01 January 2015 by John Ryan, Thenappan Thenappan, Stuart Rich, Stephen Archer

Category 1 PH due to a second case of pulmonary capillary hemangiomatosis

Ryan JJ,Thenappan T,Luo N,Ha T,Patel AR,Rich S,Archer SL.The WHO classification of pulmonary hypertension: A case-based imaging compendium.Pulmonary circulation. 2012 ;2:107-21.

Category 1 PH due to a second case of pulmonary capillary hemangiomatosis. (A) ECG showing sinus tachycardia and right atrial enlargement. (B) Chest X-ray on presentation with cardiomegaly and clear lung fields. (C) Chest X-ray after initiation of sildenafil showing pulmonary edema. (D) Ventilation-perfusion scan showing accentuation of basilar perfusion images. (E) Gross dissection of the heart shows thickened and dilated RV. (F) Patchy areas of pulmonary capillary proliferations engorged with red blood. (G) Concentric intimal fibrosis and medial hypertrophy of pulmonary arteries and arterioles.


Additional keywords: hemangiomatosis,electrocardiogram,X-ray,histology

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