Dilated cardiomyopathy

Extract

• Typical symptoms in the early stages include dyspnoea on exertion, arrhythmias and sometimes a feeling of pressure or a long-lasting, mild pain on the chest that is not related to exercise. As the disease progresses, the patient will develop heart failure and associated oedema.
• Asymptomatic incipient dilated cardiomyopathy may first manifest itself as an enlarged heart shadow on a chest x-ray or an abnormal ECG finding.
• If previously undiagnosed heart failure or left ventricular dilatation is noted, the patient must undergo echocardiography and, depending on the results, further investigations to identify the cause of heart failure.
• Pharmacological therapy is similar to that used in heart failure of any aetiology; ACE inhibitors and angiotensin receptor blockers as well as beta blockers are the drugs of choice; a combination of valsartan and sacubitril is a newer preparation.
• The identification of atrial fibrillation and initiation of anticoagulation is important in order to prevent cardiogenic embolism and worsening of the heart failure.
• Dilated cardiomyopathy is in at least 30% of the cases hereditary, i.e. running in families.
  • The most common mode of inheritance is autosomal dominant inheritance but in gene defect carriers the disease usually manifests only at middle age.
  • It is possible in some cases to detect the disease mutation by using extensive gene panel analyses. Therefore, it is recommended that the patient is referred to a unit of clinical genetics or to a cardiology department with experience in investigating the genetic background of diseases for the consideration of genetic testing.
  • Using extensive gene panels, a definite or probable disease mutation has been found in about 30% of the cases in the Helsinki University Central Hospital. Particularly familial cases should be referred for an assessment of the need for genetic testing but also in cases that seem to be non-familial, a gene defect can be found in a fair share of patients.
  • If a gene defect is not found, it does not always exclude the possibility of a hereditary disease, since not all disease genes are yet known. In such cases it is recommend that the first-degree relatives of the patient are referred to a cardiologist for clinical assessment and particularly echocardiography, if no obvious cause is identified for dilated cardiomyopathy, such as a history of chemotherapy.
  • Children are to be referred to a paediatric cardiologist for examination.

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ACE inhibitors, ATR blockers, Adrenergic beta-Antagonists, Angiotensin II Type 1 Receptor Blockers, Angiotensin-Converting Enzyme Inhibitors, Anthracyclines, Antiarrhythmia pacemaker, Anticoagulant therapy, Anticoagulants, Anticoagulation, Antineoplastic Agents, Arrhythmias, Cardiac, Atrial Fibrillation, BNP, Becker's muscular dystrophy, Beta blockers, Bisoprolol, Biventricular pacing, Cardiology, Cardiomyopathies, Cardiomyopathy, Alcoholic, Cardiomyopathy, Dilated, Cardiomyopathy, Peripartal, Cardiomyopathy, tachycardia-induced, Carvedilol, Chest Pain, Chest x-ray, Cocaine, Coronary Angiography, Cyclophosphamide, Cytostatic Agents, DCM, DNA diagnosis, Digoxin, Diuretics, Doxorubicin, Dyspnea, ECG, Echocardiography, Edema, Exertional dyspnea, Exertional dyspnoea, Familial dilated cardiomyopathy, Fluorouracil, Gene defect, Gene study, Gene test, Genetic Counseling, Genetic diagnosis, Heart Failure, Heart Murmurs, Heart Transplantation, I42.0, I42.6, I42.7, I42.8, I42.9, I43*, I50, I51.7, ICD, Implantable cardioverter defibrillator, Internal medicine, Lamin A/C gene, Left Ventricular dilatation, Metoprolol, Mitral Valve Insufficiency, Muscular Dystrophy, Duchenne, Myocarditis, Natriuretic Peptides, Oedema, Pacemaker, Pacemaker, Artificial, Pacing, Peripartal Cardiomyopathy, Pulmonary Edema, Pulmonary oedema, Radiography, Thoracic, Spironolactone, Systolic Murmurs, Tachycardia, Tachycardia cardiomyopathy, Tachycardia, Ventricular