eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Cor Triatriatum

M Silvana Horenstein, MD, Staff Physician, Department of Pediatrics, University of Texas Medical School Houston; Medical Doctor Consultant, Legacy Department, Best Doctors, Inc
Maria Victoria T Tantengco, MD, Associate Professor of Pediatrics, Division of Cardiology, Department of Pediatrics, University of Massachusetts Medical School; Medical Director, Echocardiography Laboratory, Child Heart Associates, LLC; Michael Pettersen, MD, Director of Echocardiography, Division of Cardiology, Children's Hospital of Michigan; Assistant Professor of Pediatrics, Wayne State University School of Medicine

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.

Echo available at http://img.medscape.com/pi/emed/ckb/pediatrics_cardiac/1331339-1331342-895968-1696246.flv.

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.

Echo available at http://img.medscape.com/pi/emed/ckb/pediatrics_cardiac/1331339-1331342-895968-1696249.flv.

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.

Echo available at http://img.medscape.com/pi/emed/ckb/pediatrics_cardiac/1331339-1331342-895968-1696252.flv.

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.

Echo available at http://img.medscape.com/pi/emed/ckb/pediatrics_cardiac/1331339-1331342-895968-1696253.flv.

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.

Echo available at http://img.medscape.com/pi/emed/ckb/pediatrics_cardiac/1331339-1331342-895968-1696256.flv.

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.

Differential Diagnoses

Mitral Stenosis, Supravalvular Ring
Partial Anomalous Pulmonary Venous Connection
Pulmonary Hypertension, Idiopathic
Pulmonary Hypertension, Persistent-Newborn
Pulmonary Hypoplasia
Total Anomalous Pulmonary Venous Connection

Other Problems to Be Considered

Mitral stenosis, valvar
Pulmonary vein stenosis

Workup

Laboratory Studies

• No specific laboratory studies are indicated in patients with suspected cor triatriatum.

Imaging Studies

• Chest radiography
  • Findings are usually nonspecific but may include pulmonary congestion with diffuse haziness or Kerley B lines and the ground glass pattern of acute pulmonary edema in hilar areas.
  • Patients may have mild cardiac enlargement and prominence of the pulmonary arterial segment.
  • The dilated proximal chamber may produce the appearance of left atrial enlargement.
  • Presence of an atrial septal defect or of an associated partial anomalous pulmonary venous connection adds pulmonary overcirculation to the pulmonary venous obstruction. The radiograph may then reveal significant right ventricular enlargement.
• Echocardiography
  • Echocardiography is often sufficient for diagnosis and is the diagnostic modality of choice.
    
    

    

    Mean Doppler gradient of 7-8 mm Hg across left atrial membrane indicating mildly elevated pulmonary venous pressures. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.

    
    
  • The membrane dividing the left atrium can be visualized using 2-dimensional echocardiography, as can the presence of an associated atrial septal defect. The origin of each of the pulmonary veins should be identified to exclude the presence of anomalous pulmonary venous return.
  • The distinction between cor triatriatum and a supramitral ring should be made by the location of the left-atrial appendage. Differentiating between cor triatriatum and total anomalous pulmonary venous drainage to the coronary sinus may be difficult.
  • Common cardiac anomalies can also be demonstrated
  • Transesophageal echocardiography (TEE) and intracardiac echocardiography offer precise image definition and spatial relationship of the membrane.
  • TEE is very useful in larger and older patients in whom transthoracic images are suboptimal especially in visualizing the left atrium.⁴
• Angiography
  • This test is generally indicated to assess pulmonary venous return and pulmonary arterial pressures. Because approximately 10% of patients have partial anomalous venous return, angiography is helpful in defining the precise venous anatomy.
  • When performed, catheterization generally reveals pulmonary hypertension in a degree that varies directly with the severity of obstruction to pulmonary venous drainage.⁵ Demonstration of a pressure gradient between the left atrium and capillary wedge pressure is classic.
  • The proximal chamber is visualized during the venous phase, and a delay then occurs before the true left atrium and left ventricle are visualized. The proximal chamber then remains opacified and does not contract with the distal chamber
• Cardiac CT⁶ scanning and MRI⁷ : Both imaging modalities provide with very detailed anatomic images.⁸ MRI has the advantage of not subjecting the patient to radiation.⁹

Other Tests

• ECG findings are nonspecific and may range from normal in asymptomatic older patients to mimicking those findings of a patient with the clinical picture of pulmonary hypertension.
• In some patients, ECG may reveal the following:
  • Atrial arrhythmias⁵
  • Right-axis deviation
  • Right atrial enlargement
  • Right ventricular hypertrophy

Histologic Findings

• Histology plays no part in the diagnosis; however, pulmonary hypertension results in well-defined structural changes.
• Increased pulmonary arterial muscularity is present very early, with increased thickness of the arterial wall and extension of muscle into the arterioles.

