Cardiovascular complications of sickle cell disease

Abstract

Sickle cell disease (SCD) is the most common inherited blood disorder in the United States, and a global health problem. Pathological features of the abnormal hemoglobin (HbS) result in 2 hallmarks of the disease - recurrent episodes of acute microvascular occlusion and chronic hemolytic anemia - that inflict continuous and insidious damage to multiple organs. With improved childhood survival, SCD in adults has evolved into a chronic degenerative disease with underlying damage to multiple organs including the heart and lungs. Cardiopulmonary complications, including cardiomyopathy, diastolic dysfunction, pulmonary hypertension (PH), and sudden cardiac death are the most common causes of morbidity and mortality. Awareness of the sickle-related cardiovascular phenotypes is important for screening, early diagnosis, and intervention of cardiac complications in this disorder.

Keywords: Cardiac; Diastolic dysfunction; Pulmonary hypertension; Sickle cell.

Figure 1.

60 year old man with sickle cell disease (HbSS), HTN, paroxysmal atrial fibrillation, and pulmonary hypertension. A. There is right heart dilation. B. The left atrium is severely dilated. C. Left atrial volume index is 105 ml/m² (normal ≤ 34 ml/m²). D. Septal flattening is suggestive of RV pressure overload. E. Peak TR velocity is 4 m/s, gradient of 65 mmHg, estimated RVSP 75mmHg. F. Mitral annulus tissue Doppler peak septal e’ velocity is 7.6 cm/s with E/e’ ratio of 14 suggesting elevated LV filling pressure.