Surgical repair of severe mitral valve regurgitation complicated by incomplete cor triatriatum

Abstract

A 69-year-old woman with exertional dyspnea was referred emergently to our hospital for further evaluation. Transthoracic echocardiography showed severe mitral valve regurgitation and moderate tricuspid regurgitation, which were thought to be the main cause of her heart failure. An electrocardiogram showed paroxysmal atrial fibrillation. Mitral and tricuspid repair and pulmonary vein isolation were scheduled. Intraoperative transesophageal echocardiography revealed a fibromuscular diaphragm and multiple ostia in the left atrium, strongly suggesting cor triatriatum. After left atriotomy, an incomplete transverse membrane was identified in the chamber. The membrane was resected and the mitral valve was repaired; then a tricuspid annuloplasty was performed, and the pulmonary veins were isolated bilaterally. Her postoperative course was uneventful. Cor triatriatum is a rare congenital anomaly, and in some cases is associated with mitral regurgitation. In patients with severe mitral regurgitation, we recommend preoperative transesophageal echocardiography to obtain a correct diagnosis. We should evaluate carefully moderate to severe mitral regurgitation without pulmonary hypertension or left atrial dilatation taking cor triatriatum into consideration. <Learning objective: Cor triatriatum is a rare congenital anomaly and in some cases is associated with mitral regurgitation. In patients with severe mitral regurgitation, preoperative transesophageal echocardiography is recommended to get a correct diagnosis. We should evaluate carefully moderate to severe mitral regurgitation without pulmonary hypertension or left atrial dilatation taking cor triatriatum into consideration.>

A Very Rare Case of Cor Triatriatum with Severe Mitral Regurgitation

“…Cor triatriatum is a very rare congenital heart disease. The incidence ranges 0.1 to 0.4% of heart congenital heart diseases [ 3 ] [ 4 ] [ 5 ] [ 6 ] [ 7 ] [ 8 ] [ 9 ] [ 10 ] [ 11 ]. Classic (or typical) cor triatriatum, or cor triatriatum sinister, is a rare congenital cardiac anomaly in which the pulmonary veins typically enter a “proximal” left atrial chamber separated from the “distal “ left atrial chamber by a diaphragm in which there are one or more restrictive ostia.…”

“…Another reference centre reports 28 cases in 23 years of experience [ 10 ] while the cases of surgical treatment in adults with concomitant severe mitral regurgitation are very rare. In our knowledge there are only 9 reported cases [ 3 ] [ 4 ] [ 8 ] [ 12 ] [ 13 ] [ 14 ] [ 15 ] [ 16 ].…”

Editorial: Cor triatriatum: An intraoperative diagnosis

“…In this issue of the journal, Umemura et al [4] report a case of cor triatriatum which was detected incidentally during mitral valve repair operation. Preoperative cardiac catheterization showed no evidence of pulmonary hypertension despite the presence of significant mitral regurgitation and poor left ventricular function.…”