eMedicine Specialties > Cardiology > Congenital Heart Disease in the Adult

Cor Triatriatum: Differential Diagnoses & Workup

Author: Jamshid Shirani, MD, FACC, FAHA, Consulting Staff, Director of Cardiovascular Fellowship Program, Department of Medicine, Division of Cardiology, Geisinger Medical Center
Coauthor(s): Arun Kalyanasundaram, MD, MPH, Interventional Cardiology Fellow, Department of Cardiology, Cleveland Clinic; Kamal K Pourmoghadam, MD, Associate Professor, Department of Cardiothoracic Surgery, Jefferson Medical College; Director of Pediatric Cardiac Surgery, Department of Surgery, Janet Weis Children's Hospital, Geisinger Medical Center
Contributor Information and Disclosures

Updated: Jul 15, 2009

Differential Diagnoses

Atrial Myxoma
Mitral Stenosis
Pericarditis, Constrictive
Pulmonary Hypertension, Primary
Pulmonary Hypertension, Secondary
Tricuspid Stenosis

Other Problems to Be Considered

Diagnosis of cor triatriatum is frequently made with considerable delay due to rarity of the condition and presenting signs and symptoms that mimic other more common cardiac or pulmonary disorders.

Cough and hemoptysis may be attributed to other potential causes such as bronchitis, pulmonary tuberculosis, or malignancy.

Cor triatriatum sinistrum

Bronchial asthma
Pulmonary vein stenosis
Pulmonary veno-occlusive disease
Supravalvular mitral ring
Atrial tumors

Cor triatriatum dextrum

Prominent Chiari network
Right atrial tumor (particularly right atrial myxoma)
Inferior vena cava (obstruction) syndrome

Workup

Laboratory Studies

Cor triatriatum sinistrum

  • Electrocardiography
    • Sinus rhythm
    • Frequent atrial premature complexes
    • Left and/or right atrial abnormality
    • Right axis deviation
    • Right ventricular hypertrophy and strain pattern
  • Right heart catheterization
    • Elevated right atrial, right ventricular, pulmonary arterial, and pulmonary artery wedge pressure
      • Right atrial mean pressure greater than 5 mm Hg
      • Right ventricular pressure greater than 30/5 mm Hg
      • Pulmonary arterial pressure greater than 30/12 mm Hg
      • Pulmonary artery wedge (left atrial) pressure greater than 12 mm Hg
    • Prominent V wave in right atrial pressure tracing (due to tricuspid regurgitation)
  • Left heart catheterization
    • Normal left ventricular and central aortic pressure
    • Systemic hypotension may be present if the left atrial membrane is restrictive and stroke volume is reduced as the result.
  • Coronary angiography
    • Coronary artery disease or coronary artery anomalies may be independently found but are not a part of the usual presentation of cor triatriatum.

Cor triatriatum dextrum

There are no pathognomonic electrocardiographic findings in isolated cor triatriatum dextrum. Right heart catheterization may reveal elevated pressure in the proximal right atrial chamber with a gradient across the accessory membrane.

Imaging Studies

Cor triatriatum sinistrum

  • Chest radiograph
    • Cardiomegaly
    • Pulmonary congestion
    • Prominent pulmonary arteries
    • Pleural effusion
  • Angiography: Angiographic diagnosis has been reported.
    • Successfully establishes diagnosis in about 50% of the cases
    • May help determine the severity of obstruction to left ventricular filling and assess magnitude of pulmonary hypertension
  • Echocardiography
    • Echocardiography is the most commonly used imaging technique for the diagnosis of cor triatriatum. Although the diagnosis is commonly suspected on transthoracic study, transesophageal echocardiography (TEE) is frequently needed to precisely define the anatomy of the membrane, its relation to other structures, and the pulmonary venous drainage pattern. Echocardiography allows the following:
      • Assessment of atrial morphology
      • Evaluation of characteristic anatomic relations
      • Determination of resultant flow disturbances
      • Detection of associated anomalies
      • Assessment of hemodynamic significance of the lesion
    • The typical cor triatriatum sinistrum appears as a membrane attached laterally to the junction of the left upper pulmonic vein and left atrial appendage, dividing the left atrium into 2 chambers. The proximal chamber receives blood from the pulmonary veins and the distal chamber contains the left atrial appendage and mitral valve. One or more fenestrations of varying sizes connect the 2 chambers.
    • Three-dimensional reconstruction of echocardiographic images has been used to better define the membrane and its relationship to surrounding structures.
    • Intracardiac echocardiography has been shown to have demonstrated presence of cor triatriatum in 1 patient.
  • Both computerized tomography of the heart and cardiac magnetic resonance imaging (MRI) have been used for evaluating patients with suspected cor triatriatum. The results are multiple case reports of successful diagnosis.

