Proximal pulmonary arterial and intrapulmonary radiologic features of Eisenmenger syndrome and primary pulmonary hypertension.

Extrapulmonary and intrapulmonary radiologic features of Eisenmenger syndrome and primary pulmonary hypertension (PPH) and the clinical expressions that coincide with the radiologic features of these 2 forms of pulmonary hypertension have not previously been characterized. Computed tomography (CT) and pulmonary CT angiography (CTA) utilized GE High Speed Advantage or Siemens Evolution electron beam tomographic (EBT) scanners in 31 patients with cyanotic pulmonary vascular disease (PVD) (group A: 12 men and 19 women; mean age 38 +/- 4 years) and in 13 women with PPH-acyanotic PVD (group B: mean age 30 +/- 3 years). Ten group A and 3 group B patients underwent imaging twice, yielding 57 studies. Group A patients' extrapulmonary radiologic features included proximal pulmonary arteries that were consistently enlarged, and were aneurysmal in 13%, causing bronchial compression and atelectasis. Thromboses were uniformly present and mild in 71% and moderate to massive in 29% of patients. Massive proximal thromboses caused asphyxic death and augmented right-to-left shunts by increasing flow resistance. Mild to extensive mural calcific deposits occurred in 26% of patients. Intrapulmonary radiologic features included intrapulmonary embolic infarction, intrapulmonary hemorrhage, bronchial/systemic arterial collaterals, and neovascularity. Group B patients' extrapulmonary radiologic features included proximal pulmonary arteries that were consistently enlarged but not aneurysmal and devoid of thromboses. Mild to extensive mural calcific deposits occurred in 23% of these patients. Intrapulmonary radiologic features were confined to mosaic attenuation and bronchial/systemic collaterals.