NYMC Faculty Publications

Additional Author Affiliation

New York Medical College

Author ORCID Identifier

http://orcid.org/0000-0001-7916-6625

Document Type

Article

Publication Date

6-1-2015

Department

Public Health

Abstract

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.

Comments

At the time of publication, Dr. Kim was affiliated with New England Research Institutes in Watertown, MA.

Publisher's Statement

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.

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