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Patients with sickle cell disease (SCD) are at increased risk for cardiovascular complications. Exercise testing is used as a prognostic marker in a variety of cardiovascular diseases. However, there is a lack of evidence on exercise in SCD patients, particularly regarding its safety, feasibility, and possible prognostic role.We used the maximal treadmill test to determine safety and feasibility of the exercise testing in SCD patients. Additionally, the factors associated with exercise duration, as well as the impact of exercise-induced changes on clinical outcome, were also assessed.One-hundred thirteen patients with SCD, who underwent exercise testing, were prospectively enrolled. A comprehensive cardiovascular evaluation, including echocardiography and B-type natriuretic peptide (BNP) levels, were obtained. The long-term outcome was a composite endpoint of death, severe acute painful episodes, acute chest syndrome, or hospitalization for other SCD-related complications. Cox regression analysis was performed to identify the variables associated with the outcome. A p-value0.05 was considered to be statistically significant.The mean age was 36 ± 12 years (range, 18-65 years), and 62 patients were women (52%). Ischemic electrocardiogram and abnormal blood pressure (BP) response to exercise were detected in 17% and 9%, respectively. Two patients experienced pain crises within 48 hours that required hospitalization. Factors associated with exercise duration were age, sex, tricuspid regurgitation (TR) maximal velocity, and E/e' ratio, after adjustment for markers of disease severity. During the mean follow-up of 10.1 months (ranging from 1.2 to 26), the endpoint was reached in 27 patients (23%). Independent predictors of adverse events were hemoglobin concentration, late transmitral flow velocity (A wave), and BP response to exercise.Exercise testing in SCD patients who were clinically stable is feasible. Exercise duration was associated with diastolic function and pulmonary artery pressure. Abnormal BP response was an independent predictor of adverse events.Pacientes com anemia falciforme (AF) têm risco aumentado de complicações cardiovasculares. O teste ergométrico é usado como marcador de prognóstico em uma série de doenças cardiovasculares. Entretanto, há uma escassez de evidências sobre exercícios em pacientes com AF, especialmente em relação à sua segurança, viabilidade e possível função prognóstica.Usamos o teste em esteira máximo para determinar a segurança e a viabilidade do teste ergométrico em pacientes com AF. Além disso, os fatores associados à duração do exercício, bem como o impacto das alterações causadas pelo exercício em resultados clínicos, também foram avaliados.113 pacientes com AF que passaram pelo teste ergométrico e por uma avaliação cardiovascular abrangente incluindo um ecocardiograma e os níveis do peptídeo natriurético do tipo B (BNP). O desfecho de longo prazo foi uma combinação de eventos incluindo morte, crises álgicas graves, síndrome torácica aguda ou internações hospitalares por outras complicações associadas â doença falciforme. A análise de regressão de Cox foi realizada para identificar as variáveis associadas ao resultado. Um p valor0,05 foi considerado estatisticamente significativo.A média de idade foi de 36 ± 12 anos (intervalo, 18-65 anos), e 62 pacientes eram do sexo feminino (52%). A presença de alterações isquêmicas ao esforço e resposta pressórica anormal ao exercício foram detectadas em 17% e 9 % da´população estudada respectivamente. Dois pacientes apresentaram crise álgica com necessidade de internação hospitalar no período de 48 horas da realização do exame. Fatores associados à duração do exercício foram idade, sexo, velocidade máxima de regurgitação tricúspide (RT), e relação E/e’, após a padronização quanto aos marcadores da gravidade da doença. Durante o período médio de acompanhamento de 10,1 meses (variando de 1,2 a 26), 27 pacientes (23%) apresentaram desfechos clínicos adversos. Preditores independentes de eventos adversos foram a concentração de hemoglobina, velocidade do fluxo transmitral tardio (onda A), e a resposta da PA ao exercício.A realização de testes ergométricos em pacientes com AF, clinicamente estáveis, é viável. A duração do exercício estava associada à função diastólica e a pressão arterial pulmonar. A resposta anormal da PA foi um preditor independente de eventos adversos.

