Double Outlet Right Ventricle
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Name of Disease State
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= Overview = Associate Editor-In-Chief: Contact at [ @ ]; Phone:
The Double Outlet of the Right Ventricle DORV, is a complex heart defect. It is a type of ventriculoarterial connection in which most of the aorta and the pulmonary artery arise from the right ventricle. Generally, it is associated with concordan atrio-ventricular connection.
Pathologist define the DORV when more than 50% of both great arteries arise from the right ventricle, but surgeons take a higher value: 90% .
Usually, a VSD is associated and less often an ASD, anomaly of the coronary arteries, different grades of aortic or pulmonic artery stenosis, etc. It can occur with or without transposition of the great arteries. When it occurs in conjunction with anterior VSD defect, it is called "Taussig-Bing syndrome".
- 1 References
- 2 References
- 3 Anatomy
- 4 Diagnosis
- 4.1 References
- 4.2 References
- 4.3 Appearance of the Patient
- 4.4 Eyes
- 4.5 Ear Nose and Throat
- 4.6 Heart
- 4.7 Lungs
- 4.8 Abdomen
- 4.9 Extremities
- 4.10 Neurologic
- 4.11 Other
- 4.12 Laboratory Findings
- 5 Treatment
- 5.1 Pharmacotherapy
- 5.2 Surgery and Device Based Therapy
- 5.3 Acknowledgements
- 5.4 Suggested Reading and Key General References
- 5.5 Suggested Links and Web Resources
- 5.6 For Patients
- 6 Basic Instructions on Editing This Page
= Epidemiology and Demographics = Associate Editor-In-Chief: Contact at [ @ ]; Phone:
The Double Outlet of the Right Ventricle DORV, accounts for 1-1.5% of all Congenital Heart Disease. It has an incidence of 1 in 10000 live births.
Generally, it is diagnosed during the first month of life, and there is no sex or race predilection.
Most Medical Centers have reported an approximated in-hospital mortality after surgery of 4.8% with a 3.2% for late mortality. The more complex the defect, the higher the mortality and the need for reoperation.
In the DORV, the aorta and the pulmonary artery are related to the morphologic RV. There are three essential gross morphologic features that allow the distintion among the many variants:
a) The relationship of the great arteries to the ventricles:
i. Aorta to the right and posterior to the pulmonary trunk (normal arrangement present in a 3% of the RVOT cases).
ii. The great arteries side by side with the aorta to the right of the pulmonary trunk is the usual arrangement (approximately 64%)in double outlet RV. Physiologically similar to Tetralogy of Fallot.
iii. Aorta to the right and anterior to the pulmonary trunk (24% of the RVOT cases). Physiollogically resembles TGA, specifically D-TGA with VSD.
iv. Aorta to the left and anterior to the pulmonary trunk (rarest arrangement representing a 7% of the RVOT cases _ L-TGA_ pattern).
b) The relationship of the VSD to the great arteries:
i. Provides the morphologic LV with its only outlet.
iv. Beneath both (doubly committed).
v. Beneath neither.
c) The presence or absence of outflow obstruction to RV outflow.
i. Pulmonic stenosis occurs in 41 to 72% of patients with double outlet RV.
ii. When pulmonic stenosis occurs, the VSD is almost always subaortic.
iii. RV blood is diverted into the aorta with a decrease in the oxygen saturation.
The most common combination is the side-by-side relationship of the great arteries, with the aorta directly to the right and with the VSD in the subaortic location.
a) The LV blood selectively enters the aorta because of the proximity of the VSD to the aorta.
b) In the absence of PS, the physiologic consequences are similar to those of an isolated VSD.
c) A low PVR permits RV blood to flow almost exclusively into the pulmonary trunk and permits a substantial portion of the LV blood to enter the pulmonary circulation.
d) Aortic O2 saturation is normal, and Pulmonary Artery (PA) blood flow is increased.
The Taussig-Bing anomaly involves the same great vessel relationship, but there is a subpulmonic VSD.
a) LV blood selectively enters the pulmonary trunk because of the proximity of the subpulmonic VSD to the pulmonary artery (PA).
b) Accounts for approximately 8% of cases.
