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3. Eisenmenger Syndrome
Clinical Presentation: Exertional dyspnea and fatigue in person with congenital heart defect. May have symptoms of hyperviscosity syndrome. Can present with hemoptysis due to rupture of pulmonary vessels.
cyanosis with clubbing in digits
prominent “a” wave in jugular venous pulsation
Loud P2 (pulmonic closure) sound
Diagnostic Imaging and Testing:
Chest x-ray: proximal pulmonary artery dilation and calcification of pulmonary vasculature
ECG: RVH, RA enlargement
Echo+ Doppler: identifies underlying defect, estimates pulmonary artery systolic pressure
Treatment: avoid activities that increase right to left shunt (No exercise, high altitude, vasodilators, or pregnancy.) Antibiotic prophylaxis, supportive therapy. Only long-term treatment is heart-lung transplant.
Etiology: chronic left to right shunt through congenital heart defect causes severe pulmonary vascular obstruction, increased pulmonary pressure causes reversal of shunt, causing systemic cyanosis
Pathology: RVH, RA enlargment
Pathophysiology: increased pulmonary blood flow causes media to hypertrophy, decreases cross-sectional area of vascular bed. Vessels become thrombosed, increasing pulmonary vascular resistance causes reversal of shunt. Reduced hemoglobin saturation causes increase proliferation of bone marrow.