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Double outlet right ventricle

Doble sortida del ventricle dret a Vall d'Hebron

This is a heart condition affecting babies in which the two main arteries of the heart (the aorta and the pulmonary artery) originate in the right ventricle. This kind of disorder may be associated with other alterations such as ventricular septal defect (VSD) or pulmonary valve stenosis. 

Description

What is double outlet right ventricle?

A ventricular septal defect (VSD - or interventricular communication) is a hole in the partition separating the two ventricles. Communication between them is necessary, as it allows oxygenated blood from the left chamber to pass to the aorta.

In the case of double outlet right ventricle (DORV), however, oxygenated and unoxygenated blood becomes mixed, meaning the oxygen level is lower than normal. Different corrective measures must be taken depending on the characteristics of the condition.

Besides this, some children may have pulmonary valve stenosis, which is when there is an obstruction in the blood flow to the lungs.

 

Diagnosis

Double outlet right ventricle is diagnosed via echocardiogram. It is increasingly detected prenatally or when a baby has just been born. In some cases, imaging or catheterization techniques have to be undertaken to look at the DORV in detail to determine the most appropriate surgical procedure. 

 

Typical treatment

DORV and related damage is usually surgically repaired during the first six months of life.  In this procedure, the left ventricle is connected to the aorta, and the septal defect is therefore closed off.

In babies without pulmonary stenosis, a band is also usually fitted around the pulmonary artery to reduce the excessive pulmonary blood flow.

A different procedure has to be carried out on babies with pulmonary stenosis. In this case, the valve has to be repaired with a patch enlargement, or in more serious cases, using a conduit. If stenosis is severe, it may be necessary to perform intermediary surgery to increase lung flow and reach an adequate level of oxygen in the blood before the final repair can be carried out. 

Most patients need no further intervention during their lifetime, apart from children who have been given corrective surgery such as a conduit into the pulmonary artery. In this case further surgery will certainly be required. Despite this surgery, most children treated will be able to lead a normal life, although they will have to be monitored by a cardiologist. 

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General Hospital

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Maternity and Children's Hospital

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Dr. David
García-Dorado García
Person in charge/Manager/Director
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Cardiology
Dra. Asunción
Torrents Fernández