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

Cor Triatriatum: Treatment & Medication

Author: M Silvana Horenstein, MD, Staff Physician, Department of Pediatrics, University of Texas Medical School Houston; Medical Doctor Consultant, Legacy Department, Best Doctors, Inc
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; Michael Pettersen, MD, Director of Echocardiography, Division of Cardiology, Children's Hospital of Michigan; Assistant Professor of Pediatrics, Wayne State University School of Medicine
Contributor Information and Disclosures

Updated: Jun 30, 2009

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.

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

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

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

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.

Adult

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

Pediatric

Administer as in adults

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

Documented hypersensitivity; 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

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.

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

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

Documented hypersensitivity; hypokalemia; renal 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

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

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
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References

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  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. 2nd ed. Churchill Livingstone; 1993:675-81.

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  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. 6th 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].

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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

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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.

 
 
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