Treatment

Medical Care

• The goal of medical care in cor triatriatum is to reduce the symptoms caused by pulmonary venous congestion until definitive surgical therapy can be performed.
• Patients presenting in extremis should be operated on immediately after resuscitation, without time spent for prolonged medical therapy.
• On occasion, extracorporeal membrane oxygenation is initiated to stabilize a patient.

Surgical Care

Surgery is the treatment of choice. Recently, interventional catheterization techniques have evolved and been used successfully in some patients.

• Surgical correction
  • Open correction is currently preferred over closed (percutaneous) procedures.
  • The procedure is performed on cardiopulmonary bypass through an atrial incision with complete resection of the diaphragm.
• Interventional cardiology: The role of percutaneous balloon dilation in managing this condition remains to be determined.

Consultations

• Pediatric cardiology
• Pediatric cardiac surgery

Diet

• No specific dietary restrictions are recommended.

Activity

• Physical activity should not be limited in patients with early and complete correction.
• Patients with persistent pulmonary or cardiac dysfunction secondary to long-standing disease may have moderate restriction of exercise tolerance.

Medication

Medical management in cor triatriatum is targeted toward associated elevation in pulmonary vascular resistance and heart failure. It is continued in the postoperative period until the pulmonary resistance falls and right ventricular performance improves. Mainstays of treatment are inotropic agents and diuretics.

Inotropic agents

Cardiac glycosides (eg, digoxin) increases myocardial contractility in patients with heart failure. Adrenergic and dopaminergic agents (eg, dopamine) provide myocardial support in the perioperative period for patients with heart failure. The more restrictive the connection between proximal and distal chambers, the more likely inotropic support is required. Numerous agents are available in this category.

Digoxin (Lanoxin)

Exerts its inotropic action by increasing the amount of intracellular calcium available during excitation-contraction coupling. It is one of numerous inotropic agents that can be used in infants with congenital cardiac defects. Other agents, such as dopamine (described below), are more appropriate for acute management of heart failure in ICU setting.

Dosing

Adult

Total digitalizing dose (TDD): 0.75-1.5 mg PO; 0.5-1 mg IV/IM
Administer 50% of TDD initially; remaining 2 doses at 25% TDD q6-12h
Maintenance dose: 0.125-0.5 mg/d PO; 0.1-0.4 mg/d IV/IM

Pediatric

TDD PO:
Preterm infant: 20-30 mcg/kg
Term infant: 25-35 mcg/kg
1 month to 2 years: 35-60 mcg/kg
2-5 years: 30-40 mcg/kg
5-10 years: 20-35 mcg/kg
>10 years: 10-15 mcg/kg
TDD IV/IM:
Preterm infant: 15-25 mcg/kg
Term infant: 20-30 mcg/kg
1 month to 2 years: 30-50 mcg/kg
2-5 years: 25-35 mcg/kg
5-10 years: 15-30 mcg/kg
>10 years: 8-12 mcg/kg
Administer 50% of TDD initially; remaining 2 doses at 25% TDD q6-12h
Maintenance dose PO:
Preterm infant: 5-7.5 mcg/kg/d divided bid
Term infant: 6-10 mcg/kg/d divided bid
1 month to 2 years: 10-15 mcg/kg/d divided bid
2-5 years: 7.5-10 mcg/kg/d divided bid
5-10 years: 5-10 mcg/kg/d divided bid
>10 years: 2.5-5 mcg/kg qd
Maintenance dose IV/IM:
Preterm infant: 4-6 mcg/kg/d divided bid
Term infant: 5-8 mcg/kg/d divided bid
1 month to 2 years: 7.5-12 mcg/kg/d divided bid
2-5 years: 6-9 mcg/kg/d divided bid
5-10 years: 4-8 mcg/kg/d divided bid
>10 years: 2-3 mcg/kg qd

Interactions

Levels can be markedly altered by numerous medications; cholestyramine, metoclopramide, sulfasalazine, and chemotherapy all significantly lower digoxin levels; erythromycin, tetracycline, amiodarone, verapamil, quinidine, and quinine increase serum levels

Contraindications

Documented hypersensitivity; digitalis-induced toxicity; AV block (without pacemaker); idiopathic hypertrophic subaortic stenosis; constrictive pericarditis

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Dosage adjustment is required in patients with renal impairment; can cause cardiac arrhythmias; patients are predisposed to digoxin toxicity with hypokalemia, hypomagnesemia, hypercalcemia, and hypermagnesemia; CNS effects, such as drowsiness, and GI effects, such as nausea and vomiting, are some of the more common adverse drug reactions

Dopamine (Intropin)

Adrenergic agonists are often used in the critical care setting for their rapid onset of action and rapid peak effect. They are, therefore, much easier to titrate to effect in acute settings. Their half-life is also much shorter than digoxin's, and their effects are rapidly lost when drug is discontinued.

