Diagnóstico de hipertensão pulmonar em indivíduos adultos com doença falciforme / Diagnosis of pulmonary hypertension in adults with sickle cell disease

AUTOR(ES)
DATA DE PUBLICAÇÃO

2008

RESUMO

INTRODUCTION: Patients with sickle cell disease (SCD) and other haemolytic anaemia have increased prevalence of pulmonary hypertension (PH) that is related to higher mortality. The aim of this stdy was to determine the prevalence of PH and, its clinical, laboratorial and hemodynamic features. METHODS: In a crosssectional study, we evaluated 80 consecutive patients with sickle cell anemia and Sb0thalassemia who were submitted to a Doppler echocardioghraphy performed by a single observer. Clinical and laboratorial data were collected for all patients in order to verify the presence of SCD complications and to evaluate haemolysis rate, inflammation, liver and renal function. All patients performed a six-minute walk test. Patients who had peak velocity of regurgitant flow of tricuspid (Vrft) of at least 2.5 m/s were referred to pulmonary artery catheterization (Swan-Ganz). PH was defined as a mean pulmonary artery pressure ³ 25 mmHg. Clinical, laboratorial and hemodynamic data of patients with confirmed PH were compared to those data of patients without PH. RESULTS: Forty percent of patients (32/80) had Vrft ³ 2.5m/s and hemodynamic evaluation was recommended. The group of patients with Vrft³2.5 m/s had higher average age, higher prevalence of leg ulcers, proteinuria and hepatitis C, lower values of hemoglobin and albumin, higher values of urea, creatinine, uric acid, lactic dehydrogenase, aspartate aminotransferase and gglutamyltranspeptidase than the group with Vrft<2.5 m.s. The group with Vrft³2.5 m/s had poorer performance on the walk test and had lowest oxygen saturation at rest and post-exercise. On echocardiography, this group had greater right and left atrial volume. Only 78% of patients (25/32) underwent pulmonary artery catheterization and, in 8 patients PH was confirmed. Among the patients with PH, 3 had pre-capillary hypertension and 5 had post-capillary hypertension (pulmonary artery occlusion pressure above 15 mmHg). The Vrft measured by echocardiogram showed good correlation with the value of systolic pulmonary artery pressure, measured on Swan-Ganz(r=0,77). The patients with confirmed PH had higher mean age, lower levels of haemoglobin and platelet count and higher values of lactic dehydrogenase, urea, creatinine, uric acid, iron and gglutamyltranspeptidase than the group without PH. Individuals with PH had poorer performance on walk test than the group without PH. Patients with PH showed increased right and left atrium volume and higher cardiac index than the group without PH. CONCLUSIONS: Patients with SCD had increased prevalence of PH detected by Doppler echocardiography and confirmed by pulmonary catheterization. The echocardiogram was a good tool for screening. Patients who had Vrft ³ 2.5m/s exhibited clinical and laboratorial data consistent with a higher hemolysis rate than those with Vrft<2.5 m/s. Individuals diagnosed with PH may have post-capillary or pre-capillary hypertension, suggesting the existence of several etiologies and the need for different therapeutic approaches.

ASSUNTO(S)

catheterization swan-ganz anemia falciforme/complicações sickle cell anemia/complications pulmonary hypertension/ etiology sickle cell anemia/diagnosis anemia hemolítica congênita/diagnóstico hipertensão pulmonar/etiologia anemia falciforme/diagnóstico cateterismo de swan-ganz

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