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Common Congenital Heart Lesions - page 11 / 126

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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.

Physical Findings:

  • 1.

    cyanosis with clubbing in digits

  • 2.

    prominent “a” wave in jugular venous pulsation

  • 3.

    Loud P2 (pulmonic closure) sound

  • 4.

    No murmur

Diagnostic Imaging and Testing:

  • A.

    Chest x-ray: proximal pulmonary artery dilation and calcification of pulmonary vasculature

  • B.

    ECG: RVH, RA enlargement

  • C.

    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.

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