Patho physiology: Cardiomyopathy refers to diseases of the heart muscle. These diseases have many causes, signs and symptoms, and treatments.In cardiomyopathy, the heart muscle becomes enlarged, thick, or rigid. In rare cases, the muscle tissue in the heart is replaced with scar tissue.As cardiomyopathy worsens, the heart becomes weaker. It's less able to pump blood through the body and maintain a normal electrical rhythm. This can lead to heart failure or irregular heartbeats called arrhythmias. In turn, heart failure can cause fluid to build up in the lungs, ankles, feet, legs, or abdomen.The weakening of the heart also can cause other complications, such as heart valve problems.
Treatment: People who have cardiomyopathy but no signs or symptoms may not need treatment. Sometimes, dilated cardiomyopathy that comes on suddenly may go away on its own. For other people who have cardiomyopathy, treatment is needed. Treatment depends on the type of cardiomyopathy you have, the severity of your symptoms and complications, and your age and overall health. Treatments may include: Heart-healthy lifestyle changes, Medicines, Nonsurgical procedure, Surgery and implanted devices. The main goals of treating cardiomyopathy include: • Controlling signs and symptoms so that you can live as normally as possible • Managing any conditions that cause or contribute to the disease • Reducing complications and the risk of sudden cardiac arrest • Stopping the disease from getting worse
Research: Prevalence and Disease Outcome: because recognition of inherited disease is often limited by practice patterns which are in turn heavily influenced by the urgent demands of caring for sick patients, an important proportion of inherited disease is misdiagnosed. As an example, we discovered that nearly 25% of patients undergoing heart transplant for end stage cardiomyopathy have inherited disease, whereas current recognition of inherited disease in this population is limited to 4% of patients. This research was presented at the International Society of Heart and Lung Transplant in Prague (2012).
Statistics: Overall, 43 patients (29%) died or were transplanted. Within 1 year of diagnosis, 21 patients died, and only 4 underwent transplantation (3 on mechanical circulatory support). The 1-year survival was 85% (95% confidence interval [CI] = 79-91), and 5-year survival was 84% (95% CI = 78-90). Transplantation-free survival at 1 year was 82% (95% CI = 75-88) and at 5 years was 72% (95% CI = 64-80). Within 1 year of diagnosis, with death as the main end-point (21 of 25, 84%), intensive care unit admission (hazard ratio = 2.6, p = 0.05) and mechanical circulatory support (hazard ratio = 3.2, p = 0.03) were risk factors (multivariable Cox analysis); inotropic support was longer in patients reaching an end-point. At >1 year after diagnosis, with transplantation as the main end-point (15 of 18, 83%), age >6 years (hazard ratio = 6.1, p = 0.02) was a risk factor. There were 56 (38%) children who recovered, 50% within 1 year of diagnosis. Recovery was associated with younger age; was similar in patients with myocarditis (43%) and idiopathic disease (41%); and was similar in patients initially admitted to the intensive care unit, admitted to the ward, or treated as outpatients.