Dilated Cardiomyopathy (DCMP)
This is a rare disease in pediatric age groups.
Causes of DCMP
Anomalous origin of the left coronary artery (ALCAPA), myocarditis common causes include viral infection with coxachie, echo, HIV, measles, mumps and rubella etc.
Sometimes, cause is familial with autosomal dominant, recessive and x-linked inheritance describes.
Systemic causes: can be metabolic, endocrine, storage, mitochondrial and connective tissue disorders
If DCMP is severe then child is generally in symptomatic heart failure. Child presents with difficulty in breathing, cough, cold, poor feeding, edema over feet etc.
Chest x-ray shows cardiomegaly, increased pulmonary vascularity due to fluid overload in lungs alveoli, in association with liver enlargement hepatomegaly, raises doubt of DCMP.
Transthoracic echocardiography confirms the diagnosis by showing dilatation of cardiac chambers, mitral regurgitation, decreased cardiac pumping function.
In pediatric age groups, coronary arteries should be assessed very carefully as abnormalities in coronary arteries also leads to similar picture.
ECG differentiates coronary abnormalities cause from non-coronary causes of DCMP. Generally ECG shows sinus tachycardia, left atrial enlargement etc.
This gives symptomatic relief. Drugs that commonly used are diuretic, ACE inhibitors, B-blockers. Digoxin should be used very carefully in case of myocarditis.
DCMP patients are very prone for arrhythmias so anti-arrhythmic drugs are also used whenever required.
Poor LV function is always at risk for blood clot formation in cardiac chambers so anti-clot forming drugs like aspirin is also used wherever indicated.
Mechanical and surgical treatment
Left ventricular assist devices (LVAD) and biventricular assist devices, although not widely available and quite expensive will have a role at least in short term, and act as a bridge to heart transplant.
Cardiac Transplant:indicated in end stage heart failure.
Death due to DCMP is highest in first year after diagnosis due to intractable heart failure. Late deaths are due to arrhythmia,
Kawasaki disease is a disease that affects skin, mouth, and lymph nodes. Most commonly affected age group is around 5 years of age.
Etiology of this disease is not known.
Clinical features are divided into groups:
Acute phase: Last up to 2 weeks approximately. Most common symptom in this phase is fever that usually last for 5 days.
Other symptoms may be:
- Redness in the eyes
- Red, dry, cracked lips
- Rash on the body
- Swollen lymph nodes
- Swollen tongue with a white coating and big red bumps (called "strawberry tongue")
Second Phase: Usually begins within 2 weeks of when the fever started, begins with peeling of skin. Other symptoms can be joint pain, abdominal pain, diarrhea.
Kawasaki disease diagnosed when child with fever lasting 5 or more days and at least four of these symptoms:
- redness in both eyes
- a rash in the chest, stomach, or genital area
- a large swollen lymph node in the neck
- changes around the lips, tongue, or mouth
- changes in the fingers and toes, such as swelling, color change, or peeling
- red, swollen palms of hands and soles of feet
Treatment should be started as soon as possible, ideally within 10 days of when the fever begins. It is by giving IV Immunoglobulin along with high dose of aspirin. Later on low dose aspirin to be continued for a long duration depending on how much coronary artery are affected.
Most child recover completely.
If not treated properly especially not treated in first 10 days of illness, Kawasaki disease may affect heart especially coronary arteries. Can leads to vasculitis of coronary arteries.
Cardiac arrhythmia or abnormal functioning of some heart valves also can occur.
In pediatric age groups, cardiac diseases are mostly present since birth (congenital heart diseases).
But, few cardiac issues can be thickening of cardiac muscles (hypertrophic cardiomyopathy) or decreased or restricted filling of cardiac chambers (restricted cardiomyopathy).