Winship Viljoen Leary syndrome
Overview
A syndrome that is characterised by microcephaly with mental retardation and cardiomyopathy.
Symptoms
* Small for age head * Microcephaly * Mental retardation * Learning difficulties * Concentration difficulties
Treatment
, diuretics, oxygen, and a sodium-restricted diet. Other options may involve bed rest and steroids. Vasodilators reduce preload and afterload, thereby decreasing congestion and increasing cardiac output. Acute heart failure requires vasodilation with nitroprusside or nitroglycerin I.V. Long-term treatment may include prazosin, hydralazine, isosorbide dinitrate, angiotensin-converting enzyme inhibitors, and anticoagulants. When these treatments fail, therapy may require a heart transplant for carefully selected patients. Cardiomyoplasty, which wraps the latissimus dorsi muscle around the ventricles, assists the ventricle to effectively pump blood. A cardiomyostimulator delivers bursts of electrical impulses during systole to contract the muscle. Therapeutic goals include correcting the underlying causes and improving the heart’s pumping ability with digoxin, a diuretic, oxygen, and a sodium-restricted diet. Other options may involve bed rest and a steroid. Vasodilators reduce preload and afterload, thereby decreasing congestion and increasing cardiac output. Acute heart failure requires vasodilation with I.V. nitroprusside or I.V. nitroglycerin. Long-term treatment may include hydralazine, isosorbide dinitrate, an angiotensin-converting enzyme inhibitor, and an anticoagulant. When these treatments fail, therapy may require heart transplantation, though only for carefully selected patients. Cardiomyoplasty, which wraps the latissimus dorsi muscle around the ventricles, helps the ventricle to effectively pump blood. A cardiomyostimulator delivers bursts of electrical impulses during systole to contract the muscle. Give your patient specific care instructions prior to his dimissal. (See Discharge instructions.)