Lung neovascularity in pulmonary arterial hypertension associated with congenital heart defects and idiopathic pulmonary arterial hypertension: study of 198 patients

• DOI: 10.1007/s00330-011-2347-4
• Publication Date: 2011-12-28T20:44:30Z
• Authors: Cecilia Modolon; Domenico Attinà; Francesco Buia; Fiorella De Luca; Patrizia Fughelli; Maria Letizia Bac...; Massimiliano Pala...; Alessandra Manes; Enri Leci; Nazzareno Galiè; Maurizio Zompatori
• Keywords: Heart Defects, Congenital; Male; Adolescent; Neovascularization, Pathologic; Hypertension, Pulmonary; Angiography; Reproducibility of Results; Comorbidity; Middle Aged; CONGENITAL HEART DISEASE.; Eisenmenger Syndrome; Idiopathic pulmonary arterial hypertension; Risk Assessment; Sensitivity and Specificity; 3. Good health; Young Adult; 03 medical and health sciences; 0302 clinical medicine; Italy; Risk Factors; Prevalence; Humans; Female; Tomography, X-Ray Computed; Aged

To correlate the severity of lung neovascularity (Sheehan vessels) with the cause and haemodynamic severity of pulmonary arterial hypertension (PAH), pulmonary artery (PA) size and heart disease type in patients with PH associated with congenital heart diseases (PAH-CHD) and idiopathic PH (IPAH).We reviewed the HRCT and CT pulmonary angiography studies of 87 patients with PAH-CHD and 111 with IPAH; all had undergone right heart catheterisation. We evaluated the PA size and severity of neovascularity on CT.Neovascularity, which was found in 72% of PAH-CHD (56% with Eisenmenger's syndrome) and in 22% of IPAH patients, is significantly related to the severity of PH and all patients with severe neovascularity had intermediate or high PH. All PAH-CHD patients had a dilated PA with a greater risk of developing severe dilatation (diameter >5 cm). The neovascularity correlated with the PA size only in IPAH.Neovascularity even if not pathognomonic for PAH-CHD, is significantly more common in these patients, especially in Eisenmenger's syndrome. It is often the first CT sign to indicate the severity of PH in PAH-CHD and IPAH. A neovascularity ≥5 on CT indicates a intermediate or high PH.• Large retrospective studying specific lung disorders in patients with pulmonary arterial hypertension. • Neovascularity is often the first CT sign indicating the severity of pulmonary hypertension Alterations of lung parenchyma on CT in pulmonary hypertension are described • The first study to assess the severity of pulmonary hypertension by CT • If substantiated, CT might eventually replace some cardiac catheterisation for evaluating PH.