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AUTHOR AND EDITOR INFORMATION

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Author: Jamshid Shirani, MD, FACC, FAHA, Consulting Staff, Director of Cardiovascular Fellowship Program, Department of Medicine, Division of Cardiology, Geisinger Medical Center

Jamshid Shirani 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

Coauthor(s): Arun Kalyanasundaram, MD, MPH, Fellow, Department of Cardiology, Division of Medicine, Geisinger Medical Center; 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

Editors: Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Frank M Sheridan, MD, Cardiology, Providence Everett Medical Center; Amer Suleman, MD, Consultant in Electrophysiology and Cardiovascular Medicine, Department of Internal Medicine, Division of Cardiology, Medical City Dallas Hospital; Park W Willis IV, MD, Sarah Graham Distinguished Professor of Medicine and Pediatrics, University of North Carolina at Chapel Hill School of Medicine

Author and Editor Disclosure

Synonyms and related 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

INTRODUCTION

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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

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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


DIFFERENTIALS

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Atrial Myxoma
Mitral Stenosis
Pericarditis, Constrictive
Pulmonary Hypertension, Primary
Pulmonary Hypertension, Secondary
Tricuspid Stenosis

Other Problems to be Considered

Diagnosis of cor triatriatum is frequently made with considerable delay due to rarity of the condition and presenting signs and symptoms that mimic other more common cardiac or pulmonary disorders.

Cough and hemoptysis may be attributed to other potential causes such as bronchitis, pulmonary tuberculosis, or malignancy.

Cor triatriatum sinistrum

Bronchial asthma
Pulmonary vein stenosis
Pulmonary veno-occlusive disease
Supravalvular mitral ring
Atrial tumors

Cor triatriatum dextrum

Prominent Chiari network
Right atrial tumor (particularly right atrial myxoma)
Inferior vena cava (obstruction) syndrome


WORKUP

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Lab Studies

Cor triatriatum sinistrum

  • Electrocardiography
    • Sinus rhythm
    • Frequent atrial premature complexes
    • Left and/or right atrial abnormality
    • Right axis deviation
    • Right ventricular hypertrophy and strain pattern
  • Right heart catheterization
    • Elevated right atrial, right ventricular, pulmonary arterial, and pulmonary artery wedge pressure
      • 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
      • Pulmonary artery wedge (left atrial) pressure greater than 12 mm Hg
    • Prominent V wave in right atrial pressure tracing (due to tricuspid regurgitation)
  • Left heart catheterization
    • 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 electrocardiographic 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.

Imaging Studies

Cor triatriatum sinistrum

  • Chest radiograph
    • Cardiomegaly
    • Pulmonary congestion
    • Prominent pulmonary arteries
    • Pleural effusion
  • Angiography: Angiographic diagnosis has been reported.
    • Successfully establishes diagnosis in about 50% of the cases
    • 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 for the diagnosis of cor triatriatum. 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.
    • Three-dimensional reconstruction of echocardiographic images has been used to better define the membrane and its relationship to surrounding structures.
    • Intracardiac echocardiography has been shown to have demonstrated presence of cor triatriatum in 1 patient.
  • Both computerized tomography of the heart and cardiac magnetic resonance imaging (MRI) have been used for evaluating patients with suspected cor triatriatum. The results are multiple case reports of successful diagnosis.

Frequently reported associated findings on cardiac imaging include the following:

  • Patent foramen ovale
  • Atrial septal defect (secundum-type and primum-type)
  • 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.


TREATMENT

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Medical Care

Medial care for patients with cor triatriatum includes the following:

  • For symptomatic patient
    • Stabilize hemodynamics by control of hypoxemia, fluid overload, and pulmonary congestion
    • Control ventricular rate in patients with atrial fibrillation
    • Anticoagulation prophylaxis against deep vein thrombosis and pulmonary embolism in those with right-sided heart failure
    • Full anticoagulation in those with atrial fibrillation
  • Surgical consultation
  • For cor triatriatum dextrum, observation alone is appropriate in asymptomatic patients. In others, control of fluid retention and rate-control of atrial arrhythmias may be required. Percutaneous technique of balloon septostomy of the accessory membrane has been reported.

