eMedicine Specialties > Cardiology > Congenital Heart Disease in the Adult

Cor Triatriatum: Treatment & Medication

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

Treatment

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

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.

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.


Digoxin (Lanoxin)

Exerts 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

Loading dose: 0.5-1 mg PO/IV in divided doses over 24 h
Maintenance dose: 0.125-0.5 mg/d PO/IV

Pediatric

Premature 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

Cholestyramine, metoclopramide, sulfasalazine, and chemotherapy significantly lower digoxin levels; erythromycin, tetracycline, amiodarone, verapamil, quinidine, and quinine may increase serum levels

Documented hypersensitivity or digitalis-induced toxicity, ventricular fibrillation or ventricular tachycardia (unless caused by heart failure)

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

Consider potassium supplementation in patients taking diuretics (hypokalemia predisposes patients to digitalis toxicity)


Dopamine (Intropin)

Adrenergic 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

1-5 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

Neonates: 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

Phenytoin, alpha- and beta-adrenergic blockers, general anesthesia, and MAOIs increase and prolong effects of dopamine

Documented hypersensitivity; pheochromocytoma or ventricular fibrillation

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

Protect 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

Loop diuretics

Management of right heart failure and pulmonary edema.


Furosemide (Lasix)

Highly 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

10-200 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

1-2 mg/kg/dose PO/IV, not to exceed 6 mg/kg/dose bid/qid

Metformin 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

Documented hypersensitivity; hepatic coma, anuria, and state of severe electrolyte depletion

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 (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

Anticoagulants

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


Heparin

Augments 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

Loading 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

Loading 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

Digoxin, nicotine, tetracycline, and antihistamines may decrease effects; NSAIDs, aspirin, dextran, dipyridamole, and hydroxychloroquine may increase heparin toxicity

Documented hypersensitivity; subacute bacterial endocarditis; active bleeding; history of heparin-induced thrombocytopenia

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

In 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


Warfarin (Coumadin)

Most commonly used oral anticoagulant. Interferes with hepatic synthesis of vitamin K-dependent coagulation factors; used for prophylaxis and treatment of thromboembolic disorders.

Adult

2-10 mg/d PO qd; adjust dose to an INR of 2-3 or higher depending on the condition requiring anticoagulation

Pediatric

Administer weight-based dose of 0.05-0.34 mg/kg/d PO; adjust dose according to desired INR

Drugs 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

Documented hypersensitivity; severe liver or kidney disease; open wounds or GI ulcers

Pregnancy

X - Contraindicated; benefit does not outweigh risk

Precautions

Do 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)

More on Cor Triatriatum

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Treatment & Medication: Cor Triatriatum
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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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