Cardiac complications of sickle cell disease in pediatric patients: A case report and contemporary literature review. (September 2022)

• Record Type: Journal Article
• Authors: Smerling, Jonathan; Burns, Joseph; Haimed, Abraham; Ganigara, Madhusudan; Appiah-Kubi, Abena O.; Aygun, Banu; Mitchell, Elizabeth C.; Dhar, Arushi
• Is Part Of: Progress in pediatric cardiology. Volume 66(2022)
• Publication Date: 2022-09
• Subjects: Sickle cell disease -- Pulmonary hypertension -- Pediatric hematology -- Pediatric cardiology; Pediatric cardiology -- Periodicals; Cardiovascular Diseases -- Periodicals; Infant; Child; Cardiologie pédiatrique -- Périodiques; 618.9212005
• DOI: 10.1016/j.ppedcard.2022.101517
• Languages: English
• ISSNs: 1058-9813

Abstract

Abstract: Background: Sickle cell disease (SCD) is a family of hemoglobinopathies characterized by a mutation in both β globin genes resulting in hemoglobin S as the predominant hemoglobin. This hemoglobin S polymerizes in the deoxygenated state, resulting in the formation of "sickled" erythrocytes. Cardiopulmonary complications represent the leading cause of death for those affected, though the body of literature describing cardiac complications of SCD in children is not as robust as that in adult populations. Aim of Review: It is the aim of this article to present a case to frame the cardiac complications of SCD and present a summary of contemporary literature on the topic. Key Scientific Concepts of Review: Pulmonary hypertension is a well-described complication of SCD, estimated with echocardiography and projected to occur in up to 46 % of cases. Diastolic dysfunction is poorly described in children with SCD though limited studies report a unique restrictive physiology superimposed on hyperdynamic physiology. Similarly, myocardial infarction is rare in children with SCD but it is hypothesized that increasing carotid intima thickness secondary to SCD contributes to an increased risk. Further, children with SCD demonstrate impaired systolic dipping during sleep, predisposing to the development of hypertension. Limited cases of electrophysiologic abnormalities report prolonged QTc interval, suspected to be secondary to chronic anemia. The use of disease modifying agents such as hydroxyurea likely reduces the risk and facilitates a reduction in these cardiac changes. It is critical for patients and families with SCD that there is further investigation of the cardiac complications of SCD in pediatric populations. Highlights: Pulmonary Hypertension is a well described complication of Sickle Cell Disease Chronic anemia in Sickle Cell Disease is thought to cause cardiac remodeling and diastolic dysfunction Pediatric patients with Sickle Cell Disease have been found to have impaired systolic dipping There is limited literature describing electrophysiological abnormalities in Sickle Cell Disease Hydroxyurea and other disease modifying agents likely reduce the risk of the cardiac complications of Sickle Cell Disease