Risk Factors of Pulmonary Hypertension in Brazilian Patients with Sickle Cell Anemia.
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.
Clinical Hematology Division, Instituto de Hematologia Arthur de Siqueira Cavalcanti-HEMORIO, Rio de Janeiro, RJ, Brazil.
Clarisse Lopes de Castro
CL
Lobo
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.
Emilia Matos
EM
do Nascimento
Clinical Hematology Division, Instituto de Hematologia Arthur de Siqueira Cavalcanti-HEMORIO, Rio de Janeiro, RJ, Brazil.
Renato
R
Abelha
Clinical Hematology Division, Instituto de Hematologia Arthur de Siqueira Cavalcanti-HEMORIO, Rio de Janeiro, RJ, Brazil.
Ana Maria Mach
AM
Queiroz
Laboratory CRIS-EA 647-Section "Vascular Biology and Red Blood Cell," University Claude Bernard Lyon 1, Villeurbanne, France.
Philippe
P
Connes
Universidade Federal Fluminense (UFF), Niteroi, RJ, Brazil.
Gilberto Perez
GP
Cardoso
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 States of America.
Samir K
SK
Ballas
D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000503:physiopathology; D001938:Brazil; D003430:Cross-Sectional Studies; D005080:Exercise Test; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011446:Prospective Studies; D012307:Risk Factors; D055815:Young Adult