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

Double Outlet Right Ventricle, Normally Related Great Arteries: Follow-up

Author: Rod Tarrago, MD, Pediatric Intensivist, Department of Pediatric Critical Care, Children's Hospitals and Clinics of Minnesota
Coauthor(s): Steven R Neish, MD, SM, Director of Pediatric Cardiology Fellowship Program, Associate Professor, Department of Pediatrics, Baylor College of Medicine
Contributor Information and Disclosures

Updated: Oct 6, 2008

Follow-up

Further Inpatient Care

  • Provide inpatient care if congestive heart failure (CHF) is severe. Treat patients initially with fluid restriction and alleviation of temperature and physical stress. Sedation may be required with opioids.
  • Observe and manage ventricular function for patients in immediate postoperative period. Arrhythmias may develop after repair and may require medical intervention.

Further Outpatient Care

  • After repair, children with double outlet right ventricle (DORV) are often treated with systemic afterload reduction using ACE inhibitors for several months to assist in cardiac remodeling.

Inpatient & Outpatient Medications

  • Commonly used medications are listed above (see Medication), including furosemide, digoxin, captopril, and enalapril.

Transfer

  • Transfer may be required for further diagnostic testing and medical/surgical treatment.

Complications

  • If patients undergo surgery for repair at an older age, they often develop ventricular dysfunction and elevation of pulmonary artery pressures.
  • Operative and postoperative complications depend on anatomy of lesion and type of repair.
    • Some patients develop restrictive ventricular septal defect (VSD) and require reoperation.
    • In patients with subaortic and subpulmonary VSD, the VSD diameter can decrease by 20% in the immediate postoperative period. These patients can sometimes develop subaortic obstruction.
    • Patients, especially those undergoing complex repair, can develop postoperative ventricular dysfunction associated with residual VSD, aortic insufficiency, atrioventricular valve insufficiency, and prolonged circulatory arrest at repair.
    • Some patients are at risk for late postoperative arrhythmias and sudden death.
    • Patients may develop persistent atrial tachycardia, complex ventricular ectopy, or syncope requiring electrophysiologic studies.

Prognosis

  • The long-term survival rate for children who undergo repair for a subaortic VSD type of double outlet right ventricle is 80-95%.
  • A retrospective study analyzed the pregnancy outcome of patients with previous biventricular repair of double outlet right ventricle. The study, which included 19 pregnancies, found a premature labor rate of 44% at a median of 32 weeks' gestation.12  Other complications included diminished fertility, menstrual disorders, and a higher than expected rate of neonates that were small for gestational age. However, despite these complications, 17 of the 19 pregnancies resulted in live births.

Patient Education

  • Educate parents regarding anatomic defect, surgical repair, and postoperative course. Prior to repair, parents should learn about medical therapy and signs and symptoms of CHF.
  • Institute a specific nutritional program to attain adequate weight gain.
  • For excellent patient education resources, visit eMedicine's Heart Center. Also, see eMedicine's patient education article Tetralogy of Fallot.

Miscellaneous

Medicolegal Pitfalls

  • Failure to make the correct diagnosis
  • Failure to prepare for and treat surgical complications
 


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References
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References

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  2. Brown JW, Ruzmetov M, Okada Y, et al. Surgical results in patients with double outlet right ventricle: a 20- year experience. Ann Thorac Surg. Nov 2001;72(5):1630-5. [Medline].

  3. Kirby ML, Waldo KL. Role of neural crest in congenital heart disease. Circulation. Aug 1990;82(2):332-40. [Medline].

  4. Goldmuntz E, Clark BJ, Mitchell LE, et al. Frequency of 22q11 deletions in patients with conotruncal defects. Journal of the American College of Cardiology. 1999;32:499-501. [Medline].

  5. Khositseth A, Tocharoentanaphol C, Khowsathit P, Ruangdaraganon N. Chromosome 22q11 deletions in patients with conotruncal heart defects. Pediatr Cardiol. Sep-Oct 2005;26(5):570-3. [Medline].

  6. Momma K, Kondo C, Matsuoka R, Takao A. Cardiac anomalies associated with a chromosome 22q11 deletion in patients with conotruncal anomaly face syndrome. American Journal of Cardiology. 1996;78:591-594. [Medline].

