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Combined Texas Heart Institute and Texas Childrens Hospital case presentation:

Cor Triatriatum
Brian Walton

History of Present Illness

50 year old white male with atrial fibrillation for 20 years, cor triatriatum and mitral regurgitation presents with complaints of fatigue. He remains active but has noted increased fatigue following his usual activities and more frequent rest breaks during exercise. He denies dyspnea at rest or chest discomfort. He was found to have atrial fibrillation approximately 20 years ago during a medical evaluation following a bicycle accident. He has had 4 attempted cardioversions and has been treated with a variety of anti-arrhythmic agents without success. The patient was noted to have cor triatriatum by echocardiography over a decade ago and has been followed expectantly. During a routine follow up echo several years ago he was found to have mitral regurgitation that has progressively worsened.

Past Medical History

  • Atrial fibrillation diagnosed 20 years ago
  • Cor triatriatum diagnosed 10 years ago
  • Mitral regurgitation diagnosed 1.5 years ago

Past Surgical History

Reconstruction of his left clavicle

Medications

  • Digoxin
  • Vasoretic
  • Coumadin

Patient Exam

Normal S1 S2 with III/VI SEM at apex

Hospital Course

Patient underwent successful resection of his cor triatriatum membrane, repair of his mitral valve and Maze cryoablation procedure. He tolerated the procedure well and had an uneventful post-operative course.

Transesophageal echo showing cor triatriatum and mitral regurgitation (856 KB).

Discussion

Cor triatriatum is a rare congenital anomaly first described by Church1 in 1868 and occurs in 0.1% of children with congenital heart disease2. This anomaly is comprised of a common pulmonary venous chamber that receives drainage from all four pulmonary veins but is separated from the true left atrium by a membrane.  This membrane may have an ostia connecting the common pulmonary venous chamber to the left atria that can be variable in size and location. Less frequently this membrane has no atrial ostia and instead empties via an atrial septal defect. Echocardiography is crucial to the appropriate diagnosis and differentiation from other congenital defects.

Patients may present with complaints of dyspnea and clinical evidence of heart failure or may be asymptomatic at the time of incidental discovery. Van Son et al3 reported that patients with a restrictive membrane ostia and large left atrial gradient required surgical attention earlier in life than patients with less restrictive ostia and smaller gradients. Several centers2-4 have reported short term and long term clinical success following surgical resection of the membrane and any associated atrial septal defects. Correct preoperative diagnosis and early surgical intervention offers patients with cor triatriatum favorable long-term results.

References

1. Church WS. Congenital malformation of the heart; abnormal septum in the left auricle. Trans Pathol Soc Lond 1868;19:415-20.

2. Rodefeld MD et al. Cor triatriatum: clinical presentation and surgical results in 12 patients. Ann Thorac Surg. 1990;50:562-8.

3. Van Son JAM et al. Cor triatriatum: diagnosis, operative approach, and late results. Mayo Clin Proc. 1993;68:854-859.

4. Salomone G et al. Cor triatriatum: clinical presentation and operative results. J Thorac Cardiovasc Surg. 1991;101:1088-92.

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