DORV is a congenital (condition present at birth) heart defect that is found very rarely. In this condition both the pulmonary artery and the aorta rise, completely or partly, from the right ventricle. In a normal heart only the pulmonary artery arises from the right ventricle. The aorta normally arises from the left ventricle. The pulmonary artery carries caries blood to the lungs for oxygen while the aorta carries oxygenated blood from the heart to the body.
Due to this condition oxygen rich blood gets mixed with oxygen poor blood and so oxygen levels in the blood do not maintain the optimal level which results in the heart having to work more hard to maintain circulation. This may result in congestive heart failure (CHF) or pulmonary vascular disease due to limitation of blood flow and pressure by pulmonary stenosis.
DORV almost always corresponds with a ventricular septal defect (VSD), an abnormal opening in the septum (wall that separates the two ventricles) that allows blood to pass between the right and left ventricles. The degree of the condition is related to the position of the great vessels, the size of the VSD, and any other associated conditions within the heart. Nearly all instances of DORV are diagnosed in the first month of life.
Congenital heart problems like the DORV usually appear during the first eight weeks of fetal development. It occurs mostly due to a genetic link, a defective gene or a chromosome abnormality.
The symptoms and their severity may vary from child to child and the age at which they first occur. Some of the common symptoms include fatigue, congested breathing, blue colour of skin, lips and nailbeds (cyanosis) or poor weight gain.
Diagnosis and Treatment
DORV can be identified by pediatricians or cardiologists by a bluish tint to the skin or by listening to the heart murmur. Some of the medical tests used to diagnose DORV are Echocardiogram, Cardiac Magnetic Resonance Imaging or Cardiac catheterization. Sometimes a combination of the above tests maybe required.
DORV is usually treated by surgery to connect the aorta to the left ventricle. The two major operations for this treatment are intraventricular repair and arterial switch operation. The operation procedure depends on a variety of factors such as the child’s age, medical history and extent of the condition. The site of the VSD may vary and can also affect the clinical manifestations and the options for surgery.