Double Inlet Left Ventricle

Overview

Double inlet left ventricle (DILV) is a complex congenital heart defect in which the upper chambers of the baby’s heart (the left and right atria) are both connected to the same lower chamber – the left ventricle. The baby’s right ventricle is small and underdeveloped.

The ventricles are the heart’s pumping chambers. Babies with double inlet left ventricle are left with only one developed or working pumping chamber (the left ventricle).

Treatment typically involves a series of two to three heart procedures within the first few years of life.

DILV is one of several congenital heart defects known as “single ventricle defects,” conditions in which the child only has one functioning ventricle.

How does double inlet left ventricle affect my baby?

In a normal heart, oxygen-poor blood collects in the right atrium and flows down into the right ventricle, where it is pumped to the lungs for more oxygen through the pulmonary artery. Oxygen-rich blood collects in the left atrium and flows down into the left ventricle where it is pumped out to the body through the aorta.

In babies with double inlet left ventricle, blood from both upper chambers (right and left atria) flows into the left ventricle, resulting in a mixture of oxygen-rich and oxygen-poor blood. This mixed blood then gets pumped out to the body and the lungs.

The symptoms of DILV may vary and this often depends on which of the “great arteries” – the pulmonary artery or the aorta – arises from the left ventricle.

• In cases where the pulmonary artery arises from the underdeveloped right ventricle and the aorta from the left ventricle, often there is less oxygen circulating to the lungs which may cause the baby’s skin, lips and nails to turn blue (known as cyanosis). Without adequate oxygen supplied to the body and organs, the baby may not grow at a normal rate, a condition known as “failure to thrive.”
• In cases where the great arteries are “transposed” or switched, with the aorta arising from the underdeveloped right ventricle and the pulmonary artery from the left ventricle, there may be too much blood being pumped to the lungs. This can lead to a condition called pulmonary over-circulation. Symptoms include fast breathing, sweating, feeding difficulties and poor growth. With this type of DILV, there may also be a narrowing of the aorta (known as a coarctation).

Cause

Double inlet left ventricle is a congenital heart defect, meaning it is present at birth.

The condition occurs early in pregnancy when the fetal heart doesn’t form properly. The cause is unknown.

Symptoms

Newborns with double inlet left ventricle typically show signs of the condition within the first few days or weeks after birth.

While the signs and symptoms vary for each baby, they may include:

• Difficulty breathing
• Heart murmur
• Bluish skin, lips or nails (cyanosis)
• Feeding difficulties
• Poor weight gain
• Fatigue
• Sweating
• Swelling (edema) in the legs or belly
• Heart failure

Diagnosis

Double inlet left ventricle may be detected during a routine prenatal ultrasound. Additional testing, including fetal echocardiography, is typically needed to confirm the diagnosis and learn more about the defect. A diagnosis during pregnancy enables your family and your healthcare team to plan ahead for the specialized treatment and expertise required by the baby at birth, optimizing outcomes.

In some cases, the defect may not be diagnosed until after the baby is born. The doctor may detect a heart murmur, an abnormal sound caused by blood flow problems in the heart. Additional testing to confirm the diagnosis may include an electrocardiogram (ECG or EKG), an echocardiogram, chest X-ray, cardiac catheterization, and a CT scan or MRI of the baby’s heart.

Specialized Prenatal Evaluation and Care

If double inlet left ventricle is suspected or diagnosed during pregnancy, prompt referral to a fetal cardiac center is important for further evaluation and to ensure proper delivery and treatment planning.

At Texas Children’s Fetal Center, we arrange for you to visit as quickly as possible for a comprehensive assessment by a team of specialists experienced in working together to diagnose and treat congenital heart defects. These specialists include maternal-fetal medicine (MFM) physicians (experts in high-risk pregnancies), fetal and pediatric cardiologists, fetal imaging experts, congenital heart surgeons, and neonatologists.

In addition to collecting any previous diagnostic tests, additional testing may include:

• High-resolution anatomy ultrasound to confirm the diagnosis, evaluate the condition and look for other abnormalities
• Ultra-fast MRI for a more detailed view of fetal anatomy
• Fetal echocardiogram to assess fetal heart structure and function
• Doppler ultrasound to examine blood flow patterns  
• Amniocentesis and chromosomal analysis to screen for genetic abnormalities

Our specialists will then meet with you about the results, discuss treatment recommendations, and answer any questions you have, to help your family make the most informed decisions regarding your baby’s care and treatment.

During pregnancy, mother and baby will be closely monitored for early detection of complications, including fetal non-immune hydrops (fluid accumulation in multiple areas of the baby’s body) and fetal heart failure.

Delivery

For the best possible outcome, babies with double inlet left ventricle should be delivered at a center with the highest quality congenital heart program, ensuring the expertise and resources required at birth to treat this complex condition, including an advanced neonatal intensive care unit (NICU).