Cardiovascular complications have been increasingly detected in patients with sickle cell disease (SCD). Two-dimensional speckle-tracking echocardiography (STE) detects early myocardial changes in a number of pathophysiological processes, which may be useful in SCD. This study was designed to examine the value of STE in predicting clinical outcome in adult patients with SCD. A total of 219 patients, mean age 33 ± 12 years were prospectively enrolled. Several clinical, laboratory and echocardiographic variables including left ventricular global longitudinal strain (LVGLS) by STE were assessed. The endpoint was a composite of the following events: (1) all-cause mortality, (2) three or more acute painful episodes that require hospitalization in one year, (3) acute chest syndrome and (4) hospitalization due to disease complication. The majority of the patients had enlargement of LV and left atrial (LA) with preserved ejection fraction. During the mean follow-up of 30 months, 69 patients (32%) had reached the endpoint, including eight deaths (3.7%). No difference was observed in the parameters of diastolic function comparing the patients with and without events. LVGLS ranged from − 12.25 to − 25.44 (mean − 20.26 ± 2.5), with higher values in the patients who had events compared with those who did not. In the multivariable analysis, higher LVGLS values were associated with adverse events (adjusted OR 1.25; 95% CI 1.04–1.51; p = 0.021), independently of the TR maximal velocity and LV ejection fraction. In patients with SCD, higher LV global longitudinal strain was a predictor of adverse outcome, independently of age, TR velocity and LV function.

Cardiovascular complications have been increasingly detected as a result of prolonged longevity of patients with sickle cell disease (SCD). Previous studies have focused especially on pulmonary hypertension and its consequences on the right-side heart chambers, whereas factors associated with morphological changes in left ventricle (LV) remain poorly understood. This study was designed to identify clinical, laboratorial, and echocardiographic parameters associated with LV remodeling and its impact on outcome in SCD. Ninety patients aged 28 ± 7 years and 20 age- and gender-balanced healthy subjects were enrolled. Laboratory tests, electrocardiogram, and an echocardiogram with tissue Doppler imaging were performed in all patients. Patients with SCD had larger left and right heart chambers dimensions, LV mass, and tricuspid regurgitation (TR) velocity compared to health controls with similar demographic features. Despite chambers enlargement, systolic function of both ventricles was preserved. The mitral inflow velocities were higher in the patients than in controls, whereas septal and lateral annular motion velocities were normal, suggesting normal ventricular relaxation. SCD patients who were on hydroxyurea therapy and/or hypertransfusion had higher hemoglobin concentrations, but similar echocardiographic findings in comparison to those without treatment. Systolic blood pressure, ferritin concentration, TR velocity, and parameters of diastolic function were independently associated with increased LV mass. In addition, the predictors of adverse events were ferritin concentration, lactate dehydrogenase levels, and TR velocity. LV remodeling in SCD patients seems to be influenced by a combination of factors including blood pressure, ferritin concentration, TR velocity, and parameters of LV diastolic function, and was not associated with adverse outcomes.

FAPEMIG - Fundação de Amparo à Pesquisa do Estado de Minas Gerais As complicações cardiovasculares na doença falciforme (DF) são frequentes, e têm sido atualmente mais frequentemente detectadas, em decorrência da maior longevidade dos pacientes. Como metodologia altamente informativa e confiável, a ecocardiografia possibilita análise acurada da morfologia e função cardíaca. Estudos prévios avaliaram especialmente a hipertensão pulmonar e suas consequências sobre as câmaras cardíacas direitas, permanecendo pobremente compreendidos os fatores associados às alterações morfológicas do ventrículo esquerdo (VE). O objetivo deste estudo foi investigar parâmetros clínicos, laboratoriais e ecocardiográficos associados ao remodelamento do VE, além de avaliar o valor do strain bidimensional em detectar disfunção atrial e ventricular em pacientes com DF. Método: foram investigados 90 pacientes consecutivamente recrutados em serviço de referência, com hemoglobinopatia SS ou S-beta-talassemia zero, com idade média de 287 anos (limites, 18-40 anos), sem doenças associadas, e 20 controles sadios com idade e sexo semelhantes. Foram realizados exames laboratoriais habituais, eletrocardiograma (ECG) convencional e ecocardiografia, incluindo-se doppler tecidual e strain de todos os pacientes. Resultados: nos pacientes com DF foi observado aumento nas dimensões das câmaras cardíacas direitas e esquerdas, na massa do VE e na velocidade de regurgitação tricúspide, quando