From the Guidelines
Sickle cell disease can lead to cardiomyopathy due to chronic anemia, vaso-occlusion, and other complications. The relationship between sickle cell disease and cardiomyopathy is complex, with multiple factors contributing to the development of cardiac dysfunction. According to the 2021 AHA/ACC/ASE/CHEST/SAEM/SCCT/SCMR guideline for the evaluation and diagnosis of chest pain 1, patients with sickle cell disease are at risk of acute chest syndrome, which can lead to cardiac complications, including myocardial ischemia and acute myocardial infarction (AMI).
Key factors that contribute to the development of cardiomyopathy in sickle cell disease include:
- Chronic anemia, which increases cardiac output and leads to left ventricular dilation and eccentric hypertrophy
- Repeated episodes of vaso-occlusion, causing microvascular damage and myocardial fibrosis
- Pulmonary hypertension, which increases right ventricular strain and can contribute to right-sided heart failure
- Iron overload from multiple blood transfusions, which can further damage the heart muscle
Management of cardiomyopathy in sickle cell disease typically involves treating the underlying condition with hydroxyurea to reduce sickling events, along with standard heart failure therapies, including ACE inhibitors, beta-blockers, and diuretics as clinically indicated. Regular cardiac evaluations, including echocardiograms, are essential to monitor for early signs of cardiomyopathy in patients with sickle cell disease.
From the Research
Sickle Cell Disease and Cardiomyopathy
- Sickle cell disease (SCD) can lead to cardiomyopathy due to various mechanisms, including chronic anemia, iron overload, and volume overload, which cause cardiac remodeling and left ventricular (LV) dilation 2.
- SCD-related cardiomyopathy is unique and features restrictive cardiomyopathy with LV dilation, although systolic dysfunction is uncommon in this condition 2.
- The diagnosis of SCD-associated cardiomyopathy can be challenging due to unusual echocardiographic features and the need for improved phenotyping to identify patients at an earlier stage of myocardial dysfunction 3.
Pathophysiology and Diagnosis
- The pathological features of SCD result in recurrent episodes of acute microvascular occlusion and chronic hemolytic anemia, leading to continuous damage to multiple organs, including the heart and lungs 4.
- Cardiopulmonary complications, including cardiomyopathy, diastolic dysfunction, pulmonary hypertension (PH), and sudden cardiac death, are common causes of morbidity and mortality in SCD patients 4, 5.
- Awareness of sickle-related cardiovascular phenotypes is essential for screening, early diagnosis, and intervention of cardiac complications in SCD patients 4.
Cardiovascular Manifestations
- SCD can lead to various cardiovascular manifestations, including right and left ventricular systolic and diastolic dysfunction, elevated cardiac output, cardiomegaly, and myocardial ischemia 5.
- Pulmonary hypertension resulting from intravascular hemolysis is a major complication that independently correlates with survival in SCD patients 5, 6.
- Diastolic heart failure (HF) and/or pulmonary hypertension (PH) are leading causes of death in patients with SCD, often featuring biventricular cardiac hypertrophy and left ventricular (LV) diastolic dysfunction 6.