Double Outlet Right Ventricle

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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".



References

Wikipedia. ICD-10,ICD-9



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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.

References

Anatomy

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.

ii. Subaortic.

iii. Subpulmonic.

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.

References

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

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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).

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Diagnosis

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Appearance of the Patient

Eyes

Ear Nose and Throat

Heart

Lungs

Abdomen

Extremities

Neurologic

Other

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Laboratory Findings

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.


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Monitor serum electrolytes after treating children with diuretics, glycosides, and afterload-reducing agents.


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If there is mild or no pulmonary stenosis, 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.

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Other Imaging Findings

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Other Diagnostic Studies

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Treatment

Pharmacotherapy

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Surgery and Device Based Therapy

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References

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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