Cor Triatriatum

http://www.100md.com 《新英格兰医药杂志》

     A 29-year-old man presented with severe dyspnea and hemoptysis. He said he had always had a reduced capacity for exercise as compared with his peers. He had an elevated jugular venous pressure, a right ventricular heave, a loud pulmonic second sound, and a grade 2 systolic ejection murmur at the left upper sternal border. An electrocardiogram (Panel A) showed sinus rhythm, right-axis deviation, left atrial enlargement, right ventricular hypertrophy with strain, and an S1Q3T3 pattern (an S wave in lead I and a Q wave and an inverted T wave in lead III). An echocardiogram (Panel B) showed normal left ventricular function, an enlarged and globally hypokinetic right ventricle, right atrial enlargement, and a membrane in the left atrium that extended from the intraatrial septum to the lateral wall of the left atrium (arrow). There was diastolic flow across this membrane at an elevated velocity (2.5 m per second). At cardiac catheterization, simultaneous measurements of the pulmonary-capillary wedge pressure (Panel C, arrow) and left ventricular diastolic pressure (arrowhead) showed a gradient of 18 mm Hg across the left atrial membrane. The pulmonary-artery pressure was 112/65 mm Hg. The coronary arteries were normal. The membrane was successfully resected. Six weeks after surgery, the patient was able to perform normal activities. A postoperative echocardiogram showed an estimated pulmonary-artery systolic pressure of 45 mm Hg.

    In cor triatriatum, the membrane within the left atrium results from failure of resorption of the common pulmonary vein. The membrane causes obstruction of pulmonary venous return, which leads to pulmonary hypertension and overloading of the right ventricle.

    Daniel B. Reese, M.D.

    Winchester Medical Center

    Winchester, VA 22601

    http://www.100md.com/html/DirDu/2006/11/29/30/55/07.htm