N C Joshi
Dilated Cardiomyopathy - Treatment
- Fluid restriction initially
- Diuretics to reduce preload
- Intravenous ionotropes with vasodilators - Dobutamine is a common first choice drug. Digoxin may have positive ionotropic effect but its role remains unclear.
- Angiotensin converting enzyme inhibitors improves survival and left ventricular dysfunction. Once phase of circulatory failure is over, start with 0.1 mg/kg of Captopril and it can be increased up to 1-2 mg/kg/24 hrs in divided doses.
- Anticoagulants are usually given to prevent intracavity thrombus formation.
- Antiarrhythmics should be used if patient is symptomatic but Antiarrhythmics are negative ionotropics so they have to be used with caution.

Although majority of cases have downhill course, a few especially presenting in infancy ultimately improve. It is the commonest reason for cardiac transplantation in children and actual survival after transplantation is 8% at 3 years.

No treatment is needed for asymptomatic children. Children with chest pain, syncope, and dyspnea should be treated with propranolol, which improves diastolic relaxation of left ventricle. If no improvement is seen with medical treatment, surgical resection of hypertrophied include may help.

Prognosis - Though ultimately fatal in majority, interval between discovery to death often goes in decades. There have been great advances in the molecular genetics of HOCM. Restriction fragment length polymorphic markers have localized the gene to chromosome 14.

Cardiomyopathy Cardiomyopathy 04/02/2001
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