Dosing

Adult

1-20 mcg/kg/min continuous IV infusion; not to exceed 50 mcg/kg/min

Pediatric

Administer as in adults

Interactions

Effects are prolonged and intensified by MAOIs, alpha-blockers and beta-blockers, general anesthetics, and phenytoin

Contraindications

Documented hypersensitivity; ventricular fibrillation

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Hypovolemia should be treated before infusion of this drug; administration through a central vein is recommended; do not use umbilical artery for infusion; if dosages >20 mcg/kg/min are required, a different agent should be considered (eg, epinephrine, dobutamine)

Loop diuretics

These agents are used for management of right heart failure and pulmonary edema.

Furosemide (Lasix)

First-line drug for diuresis in newborns and infants and can be expected to be highly effective. It is a sulfonamide derivative that exerts its effects on the loop of Henle and distal renal tubule, thus inhibiting reabsorption of sodium and chloride.

Dosing

Adult

10-200 mg PO/IV average dose; titrate to effect; doses as high as 600 mg/d may be used; continuous IV infusions may be more successful; usual maximum dosage approximately 0.4 mg/kg/h

Pediatric

1-2 mg/kg/dose PO/IV bid/tid/qid; titrate to effect; not to exceed 6 mg/kg/dose

Interactions

Decreases effectiveness of PO hypoglycemic agents; may enhance effects of antihypertensives; may potentiate effects of succinylcholine; potentiates ototoxicity of aminoglycosides

Contraindications

Documented hypersensitivity; hypokalemia; renal failure

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Inform patients of potential for photosensitivity; most popular strengths of digoxin and furosemide are white tabs of approximately equal size and may be confused in patients taking these medications on an outpatient basis; monitor serum potassium levels closely; may produce intravascular dehydration, severe hypokalemia, and significant hypochloremic metabolic alkalosis; may cause hyperuricemia; may produce deafness due to ototoxicity; administer oral dose with food or milk to decrease stomach upset

Follow-up

Further Inpatient Care

• Admit patients with cor triatriatum who have undergone surgical treatment to a pediatric ICU experienced in dealing with congenital cardiac defects.
• Provide postoperative treatment of heart failure and pulmonary hypertension until pulmonary vascular resistance normalizes.

Further Outpatient Care

• Serial echocardiography is a reliable and effective method for following patients on an outpatient basis. Late complications are rare.
• Recurrent membrane stenosis from incomplete surgical resection can occur and is well demonstrated by echocardiography. This imaging modality may also demonstrate residual pulmonary vein stenosis.
• Postoperative function and exercise tolerance should approach normal. Long-term activity restrictions are usually unnecessary.

Inpatient & Outpatient Medications

• Medications include inotropes in patients with low cardiac output secondary to heart failure. Agents such as dopamine predominate in the ICU, whereas agents such as digoxin are sometimes used in the outpatient setting.
• Diuretics are used in patients with pulmonary edema.
• Patients with pulmonary edema are unlikely to respond to inhaled nitric oxide preoperatively because the pulmonary hypertension is secondary to mechanical obstruction.

Transfer

• Transfer to a major medical center experienced in operative and perioperative care of patients with congenital cardiac defects is mandatory.
• If diagnosis is made in utero, delivery should be in an institution with a neonatal ICU and an ability to perform congenital cardiac surgery.

Complications

• If the condition is unrecognized and untreated, problems include pulmonary edema, right heart failure, and death.
• Late complications include recurrence of the obstructive membrane with recurrence of symptoms.³ Similar symptoms may occur later on in life if the membrane is incompletely excised.
• Ischemic stroke as a late complication from undiagnosed cor triatriatum (sinister) in an adult has been described.¹⁰

Prognosis

• Prognosis in symptomatic infants without surgical repair is poor with a mortality rate of 75%.
• Presence of associated cardiac anomalies adversely affects prognosis.
• If the connection between the proximal and distal chambers is not restrictive or if an atrial septal defect is present, prognosis is improved. In this subset of patients, the defect may escape detection in infancy and present later in life.
• Mortality associated with surgery is primarily in the immediate postoperative period and patients that survive can expect a late mortality rate of less than 10% and a near normal life expectancy. Late survivors have essentially normal lifestyles without sequelae from the anomaly or surgical correction. The short-term, 30-day mortality rate is 15-20%. Recurrence of cor triatriatum has been described.³

Patient Education

• Successful surgical correction allows a return to a normal lifestyle without restriction of activity or need for medications.

