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Congenital heart disease characterized by the association of an atrial septal defect with a mitral stenosis (left-to-right shunt at the atrial level).

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Lutembacher Disease.

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First reported by anatomist Johann Friedrich Meckel in a letter to Albrecht von Haller in 1750.

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Uncommon. Prevalence among cases of atrial septal defect is estimated to be 4%. Prevalence among cases of mitral stenosis is estimated to be 0.6 to 0.7%. Occurs more frequently in females than in males.

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Unknown. A reported kindred suggested autosomal dominant inheritance with almost complete penetrance. The condition is thought to represent a disorder of midline development.

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Lutembacher syndrome is classically described as a secundum atrial septal defect associated with mitral stenosis. The mitral stenosis is often rheumatic rather than congenital in origin. An atrial septal defect may be protective in mitral stenosis by providing a conduit for decompression of the left atrium and pulmonary venous system. However, mitral stenosis worsens the prognosis of an uncomplicated atrial septal defect by increasing right ventricular work and pulmonary blood flow, resulting in pulmonary hypertension. Central venous pressure is elevated in this syndrome.

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Clinical examination suggests the diagnosis and echocardiography confirms the diagnosis.

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Patients may not become symptomatic until adult life. Symptoms include dyspnea, orthopnea, paroxysmal nocturnal dyspnea, decreased exercise tolerance, and palpitations. There may be signs of right ventricular failure. A loud S1 (mitral stenosis) with fixed splitting of S2 (atrial septal defect) is usually heard. A diastolic parasternal murmur may be heard and represents increased flow across the tricuspid valve. Electrocardiographic findings include atrial flutter, atrial fibrillation, incomplete or complete right bundle branch block, right ventricular hypertrophy, and right-axis deviation. The chest radiograph demonstrates right atrial enlargement and prominent pulmonary vasculature and may show signs of pulmonary venous congestion.

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Full history and examination to establish cardiac and pulmonary reserve. Chest radiograph to exclude pulmonary edema. Preoperative ECG and review of recent echocardiographic examinations. Laboratory investigations as indicated by surgical procedure and patient medications.

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Maintain left ventricular preload. Avoid increases in systemic vascular resistance. Avoid tachycardia, which will decrease time for left ventricular filling through the stenotic valve. Maintain ventricular contractility. Use a technique to prevent an increase in pulmonary vascular resistance and potential reversal of the left-to-right shunt. Strict precautions regarding air in intravenous lines because of increased risk of paradoxical embolism.

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Anesthetic agents favoring a sympathomimetic discharge (e.g., pancuronium, desflurane) and anticholinergic actions (e.g., atropine) are relatively contraindicated. Consider need for antibiotic prophylaxis against bacterial endocarditis.

Megarbane A, Stephan E, Kassab R, et al: Autosomal dominant secundum atrial septal defect with various cardiac and noncardiac defects: A new midline disorder. Am J Med Genet 83:193, 1999.  [PubMed: 10096596]
Steinbrunn W, Cohn K, Selzer A: Atrial septal defect associated with mitral stenosis. The Lutembacher syndrome revisited. Am J ...

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