Patients with sickle cell disease (SCD) and pulmonary hypertension (PH) have increased mortality. SCD-PH is often complicated by high cardiac output (CO) related to anemia. The transpulmonary gradient (TPG) reflects a pressure differential across the pulmonary vascular bed without the confounding effect of CO (PVR=TPG/CO). Based on the cardiac transplant literature, a TPG ≥ 12 mmHg indicates significant pulmonary arterial hypertension (PAH). With PH, there is often morphologic adaptation by the right ventricle (RV). In idiopathic PAH, RV dilation and decreased function have been correlated with poor prognosis. We hypothesize that patients with SCD and a TPG ≥ 12 mmHg would have lower functional capacity, increased mortality, and evidence of RV dysfunction on cardiac MRI (CMR).

Document Type


Publication Date


Notes/Citation Information

Published in Journal of Cardiovascular Magnetic Resonance, v. 15 (Suppl. 1): O43.

© 2013 Nguyen et al; licensee BioMed Central Ltd.

From 16th Annual SCMR Scientific Sessions (San Francisco, CA, USA: 31 January - 3 February 2013).

Digital Object Identifier (DOI)