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Cor Triatriatum Workup

  • Author: Jamshid Shirani, MD; Chief Editor: Park W Willis IV, MD  more...
 
Updated: Dec 18, 2014
 

Laboratory Studies

Cor triatriatum sinistrum

Electrocardiography

Electrocardiographic (ECG) findings include the following:

  • Sinus rhythm
  • Frequent atrial premature complexes
  • Left and/or right atrial abnormality
  • Right axis deviation
  • Right ventricular hypertrophy and strain pattern

Right heart catheterization

Findings include elevated right atrial, right ventricular, pulmonary arterial, and pulmonary artery wedge pressure and the following:

  • 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 at rest or worsening pulmonary arterial pressure during exercise [39]
  • Pulmonary artery wedge (left atrial) pressure greater than 12 mm Hg
  • Diastolic pressure gradient between pulmonary capillary wedge pressure and left ventricular end diastolic pressure [37]
  • Prominent V wave in right atrial pressure tracing (due to tricuspid regurgitation)
  • Delay in visualization of the distal left atrial chamber in the venous phase of right ventricular (pulmonary arterial) angiography [37]

Left heart catheterization

There is 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 ECG 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.

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Imaging Studies

Cor triatriatum sinistrum

Chest radiography, computed tomography scanning, and magnetic resonance imaging

Chest x-ray findings include the following:

  • Enlarged atrium
  • Cardiomegaly
  • Pulmonary congestion
  • Prominent pulmonary arteries
  • Pleural effusion

Cardiac CT scanning and cardiac MRI have been used for evaluating patients with suspected cor triatriatum.[53] The results include multiple case reports of a successful diagnosis.[54, 55]

Angiography

Angiographic diagnosis has been reported, with a successfully established diagnosis in about 50% of the cases. Angiography 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 and the modality of choice for the diagnosis of cor triatriatum[29, 32, 40, 41, 42, 43, 44, 45, 46, 47, 48, 49, 50, 51] 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.

Unlike mitral stenosis where only a diastolic flow across the stenotic valve is present, flow across the (restrictive) accessory membrane in cor triatriatum occurs both in diastole and systole.[52]

Three-dimensional reconstruction of echocardiographic images has been used and appears to improve the definition of the accessory membrane, detection of fenestrations, and identification of the relationship of the membrane to surrounding structures.[19]

Intracardiac echocardiography has been shown to have demonstrated presence of cor triatriatum in 1 patient.

Frequently reported associated findings on cardiac imaging for cor triatriatum sinistrum include the following:

  • Patent foramen ovale
  • Atrial septal defect (secundum-type and primum-type) [56]
  • 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.[40, 41, 42, 43, 44, 45, 46, 47, 48, 50, 53, 54, 57, 58, 59, 60]

See the videos below for imaging studies of cor triatriatum.

Cor triatriatum. This film shows the classic pattern of pulmonary edema associated with pulmonary overcirculation and pulmonary venous obstruction. Patient has an anomalous pulmonary venous connection that was only obvious after a pulmonary artery shunt. The particular radiograph is not a patient with cor triatriatum, but appearance of prominent pulmonary vascularity is the same. Movie courtesy of Guido Giordano, MD, Cardiovascular Department, Azienda Ospedaliera Cannizzaro, Catania, Italy.
Cor triatriatum. Movie courtesy of Guido Giordano, MD, Cardiovascular Department, Azienda Ospedaliera Cannizzaro, Catania, Italy.
Cor triatriatum. Movie courtesy of Guido Giordano, MD, Cardiovascular Department, Azienda Ospedaliera Cannizzaro, Catania, Italy.
Cor triatriatum. Movie courtesy of Guido Giordano, MD, Cardiovascular Department, Azienda Ospedaliera Cannizzaro, Catania, Italy.
Cor triatriatum. Movie courtesy of Guido Giordano, MD, Cardiovascular Department, Azienda Ospedaliera Cannizzaro, Catania, Italy.
Cor triatriatum. Movie courtesy of Guido Giordano, MD, Cardiovascular Department, Azienda Ospedaliera Cannizzaro, Catania, Italy.
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Contributor Information and Disclosures
Author

Jamshid Shirani, MD Director of Cardiology Fellowship Program, Director of Echocardiography Laboratory, Director of Hypertrophic Cardiomyopathy Clinic, St Luke's University Health Network

Jamshid Shirani, MD is a member of the following medical societies: American Association for the Advancement of Science, American Federation for Medical Research, American Society of Echocardiography, Association of Subspecialty Professors, American College of Cardiology, American College of Physicians, American Heart Association

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 Cardiovascular Angiography and Interventions, Society of General Internal Medicine, Southern Medical Association, Society of Hospital Medicine

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, Society of Thoracic Surgeons

Disclosure: Nothing to disclose.

Yuba R Acharya, MD Fellow, Department of Cardiology, St Luke's University Hospital, Bethlehem, Pennsylvania

Yuba R Acharya, MD is a member of the following medical societies: American College of Cardiology, American College of Physicians, American Medical Association, Society of Hospital Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Frank M Sheridan, MD 

Frank M Sheridan, MD is a member of the following medical societies: American College of Cardiology, American Heart Association, Society for Cardiovascular 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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Cor triatriatum. Echocardiogram showing the proximal chamber (PC) and distal chamber (DC) of the left atrium; the right atrium (RA), left ventricle (LV), and right ventricle (RV) also are shown. Image courtesy of Guido Giordano, MD, Cardiovascular Department, Azienda Ospedaliera Cannizzaro, Catania, Italy.
Cor triatriatum. Image courtesy of Guido Giordano, MD, Cardiovascular Department, Azienda Ospedaliera Cannizzaro, Catania, Italy.
Cor triatriatum. Image courtesy of Guido Giordano, MD, Cardiovascular Department, Azienda Ospedaliera Cannizzaro, Catania, Italy.
Cor triatriatum. Image courtesy of Guido Giordano, MD, Cardiovascular Department, Azienda Ospedaliera Cannizzaro, Catania, Italy.
Cor triatriatum. Image courtesy of Guido Giordano, MD, Cardiovascular Department, Azienda Ospedaliera Cannizzaro, Catania, Italy.
Cor triatriatum. Image courtesy of Guido Giordano, MD, Cardiovascular Department, Azienda Ospedaliera Cannizzaro, Catania, Italy.
Cor triatriatum. This film shows the classic pattern of pulmonary edema associated with pulmonary overcirculation and pulmonary venous obstruction. Patient has an anomalous pulmonary venous connection that was only obvious after a pulmonary artery shunt. The particular radiograph is not a patient with cor triatriatum, but appearance of prominent pulmonary vascularity is the same. Movie courtesy of Guido Giordano, MD, Cardiovascular Department, Azienda Ospedaliera Cannizzaro, Catania, Italy.
Cor triatriatum. Movie courtesy of Guido Giordano, MD, Cardiovascular Department, Azienda Ospedaliera Cannizzaro, Catania, Italy.
Cor triatriatum. Movie courtesy of Guido Giordano, MD, Cardiovascular Department, Azienda Ospedaliera Cannizzaro, Catania, Italy.
Cor triatriatum. Movie courtesy of Guido Giordano, MD, Cardiovascular Department, Azienda Ospedaliera Cannizzaro, Catania, Italy.
Cor triatriatum. Movie courtesy of Guido Giordano, MD, Cardiovascular Department, Azienda Ospedaliera Cannizzaro, Catania, Italy.
Cor triatriatum. Movie courtesy of Guido Giordano, MD, Cardiovascular Department, Azienda Ospedaliera Cannizzaro, Catania, Italy.
 
 
 
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