A 48-year-old woman with recurrent syncope presented with acute left vision loss after another syncopal fall. She reported no prodromal symptoms and no prior syncope workup. Vital signs were normal. Ophthalmologic exam revealed left corneal ulcerations. Cardiopulmonary and neurologic examinations were unremarkable. Brain MRI, carotid ultrasound, serial troponins, telemetry, and EKG were normal. Transthoracic echocardiography uncovered a diagnosis of cor triatriatum, an obstructing patent membrane in a dilated, bisected left atrium (Fig. 1) with constricted blood flow and increased flow velocity across the restrictive orifice (Fig. 2).
Cor triatriatum represents only 0.1–0.4% of congenital cardiac malformations and has several anatomic variants.1,2 Ventricular inflow obstruction results from abnormal septation within the left or right atrium, creating two atrial chambers subdivided by a thin membrane. Many remain asymptomatic until their thirties. Most cases in adulthood are discovered incidentally.3,4,5 Clinicians should suspect cor triatriatum in young healthy patients with clinical features mimicking mitral stenosis but no cardiovascular co-morbidities. Early and severe manifestations occur in smaller communicating orifices and higher obstruction between bisected atrial chambers.1 Late symptoms results from progressive increase in pulmonary artery pressure.2 Syncope, heart failure, and sudden cardiac arrest are well cited.5,6 Early diagnosis by echocardiography is important to expedite surgical cure.2,7,8
Cor Triatriatum: Background, Pathophysiology, Epidemiology. Available at: http://emedicine.medscape.com/article/154168-overview#a6. Accessed February 8, 2018
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The authors wish to thank John Petersen, MD for his cardiology expertise and interpretation of the transthoracic echocardiographic images. This case was presented as a clinical vignette poster at the 40th Society of General Internal Medicine Annual Meeting on April 21, 2017.
Conflict of Interest
The authors declare that they do not have a conflict of interest.
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Joseph, I., Jester, G. & Lo, M.C. Cor Triatriatum as an Uncommon Cause of Recurrent Syncope. J GEN INTERN MED 33, 1201–1202 (2018). https://doi.org/10.1007/s11606-018-4429-3
- cor triatriatum
- cardiac anomaly
- congenital heart disease