Pediatric cardiomyopathy is a rare heart condition that affects infants and children. Specifically, cardiomyopathy means disease of the heart muscle (myocardium). Several different types of cardiomyopathy exist and the specific symptoms vary from case to case. In some cases, no symptoms may be present (asymptomatic); in many cases, cardiomyopathy is a progressive condition that may result in an impaired ability of the heart to pump blood; fatigue; heart block; irregular heartbeats (tachycardia); and, potentially, heart failure and sudden cardiac death.
Cardiomyopathy may be termed ischemic or nonischemic. Ischemic cardiomyopathy refers to cases that occur due to a lack of blood flow and oxygen (ischemia) to the heart. Such cases often result from hardening of the arteries (coronary artery disease). Nonischemic cardiomyopathy refers to cases that occur due to structural damage or malfunction of the heart muscle. Nearly all cases of pediatric cardiomyopathy are nonischemic. This report deals with nonischemic pediatric cardiomyopathy.
Cardiomyopathy may also be termed primary or secondary. Primary cardiomyopathy refers to cases where cardiomyopathy occurs by itself or for unknown reasons (idiopathic). Secondary cardiomyopathy refers to cases where the disease occurs secondary to a known cause such as heart muscle inflammation (myocarditis) caused by viral or bacterial infections; exposure to certain toxins such as heavy metals or excessive alcohol use; or certain disorders that affect the heart and/or additional organs systems.
According to the Pediatric Cardiomyopathy Registry, approximately 79 percent of pediatric cardiomyopathy cases occur for unknown reasons (idiopathic). Nonischemic cardiomyopathy may be further divided into four subtypes based upon the specific changes within the heart. These subtypes are: dilated, hypertrophic, restrictive and arrhythmogenic right ventricular dysplasia
Cardiomyopathy is a disease of the heart muscle. It affects people of all ages and is mostly inherited. It is not curable but can usually be treated successfully, with most of those affected going on to lead a long and full life.
There are several different types of cardiomyopathy three main types of cardiomyopathy – hypertrophic, dilated and arrhythmogenic right ventricular.
Increased awareness of the condition in recent years and recognition of subtle symptoms has helped early diagnosis and therefore early management. Without doubt, this can change the outcome of the condition and help to prevent complications. With the knowledge that the disease can be inherited, family screening has also contributed a great deal to early diagnosis. With knowledge of an existing or potential problem, lifestyle modifications and medication can be applied on time. Young people can be given appropriate advice when undergoing preventative screening and this is much better than treating an already severe condition.
Understanding of the condition
Understanding about the mechanisms and progression of heart dysfunction has significantly increased in the last few years. Studies have shown a number of mechanisms are activated when the heart function starts to go down and doctors are now trying to use these mechanisms in a beneficial way for the patient. In the context of a weak heart, the body’s reaction triggers compensatory mechanisms that can be helpful short term but damaging in the long term. Although it was previously thought that a weak heart needs medication that will make it beat more strongly, contemporary cardiology has been able to apply treatment that interferes with the more basic mechanisms.
An example of this is medication that prevents the heart condition from deteriorating further by keeping the heart rate and function mildly suppressed and under reasonable control. This is because studies have shown that the more you activate the failing heart, the worse it becomes in the long run. Whereas the more you protect the heart from its own compensatory reaction, the safer it is for the heart in the long term. So the philosophy in the management of heart dysfunction has changed over the years and isfocusing on the prognosis and prevention of complications alongside the immediate reduction of symptoms.