  7. Pitkanen OM, Hornberger LK, Miner SE, et al. Borderline left ventricles in prenatally diagnosed atrioventricular septal defect or double outlet right ventricle: echocardiographic predictors of biventricular repair. Am Heart J. Jul 2006;152(1):163.e1-7. [Medline].

  8. Tongsong T, Chanprapaph P, Sittiwangkul R, Khunamornpong S. Antenatal diagnosis of double outlet of right ventricle without extracardiac anomaly: a report of 4 cases. J Clin Ultrasound. May 2007;35(4):221-5. [Medline].

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  10. Artrip JH, Sauer H, Campbell DN, et al. Biventricular repair in double outlet right ventricle: surgical results based on the STS-EACTS International Nomenclature classification. Eur J Cardiothorac Surg. Apr 2006;29(4):545-50. [Medline].

  11. Takeuchi K, McGowan FX, Bacha EA, et al. Analysis of surgical outcome in complex double-outlet right ventricle with heterotaxy syndrome or complete atrioventricular canal defect. Ann Thorac Surg. Jul 2006;82(1):146-52. [Medline].

  12. Drenthen W, Pieper PG, van der Tuuk K, et al. Fertility, pregnancy and delivery in women after biventricular repair for double outlet right ventricle. Cardiology. 2008;109(2):105-9. [Medline].

  13. Bartelings MM, Gittenberger-de Groot AC. Morphogenetic considerations on congenital malformations of the outflow tract. Part 2: Complete transposition of the great arteries and double outlet right. International Journal of Cardiology. 1991;33:5-26. [Medline].

  14. Battistessa S, Soto B. Double outlet right ventricle with discordant atrioventricular connexion: an angiographic analysis of 19 cases. International Journal of Cardiology. 1990;27:253-267. [Medline].

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  18. Oppido G, Napoleone CP, Loforte A, et al. Complex double-outlet right ventricle repair in a neonate with complete tracheal agenesis. J Thorac Cardiovasc Surg. Jan 2004;127(1):283-5. [Medline].

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  21. Takeuchi K, McGowan FX Jr, Moran AM, et al. Surgical outcome of double-outlet right ventricle with subpulmonary VSD. Ann Thorac Surg. Jan 2001;71(1):49-52; discussion 52-3. [Medline].

  22. Tchervenkov CI, Korkola SJ, Beland MJ. Single-stage anatomical repair of complete atrioventricular canal, double-outlet right ventricle, and cor triatriatum using ventricular septal defect translocation. Ann Thorac Surg. Apr 2002;73(4):1317-20. [Medline].

  23. Walters HL, Mavroudis C, Tchervenkov CI, et al. Congenital Heart Surgery Nomenclature and Database Project: double outlet right ventricle. Ann Thorac Surg. Apr 2000;69(4 Suppl):S249-63. [Medline].

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Further Reading

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Keywords

double outlet right ventricle, normally related great arteries, DORV, both great arteries originating from the right ventricle, partial transposition complex of the great arteries, transposition of the aorta and levoposition of the pulmonary artery, congenital heart defect, CHD, ventricular septal defect, VSD, Taussig-Bing anomaly, tetralogy of Fallot, DiGeorge syndrome, DiGeorge's syndrome, neural crest, congestive heart failure, subaortic stenosis, arch obstruction, atrioventricular canal defect, mitral stenosis, coarctation of the aorta, interrupted aortic arch, mitral atresia, atrial septal defect, ASD, tricuspid regurgitation, Ebstein malformation, transposition of the great arteries, hypercyanotic spells, polycythemia, failure to thrive

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Contributor Information and Disclosures

Author

Rod Tarrago, MD, Pediatric Intensivist, Department of Pediatric Critical Care, Children's Hospitals and Clinics of Minnesota
Rod Tarrago, MD is a member of the following medical societies: Society of Critical Care Medicine
Disclosure: Nothing to disclose.

Coauthor(s)

Steven R Neish, MD, SM, Director of Pediatric Cardiology Fellowship Program, Associate Professor, Department of Pediatrics, Baylor College of Medicine
Steven R Neish, MD, SM is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Heart Association
Disclosure: Nothing to disclose.

Medical Editor

Juan Carlos Alejos, MD, Associate 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 broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Julian M Stewart, MD, PhD, Associate Chairman of Pediatrics, Director, Center for Hypotension, Westchester Medical Center; Professor of Pediatrics and Physiology, New York Medical College
Julian M Stewart, MD, PhD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

CME Editor

Gilbert Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College
Gilbert 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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