Risk Factors of Pulmonary Hypertension in Brazilian Patients with Sickle Cell Anemia.

Authors

Clarisse Lopes de Castro Lobo, Clinical Hematology Division, Instituto de Hematologia Arthur de Siqueira Cavalcanti-HEMORIO, Rio de Janeiro, RJ, Brazil
Emilia Matos do Nascimento, Clinical Hematology Division, Instituto de Hematologia Arthur de Siqueira Cavalcanti-HEMORIO, Rio de Janeiro, RJ, Brazil; UEZO-Centro Universitário Estadual da Zona Oeste, Rio de Janeiro, RJ, Brazil
Renato Abelha, Clinical Hematology Division, Instituto de Hematologia Arthur de Siqueira Cavalcanti-HEMORIO, Rio de Janeiro, RJ, Brazil
Ana Maria Mach Queiroz, Clinical Hematology Division, Instituto de Hematologia Arthur de Siqueira Cavalcanti-HEMORIO, Rio de Janeiro, RJ, Brazil
Philippe Connes, Laboratory CRIS-EA 647, Section 'Vascular Biology and Red Blood Cell', University Claude Bernard Lyon 1, Villeurbanne, FranceFollow
Gilberto Perez Cardoso, Universidade Federal Fluminense (UFF), Niteroi, RJ, Brazil
Samir K. Ballas, Clinical Hematology Division, Instituto de Hematologia Arthur de Siqueira Cavalcanti-HEMORIO, Rio de Janeiro, RJ, Brazil; Cardeza Foundation, Department of Medicine, Jefferson Medical College, Thomas Jefferson University, Philadelphia, PA, United StatesFollow

Document Type

Article

Publication Date

9-3-2015

Comments

This article has been peer reviewed. It was published in: PLoS ONE.

Volume 10, Issue 9, 3 September 2015, Article number e0137539.

The published version is available at DOI: 10.1371/journal.pone.0137539

Copyright © 2015 Lobo et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited

Abstract

This study was a prospective cross-sectional cohort study of 125 patients with sickle cell anemia (SS) between the ages of 16 to 60 years. Enrolled patients were followed-up prospectively for 15 months. Demographic, clinical, hematological and routine biochemical data were obtained on all patients. Six-minute walk test and Doppler Echocardiography were performed on all patients. A tricuspid regurgitant jet velocity (TRJV) < 2.5 m/sec was considered normal, 2.5 ≤ TRJV ≤ 3.0 was considered mild-moderate and > 3.0 m/sec, severe. Patients with abnormal TRJV were significantly older and more anemic, had significantly higher lactate dehydrogenase (LDH) levels, reticulocyte count and incidence of death. The logistic multimodal model implemented for the 125 patients indicated that age was the covariate that influenced the outcome of normal or abnormal TRJV with a cutoff age of thirty-two years. The survival rate for the group of patients with creatinine (Cr) > 1.0 mg/dL was lower than the group with Cr ≤ 1 and normal TRJV. A coefficient matrix showed that the LDH values were weakly correlated with the reticulocyte count but strongly correlated with hemoglobin suggesting that the TRJV values were not correlated with the hemolytic rate but with anemia. Ten patients died during the follow-up of whom 7 had TRJV > 2.5 m/sec. Acute chest syndrome was the most common cause of death followed by sepsis. In conclusion, this study shows that patients with SS older than thirty-two years with high LDH, elevated TRJV, severe anemia and Cr > 1 have poor prognosis and may be at risk of having pulmonary hypertension and should undergo RHC.

Recommended Citation

Lobo, Clarisse Lopes de Castro; do Nascimento, Emilia Matos; Abelha, Renato; Queiroz, Ana Maria Mach; Connes, Philippe; Cardoso, Gilberto Perez; and Ballas, Samir K., "Risk Factors of Pulmonary Hypertension in Brazilian Patients with Sickle Cell Anemia." (2015). Cardeza Foundation for Hematologic Research. Paper 29.
https://jdc.jefferson.edu/cardeza_foundation/29