Delivery and postnatal care should be carefully planned and coordinated. Our Fetal Center team works closely with Texas Children’s Heart Center, seamlessly transitioning your baby’s care at delivery to this national leader in pediatric cardiology and heart surgery. Here, the pediatric specialists treating your newborn have been an integral part of their care team since before birth.

Following delivery at Texas Children’s Pavilion for Women, newborns are transferred to Texas Children’s level IV NICU, the highest level of care available for premature and critically ill newborns, or Texas Children’s specialized cardiac intensive care unit (CICU). 

Treatment After Birth

Babies born with double inlet left ventricle typically require a series of two to three surgeries during the first few years of life. The procedures re-route and improve blood flow to the body and lungs, increasing the amount of oxygen the body receives.

The type and timing of each surgery or procedure varies based on the child’s heart condition and overall health. The first surgery may have to be performed within the first few days or weeks of the baby’s life.

Possible procedures include:

• Blalock-Taussig (BT) shunt – If the lungs are not receiving enough blood flow after birth, an infant may undergo a surgery in which a small tube (shunt) is surgically placed from an artery attached to the aorta to the pulmonary artery, creating an alternate route of blood flow 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 that restricts the amount of blood traveling to the lungs, protecting the lung vessels from being damaged by high pressure.
• Bidirectional Glenn procedure – This surgery is typically performed between 3 to 6 months of age. During the surgery, the large vein carrying blood from the upper body into the heart (known as the superior vena cava) is connected to the pulmonary artery to provide a stable source of blood flow to the lungs. For newborns who don’t require surgery immediately following birth, this is their first procedure.  
• The Fontan procedure – This additional surgery is subsequently performed between 3 to 6 years of age. During this surgery, the large vein carrying blood from the lower part of the body into the heart (known as the inferior vena cava) is connected to the pulmonary artery.

Some children with DILV do not tolerate this series of surgeries or modified circulation and ultimately require a heart transplant.

Heart medications may be required before and after surgery. Some infants may require a medication called prostaglandins (PGE) following birth in order to keep a fetal communication called a ductus arteriosus (also known as a PDA) open to preserve blood flow to the lungs, organs and tissues until they can undergo surgery.

Additional surgeries may be needed if other defects are present.

At Texas Children’s, babies with double inlet left ventricle receive comprehensive treatment through our Single Ventricle Program, a specialized program focused on the care and needs of infants with these rare congenital heart defects. The program includes critical home monitoring services and support to help families optimize their baby’s well-being between surgeries – known as the interstage period – an important and vulnerable stage in the baby’s health.

Postnatal Care Team

Your baby’s postnatal care team may include:

• Neonatologist
• Pediatric cardiologist
• Congenital heart surgeon
• Cardiovascular anesthesiologist
• Cardiac intensivist
• Nutritionist
• Failure to Thrive specialists
• Neurodevelopmental specialist

Babies born with double inlet left ventricle require lifelong care by a cardiologist.

At Texas Children’s Heart Center, our pediatric cardiologists will follow your child through adolescence, seamlessly transitioning their care at adulthood to a cardiologist who specializes in the treatment of adults with congenital heart defects. 

Why Texas Children’s Hospital?

• A single location for expert maternal, fetal and pediatric care. Texas Children’s offers mothers and babies the specialized care required for the diagnosis and treatment of congenital heart conditions all in one location, for highly coordinated care and treatment planning.
• A skilled, experienced team with proven outcomes. We have a dedicated team of maternal-fetal medicine specialists, fetal imaging experts, fetal and pediatric cardiologists, congenital heart surgeons, neonatologists and others who work in concert to care for you and your baby every step of the way, using proven protocols we’ve developed over the years. With their combined expertise and unified approach, this team offers the best possible care for pregnancies involving double inlet left ventricle.
• We care for your child’s cardiac needs at every stage of life, from fetus to adulthood. Our comprehensive approach starts with your first prenatal visit and continues through delivery, postnatal care, childhood and adulthood, thanks to one of the nation’s leading teams of fetal, pediatric and adult congenital heart specialists.

Texas Children’s – #1 in the Nation for Pediatric Cardiology and Heart Surgery

Our Fetal Cardiology Program is a collaboration between Texas Children’s Fetal Center and Texas Children’s Heart Center, ranked #1 in the nation for pediatric cardiology and heart surgery by U.S. News & World Report, with outcomes among the best in the country.

In the News

• Best hospitals for children with severe congenital heart disease
• 10 ways to get your child the best heart surgeon

Videos

• Critical congenital heart disease screening now required for all newborns in Texas
• Texas Children’s Fetal Center: Our care began before he was born

Volumes and Outcomes

• Volumes and outcomes for patients treated by the Fetal Cardiology program
• Texas Children’s Heart Center Outcomes

Additional Resources

• 6 things to consider when choosing a fetal center

Research and Clinical Trials

Texas Children’s, together with our partner institution Baylor College of Medicine, constantly strives to seek new and better treatments for babies with congenital heart defects.

For more information, please contact the Cardiovascular Clinical Research Core at 832-826-2064 or email sandrea@texaschildrens.org.

Learn more about our fetal cardiology research.