Sickle cell related cardiomyopathy and cardiovascular autonomic dysfunction

TL;DR

This paper reviews how sickle cell disease affects heart function and autonomic control, contributing to sudden death.

Contribution

The paper provides a comprehensive review linking sickle cell cardiomyopathy, autonomic dysfunction, and sudden mortality.

Findings

Sickle cell cardiomyopathy involves ventricular dilatation, hypertrophy, and pulmonary hypertension.

Autonomic dysfunction in SCD patients may contribute to vaso-occlusive crises and sudden death.

Longer survival in SCD patients has increased focus on chronic complications like heart disease.

Abstract

Sickle cell disease (SCD) is the most common genetic haemoglobinopathy worldwide. Due to advancements in care, SCD patients are living longer. Consequently, there is increased interest in long term sequalae of chronic micro-vascular sickling and resultant end organ damage. Sickle cell cardiomyopathy is an emerging clinical entity characterised by a unique combination of ventricular dilatation, ventricular hypertrophy, diastolic dysfunction and pulmonary hypertension. Additionally, SCD patients have impaired autonomic function which is thought to pre-dispose to vaso-occlusive crises through sympathetic activation and parasympathetic withdrawal during times of physiologic stress. Furthermore, sudden death is a major cause of mortality among patients with SCD, however the mechanism has not been elucidated. This review summarizes the sickle cell cardiomyopathy literature, its relationship with autonomic dysfunction and its association with sudden death.

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