Surgical Care

  • Surgical resection of the accessory membrane has been successful in symptomatic patients with cor triatriatum.
  • Complete resection of the membrane and closure of the atrial septum with a pericardial patch is a common approach.
  • Associated congenital defects need to be corrected at the same time.

Consultations

  • Cardiology consultation for medical management and echocardiographic, hemodynamic, and angiographic evaluation
  • Radiology consultation for advanced cardiac imaging with computerized tomography or cardiac magnetic resonance techniques

Diet

  • A low-salt diet is appropriate in those with significant fluid retention

Activity

  • Bed rest is appropriate in symptomatic patients with pulmonary congestion or significant right-sided heart failure and pulmonary hypertension.


MEDICATION

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

Drug Category: Inotropic agents

Provide myocardial support in the perioperative period for patients with right heart failure. The more restrictive the connection between the proximal and distal chambers, the more likely inotropic support will be required. A number of agents are available in this category. Adrenergic agonists increase myocardial contractility in patients with heart failure.

Drug NameDigoxin (Lanoxin)
DescriptionExerts inotropic action by increasing amount of intracellular calcium available during excitation-contraction coupling. One of numerous inotropic agents used in infants with congenital cardiac defects. Other agents, such as dopamine, are more appropriate for the acute management of heart failure in the ICU setting.
Adult DoseLoading dose: 0.5-1 mg PO/IV in divided doses over 24 h
Maintenance dose: 0.125-0.5 mg/d PO/IV
Pediatric DosePremature neonates: Load with 0.015-0.025 mg/kg IV in 3 doses over 24 h; maintenance dose is 0.01 mg/kg/d divided bid
Neonates: Load with 0.025-0.035 mg/kg PO/IV in 3 doses over 24 h; maintenance dose is 0.01 mg/kg/d divided bid
Infants: Load with 0.035-0.06 mg/kg IV in 3 doses over 24 h; maintenance dose is 0.01-0.02 mg/kg/d divided bid
ContraindicationsDocumented hypersensitivity or digitalis-induced toxicity, ventricular fibrillation or ventricular tachycardia (unless caused by heart failure)
InteractionsCholestyramine, metoclopramide, sulfasalazine, and chemotherapy significantly lower digoxin levels; erythromycin, tetracycline, amiodarone, verapamil, quinidine, and quinine may increase serum levels
PregnancyC - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
PrecautionsConsider potassium supplementation in patients taking diuretics (hypokalemia predisposes patients to digitalis toxicity)

Drug NameDopamine (Intropin)
DescriptionAdrenergic agonists often are used in the critical care setting for rapid onset of action and rapid time to peak effect. Are much easier to titrate to effect in acute setting. Half-life is much shorter than digoxin, and effects are rapidly lost when drug is discontinued.
Adult Dose1-5 mcg/kg/min as continuous IV infusion, not to exceed 50 mcg/kg/min; at doses higher than 30 mcg/kg/min, consider using another agent for inotropic effect
Pediatric DoseNeonates: 1-20 mcg/kg/min as continuous IV infusion
Children: 1-20 mcg/kg/min as continuous IV infusion, not to exceed 50 mcg/kg/min
ContraindicationsDocumented hypersensitivity; pheochromocytoma or ventricular fibrillation
InteractionsPhenytoin, alpha- and beta-adrenergic blockers, general anesthesia, and MAOIs increase and prolong effects of dopamine
PregnancyC - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
PrecautionsProtect solution from light; monitor urine flow, cardiac output, pulmonary wedge pressure, and blood pressure closely during infusion; prior to infusion, correct hypovolemia with either whole blood or plasma, as indicated; monitoring central venous pressure or left ventricular filling pressure may be helpful in detecting and treating hypovolemia

Drug Category: Loop diuretics

Management of right heart failure and pulmonary edema.

