Double Outlet Right Ventricle With Normally Related Great Arteries

No Results

No Results


Double outlet right ventricle (DORV) was first pathologically described in the late 19th century as partial transposition. In 1957, Witham first used the term double outlet right ventricle to describe a partial transposition of the great arteries. [1] He described 4 hearts with 2 varieties of “complete aortic transposition with the pulmonary artery in normal position.”

Double outlet right ventricle is defined as a form of ventriculoarterial connection in which both great arteries arise completely or predominantly from the morphologic right ventricle. This definition is still controversial. For example, some researchers require that the aorta and the pulmonary artery arise entirely from the right ventricle. Others require that 90% of the great vessels arise from the morphologic right ventricle. Alternatively, the 50% rule states that more than one half of both arterial trunks must arise from the morphologic right ventricle. Some require only the presence of fibrous discontinuity between the mitral and semilunar valves. This is present in most specimens and is referred to as subpulmonic and subaortic conus.

Double outlet right ventricle, with a large variability in anatomy, represents a continuum of congenital heart defects (CHDs) that includes ventricular septal defect (VSD) with significant override of the aorta, origin of both great arteries from the right ventricle, and transposition of the great arteries with pulmonary override of the VSD. A common arterial trunk may also arise completely from the right ventricle. This is actually a type of truncus arteriosus.

Pathophysiologic description and classification is accomplished by relating the location of the VSD to the arrangement of the great vessels. Each combination results in a physiologic behavior similar to that of other CHDs. The VSD in double outlet right ventricle can be subaortic, subpulmonary, noncommitted, or doubly committed. Most VSDs are nonrestrictive, but as many as 17% of patients may require VSD enlargement during repair to allow unrestricted systemic blood flow.

The most common type of VSD found in double outlet right ventricle is a subaortic type. The aortic orifice is usually posterior and to the right of the pulmonary orifice, with a spiral arterial relationship. Because the great arteries are normally related, the left ventricular outflow is directed toward the aorta, resulting in aortic oxygen saturations that exceed pulmonary saturations. Associated pulmonary stenosis is present in as many as 50% of patients with double outlet right ventricle. The resulting physiology is similar to tetralogy of Fallot, in which the aorta completely overrides the right ventricle.

Systolic pressures are equal in both ventricles and in the aorta. In the absence of pulmonary stenosis, the physiology resembles that of a large isolated VSD, in which the ratio of pulmonary to systemic blood flow is determined by the pulmonary vascular resistance. Systemic and pulmonary saturations are also affected by the degree of mixing in the right ventricle. This anatomy may result in congestive heart failure (CHF) and pulmonary vascular disease.

In double outlet right ventricle with subpulmonary VSD (Taussig-Bing anomaly), the left ventricular outflow is directed toward the pulmonary artery. This preferential streaming results in pulmonary artery saturations greater than aortic saturations. The aortic and pulmonary orifices are usually positioned side by side but are described as transposed or malposed. The rare presence of pulmonary stenosis results in physiology similar to tetralogy of Fallot. However, in the absence of pulmonary obstruction or stenosis, patients with double outlet right ventricle and subpulmonary VSD have physiology similar to transposition of the great arteries and VSD. In this case, pulmonary vascular resistance (PVR) determines pulmonary blood flow. Early-onset pulmonary obstructive vascular disease commonly develops because of increased pulmonary blood flow and pressures, yet cyanosis may be absent with high pulmonary blood flow. This type of double outlet right ventricle is frequently associated withsubaortic stenosis and arch obstruction.

Double outlet right ventricle with noncommitted or remote VSD has anatomy and physiology similar to that of an isolated VSD or atrioventricular canal defect. To meet the criteria for double outlet right ventricle with noncommitted VSD, some have suggested that the distance between the VSD and the aortic and pulmonary outflow tracts should be at least equal to the aortic valve diameter. Most commonly, the great arteries are normally related in this type of double outlet right ventricle. Pulmonary and systemic blood flow and saturations are determined by the ratio of pulmonary to systemic vascular resistance and by the degree of right ventricular mixing.

Finally, double outlet right ventricle with doubly committed VSD displays physiology in which the left ventricular outflow is equally shared by the aorta and pulmonary artery. The systemic and pulmonary vascular resistances determine the ratio of pulmonary-to-systemic blood flow. This is a relatively rare form of double outlet right ventricle that typically has normally related great arteries. Right ventricular mixing affects oxygen saturations.

Because double outlet right ventricle is the only defect in less than 50% of patients with double outlet right ventricle, classification and description may also take into consideration obstruction of the systemic circulation, ventricular anomalies, coronary artery anomalies, and conduction system abnormalities. Upon further investigation, findings of additional VSDs, anomalies of ventricular rotation, and anomalies of insertion of the subvalvar apparatus of atrioventricular valves are not uncommon.

Systemic circulation may be obstructed at the aortic valve or the obstruction may be subaortic; subaortic obstruction develops in approximately 10% of patients. Aortic valve anomalies are usually associated with mitral valve anomalies that may also be present in the form of a restrictive VSD. Coarctation of the aorta is the most common associated lesion, and interrupted aortic arch may also be present.

Patients with double outlet right ventricle can have coexisting ventricular anomalies. Left ventricular inflow anomalies are less frequent yet can be severe. Mitral stenosis or atresia is often associated with a hypoplastic left ventricle and intact ventricular septum. Left ventricular hypoplasia is present if decreased pulmonary venous return, restrictive VSD, and large atrial septal defect (ASD) are present. Misalignment of atrioventricular valves is also visible. This is very important for surgical correction and must be investigated. Finally, straddling of the atrioventricular valve annuli or straddling of the chordae may be present. Right ventricular abnormalities including tricuspid regurgitation, tricuspid stenosis, and Ebstein malformation may develop.

