eMedicine Specialties > Cardiology > Congenital Heart Disease in the Adult

Cor Triatriatum

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

Introduction

Background

First reported in 1868, cor triatriatum, that is, a heart with 3 atria (triatrial heart), is a congenital anomaly in which the left atrium (cor triatriatum sinistrum) or right atrium (cor triatriatum dextrum) is divided into 2 parts by a fold of tissue, a membrane, or a fibromuscular band. Classically, the proximal (upper or superior) portion of the corresponding atrium receives venous blood, whereas the distal (lower or inferior) portion is in contact with the atrioventricular valve and contains the atrial appendage and the true atrial septum that bears the fossa ovalis. The membrane that separates the atrium into 2 parts varies significantly in size and shape. It may appear similar to a diaphragm or be funnel-shaped, bandlike, entirely intact (imperforate) or contain 1 or more openings (fenestrations) ranging from small, restrictive-type to large and widely open.

In the pediatric population, this anomaly may be associated with major congenital cardiac lesions such as tetralogy of Fallot, double outlet right ventricle, coarctation of the aorta, partial anomalous pulmonary venous connection, persistent left superior vena cava with unroofed coronary sinus, ventricular septal defect, atrioventricular septal (endocardial cushion) defect, and common atrioventricular canal. Rarely, asplenia or polysplenia has been reported in these patients. In the adult, cor triatriatum is frequently an isolated finding.

Cor triatriatum dextrum is extremely rare and results from the complete persistence of the right sinus valve of the embryonic heart. The membrane divides the right atrium into a proximal (upper) and a distal (lower) chamber. The upper chamber receives the venous blood from both vena cavae and the lower chamber is in contact with the tricuspid valve and the right atrial appendage.

Pathophysiology

Cor triatriatum sinistrum

The most popular theory holds that cor triatriatum sinister occurs when the common pulmonary vein fails to incorporate the pulmonary circulation into the left atrium and the common pulmonary venous ostium remains narrow (malincorporation theory). The result is a septum-like structure that divides the left atrium into 2 compartments. However, this theory fails to explain the presence of fossa ovalis and atrial muscle fibers within the walls of the proximal chamber where only a venous wall is supposed to be present.

In addition, several cases have been reported in which 1 or 2 pulmonary veins drain into the proximal (accessory) chamber and the others drain directly into the true left atrium. Others believe that the membrane dividing the left atrium is an abnormal growth of the septum primum (malseptation theory) or that the right horn of the embryonic sinus venosus entraps the common pulmonary vein and thereby prevents its incorporation into the left atrium (entrapment theory). The significance of a prominent or persistent left superior vena cava in the pathogenesis of cor triatriatum is unclear.

Cor triatriatum dextrum

During embryogenesis, the right horn of the sinus venosus gradually incorporates into the right atrium to form the smooth posterior portion of the right atrium, whereas the original embryologic right atrium forms the trabeculated anterior portion. The right horn of the sinus venosus and the embryologic right atrium are then connected through the sinoatrial orifice, which has on either side the 2 valvular folds called the right and left venous valves. During this incorporation, the right valve of the right horn of the sinus venosus divides the right atrium in 2. This right valve forms a sheet that serves to direct the oxygenated venous return from the inferior vena cava across the foramen ovale to the left side of the heart during fetal life (Chiari network).

Normally, this network regresses and leaves behind the crista terminalis superiorly and the eustachian valve of the inferior vena cava and the thebesian valve of the coronary sinus inferiorly. Complete persistence of the right sinus valve of embryonic life results in separation of the smooth and trabeculated portions of the right atrium and constitutes cor triatriatum dextrum. If this membrane is extensively fenestrated and weblike in appearance, then it is referred to as the Chiari network.

Frequency

United States

  • The incidence of cor triatriatum has been variously reported as 0.1-0.4%.
  • An incidence of 0.4% has been reported at autopsy of patients with congenital cardiac disease.
  • An incidence of 0.2% was reported among patients undergoing transesophageal echocardiography.
  • In high-volume echocardiographic laboratories, the incidence of cor triatriatum is less than 1 in 10,000.
  • Cor triatriatum dextrum is extremely rare.
  • Cor triatriatum sinistrum can be misdiagnosed as other common cardiac or pulmonary conditions such as bronchial asthma or mitral stenosis.

Mortality/Morbidity

  • The morbidity and mortality of cor triatriatum sinistrum is high in those who are symptomatic in infancy. This is due to the severely restrictive opening in the accessory membrane and the association with major cyanotic or acyanotic congenital heart lesions. Mortality may exceed 75% in untreated symptomatic infants.
  • Significant sequelae is unusual with cor triatriatum dextrum as it is not commonly associated with life-threatening symptoms or major congenital cardiac defects.

Sex

No clear difference has been noted in incidence or clinical presentation among men or women.

Age

  • In symptomatic infants, cor triatriatum sinistrum is often associated with other major congenital cardiovascular defects.
  • In the adult, cor triatriatum sinistrum can be as follows:
    • Asymptomatic (found incidentally on cardiac imaging)
    • An isolated finding with a large non-restrictive communication between the superior and inferior left atrial chambers
    • Associated with minor congenital defects such as patent foramen ovale, atrial septal defect, or persistent left superior vena cava
  • Cor triatriatum dextrum can be diagnosed at any age, especially if incidentally discovered. Other congenital cardiac defects, such as atrial septal defect, may be present and demand evaluation. Cor triatriatum can also be misdiagnosed as other common cardiac conditions such as constrictive pericarditis.

Clinical

History

Cor triatriatum sinistrum

  • Classified based on the size of the openings in the accessory membrane into the left atrium. 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.
  • Asymptomatic
    • Heart murmur
    • Incidental finding on routine cardiac imaging
  • Symptomatic: 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 (see Medscape's Atrial Fibrillation Resource Center). 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)

Physical

Cor triatriatum sinistrum

  • Findings related directly to the presence of an accessory atrial membrane include the following:
    • Murmur
    • Pulmonary congestion
    • Tachypnea
    • Respiratory distress
    • Pulmonary rales
    • Pleural effusion
    • Hypoxemia
  • Pulmonary hypertension
    • Loud second component (P2) of the second heart sound
  • Right ventricular failure
    • 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
    • Sinus
    • Rapid irregular heart rate
      • Frequent premature atrial complexes
      • Atrial fibrillation with rapid ventricular response
  • Findings related to associated cyanotic or acyanotic congenital cardiac defects 

Cor triatriatum dextrum

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

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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