eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Cor Triatriatum: Differential Diagnoses & Workup

Author: M Silvana Horenstein, MD, Staff Physician, Department of Pediatrics, University of Texas Medical School Houston; Medical Doctor Consultant, Legacy Department, Best Doctors, Inc
Coauthor(s): Maria Victoria T Tantengco, MD, Associate Professor of Pediatrics, Division of Cardiology, Department of Pediatrics, University of Massachusetts Medical School; Medical Director, Echocardiography Laboratory, Child Heart Associates, LLC; Michael Pettersen, MD, Director of Echocardiography, Division of Cardiology, Children's Hospital of Michigan; Assistant Professor of Pediatrics, Wayne State University School of Medicine
Contributor Information and Disclosures

Updated: Jun 30, 2009

Differential Diagnoses

Mitral Stenosis, Supravalvular Ring
Partial Anomalous Pulmonary Venous Connection
Pulmonary Hypertension, Idiopathic
Pulmonary Hypertension, Persistent-Newborn
Pulmonary Hypoplasia
Total Anomalous Pulmonary Venous Connection

Other Problems to Be Considered

Mitral stenosis, valvar
Pulmonary vein stenosis

Workup

Laboratory Studies

  • No specific laboratory studies are indicated in patients with suspected cor triatriatum.

Imaging Studies

  • Chest radiography
    • Findings are usually nonspecific but may include pulmonary congestion with diffuse haziness or Kerley B lines and the ground glass pattern of acute pulmonary edema in hilar areas.
    • Patients may have mild cardiac enlargement and prominence of the pulmonary arterial segment.
    • The dilated proximal chamber may produce the appearance of left atrial enlargement.
    • Presence of an atrial septal defect or of an associated partial anomalous pulmonary venous connection adds pulmonary overcirculation to the pulmonary venous obstruction. The radiograph may then reveal significant right ventricular enlargement.
  • Echocardiography
    • Echocardiography is often sufficient for diagnosis and is the diagnostic modality of choice.

      Mean Doppler gradient of 7-8 mm Hg across left at...

      Mean Doppler gradient of 7-8 mm Hg across left atrial membrane indicating mildly elevated pulmonary venous pressures. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.

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      Mean Doppler gradient of 7-8 mm Hg across left at...

      Mean Doppler gradient of 7-8 mm Hg across left atrial membrane indicating mildly elevated pulmonary venous pressures. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.

    • The membrane dividing the left atrium can be visualized using 2-dimensional echocardiography, as can the presence of an associated atrial septal defect. The origin of each of the pulmonary veins should be identified to exclude the presence of anomalous pulmonary venous return.
    • The distinction between cor triatriatum and a supramitral ring should be made by the location of the left-atrial appendage. Differentiating between cor triatriatum and total anomalous pulmonary venous drainage to the coronary sinus may be difficult.
    • Common cardiac anomalies can also be demonstrated
    • Transesophageal echocardiography (TEE) and intracardiac echocardiography offer precise image definition and spatial relationship of the membrane.
    • TEE is very useful in larger and older patients in whom transthoracic images are suboptimal especially in visualizing the left atrium.4
  • Angiography
    • This test is generally indicated to assess pulmonary venous return and pulmonary arterial pressures. Because approximately 10% of patients have partial anomalous venous return, angiography is helpful in defining the precise venous anatomy.
    • When performed, catheterization generally reveals pulmonary hypertension in a degree that varies directly with the severity of obstruction to pulmonary venous drainage.5 Demonstration of a pressure gradient between the left atrium and capillary wedge pressure is classic.
    • The proximal chamber is visualized during the venous phase, and a delay then occurs before the true left atrium and left ventricle are visualized. The proximal chamber then remains opacified and does not contract with the distal chamber
  • Cardiac CT6 scanning and MRI7 : Both imaging modalities provide with very detailed anatomic images.8 MRI has the advantage of not subjecting the patient to radiation.9

Other Tests

  • ECG findings are nonspecific and may range from normal in asymptomatic older patients to mimicking those findings of a patient with the clinical picture of pulmonary hypertension.
  • In some patients, ECG may reveal the following:
    • Atrial arrhythmias5
    • Right-axis deviation
    • Right atrial enlargement
    • Right ventricular hypertrophy

Histologic Findings

  • Histology plays no part in the diagnosis; however, pulmonary hypertension results in well-defined structural changes.
  • Increased pulmonary arterial muscularity is present very early, with increased thickness of the arterial wall and extension of muscle into the arterioles.

