Double Inlet Left Ventricle
What You Need to Know
- DILV is rare and occurs in 5 out of every 100,000 births.
- Babies and children with DILV may have additional heart defects, including coarctation of the aorta, pulmonary atresia or pulmonary valve stenosis.
- Treatment involves a series of open-heart surgeries after birth over the first few years of life
What is double inlet left ventricle?
Double inlet left ventricle is a type of single ventricle congenital heart defect, meaning only one of the heart’s two pumping chambers (called a ventricle) is working.
A normal functioning heart has two working ventricles — the right ventricle, which pumps oxygen-poor blood to the lungs, and the left ventricle, which pumps oxygen-rich blood to the body. The heart also has the mitral valve, a pathway that keeps blood flowing into the left ventricle, and the tricuspid valve, a pathway that keeps blood flowing into the right ventricle.
When DILV occurs, both valves lead into the left ventricle. This causes oxygen-rich blood and oxygen-poor blood to mix in the left ventricle and pump into the lungs and body. The right ventricle, which would normally pump blood to the lungs, is small and underdeveloped and does not function as needed.
DILV is rare, occurring in 5 out of every 100,000 births. Babies with DILV may have additional heart defects, such as coarctation of the aorta, pulmonary atresia or pulmonary valve stenosis.
What causes double inlet left ventricle?
The cause of double inlet left ventricle is unknown. There may be a genetic component, but potential genes involved are unknown.
Double Inlet Left Ventricle Symptoms
Symptoms of DILV usually appear within the first days to weeks of the baby’s life and can include:
- Heart murmur
- Cyanosis, a blue discoloration of the skin and lips due to low oxygen
- Difficulty feeding
- Fatigue
- Failure to gain weight
- Difficulty breathing
- Pale or dusky color to the skin
- Sweating
- Swollen legs or abdomen
- Heart failure
Double Inlet Left Ventricle Diagnosis
Before birth
In most cases, DILV is diagnosed before the baby is born. This is performed by:
- Fetal Ultrasound: A test that creates an image of the baby in the mother's womb (uterus) and evaluates the baby’s heart, head, spine and other parts of the body.
- Fetal Echocardiogram: A test that uses sound waves to check the heart of the developing baby. This test can confirm the diagnosis of DILV.
After birth
- Postnatal Echocardiogram: An ultrasound done after birth that demonstrates the anatomy and flow in the heart and blood vessels.
- Electrocardiogram (EKG): A recording of the electrical conduction in the heart.
- CT scan and MRI: 2D and 3D imaging of the heart anatomy, function and blood flow.
- Chest X-ray: Can be used to examine heart shape and size.
- Cardiac catheterization: A small, flexible hollow tube (catheter) is inserted into a blood vessel in the groin, arm or wrist to do several tests that can provide information about the structure and function of the heart.
Double Inlet Left Ventricle Treatment
Treatment for DILV involves a series of open-heart surgeries after birth over the first few years of life to improve blood flow from the heart to the body and lungs. Some patients may also need medication before and after surgeries that help to keep enough blood flowing to the lungs or the body.
Surgery for double inlet left ventricle
The type and number of procedures needed to treat DILV will depend on the specifics of the child’s condition and additional defects that may be present.
Neonatal procedures: Babies with DILV will usually need a procedure within the first few days to weeks of life to help balance the flow of blood to the lungs and the body. The surgery required will depend on the issue caused by the DILV.
- Blalock-Taussig-Thomas (BTT) shunt. If the baby’s lungs are not receiving enough blood but the rest of the body is receiving proper blood, a surgery can be performed by placing a small tube (shunt) from an artery attached to the aorta to the pulmonary artery. This shunt provides a new blood flow path to the lungs.
- Pulmonary artery band. If there is too much blood flow to the lungs, the surgeon may perform another type of surgery called a pulmonary artery band. This procedure restricts the amount of blood flow to the lungs by placing a band around the main pulmonary artery and tightening until blood flow is reduced to desired levels.
Glenn procedure: The Glenn procedure is typically performed when the child is around 4-6 months old. It reroutes blood from the upper body directly to the pulmonary arteries, so the blood can receive oxygen without going through the single ventricle. This results in the single ventricle pumping blood only from the lower half of the body, reducing strain on the heart.
Fontan procedure: The Fontan procedure is the final procedure and is typically performed when the child is 3-5 years old. It redirects the flow of oxygen-poor blood from the lower body directly into the lungs, without passing through the heart. The procedure does not create normal circulation in the child’s body, but does improve blood flow and adjusts the oxygen levels so they are similar to that of a child without the defect.
The child may also need additional surgeries or procedures to treat other defects.
Some children may develop heart failure despite the surgeries and require a heart transplant.
Surgery Recovery and Follow-up Care
After surgery, children may need:
- Aspirin or anticoagulation medication to prevent blood clots
- Diuretic medication to help reduce fluid buildup
- Other heart medications, depending on their condition
Children with DILV are considered to be at higher risk for serious health issues in between these surgeries and will be regularly monitored by the child’s care team to make sure no complications arrive and to determine when the next surgery should take place.
After all surgeries are complete, children with DILV require regular follow-ups. Generally, children need close follow-up care while they are young, followed by regular follow-up visits every six to 12 months, in addition to care from other specialists they may need.
Outlook and Prognosis
Many children treated for DILV will live into adulthood but will need lifelong cardiac care. The child’s follow-up care plan will depend on their condition.
Children may face additional complications from DILV, such as:
Clubbing of the fingers and toes
Frequent pneumonia
Heart rhythm issues
Heart failure
Death. DILV can be fatal if not properly treated.
Physical Activity and Exercise After Repaired DILV
Safe levels of physical activity for those with repaired DILV should be determined by consulting with a cardiologist.
Pregnancy After Repaired DILV
Pregnancy is high-risk for patients with repaired DILV. A cardiologist should be consulted to review the risks.
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