Double Outlet Right Ventricle, Chapter 19, in Perloff, Congenital Heart Disease, pp. 443-466.
M Silvana Horenstein, MD, Double Outlet Right Ventricle, With Transposition. e medicine.2005
= Risk Factors = Associate Editor-In-Chief: Contact at [ @ ]; Phone:
a) Most drugs affect the development of the cardiovascular system, because it appears earlier during the embrionic period. Drugs such as busulfan, lithium, retinoids, thalidomide, and trimethadione can cause cardiac defects. Busulfan is an animal teratogen, and there are not definitive data that exposures have resulted in birth defects in humans. The other drugs are proven human teratogens, depending on the dose. The teratogenic risk of lithium is low and controversial.
b) Maternal use of alcohol during the first trimester of pregnancy.
c) Exposure to solvents during pregnancy.
d) Approximately, a 40%–80% increased risk with first-trimester febrile illness.
e) Fetal "nutrition" which is affected by maternal diet, uterine blood flow, placental function, and the fetal genome.
Suzanne M. Mone, MD et al. Effects of Environmental Exposures on the Cardiovascular System: Prenatal Period Through Adolescence. Pediatrics. vol 113. 2004
Tikkanen J, Heilonen OP. Maternal hyperthermia during pregnancy and cardiovascular malformations in the offspring. Eur J Epidemiol.1991; 7 :628 –635.
DeSantis M, Carducci B, Cavaliere AF, DeSantis L, Straface G, Caruso A. Drug-induced congenital defects. Strategies to reduce the incidence. Drug Saf.2001; 24 :889 –901
= Screening = Associate Editor-In-Chief: Contact at [ @ ]; Phone:
= Pathophysiology & Etiology= Associate Editor-In-Chief: Contact at [ @ ]; Phone:
The pathophysiology of the DORV is very variable, changing from case to case. It depends on the great artery relationship (side by side, d-TGA, l-TGA, normally related arteries), the relationship of the VSD to the great arteries (subaortic or subpulmonary VSD), and on the presence or absence of RV outflow tract obstruction or pulmonary stenosis (PS).
The most common type of DORV, referes to the one with similar physiopathology than Tetralogy of Fallot and great arteries lying side by side. The aorta is located to the right of the pulmonary artery and both semilunar valves lying in the same transverse plane. The VSD in the subaortic location. If the VSD is a subpulmonic VSDis called Taussig-Bing anomaly.
The second type of DORV is the type where the aorta is anterior and to the right of the pulmonary artery (PA), funtionally resembling transposition of the great arteries (TGA), D-TGA, with a VSD. A less frequent type is where the aorta (AO) is anterior and to the left of the PA (left-transposition of the great arteries [L-TGA]).
In rare cases of DORV, there is a normal great artery relationship with the aorta arising posterior and to the right of the PA.
Clinical manifestations depends on the size, but mostly on the location of the VSD in relation to the great arteries. Also the clinical symptoms will depend on the presence or absence of a pulmonary stenosis.
When the VSD is subaortic, the oxygenated blood is pump from the LV into the Aorta, and deoxygenated blood flow from the right atrium to the pulmonary artery (closer to its location).
= Molecular Biology = Associate Editor-In-Chief: Contact at [ @ ]; Phone:
= Genetics = Associate Editor-In-Chief: Contact at [ @ ]; Phone:
= Natural History = Associate Editor-In-Chief: Contact at [ @ ]; Phone:
== Differential Diagnosis == Associate Editor-In-Chief: Contact at [ @ ]; Phone:
== History and Symptoms == Associate Editor-In-Chief: Contact at [ @ ]; Phone:
== Physical Examination == Associate Editor-In-Chief: Contact at [ @ ]; Phone:
Appearance of the Patient
Ear Nose and Throat
During the initial diagnosis and management of children with double outlet right ventricle (DORV), routine laboratory studies are not required.
If Polycytemia is suspected, order a hemoglobin and hematocrit assessment.