More on Cor Triatriatum

Overview: Cor Triatriatum
Differential Diagnoses & Workup: Cor Triatriatum
Treatment & Medication: Cor Triatriatum
Follow-up: Cor Triatriatum
Multimedia: Cor Triatriatum
References
Further Reading
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References

  1. Bladt O, Vanhoenacker R. Cor triatriatum. JBR-BTR. Mar-Apr 2008;91(2):62. [Medline].

  2. Vaideeswar P, Tullu MS, Sathe PA, Nanavati R. Atresia of the common pulmonary vein--a rare congenital anomaly. Congenit Heart Dis. Nov-Dec 2008;3(6):431-4. [Medline].

  3. Ito M, Kikuchi S, Hachiro Y, Abe T. Congenital pulmonary vein stenosis associated with cor triatriatum. Ann Thorac Surg. Feb 2001;71(2):722-3. [Medline].

  4. Modi KA, Annamali S, Ernest K, Pratep CR. Diagnosis and surgical correction of cor triatriatum in an adult: combined use of transesophageal and contrast echocardiography, and a review of literature. Echocardiography. Jul 2006;23(6):506-9. [Medline].

  5. Yamada T, Tabereaux PB, McElderry HT, Doppalapudi H, Kay GN. Transseptal catheterization in the catheter ablation of atrial fibrillation in a patient with cor triatriatum sinister. J Interv Card Electrophysiol. Jun 2009;25(1):79-82. [Medline].

  6. Saremi F, Gurudevan SV, Narula J, Abolhoda A. Multidetector computed tomography (MDCT) in diagnosis of "cor triatriatum sinister". J Cardiovasc Comput Tomogr. Dec 2007;1(3):172-4. [Medline].

  7. Dillman JR, Yarram SG, Hernandez RJ. Imaging of pulmonary venous developmental anomalies. AJR Am J Roentgenol. May 2009;192(5):1272-85. [Medline].

  8. Su CS, Tsai IC, Lin WW, Lee T, Ting CT, Liang KW. Usefulness of multidetector-row computed tomography in evaluating adult cor triatriatum. Tex Heart Inst J. 2008;35(3):349-51. [Medline].

  9. Locca D, Hughes M, Mohiaddin R. Cardiovascular magnetic resonance diagnosis of a previously unreported association: Cor triatriatum with right partial anomalous pulmonary venous return to the azygos vein. Int J Cardiol. Jul 17 2008;[Medline].

  10. Spengos K, Gialafos E, Vassilopoulou S. Ischemic stroke as an uncommon complication of Cor triatriatum. J Stroke Cerebrovasc Dis. Nov-Dec 2008;17(6):436-8. [Medline].

  11. [Guideline] Paridon SM, Alpert BS, Boas SR, et al. Clinical stress testing in the pediatric age group: a statement from the American Heart Association Council on Cardiovascular Disease in the Young, Committee on Atherosclerosis, Hypertension, and Obesity in Youth. Circulation. Apr 18 2006;113(15):1905-20. [Medline].

  12. Bartel T, Muller S, Erbel R. Dynamic three-dimensional echocardiography using parallel slicing: a promising diagnostic procedure in adults with congenital heart disease. Cardiology. 1998;89(2):140-7. [Medline].

  13. Citro R, Bossone E, Provenza G, Patella MM, Gregorio G. Isolated left cor triatriatum: a rare cause of effort dyspnoea in the adult. J Cardiovasc Med (Hagerstown). Sep 2008;9(9):926-8. [Medline].

  14. Gharagozloo F, Bulkley BH, Hutchins GM. A proposed pathogenesis of cor triatriatum: impingement of the left superior vena cava on the developing left atrium. Am Heart J. Nov 1977;94(5):618-26. [Medline].

  15. Jeiger W, Gibbons JE, Wigglesworth FW. Cor triatriatum: Clinical, hemodynamic and pathologic studies: Surgical correction in early life. Pediatrics. 1963;31:255-64.

  16. Kerkar P, Vora A, Kulkarni H, et al. Percutaneous balloon dilatation of cor triatriatum sinister. Am Heart J. Oct 1996;132(4):888-91. [Medline].

