Background: We sought to characterize the distinctive pulmonary vascular abnormalities seen on chest radiographs and computed tomography (CT) scans in Eisenmenger syndrome.
Methods and results: Thoracic CT scans, chest radiographs, and clinical data were reviewed for 24 Eisenmenger syndrome patients subdivided into those with interatrial (pretricuspid) versus interventricular or great arterial (posttricuspid) communications and in 14 acyanotic patients with pulmonary arterial hypertension (PAH) and no congenital heart disease. CT scans were scored blindly by 2 thoracic radiologists for the presence and severity of small, tortuous, intrapulmonary vessels, termed "neovascularity," lobular ground-glass opacification, and systemic perihilar and intercostal vessels. Histopathologic lung sections from 5 patients with Eisenmenger syndrome and from 3 patients with acyanotic PAH were reviewed. Associations between clinical and imaging features were tested by ANOVA and chi2 tests. Kendall's rank-order coefficient and the Kruskal-Wallis test were used to test for significant differences in imaging features between Eisenmenger syndrome and acyanotic PAH. Neovascularity on chest radiographs was more common in Eisenmenger syndrome than acyanotic PAH, but differences were not significant. On CT, neovascularity, lobular ground-glass opacification, and hilar and intercostal systemic collaterals were more prevalent in Eisenmenger syndrome, with severity greater in posttricuspid communications. Three previously undescribed vascular lesions were identified histologically in Eisenmenger syndrome: malformed, dilated, muscular arteries within alveolar septa; congested capillaries within alveolar walls; and congested capillaries within the walls of medium-size, muscular pulmonary arteries. These lesions may correspond to the distinctive vascular abnormalities observed on chest radiographs and CT scans.
Conclusions: Distinctive vascular lesions on chest radiographs and CT scans in Eisenmenger syndrome appear to be correlated histologically with collateral vessels that develop more extensively with posttricuspid communications.