All ETDs from UAB

Advisory Committee Chair

Virginia Howard

Advisory Committee Members

Julie Kanter

Walter Johnson

April Carson

Gerhard Hellemann

Document Type

Dissertation

Date of Award

2021

Degree Name by School

Doctor of Philosophy (PhD) School of Public Health

Abstract

Cardiopulmonary disorders, including pulmonary hypertension, chronic lung disease, diastolic dysfunction and congestive heart failure, are among the most common complications in sickle cell disease (SCD). Many of these complications are identified in adulthood when patients develop irreversible clinical symptoms. General improvement in healthcare and disease management is allowing more children with SCD to reach adulthood, leading to the growing interest in preventing disease-specific complications to improve morbidity and mortality rates. Although cardiac abnormalities are common, it is unknown when cardiac dysfunction appears in the course of SCD.This dissertation utilized the Dissemination and Implementation of Stroke Prevention Looking at the Care Environment (DISPLACE) study to assess cardiopulmonary outcomes in children with SCD, determine the risk factors associated with left ventricular hypertrophy in children with SCD and evaluate the changes in left ventricular function for children receiving disease modifying therapy. Further, this work also investigated the association between PFO and ischemic stroke among children with SCD. Results of retrospective crossectional studies showed a significant proportion of children with SCD have echocardiographic abnormalities, with 61% having left atrial dilatation, 24% having high tricuspid regurgitant jet velocity, 21% having left ventricular dilation and 20% had Left ventricular hypertrophy. The median ages of children with the echocardiographic abnormalities were found to be similar for all the abnormalities ~9years (IQR: 5-13years), except for left atrial dilatation with a lower median age of 8 years (IQR:4-12years). Children with SCD with lower hemoglobin levels were more likely to have echocardiographic abnormalities, consistent with the physiological cardiac volume overload imposed by anemia. The results showed children on HU had a significantly higher odds of LVH compared to those on no therapy (OR: 1.83, 95% CI: 1.41 – 2.37). The results showed a lower-than-expected prevalence of PFO. Only 6% of the children having PFO. The results didn’t support the hypothesis that PFO is associated with ischemic stroke in children with SCD. The study provides pertinent information to support initiating echocardiographic screening at earlier ages and the need to investigate other cardiopulmonary markers of morbidity in children than is currently practiced. The study provides preliminary information to support prospective cohort studies to assess cardiovascular outcomes in SCD.

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