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

Cor Triatriatum: Follow-up

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

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.
 
Acknowledgments

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.



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].

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  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].

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  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].

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  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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