vascular disease can be accurately predicted and lung biopsy may be avoided.