eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology
Cor Triatriatum
Updated: Jun 30, 2009
Introduction
Background
Cor triatriatum is a rare congenital cardiac anomaly in which a fibromuscular membrane divides the atrium in two. In its most common form, cor triatriatum sinister, the left atrium is divided into an upper chamber that receives the pulmonary veins and a lower chamber that is related to the left atrial appendage and the mitral valve orifice. However, variable types of subtotal cor triatriatum are also noted, with only the right or left pulmonary veins draining into the upper chamber.1
Cor triatriatum represents 0.1% of all congenital cardiac malformations and may be associated with other cardiac defects in as many as 50% of cases. Examples of associated cardiac defects include atrial septal defect, persistent left superior vena cava with an unroofed coronary sinus, partial anomalous pulmonary venous connection, ventricular septal defect, and more complex cardiac lesions, such as tetralogy of Fallot, atrioventricular canal, and double outlet right ventricle. Associated bicuspid pulmonary valve, aortic valve atresia, and heterotaxy have also been described.2
Congenital pulmonary vein stenosis is a very rare association with cor triatriatum.3
An embryologically unrelated membrane may rarely divide the right atrium; this finding, so-called cor triatriatum dexter, is usually asymptomatic and is mostly reported as an incidental finding.
The location of the atrial appendage is a key landmark in this congenital malformation. It differentiates cor triatriatum from a physiologically similar condition, supravalvular mitral stenosis. In cor triatriatum the left atrial appendage is invariably associated with the lower chamber, which is below the membrane.
The natural history of this defect depends on the size of the communicating orifice between the upper and lower atrial chambers. If the communicating orifice is small, the patient is critically ill and may succumb at a young age (usually during infancy) to congestive heart failure and pulmonary edema. If the connection is larger, patients may present in childhood or young adulthood with a clinical picture similar to that of mitral stenosis. Cor triatriatum may also be an incidental finding when it is nonobstructive.
Pathophysiology
Initially, the fetal lungs and pulmonary veins are connected to the systemic venous circulation. Subsequently, a dorsal outgrowth from the common atria, also referred as common pulmonary vein, evaginates and joins the pulmonary veins, whereas the connection to the systemic circulation disappears. As the fetal heart grows, the common pulmonary vein is completely absorbed. Failure of this dorsal outgrowth to join the pulmonary veins results in total anomalous pulmonary venous drainage (TAPVD). An abnormal connection between the common pulmonary vein and the atria results in any of the variants of cor triatriatum.
The critical anatomic feature of cor triatriatum is a diaphragm that divides the left atrium into 2 chambers (see Media files 1-5).
Long-axis parasternal view demonstrating a left atrial membrane separating pulmonary vein inflow from left ventricular (mitral valve) inflow. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.
Long-axis parasternal view depicting 2D image of cor triatriatum sinister membrane and color Doppler of pulmonary venous flow through the orifice of the membrane. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.
Short-axis parasternal view depicting right and left pulmonary vein flow proximal to the cor triatriatum left atrial membrane and left atrial appendage orifice distal to the cor triatriatum left atrial membrane. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.
Subxiphoid coronal image of the posterior left atrial chamber that receives pulmonary venous flow separated from the rest of the left atrium by the cor triatriatum membrane. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.
Apical 5-chamber view demonstrating a 4-5 mm left atrial membrane orifice with mild pulmonary venous inflow restriction. Note the presence of an associated perimembranous ventricular septal defect (VSD) with tricuspid septal aneurysmal tissue. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.
It consists of fibromuscular tissue, and the proximal chamber that is created represents a vestigial common pulmonary vein. The pulmonary veins thus drain into the proximal chamber.
Outcome depends on the size of the communication to the distal chamber, which communicates with the mitral valve. If present, a patent fossa ovalis or secundum atrial septal defect permits decompression of the proximal chamber into the right atrium.
