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Cor Triatriatum Clinical Presentation

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

History

Cor triatriatum sinistrum

This is classified based on the size and the number of the openings in the accessory membrane into the left atrium. The most commonly used classification is the one developed by Loeffler in 1949. According to that classification, cor triatriatum is divided into 3 distinct groups: type 1 has no opening in the accessory membrane, with the proximal left atrium draining into the right atrium; type 2 has one or more small restrictive openings (fenestrations), resulting in significant left ventricular inflow obstruction; and type 3 has a large (nonrestrictive) opening in the membrane.[24] No opening or 1 or more restrictive opening is commonly seen in the newborn, infants, and children. In the adult, cor triatriatum often contains a relatively wide opening.

Clinical manifestations depend upon the size of the opening in the septum and the presence of associated congenital cardiac defects. In most patients, the opening is severely restrictive and produces symptoms similar to those of severe mitral stenosis. In the adult, clinical manifestations are often delayed due to the presence of a large opening.

In asymptomatic patients, a heart murmur may be detected. Cor triatriatum may be detected as an incidental finding on routine cardiac imaging.

In symptomatic patients, the transition from asymptomatic to symptomatic in the adult occurs mainly because of fibrosis and calcification of the orifice in the accessory membrane or development of mitral regurgitation and/or atrial fibrillation. Symptoms include the following:

  • Exertional dyspnea
  • Effort intolerance and easy fatigability
  • Hemoptysis
  • Orthopnea
  • Palpitation (atrial fibrillation)
  • Signs of systemic embolism
  • Systemic thromboembolism (especially in those with atrial fibrillation and/or restrictive orifice, as both conditions promote thrombus formation in the left atrium)

Cor triatriatum dextrum

Clinical presentation depends on the following:

  • The degree of septation of the right atrium
  • Size of the sinoatrial orifice

In asymptomatic patients, incidental findings occur during the following:

  • Cardiac imaging studies
  • Right heart catheterization
  • Surgery for other cardiac defects
  • Postmortem examination
  • Palpitation (recurrent supraventricular arrhythmias)
  • Increased abdominal girth (ascites)
  • Swelling of the lower extremities (edema)
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Physical

Cor triatriatum sinistrum

Findings related directly to the presence of a restrictive accessory atrial membrane include the following:

  • Murmur
  • Pulmonary congestion
  • Tachypnea
  • Respiratory distress
  • Pulmonary rales
  • Pleural effusion
  • Hypoxemia

Pulmonary hypertension may be noted as a loud second component (P2) of the second heart sound.

Right ventricular failure may manifest with the following:

  • Elevated jugular venous pressure
  • Right ventricular heave
  • Tricuspid regurgitation (systolic murmur along sternal border with respiratory variations in intensity, prominent V wave in jugular venous pulsation)
  • Right upper quadrant abdominal tenderness due to liver congestion
  • Hepatomegaly
  • Jaundice
  • Ascites
  • Peripheral edema

Tachycardia may have the following presentation:

  • Sinus rhythm
  • Rapid irregular heart rate (frequent premature atrial complexes, atrial fibrillation with rapid ventricular response)

There may be findings related to associated cyanotic or acyanotic congenital cardiac defects.

Cor triatriatum dextrum

Signs include the following:

  • Heart murmur
  • Elevated jugular venous pressure
  • Abdominal tenderness (hepatic congestion)
  • Ascites
  • Peripheral edema
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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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