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Journal of General Internal Medicine

, Volume 33, Issue 7, pp 1201–1202 | Cite as

Cor Triatriatum as an Uncommon Cause of Recurrent Syncope

  • Islande Joseph
  • Grant Jester
  • Margaret C. LoEmail author
Clinical Practice: Clinical Images

KEY WORDS

cor triatriatum syncope cardiac anomaly congenital heart disease 
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).
Fig. 1

Transthoracic echocardiographic image (apical 4-chamber view) displaying a thin, linear membrane (orange arrow), traversing and dividing the left atrium into two abnormal left atrial chambers (labeled as LA 1 proximally and LA 2 distally). LA left atrium, LV left ventricle, RA right atrium, RV right ventricle

Fig. 2

Transthoracic echocardiographic image (apical 2-chamber view, color-flow Doppler mode) demonstrating constricted blood flow and increased flow velocity (orange arrow) across the restricted opening in the left atrium caused by the cor triatriatum

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

Notes

Acknowledgements

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.

Compliance with Ethical Standards

Conflict of Interest

The authors declare that they do not have a conflict of interest.

References

  1. 1.
    Cor Triatriatum: Background, Pathophysiology, Epidemiology. Available at: http://emedicine.medscape.com/article/154168-overview#a6. Accessed February 8, 2018
  2. 2.
    Eichholz JL, Hodroge SS, Crook JJ, Mack JW Jr, Wortham DC. Cor triatriatum sinister in a 43-year-old man with syncope. Tex Heart Inst J. 2013;40(5):602–5.PubMedPubMedCentralGoogle Scholar
  3. 3.
    Mamunur Rashid AKM, Wahab MA, Haque M, Goswami BC. A Rare Congenital Heart Disease – Cor Triatriatum. Int J Case Rep Med 2013;2013:1–7. Article ID 802275.  https://doi.org/10.5171/2013.802275
  4. 4.
    Hamdan R, Mirochnik N, Celermajer D, Nassar P, Iserin L. Cor Triatriatum Sinister diagnosed in adult life with three dimensional transesophageal echocardiography. BMC Cardiovasc Disord. 2010;10:54.CrossRefGoogle Scholar
  5. 5.
    Baris L, Bogers AJJC, van den Bos EJ, Kofflard MJM. Adult cor triatriatum sinistrum: A rare cause of ischaemic stroke. Neth Heart J. 2017;25(2):217–220.CrossRefGoogle Scholar
  6. 6.
    Jha A, Makhija N. Cor Triatriatum: A review. Semin Cardiothorac Vasc Anesth. 2017;21(2):178–185.CrossRefGoogle Scholar
  7. 7.
    Saxena P, Burkhart HM, Schaff HV, Daly R, Joyce LD, Dearani JA. Surgical repair of cor triatriatum sinister: the Mayo Clinic 50-year experience. Ann Thorac Surg. 2014;97(5):1659–63.CrossRefGoogle Scholar
  8. 8.
    Isik O, Akyuz M, Ayik MF, Levent E, Atay Y. Cor triatriatum sinister: a case series. Turk Kardiyol Dern Ars. 2016;44(1): 20–23.PubMedGoogle Scholar

Copyright information

© Society of General Internal Medicine 2018

Authors and Affiliations

  1. 1.Department of Medicine University of Florida College of MedicineGainesvilleUSA

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