Yesterday, I performed an echo on an adult female, whose child (age 14) had suffered from a fatal arrhythmia that almost cost the child her life. Fortunately, family members knew CPR and kept her alive until the Paramedics arrived.
We’ve all heard about young athletes that die suddenly during strenuous exercise; I’ve written about this in previous blogs and it is usually due to anomalous coronary arteries, IHSS (idiopathic hypertrophic sub aortic stenosis). In other words, during exercise the septum is so enlarged that blood cannot flow into the aorta.
Congenital arrhythmias are a little different. This may involve a buildup of fatty-fibrous tissue that begins in the right ventricle and tends to spread to the rest of the heart, usually over a long period of time. Eventually, this fatty-fibrous tissue tends to interfere with the conduction system of the heart, leading to PVC’S or other conductivity issues.
This can be imaged by echocardiography and usually presents itself as a right ventricular cardiomyopathy. Furthermore, if it spreads to the left ventricle it may appear as a dilated cardiomyopathy.
Unfortunately, sudden death (fatal arrhythmia) may occur at any stage of the disease. This is typically an inherited disease. If you are doing an echo on one of these patients, look for right ventricular cardiomyopathy or involvement that includes the septum and the infundibulum (RVOT).
Also, look for left heart involvement. If you find left heart involvement, this disease is in its advanced stage.
Aneurysms are commonly seen in the ventricles, along with thinning of the ventricular wall.
If your child (or patient) is suffering from syncope or abnormal arrhythmias, it is important that a pediatric cardiologist should be consulted.
Treatment of patients with this disease are often given anti-arrhythmic agents, such as beta-blockers. In advanced stages, the patient should be treated as if they have advanced cardiac failure.
Unlike exercise-induced death, these patients tend to die at rest, from a fatal arrhythmia.
Treatment generally involves the implantation of a defibrillator.
Ken Heiden, (RDCS, RVT)