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General Reference

(Nejm 2000;342:339; Ann IM 1998;128:745)

Pathophys and Cause

Pathophys:Left-to-right shunting from ASD, VSD, or PDA

Signs and Symptoms

Sx:Dizziness

Si:Clubbing, cyanosis, RVH, pulmonary insufficiency murmur (Graham-Steell)

Course

45% 25-yr survival; worsened by higher altitudes

Complications

Thromboembolism (PE, CVA); high mortality w surgery or pregnancy; gout; gallstones; renal failure

Lab and Xray

Lab:Echo, Holter for arrythmias

Treatment

Rx:Phlebotomy to keep crit <60%; heart-lung transplant if medical management failing