In double outlet right ventricle (DORV), a congenital heart disease, something goes wrong during the formation of the heart in the womb and both great arteries are attached to the right ventricle. No arteries, or only a part of the aorta, are attached to the left ventricle.
The human heart has four chambers. The top chambers, the right and left atria, receive blood flowing into the heart. The bottom chambers, the right and left ventricles, pump blood out of the heart.
In a normal heart, the right atrium receives blood that has traveled through the body and has little oxygen left in it. The right ventricle pumps that blood out to the lungs through a large blood vessel called the pulmonary artery. The blood picks up oxygen in the lungs, travels back to the left side of the heart and is pumped out to the body through a large blood vessel called the aorta. Because the pulmonary artery and the aorta are the largest blood vessels in the body, doctors sometimes call them the great vessels or the great arteries.
Children born with a double outlet right ventricle heart defect always have another heart defect called a ventricular septal defect (VSD). This is a hole in the septum, the wall that divides the right and left ventricle. The VSD is actually helpful — it allows the child to live. Without the VSD, blood that traveled to the lungs and back to the left side of the heart would have no place to go, since the aorta isn’t attached to the left ventricle, and no oxygenated blood would reach the body.
The VSD allows oxygenated blood flowing into the left side of the heart to cross over to the right ventricle, and be pumped out to the body through the aorta. However, because it’s a mix of the de-oxygenated blood in the right ventricle and the oxygenated blood from the left ventricle, the blood isn’t as high in oxygen as it should be, and this causes problems.
In some cases, because more blood than normal is flowing into the right ventricle, this heart defect means that too much blood is pumped to the lungs. Over time, this uncontrolled flow can damage the lungs and heart, and heart failure can result.
In some cases, children with double outlet right ventricle also have pulmonary stenosis, a problem with the valve that leads out of the heart to the pulmonary artery. There may also be irregularities with the mitral valve, which separates the left atrium and left ventricle.
There are different types of double outlet right ventricle, classified according to the location of the VSD and how the great arteries are arranged with respect to each other. The Cardiac Center team will explain your child’s heart defect to you in detail.
Double outlet right ventricle symptoms vary depending on how much oxygenated blood is reaching the body. In most cases, children will have symptoms in the first days or weeks of life, including:
- Blue or purple tint to lips, skin and nails (cyanosis)
- Poor eating and poor weight gain
- Rapid breathing or shortness of breath
- Profuse sweating, especially with feeding
- More sleepiness than normal
- Unresponsiveness: the baby seems “out of it”
- Heart murmur: the heart sounds abnormal when a doctor listens with a stethoscope
In some cases, double outlet right ventricle is diagnosed prenatally. The Fetal Heart Program at Children's Hospital can monitor both baby and mother throughout the pregnancy and create a plan for labor and care after the birth. CHOP has its own delivery unit, on the same floor as cardiac operating rooms and cardiac patient care units. Newborns can be in the care of pediatric cardiologists and specially trained cardiac nurses immediately.
After the child is born, diagnosis may include:
- Electrocardiogram (EKG or ECG): a record of the electrical activity of the heart
- Echocardiogram (also called “echo” or ultrasound): sound waves create an image of the heart
- Chest x-ray
- Cardiac MRI: a three-dimensional image shows the heart's abnormalities
Sometimes, cardiac catheterization will be required. A thin tube is inserted into the heart through a vein and/or artery in either the leg or through the umbilicus (“belly button”).
Medications may be necessary early on to help the heart function better prior to and after surgical intervention. However, double outlet right ventricle requires open heart surgery.
Surgeons have a variety of methods to repair this heart defect, depending on the anatomy (structure) of the child’s heart. Sometimes they use a patch made of a synthetic fiber called Dacron to create a tunnel (called a baffle) through the VSD (the hole between the right and left ventricles). The tunnel connects to the aorta. This means all of the oxygenated blood from the left ventricle is channeled through the right ventricle into the aorta, without mixing with the deoxygenated blood in the right ventricle.
In other cases, surgeons reverse the double outlet right ventricle by detaching the aorta from the right ventricle and reattaching it to the left ventricle. They also move the coronary arteries to the new aorta and close the VSD. This surgery is called the arterial switch.
In other cases, the left or right ventricle will be too small or malformed to ever function correctly. Surgeons will reconfigure the heart and circulatory system so that the heart functions with one ventricle (pumping chamber), instead of two. This is called Fontan circulation and requires three open heart surgeries, called staged reconstruction. The procedures include the Norwood procedure, hemi-Fontan or Glenn operation, and Fontan procedure.
Because of enormous strides in medicine and technology, today most children born with double outlet right ventricle go on to lead productive lives as adults. Some patients won’t experience additional heart problems after surgery. However, in some cases, problems with the heart valves, heart rhythm or the baffle may occur, and additional surgeries or catheterization procedures may be required.
Between the Norwood and Glenn operations
Though early outcomes for patients with single ventricle heart defects after staged reconstruction have improved dramatically, the period between the Norwood procedure and the Glenn operation remains a very vulnerable time for infants. CHOP created the Infant Single Ventricle Monitoring Program to focus on the care and monitoring of infants with single ventricle heart defects between the first and second reconstructive surgeries.
Through age 18
Patients with Fontan circulation are referred to as single ventricle patients. As these patients get older, doctors are recognizing that, while some do fine, many experience complications, including lung, liver and gastrointestinal diseases. CHOP created the Single Ventricle Survivorship Clinic, part of the Fontan Rehabilitation, Wellness, Activity and Resilience Development (FORWARD) Program, which specializes in the care of these patients, and is researching improved treatments for them.
In addition, as a group, children with complex congenital heart defects who have had open heart surgery as infants are at a higher risk for neurodevelopmental issues when compared to children without congenital heart defects. The Cardiac Center at CHOP created the Cardiac Kids Developmental Follow-up Program to provide evaluation, screening and clinical care for children with complex congenital heart disease who are at risk for neurodevelopmental problems.
Children who were born with double outlet right ventricle must continue to see a pediatric cardiologist regularly. Children who were critically ill as newborns may also have a longer road to recovery.
It's important that adults who were born with double outlet right ventricle continue to see a cardiologist. We will help patients transition care to an adult congenital heart disease specialist.
The Philadelphia Adult Congenital Heart Center, a joint program of Children’s Hospital of Philadelphia and Penn Medicine, meets the unique needs of adults who were born with heart defects.