Or register here
Global Search

Trusted Resources: Evidence & Education

Scientific literature and patient education texts

Back to Evidence & Education / Scientific Articles

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 Sixteen Years of Additional Evidence

key information

source: American Journal of Hematology

year: 2020

authors: Payal Shah, Silvie Suriany, Roberta Kato, Adam Michael Bush, Patjanaporn Chalacheva, Saranya Veluswamy, Christopher Clement Denton, Kelly Russell, Maha Khaleel, Henry Jay Forman, Michael C. K. Khoo, Richard Sposto, Thomas D. Coates, John C. Wood, Jon Detterich

summary/abstract:

Sickle cell disease (SCD) is a monogenic hemoglobinopathy associated with significant morbidity and mortality. Cardiopulmonary, vascular and sudden death underlies 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. 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.

organization: Children's Hospital Los Angeles, USA; Stanford University, USA; University of Southern California, USA

DOI: doi.org/10.1002/ajh.26003

read more

Related Content

To improve your experience on this site, we use cookies. This includes cookies essential for the basic functioning of our website, cookies for analytics purposes, and cookies enabling us to personalize site content. By clicking on 'Accept' or any content on this site, you agree that cookies can be placed. You may adjust your browser's cookie settings to suit your preferences. More Information

The cookie settings on this website are set to "allow cookies" to give you the best browsing experience possible. If you continue to use this website without changing your cookie settings or you click "Accept" below then you are consenting to this.

Close

© 2024 rareLife solutions Inc.

To improve your experience on this site, we use cookies. This includes cookies essential for the basic functioning of our website, cookies for analytics purposes, and cookies enabling us to personalize site content. By clicking on 'Accept' or any content on this site, you agree that cookies can be placed. You may adjust your browser's cookie settings to suit your preferences. More Information

The cookie settings on this website are set to "allow cookies" to give you the best browsing experience possible. If you continue to use this website without changing your cookie settings or you click "Accept" below then you are consenting to this.

Close