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General Reference ⬇

Nejm 2000;342:256

Pathophys and Cause ⬆ ⬇

Cause:Congenital malformations

Pathophys:Associated with PDA often, and bicuspid aortic valve (70-90%). Hypertension is renal via angiotensin (Nejm 1976;295:145)

Epidemiology ⬆ ⬇

5% of all CHD; male/female = 4:1

Signs and Symptoms ⬆ ⬇

Sx:Rarely sx (CHF, leg pains, headache) in childhood; CHF in 3rd-4th decades

Si:Hypertension in arms, normal or low BP in legs; decreased/delayed femoral pulses; systolic murmur (75%) in left upper back or pulmonic areas

Complications ⬆ ⬇

SBE on bicuspid aortic valve; intracranial bleeding; hypertensive encephalopathy; ruptured/dissected aorta; hypertensive cardiovascular disease

Lab and Xray ⬆ ⬇

Lab: Noninv:EKG normal, or LVH; if RVH, suggests a PDA beyond the coarc ("fetal type")

Xray:Chest, normal heart, and pulmonary vasculature; coarctation visible on plain chest occasionally; rib notching in older patients

Treatment ⬆

Rx:Surgical resection of coarcted segment and end-to-end aortic anastomosis ideally done at age 5-15 yr; operative mortalities >20% in infancy, <1% age 5-15 yr, 10% over age 30 yr; cmplc: spinal cord ischemia (rare); acute necrotizing arteritis of mesenteric vessels (rare); hypertension postop in most