Cardiomyopathy - The Facts
1. Different forms of the disease. Cardiomyopathy is a chronic disease of the heart muscle that can present in four forms and may in severe cases lead to heart failure and/or sudden death. Dilated and hypertrophic Cardiomyopathy are the most common.
2. Can affect any child. Cardiomyopathy predominately affects adults but in rare instances does affect infants and children. Cardiomyopathy can occur in any child regardless of age, race, gender or socioeconomic background. Recent studies show that children are about 10 times more likely to develop Cardiomyopathy during the first year of life than ages 2 to 18 combined.
3. Search for a cure continues. Unlike other congenital heart conditions, there is no surgical treatment or cure that can repair the damaged heart or the stop the progression of the disease. The first treatment option is usually medication to improve the functioning of the heart and for those with arrhythmia (irregular heart rhythm) an implantable defibrillator may be recommended. For children that fail medical management, a heart transplant may need to be considered.
Cardiomyopathy is the top reason for heart transplants in children.
4. Few medical breakthroughs for paediatric Cardiomyopathy. Since Cardiomyopathy was first recognized in 1950, there have been few medical breakthroughs in understanding this complex disease. Surprisingly, the outcome for children with Cardiomyopathy is the same now as it was three decades ago. Most of the research to date has been performed on adults with a strong family history of the disease. However, only 20-30% of children with Cardiomyopathy have a family history of the disease.
Paediatric Cardiomyopathy Defined
With the paediatric population, Cardiomyopathy occurs in approximately 12 children out of every million with about 1,000-5,000 new cases diagnosed each year. The majority of diagnosed children are infants under the age of 12 months with fewer cases between the ages of 1 to 12 years. When Cardiomyopathy is diagnosed in children before puberty, it is considered extremely unusual and it may not have the same causes, manifestations or disease progression as Cardiomyopathy in adults.
Unfortunately, there has been little research and focus on paediatric Cardiomyopathy over the years.
When children are diagnosed at an early age, the prognosis may be poor depending on the form of Cardiomyopathy and the stage of the disease. For example, dilated Cardiomyopathy can progress quite rapidly when diagnosed in young children. Up to 40% of diagnosed children with dilated Cardiomyopathy fail medical management within the first year of diagnosis and of those that survive many have permanently impaired heart function.
Mortality and heart transplant rates of childhood cardiomyopathies are much higher than in adults due to the rarity and uncertainty of the disease. Less than optimal outcomes may be attributed to the more fragile state of infants and young children or it may be a function of the disease's advanced progression associated with another genetic disorder.
Another unfortunate reason is that Cardiomyopathy is not usually detected until the end stage when obvious symptoms of heart failure are apparent. Cardiomyopathy can be easily missed in routine check-ups when there are no obvious symptoms (i.e. heart murmur) or when there is no reason for diagnostic testing (i.e. no family history of the disease).
Permission to re-print kindly given by The Children's Cardiomyopathy Foundation