Miscellaneous

Medicolegal Pitfalls

• Failure to diagnose cor triatriatum and other structural abnormalities in children with pulmonary hypertensive disease

Special Concerns

• Patients should have an experienced congenital cardiac surgeon to treat this rare lesion. One of the few late complications is recurrence secondary to incomplete resection of the intra-atrial diaphragm.

Multimedia

Media file 1: 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.

Echo available at http://img.medscape.com/pi/emed/ckb/pediatrics_cardiac/1331339-1331342-895968-1696246.flv.

Media file 2: 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.

Echo available at http://img.medscape.com/pi/emed/ckb/pediatrics_cardiac/1331339-1331342-895968-1696249.flv.

Media file 3: 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.

Echo available at http://img.medscape.com/pi/emed/ckb/pediatrics_cardiac/1331339-1331342-895968-1696252.flv.

Media file 4: 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.

Echo available at http://img.medscape.com/pi/emed/ckb/pediatrics_cardiac/1331339-1331342-895968-1696253.flv.

Media file 5: 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.

Echo available at http://img.medscape.com/pi/emed/ckb/pediatrics_cardiac/1331339-1331342-895968-1696256.flv.

Media file 6: Mean Doppler gradient of 7-8 mm Hg across left atrial membrane indicating mildly elevated pulmonary venous pressures. With permission from Michael Pettersen, MD, Pediatric Cardiology, Children's Hospital of Michigan, Detroit, MI.

References

1. Bladt O, Vanhoenacker R. Cor triatriatum. JBR-BTR. Mar-Apr 2008;91(2):62. [Medline].

2. Vaideeswar P, Tullu MS, Sathe PA, Nanavati R. Atresia of the common pulmonary vein--a rare congenital anomaly. Congenit Heart Dis. Nov-Dec 2008;3(6):431-4. [Medline].

3. Ito M, Kikuchi S, Hachiro Y, Abe T. Congenital pulmonary vein stenosis associated with cor triatriatum. Ann Thorac Surg. Feb 2001;71(2):722-3. [Medline].

4. Modi KA, Annamali S, Ernest K, Pratep CR. Diagnosis and surgical correction of cor triatriatum in an adult: combined use of transesophageal and contrast echocardiography, and a review of literature. Echocardiography. Jul 2006;23(6):506-9. [Medline].

5. Yamada T, Tabereaux PB, McElderry HT, Doppalapudi H, Kay GN. Transseptal catheterization in the catheter ablation of atrial fibrillation in a patient with cor triatriatum sinister. J Interv Card Electrophysiol. Jun 2009;25(1):79-82. [Medline].

6. Saremi F, Gurudevan SV, Narula J, Abolhoda A. Multidetector computed tomography (MDCT) in diagnosis of "cor triatriatum sinister". J Cardiovasc Comput Tomogr. Dec 2007;1(3):172-4. [Medline].

7. Dillman JR, Yarram SG, Hernandez RJ. Imaging of pulmonary venous developmental anomalies. AJR Am J Roentgenol. May 2009;192(5):1272-85. [Medline].

8. 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].

9. Locca D, Hughes M, Mohiaddin R. Cardiovascular magnetic resonance diagnosis of a previously unreported association: Cor triatriatum with right partial anomalous pulmonary venous return to the azygos vein. Int J Cardiol. Jul 17 2008;[Medline].

10. Spengos K, Gialafos E, Vassilopoulou S. Ischemic stroke as an uncommon complication of Cor triatriatum. J Stroke Cerebrovasc Dis. Nov-Dec 2008;17(6):436-8. [Medline].

11. [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].

12. Bartel T, Muller S, Erbel R. Dynamic three-dimensional echocardiography using parallel slicing: a promising diagnostic procedure in adults with congenital heart disease. Cardiology. 1998;89(2):140-7. [Medline].

13. Citro R, Bossone E, Provenza G, Patella MM, Gregorio G. Isolated left cor triatriatum: a rare cause of effort dyspnoea in the adult. J Cardiovasc Med (Hagerstown). Sep 2008;9(9):926-8. [Medline].

14. Gharagozloo F, Bulkley BH, Hutchins GM. A proposed pathogenesis of cor triatriatum: impingement of the left superior vena cava on the developing left atrium. Am Heart J. Nov 1977;94(5):618-26. [Medline].