comparados com os controles. Apesar da dilatação das câmaras, a função sistólica de ambos os ventrículo estava preservada. As velocidades de fluxo mitral estavam aumentadas nos pacientes, em relação aos controles, enquanto que velocidades do anel mitral septal e lateral estavam normais, sugerindo relaxamento normal do VE. Os pacientes que estavam em hipertransfusão ou em uso de hidroxiureia apresentaram maiores concentrações de hemoglobina, mas achados ecocardiográficos similares, em comparação àqueles sem tratamento. Pressão arterial sistólica, concentração de ferritina, velocidade de regurgitação tricúspide (TRV), tempo de desaceleração da onda E mitral e razão E/e’ foram independentemente associados ao aumento da massa do VE. O strain dos dois ventrículos e átrios não diferiu daquele dos controles. Conclusão: o remodelamento do VE nos pacientes com DF parece estar associado a uma combinação de fatores, incluindo-se pressão arterial sistêmica, concentração de ferritina, VRT e parâmetros ecocardiográficos de função diastólica. A análise do strain de ambos os ventrículos e átrios não evidenciou disfunção sistólica incipiente nesse grupo de pacientes com DF. Cardiovascular complications in sickle cell disease (SCD) are common, and have been frequently found nowadays, due to increased longevity of the patients. As a highly informative and reliable method, echocardiography allows an accurate analysis of cardiac morphology and function. Previous studies have focused especially on pulmonar hypertension and its consequence on the rigth-side heart chambers, whereas factors associated with morphological changes in left ventricle (LV) remain poorly understood. The present study was designed to identify clinical, laboratorial and echocardiographic parametes associated with LV remodeling in SCD patients. Methods: ninety consecutive SCD patients, mean age of 28 ± 7 years (range, 18-40 years) without any associated diseases and 20 age- and gender-balanced healthy subjects were enrolled into the study. Standard laboratory tests, 12-lead ECG and a comprehensive echocardiogram with tissue Doppler imaging were performed in all patients. Results: patients with SCD had larger left and rigth heart chambers dimensions, LV mass and tricuspid regurgitation (TR) velocity compared to controls. Despiste chambers enlargement, systolic function of both ventricles was preserved. The mitral inflow velocites were higher in the pacientes than in controls, whereas septal and lateral anular motion velocities were normal, suggesting normal LV relaxation. SCD pacientes who were on hydroxyurea therapy and/or hypertransfusion had higer hemoglobina concentrations, but similar echocardiographic findings in comparison to the individuals without treatment. Systolic blood pressure, ferritin concentration, TR velocity, deceleration time of early mitral inflow velocity, and E/e’ratio were independently associated with increased LV mass. Speckle tracking of both ventricles and atria were not diferent from those of the controls. Conclusions: LV remodeling in SCD pacientes seems to be influenced by a combination of factors including blood pressure, ferritin concentration, TR velocity, and parameters of LV diastolic function. Speckle tracking of both ventricles and atria could not detect any incipiente systolic dysfunction in this group of SCD patients.

Background: Sickle cell disease (SCD) is a hemoglobinopathy that is common worldwide. It usually presents with cardiac involvement, although data on systolic function are somewhat controversial. The aim of this study was to investigate the value of speckle-tracking strain, a deformation index, in detecting ventricular dysfunction in SCD. Methods: Ninety adult patients with SCD were compared with 20 healthy controls. Doppler echocardiography with Doppler tissue imaging was performed in all, and the left and right ventricles were analyzed by the use of two-dimensional speckle-tracking strain. Results: The mean age of the patients with SCD was 26 years, and 43% were men. Left ventricular (LV) dimensions and mass were higher in patients with SCD, whereas LV ejection fraction did not differ from the controls. E and A waves, as well as E/e0 ratio, were also higher in patients with SCD. Two-dimensional speckle-tracking strain of both ventricles in the patients with SCD was not different from that of controls. The factors independently associated with LV longitudinal strain were age (P = .009), oximetry (P = .001), lactate dehydrogenase (P = .014), LV ejection fraction (P < .001), and right ventricular systolic annular velocity (P = .010). Conclusions: Ventricular enlargement with normal ventricular function was a frequent finding in SCD. Twodimensional speckle-tracking strain of both ventricles was similar in patients and controls, suggesting normal myocardial contractility in patients with SCD. LV global longitudinal strain was associated with age, intensity of hemolysis, and ventricular function. (J Am Soc Echocardiogr 2014;27:1216-22.)