Dilated cardiomyopathy.
Dilated cardiomyopathy is characterised by left ventricular dilation that is associated with systolic dysfunction. Diastolic dysfunction and impaired right ventricular function can develop. Affected individuals are at risk of left or right ventricular failure, or both. Heart failure symptoms can be exercise-induced or persistent at rest. Many patients are asymptomatic. Chronically treated patients sometimes present acutely with decompensated heart failure. Other life-threatening risks are ventricular arrhythmias and atrioventricular block, syncope, and sudden death. Genetic inheritance arises in 30-48% of patients, and inflammatory disorders such as myocarditis or toxic effects from medications, alcohol, or illicit drugs also result in dilated cardiomyopathy. Genes that cause dilated cardiomyopathy generally encode cytoskeletal and sarcomeric (contractile apparatus) proteins, although disturbance of calcium homeostasis also seems to be important. In children, disrupted mitochondrial function and metabolic abnormalities have a causal role. Treatments focus on improvement of cardiac efficiency and reduction of mechanical stress. Arrhythmia therapy and prevention of sudden death continue to be mainstays of treatment. Despite progress over the past 10 years, outcomes need to be improved.
Jefferies
John Lynn
JL
Pediatric Cardiology, Texas Children's Hospital, Baylor College of Medicine, Houston, TX, USA.
Towbin
Jeffrey A
JA
D002311:Cardiomyopathy, Dilated / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics / Q000503:physiopathology / Q000628:therapy; D003937:Diagnosis, Differential; D004452:Echocardiography; D004562:Electrocardiography; D017079:Exercise Tolerance; D006333:Heart Failure / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics / Q000628:therapy; D006801:Humans; D007239:Infections / Q000150:complications; D009200:Myocardial Contraction / Q000235:genetics; D009205:Myocarditis / Q000150:complications / Q000175:diagnosis / Q000235:genetics; D011379:Prognosis; D018487:Ventricular Dysfunction, Left / Q000175:diagnosis* / Q000453:epidemiology / Q000235:genetics