Dilated cardiomyopathy

• Published in: The Lancet (British edition) Vol. 390; no. 10092; pp. 400 - 414
• Main Authors: Weintraub, Robert G, Dr, Semsarian, Christopher, Prof, Macdonald, Peter, MD
• Format: Journal Article
• Language: English
• Published: England Elsevier Ltd 22.07.2017; Elsevier Limited
• Subjects: Adrenergic beta-Antagonists - therapeutic use; Angiotensin; Angiotensin-Converting Enzyme Inhibitors - therapeutic use; Arrhythmia; Cardiology; Cardiomyopathy; Cardiomyopathy, Dilated - diagnosis; Cardiomyopathy, Dilated - etiology; Cardiomyopathy, Dilated - genetics; Cardiomyopathy, Dilated - therapy; Cytoskeleton; Dilated cardiomyopathy; Echocardiography; Electrocardiography; Enzyme inhibitors; Extracorporeal Membrane Oxygenation; Genetic Predisposition to Disease; Genetic Testing - methods; Heart; Heart failure; Heart Transplantation; Humans; Immunosuppressive agents; Internal Medicine; Medical prognosis; Muscle contraction; Mutation; Myocarditis; Patients; Peptidyl-dipeptidase A; Proteins; Risk Factors; Stem cells; Thromboembolism; Toxins; Transplantation; Transplants & implants; Ventricle
• ISSN: 0140-6736; 1474-547X
• DOI: 10.1016/S0140-6736(16)31713-5

Summary Dilated cardiomyopathy is defined by the presence of left ventricular dilatation and contractile dysfunction. Genetic mutations involving genes that encode cytoskeletal, sarcomere, and nuclear envelope proteins, among others, account for up to 35% of cases. Acquired causes include myocarditis and exposure to alcohol, drugs and toxins, and metabolic and endocrine disturbances. The most common presenting symptoms relate to congestive heart failure, but can also include circulatory collapse, arrhythmias, and thromboembolic events. Secondary neurohormonal changes contribute to reverse remodelling and ongoing myocyte damage. The prognosis is worst for individuals with the lowest ejection fractions or severe diastolic dysfunction. Treatment of chronic heart failure comprises medications that improve survival and reduce hospital admission—namely, angiotensin converting enzyme inhibitors and β blockers. Other interventions include enrolment in a multidisciplinary heart failure service, and device therapy for arrhythmia management and sudden death prevention. Patients who are refractory to medical therapy might benefit from mechanical circulatory support and heart transplantation. Treatment of preclinical disease and the potential role of stem-cell therapy are being investigated.