This is a congenital heart disease, meaning the individual is born with the condition. The infants have problems with the formation of the heart in the womb where both the great arteries known as pulmonary artery and the aorta are attached to the right ventricle. In healthy individuals, the pulmonary artery that carries the blood to the lungs arises from the right ventricle and the aorta that delivers the oxygen-rich blood to the body arises from the left ventricle. In addition, they also present another condition known as ventricular septal defect (VSD) where a hole occurs in the wall of the heart that divides the ventricle. The congenital heart defect appears to develop sporadically with no clear reason identified. Although the prognosis appears good when the defect is corrected but long-term complications can develop particularly if the treatment occurs in their later age with the risk of arrhythmias and heart infections.
There are four types of DORV that consist of
- DORV with a subaortic VSD where the defect is located just below the aorta
- DORV with a subpulmonary VSD that presents problem below the pulmonary artery
- DORV with a double committed VSD presents two VSDs, one below the aorta and the other below the pulmonary artery
- DORV with a non-committed VSD where the defect is not present near the aorta or the pulmonary artery
Symptoms
The symptoms of double outlet right ventricle are based on the amount of oxygenated blood that is delivered throughout the body. Usually, most infants may present symptoms within the first few days of their life and can include poor weight gain, blue tint on their lips, unresponsiveness of the infants, difficulty with breathing, profuse sweating, loss of interest in feeding, extreme tiredness and heart murmur. It is important to get medical help, particularly when the child has difficulty with breathing, exhibits rapid breathing, has bluish color or is uninterested in feeding.
Diagnosis
Double outlet right ventricle can be detected prenatally. After the birth, the condition may be diagnosed by electrocardiogram that assesses the electrical activity of the heart, chest x-ray, cardiac MRI to identify heart abnormalities and echocardiogram.
Treatment
The treatment is based on the type of DORV, the age of the child and the severity of the condition. Most cases of double outlet right ventricle require surgery to correct the heart defect that focuses on connecting the aorta to the left ventricle and the pulmonary artery to the right ventricle. The VSD that are identified are also closed during the surgical procedure. Medications may also be used to assist the heart function prior to and after the surgery.
References
http://www.texasheart.org/HIC/Topics/Cond/dorv.cfm
http://www.chop.edu/conditions-diseases/double-outlet-right-ventricle
