Avaliação cardiológica em pacientes com doença falciforme

Abstract

Introduction: Sickle cell disease (SCD) is one of the most common monogenic hereditary disease in the world. It affects several organs and systems and is characterized by hemolytic anemia and vaso-occlusive phenomena that lead to painful acute crises and to chronic and progressive systemic damage. In view of the impact on quality of life and survival of these patients, it is essential to search for the early detection of clinical involvement and prognostic markers. Objectives: The primary outcome was to study and to describe the main findings associated with adverse outcome in SCD. Methods: Patients with SCD conrmed by hemoglobin electrophoresis were prospectively enrolled into the study. We selected 120 patients who underwent exercise testing under standard protocol, transthoracic echocardiography, BNP measurement and assessment of hematological parameters. The endpoint was a combined endpoint including the following clinical events: (1) Death related to SCD, (2) All cause mortality, (3) Three or more acute painful episodes that require hospitalization or parenteral medication (4) Acute chest syndrome characterized by a newly pulmonary infiltrate detected by chest radiography with sudden onset of signs and symptoms like chest pain, fever, tachypnea, wheezing, cough and hypoxemia, (5) Hospitalization for another SCDrelated complication, especially life-threatening infection. Follow-up data were obtained during clinical follow-up appointment or telephone interviews. Secondary outcomes were to describe the findings of exercise testing and the main factors associated with exercise time. Results: During the mean follow-up of 10.1 months (ranging from 1.2 to 26), the endpoint was reached in 27 patients (23%): 4 patients died (one death was unrelated to SCD), 8 were hospitalized due to 3 acute painful episodes, 11 had acute chest syndrome, and 4 were hospitalized with other SCDrelated complications. The independent predictors of adverse events were hemoglobin concentration, late transmitral flow velocity (A wave), and abnormal blood pressure response to exercise. During exercise testing, significant ischemic ST abnormalities in the electrocardiogram were detected in 20 patients (17%), supraventricular premature contractions were frequent, isolated in 16% of the cases, and complexes with some episodes of paroxysmal supraventricular tachycardia in 17% of the patients. Abnormal blood pressure response was found in 11 patients (9%). In the multivariable linear regression analysis including the laboratorial markers of disease severity, TR maximal velocity and E/e ratio emerged as important factors associated with exercise duration, after adjustment for age, hemoglobin and gender. Conclusions: Comprehensive cardiologic evaluation including exercise testing and echocardiogram is useful clinical tool to evaluate SCD patients and can add prognostic clinical information.