Double-outlet right ventricle (DORV)
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Double-outlet right ventricle (DORV) is a congenital heart defect where two major arteries, the aorta (which carries blood to the body) and the pulmonary artery (which carries blood to the lungs), are not connected as they should be in the heart. Normally, the aorta connects to the left lower heart chamber, while the pulmonary artery connects to the right lower chamber. But in babies with DORV, both arteries partially or fully connect to the right lower heart chamber, creating a hole between the lower chambers called a ventricular septal defect (VSD). This leads to a mixing of oxygen-rich and oxygen-poor blood, causing symptoms like skin discoloration.
Symptoms of DORV usually appear in the first few weeks after birth. Most infants born with DORV need open-heart surgery within their first year to fix the heart’s malformation and improve blood flow. Additionally, DORV can sometimes occur along with other heart issues, like additional defects or problems with heart valves or blood vessels. This makes it important for affected infants to receive comprehensive medical evaluation and treatment.
In DORV, there’s always a VSD (hole in the septum between the ventricles). This hole allows blood to flow between the ventricles. DORV can be categorized based on the location of the VSD:
- DORV with subaortic VSD: In this type, the VSD is right under the aorta.
- DORV with subpulmonary VSD (Taussig-Bing Anomaly): Here, the VSD is located under the pulmonary artery.
- DORV with doubly committed VSD: This variation involves a VSD positioned under both major arteries, the aorta and pulmonary artery.
- DORV with non-committed (or Remote) VSD: In this case, the VSD is not close to either the aorta or the pulmonary artery.
Symptoms
Symptoms of DORV (Double Outlet Right Ventricle) typically become noticeable shortly after birth and resemble those of other congenital heart conditions. These signs may include:
- Bluish or purplish skin, lips, or nails (cyanosis).
- Rapid breathing (tachypnea) or difficulty in breathing.
- Feeding difficulties and poor weight gain.
- Detection of a heart murmur by a healthcare provider using a stethoscope.
- Excessive sweating, especially during feeding.
- Elevated heart rate (tachycardia), exceeding 100 beats per minute.
- Unusual sleepiness or appearing lethargic.
In addition to these symptoms, infants with DORV may have other associated congenital issues, such as:
- Ciliary dysfunction, affecting tiny hair-like structures in the airway.
- Heterotaxy, where organs in the chest and abdomen are not in their usual positions.
- Intestinal malrotation, involving abnormal twisting of the baby’s intestines.
- Pulmonary stenosis, which is a narrowing or stiffening of the pulmonary valve, situated between the right ventricle and the pulmonary artery.
- Ventricular hypoplasia, where one or both ventricles are smaller than normal.
Causes
The exact cause of DORV is not fully understood, but it can occur in babies with chromosomal abnormalities.
Diagnosis
After birth, a healthcare provider might suspect a congenital heart defect if a child experiences growth delays or exhibits changes in lip, tongue, or fingernail color.
The provider may also detect a heart murmur during a stethoscope examination. Most heart murmurs are harmless and not indicative of a heart defect. However, some murmurs result from abnormal blood flow patterns in the heart.
Diagnostic tests for congenital heart defects include:
- Pulse oximetry: A fingertip sensor measures blood oxygen levels. Low oxygen levels may suggest heart or lung issues.
- Electrocardiogram (ECG or EKG): This noninvasive test records the heart’s electrical activity using chest electrodes. It helps identify irregular heart rhythms (arrhythmias).
- Echocardiogram: Sound waves (ultrasound) produce moving images of the heart, showing blood flow and valve function. When performed before birth, it’s called a fetal echocardiogram.
- Chest X-ray: This reveals heart and lung conditions, including heart enlargement or signs of heart failure, such as fluid in the lungs.
- Cardiac catheterization: A thin tube (catheter) inserted into a blood vessel, often in the groin, is guided to the heart. This provides detailed information on blood flow and heart function and allows for certain heart treatments.
- Heart magnetic resonance imaging (MRI): Used in adolescents and adults, it creates 3D images of the heart for precise measurement and evaluation of congenital heart defects.
Treatment
Most babies born with DORV (Double Outlet Right Ventricle) typically require open-heart surgery during their first year of life. Your healthcare provider will help you decide on the best course of action for surgery by considering:
- Any other heart or organ issues your baby may have.
- The overall health of your baby.
- The specific type of DORV your baby has.
The surgeon may choose from these approaches:
- Biventricular repair: If both ventricles are healthy and the DORV is not complex, the surgeon may recommend this option. It involves moving the aorta to the left ventricle.
- Intraventricular repair: In this procedure, the surgeon creates a tunnel through the VSD (Ventricular Septal Defect) to connect the aorta to the left ventricle. This tunnel is called a baffle.
- Univentricular repair: For more complex cases of DORV, the surgeon may suggest this approach, which redirects blood flow from the lower body directly to the lungs.
These surgical options are chosen based on the specific condition of your baby’s heart and overall health. Your healthcare team will guide you in making the best decision for your baby’s well-being.