Coronary artery abnormalities are related to the relationship of the great arteries with several variations, including anomalous origin of the right coronary artery (RCA) from the left main coronary artery (LMCA), duplication of left anterior descending coronary artery (LAD), anomalous origin of LAD from RCA (associated with a subaortic VSD and pulmonary stenosis), anterior origin of LAD, RCA immediately beneath pulmonary annulus (seen with L-malposed aorta), and RCA from the posterior sinus of Valsalva/LMCA from the left sinus, which is seen with an anterior aorta and subpulmonary VSD and is similar to transposition of the great arteries.

Conduction system abnormalities develop because of alterations in anatomy. Anatomy of the atrioventricular node and His-Purkinje system is similar to that in an isolated perimembranous VSD. In subaortic, subpulmonary, and doubly committed VSD, conduction tissues are displaced from the superior margin of the VSD.

Other abnormalities and associations are rare and can include dextrocardia and atrioventricular discordance, superior and inferior ventricles, and single atrioventricular valve connection.

Double outlet right ventricle accounts for 1-1.5% of all CHDs, with an incidence of 1 per 10,000 live births.

Incidence is the same internationally as in the United States.

One review found early in-hospital mortality after operation to be 4.8%. [2] The rate was significantly higher in patients with complex lesions. Late mortality was 3.2% with a mean follow-up time of 5.3 years. Overall 15-year survival ranged from 89.5-95.8%, with more complex lesions exhibiting higher mortality rates. Reoperation was required in 11.2% of surviving patients. This occurred a mean of 4.1 years after the original definitive repair. The most likely cause of reoperation was right ventricular outflow tract obstruction. Fifteen-year freedom from reoperation rates in surviving patients ranged from 72-100%. The reoperation rate was higher in patients with subpulmonary VSDs.

No race predilection has been reported.

No sex predilection has been reported.

Most cases of double outlet right ventricle are diagnosed in the first month of life.

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. [3] 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.

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 patient education resources, see the Heart Health Center and Tetralogy of Fallot.

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

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].

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].

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].

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

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].

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].

Momma K, Kondo C, Matsuoka R, Takao A. Cardiac anomalies associated with a chromosome 22q11 deletion in patients with conotruncal anomaly face syndrome. Am J Cardiol. 1996 Sep 1. 78(5):591-4. [Medline].

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].

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].

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].

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].

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].

Dirks V, Pretre R, Knirsch W, et al. Modified Blalock Taussig shunt: a not-so-simple palliative procedure. Eur J Cardiothorac Surg. 2013 Dec. 44(6):1096-102. [Medline].

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

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].

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].

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 ventricle. Int J Cardiol. 1991 Oct. 33(1):5-26. [Medline].

Battistessa S, Soto B. Double outlet right ventricle with discordant atrioventricular connexion: an angiographic analysis of 19 cases. Int J Cardiol. 1990 May. 27(2):253-63; discussion 265-7. [Medline].

Belli E, Serraf A, Lacour-Gayet F, et al. Biventricular repair for double-outlet right ventricle. Results and long-term follow-up. Circulation. 1998 Nov 10. 98(19 Suppl):II360-5; discussion II365-7. [Medline].

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].

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].

De Luca A, Sarkozy A, Ferese R, 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].

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].

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].

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].

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].

Silka MJ. Double-outlet ventricles. Garson A Jr, Bricker JT, Fisher DJ, Neish SR, eds. The Science and Practice of Pediatric Cardiology. 2nd ed. Philadelphia: Williams & Wilkins; 1997. 1132, 1505-23.

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].

Tan ZP, Huang C, Xu ZB, Yang JF, Yang YF. Novel ZFPM2/FOG2 variants in patients with double outlet right ventricle. Clin Genet. 2012 Nov. 82(5):466-71. [Medline].

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].

Walters HL 3rd, Mavroudis C, Tchervenkov CI, Jacobs JP, Lacour-Gayet F, Jacobs ML. Congenital Heart Surgery Nomenclature and Database Project: double outlet right ventricle. Ann Thorac Surg. 2000 Apr. 69(4 Suppl):S249-63. [Medline].

Wernovsky G, Hanley FL. Double outlet right ventricle. Chang A, Hanley FL, Wernovsky G, Wessel DL, eds. Pediatric Cardiac Intensive Care. Philadelphia: Lippincott Williams & Wilkins; 1998. 301-3.

Lee MY, Won HS, Baek JW, et al. Variety of prenatally diagnosed congenital heart disease in 22q11.2 deletion syndrome. Obstet Gynecol Sci. 2014 Jan. 57(1):11-6. [Medline].

Goo HW. Coronary artery anomalies on preoperative cardiac CT in children with tetralogy of Fallot or Fallot type of double outlet right ventricle: comparison with surgical findings. Int J Cardiovasc Imaging. 2018 Jul 26. [Medline].

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.

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 research grant from: Lundbeck Pharmaceuticals<br/>Received grant/research funds from Lundbeck Pharmaceuticals for none.

Stuart Berger, MD Executive Director of The Heart Center, Interim Division Chief of Pediatric Cardiology, Lurie Childrens Hospital; Professor, Department of Pediatrics, Northwestern University, The Feinberg School of Medicine

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.

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.

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.

Double Outlet Right Ventricle With Normally Related Great Arteries

Research & References of Double Outlet Right Ventricle With Normally Related Great Arteries|A&C Accounting And Tax Services