  17. Kirklin JW, Barratt-Boyes BG. Cardiac Surgery. 2nd ed. Churchill Livingstone; 1993:675-81.

  18. Marini D, Ou P. Cor triatriatum in a newborn. Pediatr Radiol. Mar 10 2009;[Medline].

  19. McLean MK, Kung GC, Polimenakos A, Wells WJ, Reemtsen BL. Cor triatriatum associated with ASD and common atrium in 7-month-old with tachypnea and failure to thrive. Ann Thorac Surg. Dec 2008;86(6):1999. [Medline].

  20. Oglietti J, Cooley DA, Izquierdo JP, et al. Cor triatriatum: operative results in 25 patients. Ann Thorac Surg. Apr 1983;35(4):415-20. [Medline].

  21. Richardson JV, Doty DB, Siewers RD, Zuberbuhler JR. Cor triatriatum (subdivided left atrium). J Thorac Cardiovasc Surg. Feb 1981;81(2):232-8. [Medline].

  22. Rodefeld MD, Brown JW, Heimansohn DA, et al. Cor triatriatum: clinical presentation and surgical results in 12 patients. Ann Thorac Surg. Oct 1990;50(4):562-8. [Medline].

  23. Salomone G, Tiraboschi R, Bianchi T, et al. Cor triatriatum. Clinical presentation and operative results. J Thorac Cardiovasc Surg. Jun 1991;101(6):1088-92. [Medline].

  24. Spencer FC, Sabiston DC. Surgery of the Chest. 6th ed. WB Saunders; 1995:1420-4.

  25. Sritippayawan S, Margetis MF, MacLaughlin EF, et al. Cor triatriatum: a cause of hemoptysis. Pediatr Pulmonol. Nov 2002;34(5):405-8. [Medline].

  26. Su CS, Tsai IC, Lin WW, Lee T, Ting CT, Liang KW. Usefulness of multidetector-row computed tomography in evaluating adult cor triatriatum. Tex Heart Inst J. 2008;35(3):349-51. [Medline].

  27. Tantibhedhyangkul W, Godoy I, Karp R, Lang RM. Cor triatriatum in a 70-year-old woman: role of transesophageal echocardiography and dynamic three-dimensional echocardiography in diagnostic assessment. J Am Soc Echocardiogr. Aug 1998;11(8):837-40. [Medline].

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Keywords

cor triatriatum, cor triatriatum sinister, atrial septal defect, ASD, persistent left superior vena cava with an unroofed coronary sinus, partial anomalous pulmonary venous connection, ventricular septal defect, VSD, tri-atrial heart, subdivided atrium, accessory atrium, supravalvular mitral stenosis, congestive heart failure, total anomalous pulmonary venous drainage, TAPVD, pulmonary venous obstruction, respiratory distress, pulmonary hypertension, pulmonary insufficiency, rales, right-sided heart failure, hepatomegaly, treatment, diagnosis

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Contributor Information and Disclosures

Author

M Silvana Horenstein, MD, Staff Physician, Department of Pediatrics, University of Texas Medical School Houston; Medical Doctor Consultant, Legacy Department, Best Doctors, Inc
M Silvana Horenstein, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Maria Victoria T Tantengco, MD, Associate Professor of Pediatrics, Division of Cardiology, Department of Pediatrics, University of Massachusetts Medical School; Medical Director, Echocardiography Laboratory, Child Heart Associates, LLC
Maria Victoria T Tantengco, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Society of Echocardiography, Massachusetts Medical Society, and Society of Pediatric Echocardiography
Disclosure: Nothing to disclose.

Michael Pettersen, MD, Director of Echocardiography, Division of Cardiology, Children's Hospital of Michigan; Assistant Professor of Pediatrics, Wayne State University School of Medicine
Michael Pettersen, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, and American Society of Echocardiography
Disclosure: Nothing to disclose.

Medical Editor

Juan Carlos Alejos, MD, Clinical Professor, Department of Pediatrics, Division of Cardiology, University of California at Los Angeles
Juan Carlos Alejos, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, and International Society for Heart and Lung Transplantation
Disclosure: Actelion Honoraria Speaking and teaching

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Ameeta Martin, MD, Clinical Associate Professor, Department of Pediatric Cardiology, University of Nebraska College of Medicine
Ameeta Martin, MD is a member of the following medical societies: American College of Cardiology
Disclosure: Nothing to disclose.

CME Editor

Gilbert Z Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center
Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD, Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin
Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

 
 
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