Tricuspid regurgitant jet velocity and myocardial tissue Doppler parameters predict mortality in a cohort of patients with sickle cell disease spanning from pediatric to adult age groups ‐ revisiting this controversial concept after 16 years of additional evidence

Abstract

Sickle cell disease (SCD) is a monogenic hemoglobinopathy associated with significant morbidity and mortality. Cardiopulmonary, vascular and sudden death are the reasons for the majority of young adult mortality in SCD. To better understand the clinical importance of multi‐level vascular dysfunction, in 2009 we assessed cardiac function including tricuspid regurgitant jet velocity (TRV), tissue velocity in systole(S′) and diastole (E′), inflammatory, rheologic and hemolytic biomarkers as predictors of mortality in patients with SCD. With up to 9 years of follow up, we determined survival in 95 children, adolescents and adults with SCD. Thirty‐eight patients (40%) were less than 21 years old at initial evaluation. Survival and Cox proportional‐hazards analysis were performed. There was 19% mortality in our cohort, with median age at death of 35 years. In the pediatric subset, there was 11% mortality during the follow up period. The causes of death included cardiovascular and pulmonary complications in addition to other end‐organ failure. On Cox proportional‐hazards analysis, our model predicts that a 0.1 m/s increase in TRV increases risk of mortality 3%, 1 cm/s increase in S′ results in a 91% increase, and 1 cm/s decrease in E′ results in a 43% increase in mortality. While excluding cardiac parameters, higher plasma free hemoglobin was significantly associated with risk of mortality (p=.049). In conclusion, elevated TRV and altered markers of cardiac systolic and diastolic function predict mortality in a cohort of adolescents and young adult patients with SCD. These predictors should be considered when counseling cardiovascular risk and therapeutic optimization at transition to adult providers.

Cardiovascular phenotypes predict clinical outcomes in sickle cell disease: An echocardiography‐based cluster analysis

“…Recent reports have underlined the clinical importance of integrating inflammatory, rheological and hemolytic biomarkers, in addition to cardiac function including left and right heart exploration and focusing on diastolic function and TRV, as predictors of mortality in patients with SCD. 6,29,30 The complexity of predicting prognosis in SCD patients is that echocardiographic parameters are usually within normal ranges, as defined by international recommendations and cannot discriminate pathological cardiac processes. 17,31 A recent report on survival in 95 children, adolescents and adults with SCD (40% less than 21 years old) showed a 19% mortality at a nine-year follow-up that was mainly related to cardiovascular and pulmonary complications.…”

“…17,31 A recent report on survival in 95 children, adolescents and adults with SCD (40% less than 21 years old) showed a 19% mortality at a nine-year follow-up that was mainly related to cardiovascular and pulmonary complications. 30 These authors underlined the importance of combining elevated TRV and altered markers of cardiac systolic and diastolic function to predict mortality in adolescents and young adult patients with SCD.…”

Elevated tricuspid regurgitation velocity is associated with increased adverse haematologic events during pregnancy in women with sickle cell disease

“…1,2 A tricuspid regurgitation velocity (TRV) of at least 2.5 m/s by transthoracic echocardiogram (TTE) is a marker for underlying PH in patients with SCD, and despite a positive predictive value for catheterization confirmed PH of only 31%, 1 is associated with increased mortality. 3,4 Women with PH as defined by TTE have increased risk for adverse events in pregnancy; 5,6 however, data on pregnancy outcomes in women with both SCD and elevated TRV are sparse. We sought to evaluate the impact of elevated TRV on adverse events during pregnancy.…”

“…Pulmonary hypertension (PH) in patients with sickle cell disease (SCD) is multifactorial, with pulmonary vascular disease, thromboembolic disease, diastolic dysfunction and anaemia‐related hyperdynamic circulation described as potential mechanisms 1,2 . A tricuspid regurgitation velocity (TRV) of at least 2.5 m/s by transthoracic echocardiogram (TTE) is a marker for underlying PH in patients with SCD, and despite a positive predictive value for catheterization confirmed PH of only 31%, 1 is associated with increased mortality 3,4 . Women with PH as defined by TTE have increased risk for adverse events in pregnancy; 5,6 however, data on pregnancy outcomes in women with both SCD and elevated TRV are sparse.…”

Clinical-Epidemiological Characteristics and Mortality in Patients with Sickle Cell Anemia: A Retrospective Cohort Study of 1980 at 2018

“…50,52,60 In addition to that, heart disease was also observed as the leading cause of death in several other studies. 61,62 Among the main complaints referred to in the period prior to death, pain stood out, a common manifestation in SCA due to vasoclusive episodes and damage to organs and tissues. 63 The use of analgesic medications was also associated with a higher risk of death, especially in the case of regular analgesics.…”