Pediatric emergency medicine trisk 1744 1744
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TABLE 86.10
ETIOLOGIES OF HYPERTROPHIC CARDIOMYOPATHY
Autoimmune disease
Nutritional deficiencies
Endocrine dysfunction
Infiltrative diseases
Toxins
Drugs
Other
Selenium, carnitine, thiamine
GH deficiency, thyroid disease,
hypocalcemia, diabetes mellitus,
pheochromocytoma
Glycogen storage disease,
hemochromatosis, amyloidosis
Cobalt, lead
Alcohol, sympathomimetics,
anthracyclines
Chronic tachycardia, muscular
dystrophies
Restrictive Cardiomyopathy (RCM) is due to increased stiffness of the
myocardium leading to a rise in ventricular filling pressures. RCM is the least
common type of cardiomyopathy, although patients with HCM and end-stage
DCM may develop restrictive physiology. RCM is commonly idiopathic or
familial with predominately autosomal dominant inheritance. Other causes of
RCM are rheumatologic (sarcoidosis, scleroderma), infectious (parasitic),
infiltrative (glycogen storage disease, hemochromatosis, amyloidosis), carcinoid,
nutrition, and drugs. A gallop and loud P2 may be appreciated on cardiac
examination. The EKG commonly has abnormal right or left atrial enlargement,
ST depression, and ST–T wave abnormalities. Cardiomegaly and pulmonary
venous congestion will be noted on CXR. ECHO is diagnostic for RCM. These
patients require adequate preload and do not tolerate dehydration well.
Unclassified cardiomyopathies such as Left Ventricular Noncompaction
(LVNC) are now being recognized. In LVNC the heart has prominent LV
trabeculae and deep intertrabecular recesses. LVNC occurs independent of or in
conjunction with CHD such as Ebstein anomaly, complex heart disease, or
neuromuscular disease. LVNC may be transient in neonates. It may be associated
with SCD.
MYOCARDITIS