=== Electrolyte and Biomarker Studies === Associate Editor-In-Chief: Contact at [ @ ]; Phone:
Monitor serum electrolytes after treating children with diuretics, glycosides, and afterload-reducing agents.
=== Electrocardiogram === Associate Editor-In-Chief: Contact at [ @ ]; Phone:
The ECG often will present with a normal sinus rhythm, but associated with conduction defects with a prolong PR intervale, usually first degree A-v block. P pulmonale is present in 75% of cases (tall P wave in Leads II, III and AVF).
Due to the fact that the right ventricle has to work for the two great arteries at the same time, usually the RV is hyperthrophic and there is a right axis deviation
There are a lot of ECG variations depending on the variety of DORV with TGA. In that regard, if there is a Subaortic VSD with no PS may has superior QRS axis (-30° to -170°) with either RVH or biventricular hypertrophy and left atrial enlargement. First-degree AV block may be present with this lesion. On the other hand, if there is subpulmonic VSD or in those with subaortic VSD and PS shows right axis deviation, RVH, and often right atrial enlargement.
Oliver W. Caminos, M.D.Double outlet right ventricle. Chapter 21.
=== Chest X Ray === Associate Editor-In-Chief: Contact at [ @ ]; Phone:
If there is mild or no pulmonary stenosis, subpulmonary or subaortic VSD, cardiomegaly can be present due to an increase in pulmonary blood flow. When transposition of the great arteries is present, the mediastinum may be narrow. These feaures are not specific for DORV.
If PS is present, chest radiography shows a normal heart size and normal-to-decreased pulmonary vascular markings
=== MRI and CT === Associate Editor-In-Chief: Contact at [ @ ]; Phone:
It allows to determine the visceral and atrial situs as vasculo-vascular and vasculo-visceral relationships. MRI may aid in defining the spatial relationship between VSD and the semilunar valves.
=== Echocardiography or Ultrasound === Associate Editor-In-Chief: Contact at [ @ ]; Phone:
The Subxiphoid view allows to characterize the origin of both great vessels emerging from the RV, and also their relationship to one another. In addition to the anatomy mentioned before it allows the evaluation of the sub-pulmonic conus, the severity of the pulmonary stenosis and the relationship of the VSD to the aortic valve.
Other Imaging Findings
Other Diagnostic Studies
The cardiac catheterization is a diagnostic used particularly prior to complete repair, or prior to B-T shunt placement in cases of severe pulmonary stenosis.
= Risk Stratification and Prognosis= Associate Editor-In-Chief: Contact at [ @ ]; Phone:
=== Acute Pharmacotherapies === Associate Editor-In-Chief: Contact at [ @ ]; Phone:
=== Chronic Pharmacotherapies === Associate Editor-In-Chief: Contact at [ @ ]; Phone:
Surgery and Device Based Therapy
=== Indications for Surgery === Associate Editor-In-Chief: Contact at [ @ ]; Phone:
=== Pre-Operative Assessment === Associate Editor-In-Chief: Contact at [ @ ]; Phone:
=== Post-Operative Management === Associate Editor-In-Chief: Contact at [ @ ]; Phone:
=== Transplantation === Associate Editor-In-Chief: Contact at [ @ ]; Phone:
== Primary Prevention == Associate Editor-In-Chief: Contact at [ @ ]; Phone:
== Secondary Prevention == Associate Editor-In-Chief: Contact at [ @ ]; Phone:
== Cost-Effectiveness of Therapy == Associate Editor-In-Chief: Contact at [ @ ]; Phone:
== Future or Investigational Therapies == Associate Editor-In-Chief: Contact at [ @ ]; Phone:
==Suggested Revisions to the Current Guidelines== Associate Editor-In-Chief: Contact at [ @ ]; Phone:
J. Stark and M. de Laval. Surgery for Congenital Heart Defects. Grune & Stratton.
Gatzoulis, Swan, Therrien, and Pantely. Adult Congenital Heart Disease. Blackwell Publishing.
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Suggested Reading and Key General References
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