The presentation of cor triatriatum is one of decreased cardiac output and pulmonary venous hypertension. If a connection between the common pulmonary venous chamber and the right atrium is present, pulmonary overcirculation may result in significant right ventricular enlargement.
Frequency
United States
This is a very rare malformation. Incidence is approximately 0.1-0.4% of all infants with congenital cardiac disease.
Mortality/Morbidity
Approximately 75% of patients die in infancy (generally from pulmonary hypertension) if the defect is unrepaired. If the communication between the proximal and distal chambers is not restrictive or if an atrial septal defect allows decompression of the hypertensive left atrium, the prognosis is significantly improved.
The only treatment is surgical correction. Most postoperative deaths occur in the first 30 days. The early mortality rate in each large series was consistently 15-20%. Early deaths had a higher rate of associated severe cardiac anomalies.
Long-term results are excellent, with long term survival of 80-90% in patients surviving surgery. Survivors have excellent functional results without residual sequelae and a life expectancy that approaches that of the general population. This is particularly true when repair is performed in infancy.
Race
No race predilection is reported.
Sex
A slight male predilection is observed, with a male-to-female ratio of 1.4:1.
Age
Diagnosis is primarily made in infancy. Later presentation does occur but is usually in childhood or early adulthood; in this patient population, 85% are younger than 40 years. Rarely, patients presenting in their eighth or ninth decade of life have been reported.
Clinical
History
- Cor triatriatum is essentially a form of left atrial inflow obstruction and presents with signs and symptoms of pulmonary venous obstruction.
- Most patients present during infancy with a restrictive opening in the membrane. These infants usually present with evidence of low cardiac output, including pallor, diminished peripheral pulses, and tachypnea.
- Feeding difficulties, poor weight gain, and respiratory distress are common.
- Presentation later in life is less classic; however, when the patient becomes symptomatic, evidence of pulmonary venous obstruction predominates. In these patients, the membrane may become calcified with its orifice becoming smaller or the patient may develop mitral valve insufficiency. These patients may also present with arrhythmias secondary to an enlarged, hypertensive atrium.
Physical
- Despite the intracardiac defect, no characteristic murmur or pathognomonic physical characteristics are present. This often leads to incorrect diagnosis of primary pulmonary vascular or parenchymal disease.
- Signs of pulmonary venous obstruction and pulmonary hypertension are present. A right ventricular lift and accentuation of the pulmonary second sound are frequent and may be accompanied by an early diastolic murmur of pulmonary insufficiency. Rales may be present in the lung bases.
- A soft, continuous murmur may be present due flow across the membrane. A murmur at the left sternal border is heard in patients with an atrial septal defect and a left-to-right shunt. A diastolic rumble of mitral stenosis at the apex is generally not heard with cor triatriatum.
- Low cardiac output manifests as pallor, tachypnea, and diminished peripheral pulses.
- Children are typically small, suffering from poor weight gain.
- Patients presenting later in life may be dyspneic with a history of frequent pulmonary infections. They may have signs of right-sided heart failure, including distended peripheral veins and hepatomegaly. Signs and symptoms of pulmonary hypertension may be severe.
Causes
- No known risk factors or associated genetic abnormalities have been reported.
- Embryologically, the common pulmonary vein is normally absorbed and incorporated into the left atrium. Incomplete absorption results in a fibromuscular membrane that subdivides the left atrium into 2 chambers resulting in cor triatriatum.
- Observation that a left superior vena cava is frequently associated with this lesion has led some to propose impingement of the left superior vena cava to the developing left atrium as a potential pathogenesis.
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 |
| Next Page » |
References
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[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].
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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].
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].
Further Reading
- Relevant clinical guidelines and clinical trials include the following:
- 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 11
- The Pharmacology and Hemodynamics of Dexmedetomidine in Children With Congenital Heart Disease
- Cardiac Resynchronization Therapy in Congenital Heart Defects
- Related eMedicine topics include the following:
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