Drug NameFurosemide (Lasix)
DescriptionHighly effective first-line drug for diuresis in newborns and infants. Sulfonamide derivative that exerts effects on loop of Henle and distal renal tubule, inhibiting reabsorption of sodium and chloride.
Adult Dose10-200 mg PO/IV; doses as high as 600 mg/d may be used; continuous IV infusions may be more successful; usual maximum dose is approximately 0.4 mg/kg/h
Pediatric Dose1-2 mg/kg/dose PO/IV, not to exceed 6 mg/kg/dose bid/qid
ContraindicationsDocumented hypersensitivity; hepatic coma, anuria, and state of severe electrolyte depletion
InteractionsMetformin decreases furosemide concentrations; interferes with hypoglycemic effect of antidiabetic agents and antagonizes muscle-relaxing effect of tubocurarine; auditory toxicity appears to be increased with coadministration of aminoglycosides and furosemide; hearing loss of varying degrees may occur; anticoagulant activity of warfarin may be enhanced when taken concurrently; increased plasma lithium levels and toxicity are possible when taken concurrently
PregnancyC - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
PrecautionsInform patients of potential for photosensitivity; most popular strengths of digoxin and furosemide are white tabs of approximately equal size (they may be confused by patients taking these medications on an outpatient basis); close medical supervision and dose evaluation is required to prevent fluid and electrolyte imbalance; may cause excessive dehydration during ascent but no reports of deleterious effects; observe for blood dyscrasias and liver or kidney damage; loop diuretics may increase urinary excretion of magnesium and calcium

Drug Category: Anticoagulants

These agents are used in the prophylaxis and treatment of thromboembolic disorders.

Drug NameHeparin
DescriptionAugments activity of antithrombin III and prevents conversion of fibrinogen to fibrin. Does not actively lyse but is able to inhibit further thrombogenesis. Prevents reaccumulation of clot after spontaneous fibrinolysis.
Adult DoseLoading dose: 40-170 U/kg IV
Maintenance infusion: 18 U/kg/h
Alternative dose: 50 U/kg/h followed by continuous infusion of 15-25 U/kg/h; increase dose by 5 U/kg/h q4h prn using PTT results
aPTT goal of 60-90 s for untreated patients with cor triatriatum, documented systemic embolization, intracardiac thrombi, or those with atrial fibrillation
Pediatric DoseLoading dose: 50 U/kg/h
Maintenance infusion: 15-25 U/kg/h; using PTT results, increase by 2-4 U/kg/h q6-8h prn to 25 U/kg/h; increase dose by 5 U/kg/h q4h prn using PTT results
ContraindicationsDocumented hypersensitivity; subacute bacterial endocarditis; active bleeding; history of heparin-induced thrombocytopenia
InteractionsDigoxin, nicotine, tetracycline, and antihistamines may decrease effects; NSAIDs, aspirin, dextran, dipyridamole, and hydroxychloroquine may increase heparin toxicity
PregnancyC - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
PrecautionsIn neonates, preservative-free heparin is recommended to avoid possible toxicity (gasping syndrome) by benzyl alcohol, which is used as preservative; caution in severe hypotension and shock; monitor for bleeding in peptic ulcer disease, menstruation, increased capillary permeability, and when giving IM injections

Drug NameWarfarin (Coumadin)
DescriptionMost commonly used oral anticoagulant. Interferes with hepatic synthesis of vitamin K-dependent coagulation factors; used for prophylaxis and treatment of thromboembolic disorders.
Adult Dose2-10 mg/d PO qd; adjust dose to an INR of 2-3 or higher depending on the condition requiring anticoagulation
Pediatric DoseAdminister weight-based dose of 0.05-0.34 mg/kg/d PO; adjust dose according to desired INR
ContraindicationsDocumented hypersensitivity; severe liver or kidney disease; open wounds or GI ulcers
InteractionsDrugs that may decrease anticoagulant effects include griseofulvin, carbamazepine, glutethimide, estrogens, nafcillin, phenytoin, rifampin, barbiturates, cholestyramine, colestipol, vitamin K, spironolactone, oral contraceptives, and sucralfate
Medications that may increase anticoagulant effects of warfarin include oral antibiotics, capecitabine, phenylbutazone, salicylates, sulfonamides, chloral hydrate, clofibrate, diazoxide, anabolic steroids, ketoconazole, ethacrynic acid, miconazole, nalidixic acid, sulfonylureas, allopurinol, chloramphenicol, cimetidine, disulfiram, metronidazole, phenylbutazone, phenytoin, propoxyphene, sulfonamides, gemfibrozil, acetaminophen, and sulindac
PregnancyX - Contraindicated; benefit does not outweigh risk
PrecautionsDo not switch brands after achieving therapeutic response; caution in active tuberculosis or diabetes; patients with protein C or S deficiency are at risk of developing skin necrosis; caution when initiating or discontinuing enteral feeding or vitamin supplement containing vitamin K (adjust dose)


