Cardiovascular complications and risk of death in sickle-cell disease

• Published in: The Lancet (British edition) Vol. 387; no. 10037; pp. 2565 - 2574
• Main Author: Gladwin, Mark T, Dr
• Format: Journal Article
• Language: English
• Published: England Elsevier Ltd 18.06.2016; Elsevier Limited
• Subjects: Anemia, Sickle Cell - complications; Anemia, Sickle Cell - mortality; Blood pressure; Cardiovascular diseases; Cell adhesion & migration; Death, Sudden, Cardiac - etiology; Death, Sudden, Cardiac - prevention & control; Developed countries; Early Diagnosis; Epidemiology; Erythrocytes; Health risk assessment; Heart attacks; Heart Diseases - diagnosis; Heart Diseases - etiology; Heart Diseases - mortality; Hemolysis - physiology; Humans; Hypertension; Hypertension, Pulmonary - diagnosis; Hypertension, Pulmonary - etiology; Hypertension, Pulmonary - mortality; Internal Medicine; Morbidity; Mortality; Pulmonary arteries; Pulmonary hypertension; Rheology; Risk Factors; Sickle cell disease; Stroke
• ISSN: 0140-6736; 1474-547X
• DOI: 10.1016/S0140-6736(16)00647-4

Summary In sickle-cell disease, a point mutation in the β-globin chain causes haemoglobin to polymerise within erythrocytes during deoxygenation, altering red blood cell rheology and causing haemolysis. Improvements in health infrastructure, preventive care, and clinical treatments have reduced the morbidity and mortality of sickle-cell disease in developed countries. However, as these patients live longer, the chronic effects of sustained haemolytic anaemia and episodic vaso-occlusive events drive the development of end-organ complications. Cardiopulmonary organ dysfunction and chronic kidney injury have a large effect on morbidity and premature mortality, and typically accelerate in the second decade of life. These processes culminate in the development of pulmonary hypertension, left ventricular diastolic heart disease, dysrhythmia, and sudden death. In this Series paper, we review the mechanisms, clinical features, and epidemiology of major cardiovascular complications in patients with sickle-cell disease and discuss how screening and intervention could reduce their incidence.