Frequently reported associated findings on cardiac imaging include the following:

  • Patent foramen ovale
  • Atrial septal defect (secundum-type and primum-type)
  • Partial anomalous pulmonary venous return
    • Also called subtotal cor triatriatum sinister
    • Occurs in nearly one fourth of the cases
    • The pulmonary veins may drain into the coronary sinus, superior vena cava, left superior vena cava, directly into the right atrium, or into the innominate vein.
  • Left ventricular dilation (due to chronic mitral regurgitation of poorly defined cardiomyopathy)
  • Right ventricular dilation
  • Pulmonary hypertension
  • Tricuspid regurgitation
  • Persistent left superior vena cava with or without unroofed coronary sinus
  • Partial or complete atrioventricular canal defect
  • Mitral regurgitation
  • Ascending aortic aneurysm with or without dissection or aortic regurgitation

Cor triatriatum dextrum

In general, advanced cardiac imaging (transthoracic and transesophageal echocardiography, cardiac MRI, and right ventriculography) demonstrates the presence of a membrane within the right atrium and may also be diagnostic of other concomitant congenital or acquired cardiac abnormalities.

More on Cor Triatriatum

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

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Keywords

cor triatriatum, heart with 3 atria, triatrial heart, subdivided left atrium, cor triatriatum sinister, cor triatriatum sinistrum, cor triatriatum dexter, tetralogy of Fallot, double outlet right ventricle, coarctation of the aorta, common atrioventricular canal, Chiari network, patent foramen ovale, atrial septal defect, partial anomalous pulmonary venous return, left ventricular dilation, right ventricular dilation, pulmonary hypertension, tricuspid regurgitation, persistent left superior vena cava, partial atrioventricular canal defect, complete atrioventricular canal defect, mitral regurgitation, ascending aortic aneurysm

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Contributor Information and Disclosures

Author

Jamshid Shirani, MD, FACC, FAHA, Consulting Staff, Director of Cardiovascular Fellowship Program, Department of Medicine, Division of Cardiology, Geisinger Medical Center
Jamshid Shirani, MD, FACC, FAHA is a member of the following medical societies: American Association for the Advancement of Science, American College of Cardiology, American College of Physicians, American Federation for Medical Research, American Heart Association, American Society of Echocardiography, and Association of Subspecialty Professors
Disclosure: Nothing to disclose.

Coauthor(s)

Arun Kalyanasundaram, MD, MPH, Interventional Cardiology Fellow, Department of Cardiology, Cleveland Clinic
Arun Kalyanasundaram, MD, MPH is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Heart Association, Society for Cardiac Angiography and Interventions, Society of General Internal Medicine, Society of Hospital Medicine, and Southern Medical Association
Disclosure: Nothing to disclose.

Kamal K Pourmoghadam, MD, Associate Professor, Department of Cardiothoracic Surgery, Jefferson Medical College; Director of Pediatric Cardiac Surgery, Department of Surgery, Janet Weis Children's Hospital, Geisinger Medical Center
Kamal K Pourmoghadam, MD is a member of the following medical societies: American College of Surgeons, Phi Beta Kappa, Sigma Xi, and Society of Thoracic Surgeons
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

Frank M Sheridan, MD, Cardiology, Providence Everett Medical Center
Frank M Sheridan, MD is a member of the following medical societies: American College of Cardiology, American Heart Association, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

CME Editor

Amer Suleman, MD, Consultant in Electrophysiology and Cardiovascular Medicine, Department of Internal Medicine, Division of Cardiology, Medical City Dallas Hospital
Amer Suleman, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Institute of Stress, American Society of Hypertension, Federation of American Societies for Experimental Biology, Royal Society of Medicine, and Society of Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

Chief Editor

Park W Willis IV, MD, Sarah Graham Distinguished Professor of Medicine and Pediatrics, University of North Carolina at Chapel Hill School of Medicine
Park W Willis IV, MD is a member of the following medical societies: American Society of Echocardiography
Disclosure: Nothing to disclose.

 
 
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