Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Double Outlet Right Ventricle With Normally Related Great Arteries Follow-up

  • Author: Maggie L Likes, MD; Chief Editor: Stuart Berger, MD  more...
 
Updated: Jan 08, 2016
 

Further Outpatient Care

See the list below:

  • 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.
Next

Further Inpatient Care

See the list below:

  • 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.
Previous
Next

Inpatient & Outpatient Medications

See the list below:

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

Transfer

See the list below:

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

Complications

See the list below:

  • 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.
Previous
Next

Prognosis

See the list below:

  • 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.[17] 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.
Previous
Next

Patient Education

See the list below:

  • 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 eMedicineHealth's Heart Health Center. Also, see eMedicineHealth's patient education article Tetralogy of Fallot.
Previous
 
Contributor Information and Disclosures
Author

Maggie L Likes, MD Pediatric Cardiologist, Seattle Children's Heart Center; Assistant Professor of Pediatrics, University of Washington School of Medicine

Maggie L Likes, MD is a member of the following medical societies: American Society of Echocardiography

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

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, American Autonomic Society, American Physiological Society

Disclosure: Received grant/research funds from Lundbeck Pharmaceuticals for none.

Chief Editor

Stuart Berger, MD Medical Director of The Heart Center, Children's Hospital of Wisconsin; Associate Professor, Department of Pediatrics, Section of Pediatric Cardiology, Medical College 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, Society for Cardiovascular Angiography and Interventions

Disclosure: Nothing to disclose.

Additional Contributors

Juan Carlos Alejos, MD Clinical Professor, Department of Pediatrics, Division of Cardiology, University of California, Los Angeles, David Geffen School of Medicine

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, International Society for Heart and Lung Transplantation

Disclosure: Received honoraria from Actelion for speaking and teaching.

Acknowledgements

Rod Tarrago, MD Pediatric Intensivist, Children's Respiratory and Critical Care Specialists; Chief Medical Information Officer, 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.

References
  1. Witham AC. Double outlet right ventricle; a partial transposition complex. Am Heart J. 1957 Jun. 53(6):928-39. [Medline].

  2. Al-Muhaya MA, Ismail SR, Abu-Sulaiman RM, Kabbani MS, Najm HK. Short- and mid-term outcomes of total correction of Taussig-Bing anomaly. Pediatr Cardiol. 2012 Feb. 33(2):258-63. [Medline].

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

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

  5. 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].

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

  7. 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].

  8. 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. 2006 Jul. 152(1):163.e1-7. [Medline].

  9. 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. 2007 May. 35(4):221-5. [Medline].

  10. Shi K, Yang ZG, Chen J, Zhang G, Xu HY, Guo YK. Assessment of Double Outlet Right Ventricle Associated with Multiple Malformations in Pediatric Patients Using Retrospective ECG-Gated Dual-Source Computed Tomography. PLoS One. 2015. 10 (6):e0130987. [Medline]. [Full Text].

  11. Beekmana RP, Roest AA, Helbing WA, et al. Spin echo MRI in the evaluation of hearts with a double outlet right ventricle: usefulness and limitations. Magn Reson Imaging. 2000 Apr. 18(3):245-53. [Medline].

  12. Li S, Ma K, Hu S, et al. Biventricular repair for double outlet right ventricle with non-committed ventricular septal defect. Eur J Cardiothorac Surg. 2015 Oct. 48 (4):580-7; discussion 587. [Medline].

  13. Tan LH, Du LZ, Carr MR, Kuzin JK, Moffett BS, Chang AC. Captopril induced reversible acute renal failure in a premature neonate with double outlet right ventricle and congestive heart failure. World J Pediatr. 2011 Feb. 7(1):89-91. [Medline].

  14. Dirks V, Prêtre R, Knirsch W, Valsangiacomo Buechel ER, Seifert B, Schweiger M, et al. Modified Blalock Taussig shunt: a not-so-simple palliative procedure. Eur J Cardiothorac Surg. 2013 Mar 28. [Medline].

  15. Lacour-Gayet F. Complexity stratification of the arterial switch operation: a second learning curve. Cardiol Young. 2012 Dec. 22(6):739-44. [Medline].

  16. 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. 2006 Apr. 29(4):545-50. [Medline].

  17. 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. 2006 Jul. 82(1):146-52. [Medline].

  18. 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].

  19. 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].

  20. Belli E, Serraf A, Lacour-Gayet F, et al. Biventricular repair for double-outlet right ventricle. Results and long-term follow-up. Circulation. 1998. 98 (19 supplement):360-367. [Medline].

  21. Belli E, Serraf A, Lacour-Gayet F, et al. Double-outlet right ventricle with non-committed ventricular septal defect. Eur J Cardiothorac Surg. 1999 Jun. 15(6):747-52. [Medline].

  22. D'Alessandro LC, Latney BC, Paluru PC, Goldmuntz E. The phenotypic spectrum of ZIC3 mutations includes isolated d-transposition of the great arteries and double outlet right ventricle. Am J Med Genet A. 2013 Apr. 161A(4):792-802. [Medline]. [Full Text].

  23. De Luca A, Sarkozy A, Ferese R, Consoli F, Lepri F, Dentici ML, et al. New mutations in ZFPM2/FOG2 gene in tetralogy of Fallot and double outlet right ventricle. Clin Genet. 2011 Aug. 80(2):184-90. [Medline].

  24. 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].

  25. Manner J, Seidl W, Steding G. Embryological observations on the morphogenesis of double-outlet right ventricle with subaortic ventricular septal defect and normal arrangement of the great arteries. Thorac Cardiovasc Surg. 1995 Dec. 43(6):307-12. [Medline].

  26. 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. 2004 Jan. 127(1):283-5. [Medline].

  27. Patel CR, Steele MA, Stewart JW. Double-outlet right ventricle with partial anomalous pulmonary venous connection:prenatal diagnosis. J Ultrasound Med. 2005 Jun. 24(6):861-4. [Medline].

  28. Silka MJ. Double-outlet ventricles. The Science and Practice of Pediatric Cardiology. 2nd ed. 1997. 1132, 1505-23.

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

  30. Tan ZP, Huang C, Xu ZB, Yang JF, Yang YF. Novel ZFPM2/FOG2 variants in patients with double outlet right ventricle. Clin Genet. 2011 Sep 15. [Medline].

  31. 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. 2002 Apr. 73(4):1317-20. [Medline].

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

  33. Wernovsky G, Hanley FL. Double outlet right ventricle. Pediatric Cardiac Intensive Care. 1st ed. 1998. 301-3.

Previous
Next
 
Neonate with double outlet right ventricle. Chest radiograph shows a mildly enlarged heart with symmetrically slightly increased pulmonary vasculature.
Double outlet right ventricle with subaortic ventricular septal defect. Arrow shows flow of oxygenated blood from left ventricle to aorta.
Repair of double outlet right ventricle with subaortic ventricular septal defect.
Double outlet right ventricle with subpulmonary ventricular septal defect (Taussig-Bing anomaly).
Complex repair of double outlet right ventricle with subpulmonary ventricular septal defect.
Double outlet right ventricle with doubly committed ventricular septal defect.
Repair of double outlet right ventricle with doubly committed ventricular septal defect showing VSD patch and intraventricular baffle.
Double outlet right ventricle with noncommitted ventricular septal defect.
Repair of double outlet right ventricle with noncommitted ventricular septal defect using a long ventricular septal defect patch.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.