15. Jeiger W, Gibbons JE, Wigglesworth FW. Cor triatriatum: Clinical, hemodynamic and pathologic studies: Surgical correction in early life. Pediatrics. 1963;31:255-64.

16. Kerkar P, Vora A, Kulkarni H, et al. Percutaneous balloon dilatation of cor triatriatum sinister. Am Heart J. Oct 1996;132(4):888-91. [Medline].

17. Kirklin JW, Barratt-Boyes BG. Cardiac Surgery. 2^nd ed. Churchill Livingstone; 1993:675-81.

18. Marini D, Ou P. Cor triatriatum in a newborn. Pediatr Radiol. Mar 10 2009;[Medline].

19. McLean MK, Kung GC, Polimenakos A, Wells WJ, Reemtsen BL. Cor triatriatum associated with ASD and common atrium in 7-month-old with tachypnea and failure to thrive. Ann Thorac Surg. Dec 2008;86(6):1999. [Medline].

20. Oglietti J, Cooley DA, Izquierdo JP, et al. Cor triatriatum: operative results in 25 patients. Ann Thorac Surg. Apr 1983;35(4):415-20. [Medline].

21. Richardson JV, Doty DB, Siewers RD, Zuberbuhler JR. Cor triatriatum (subdivided left atrium). J Thorac Cardiovasc Surg. Feb 1981;81(2):232-8. [Medline].

22. Rodefeld MD, Brown JW, Heimansohn DA, et al. Cor triatriatum: clinical presentation and surgical results in 12 patients. Ann Thorac Surg. Oct 1990;50(4):562-8. [Medline].

23. Salomone G, Tiraboschi R, Bianchi T, et al. Cor triatriatum. Clinical presentation and operative results. J Thorac Cardiovasc Surg. Jun 1991;101(6):1088-92. [Medline].

24. Spencer FC, Sabiston DC. Surgery of the Chest. 6^th ed. WB Saunders; 1995:1420-4.

25. Sritippayawan S, Margetis MF, MacLaughlin EF, et al. Cor triatriatum: a cause of hemoptysis. Pediatr Pulmonol. Nov 2002;34(5):405-8. [Medline].

26. 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].

27. 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].

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

Contributor Information and Disclosures

Author

M Silvana Horenstein, MD, Staff Physician, Department of Pediatrics, University of Texas Medical School Houston; Medical Doctor Consultant, Legacy Department, Best Doctors, Inc
M Silvana Horenstein, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Maria Victoria T Tantengco, MD, Associate Professor of Pediatrics, Division of Cardiology, Department of Pediatrics, University of Massachusetts Medical School; Medical Director, Echocardiography Laboratory, Child Heart Associates, LLC
Maria Victoria T Tantengco, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Society of Echocardiography, Massachusetts Medical Society, and Society of Pediatric Echocardiography
Disclosure: Nothing to disclose.

Michael Pettersen, MD, Director of Echocardiography, Division of Cardiology, Children's Hospital of Michigan; Assistant Professor of Pediatrics, Wayne State University School of Medicine
Michael Pettersen, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, and American Society of Echocardiography
Disclosure: Nothing to disclose.

Medical Editor

Juan Carlos Alejos, MD, Clinical Professor, Department of Pediatrics, Division of Cardiology, University of California at Los Angeles
Juan Carlos Alejos, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, American Medical Association, and International Society for Heart and Lung Transplantation
Disclosure: Actelion Honoraria Speaking and teaching

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Ameeta Martin, MD, Clinical Associate Professor, Department of Pediatric Cardiology, University of Nebraska College of Medicine
Ameeta Martin, MD is a member of the following medical societies: American College of Cardiology
Disclosure: Nothing to disclose.

CME Editor

Gilbert Z Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center
Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Chief Editor

Stuart Berger, MD, Professor of Pediatrics, Division of Cardiology, Medical College of Wisconsin; Chief of Pediatric Cardiology, Medical Director of Pediatric Heart Transplant Program, Medical Director of The Heart Center, Children's Hospital of Wisconsin
Stuart Berger, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American College of Chest Physicians, American Heart Association, and Society for Cardiac Angiography and Interventions
Disclosure: Nothing to disclose.

The authors and editors of eMedicine gratefully acknowledge the contributions of previous coauthors Manuel Caceres, MD; James Jaggers, MD; and Jeff L Myers, MD, PhD, to the writing and development of this article.

• 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:
  • Atrial Septal Defect, Unroofed Coronary Sinus
  • Cor Triatriatum (Cardiology)
  • Mitral Stenosis
  • Mitral Stenosis, Congenital

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