Internal Medicine

(Wang) #1

0521779407-14 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:16


954 Marfan’s Syndrome Measles

■refraction for myopia
■psychological and genetic counseling

Specific Treatment
■Beta-blockers for all patients to reduce rate of aortic root dilatation
and risk of dissection as blood pressure tolerates

Side Effects and Contraindications
■side effects: fatigue, excessive bradycardia or hypotension, bron-
chospasm, depression, erectile dysfunction, insensitivity to hypo-
glycemic symptoms
■contraindications:
➣absolute: decompensated congestive heart failure, severe COPD,
heart block
➣relative: CHF, COPD, diabetes with frequent hypoglycemia
follow-up
During Treatment
■Annual EKG and echocardiogram to monitor aortic root size until
aortic root=45 mm, then more frequently
■Prophylactic aortic valve replacement and ascending aortic conduit
surgery if moderate aortic insufficiency, aortic root >50 mm, or aortic
root increases >1 cm/year

complications
■dilated aortic root in 50% of children and 80% of adults
■aortic dissection (usually ascending) in 44% at autopsy
■pregnancy increases risk of dissection; avoid if aortic root >40 mm
■nondissecting aortic aneurysms common

prognosis
■Mortality from untreated heart disease or aortic dissection
■20-year survival after aortic valve and root surgery 65%

MEASLES


CAROL A. GLASER, MD


history & physical
History
■RNA virus, morbilliform virus within the Paramyxovirus family
■Humans only host
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