FOLLOW-UP

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Further Inpatient Care

Further inpatient care of patients with cor triatriatum includes routine postoperative surgical wound care and management of postoperative arrhythmias.

Transfer

Symptomatic patients may have to be transferred to tertiary care centers for advanced cardiac imaging, angiographic evaluation, and surgical correction.

Complications

  • Pulmonary edema
  • Pulmonary hypertension
  • Right-sided heart failure
  • Atrial arrhythmia
  • Systemic and pulmonary embolism
  • Death

Prognosis

  • The highest mortality is in untreated symptomatic infants with cor triatriatum sinistrum associated with complex congenital cardiac defects.
  • Surgical outcome is excellent in adults with isolated cor triatriatum.


MISCELLANEOUS

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Medical/Legal Pitfalls

  • The rarity of cor triatriatum has lead to frequent misdiagnosis of the condition as bronchial asthma, primary or secondary pulmonary hypertension, or mitral stenosis.
  • A high index of suspicion can prevent delayed diagnosis and unfavorable outcomes.


MULTIMEDIA

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Media file 1:  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.
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Media type:  X-RAY

Media file 2:  Cor triatriatum. Image courtesy of Guido Giordano, MD, Cardiovascular Department, Azienda Ospedaliera Cannizzaro, Catania, Italy.
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Media type:  ECG

Media file 3:  Cor triatriatum. Image courtesy of Guido Giordano, MD, Cardiovascular Department, Azienda Ospedaliera Cannizzaro, Catania, Italy.
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Media type:  Image

Media file 4:  Cor triatriatum. Image courtesy of Guido Giordano, MD, Cardiovascular Department, Azienda Ospedaliera Cannizzaro, Catania, Italy.
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Media type:  Image

Media file 5:  Cor triatriatum. Image courtesy of Guido Giordano, MD, Cardiovascular Department, Azienda Ospedaliera Cannizzaro, Catania, Italy.
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Media type:  Image

Media file 6:  Cor triatriatum. Image courtesy of Guido Giordano, MD, Cardiovascular Department, Azienda Ospedaliera Cannizzaro, Catania, Italy.
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Media type:  Image

Media file 7:  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.
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Media type:  Movie

Media file 8:  Cor triatriatum. Movie courtesy of Guido Giordano, MD, Cardiovascular Department, Azienda Ospedaliera Cannizzaro, Catania, Italy.
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Media type:  Movie

Media file 9:  Cor triatriatum. Movie courtesy of Guido Giordano, MD, Cardiovascular Department, Azienda Ospedaliera Cannizzaro, Catania, Italy.
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Media type:  Movie

Media file 10:  Cor triatriatum. Movie courtesy of Guido Giordano, MD, Cardiovascular Department, Azienda Ospedaliera Cannizzaro, Catania, Italy.
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Media type:  Movie

Media file 11:  Cor triatriatum. Movie courtesy of Guido Giordano, MD, Cardiovascular Department, Azienda Ospedaliera Cannizzaro, Catania, Italy.
Click to see larger pictureClick to see detailView Full Size Image
 
Media type:  Movie

Media file 12:  Cor triatriatum. Movie courtesy of Guido Giordano, MD, Cardiovascular Department, Azienda Ospedaliera Cannizzaro, Catania, Italy.
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Media type:  Movie


REFERENCES

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  • Alboliras ET, Edwards WD, Driscoll DJ, et al. Cor triatriatum dexter: two-dimensional echocardiographic diagnosis. J Am Coll Cardiol. Feb 1987;9(2):334-7. [Medline].
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Cor Triatriatum excerpt

Article Last Updated: Sep 18, 2008