Additional Author Affiliation
New York Medical College
Author ORCID Identifier
Pulmonary hypertension (PH) commonly develops in thalassaemia syndromes, but is poorly characterized. The goal of this study was to provide a comprehensive description of the cardiopulmonary and biological profile of patients with thalassaemia at risk for PH. A case-control study of thalassaemia patients at high versus low PH-risk was performed. A single cross-sectional measurement for variables reflecting cardiopulmonary status and biological pathophysiology were obtained, including Doppler-echocardiography, 6-min-walk-test, Borg Dyspnoea Score, New York Heart Association functional class, cardiac magnetic resonance imaging (MRI), chest-computerized tomography, pulmonary function testing and laboratory analyses targeting mechanisms of coagulation, inflammation, haemolysis, adhesion and the arginine-nitric oxide pathway. Twenty-seven thalassaemia patients were evaluated, 14 with an elevated tricuspid-regurgitant-jet-velocity (TRV) ≥ 2·5 m/s. Patients with increased TRV had a higher frequency of splenectomy, and significantly larger right atrial size, left atrial volume and left septal-wall thickness on echocardiography and/or MRI, with elevated biomarkers of abnormal coagulation, lactate dehydrogenase (LDH) levels and arginase concentration, and lower arginine-bioavailability compared to low-risk patients. Arginase concentration correlated significantly to several echocardiography/MRI parameters of cardiovascular function in addition to global-arginine-bioavailability and biomarkers of haemolytic rate, including LDH, haemoglobin and bilirubin. Thalassaemia patients with a TRV ≥ 2·5 m/s have additional echocardiography and cardiac-MRI parameters suggestive of right and left-sided cardiac dysfunction. In addition, low arginine bioavailability may contribute to cardiopulmonary dysfunction in β-thalassaemia.
Morris, C., Kim, H., Klings, E., Wood, J., Porter, J., Trachtenberg, F., Sweeters, N., Olivieri, N., Kwiatkowski, J., Virzi, L., Hassell, K., Taher, A., Neufeld, E., Thompson, A., Larkin, S., Suh, J., Vichinsky, E., & Kuypers, F. (2015). Dysregulated Arginine Metabolism and Cardiopulmonary Dysfunction in Patients with Thalassaemia. British Journal of Haematology, 169 (6), 887-898. https://doi.org/10.1111/bjh.13452
This is the peer reviewed version of the following article: Morris, C. R., Kim, H. Y., Klings, E. S., Wood, J., Porter, J. B., Trachtenberg, F., … Thalassemia Clinical Research Network (2015). Dysregulated arginine metabolism and cardiopulmonary dysfunction in patients with thalassaemia. British journal of haematology, 169(6), 887–898. doi:10.1111/bjh.13452, which has been published in final form at https://doi.org/10.1111/bjh.13452. This article may be used for non-commercial purposes in accordance with Wiley Terms and Conditions for Use of Self-Archived Versions.