Double-Outlet Right Ventricle

Double-outlet right ventricle (DORV) is a birth defect where the main artery (aorta) and the artery to the lungs (pulmonary artery) aren't connected to the usual parts of the heart. Instead, they connect, either partially or fully, to the right lower heart chamber (right ventricle). This is different from a normal heart where the aorta connects to the left lower chamber (left ventricle) and the pulmonary artery connects to the right lower chamber.

This means the blood vessels are not in their usual positions. In a normal heart, the pulmonary artery carries oxygen-poor blood to the lungs, and the aorta carries oxygen-rich blood to the rest of the body. In DORV, this flow can be disrupted.

Another important part of DORV is a hole between the two lower heart chambers (ventricles). This hole is called a ventricular septal defect (VSD). The VSD allows oxygen-rich blood and oxygen-poor blood to mix, which can lead to less oxygen in the blood. Babies with DORV may have bluish or grayish skin, a sign that their bodies aren't getting enough oxygen.

DORV is often present at birth, meaning it's a congenital heart defect. Sometimes, it occurs along with other heart problems, such as other holes in the heart, issues with the heart valves, or problems with the blood vessels. These extra problems can affect how the condition is treated.

Double-outlet right ventricle (DORV) is a heart condition present at birth. In DORV, the blood flow to the lungs is sometimes too low. This can make it hard for the heart to pump blood efficiently, potentially leading to heart failure and slowed growth in babies.

Diagnosing DORV often starts with an echocardiogram. This test uses sound waves to create pictures of the heart, showing how blood moves through the heart and its valves. If more information is needed, other tests like a heart CT scan (using X-rays to create cross-sectional images of the heart) or a heart MRI scan (using magnetic fields and radio waves for detailed images) might be done.

Treatment for DORV often involves surgery. Some babies need surgery soon after birth, while others might wait a few months. The type of surgery depends on the specific problems with the heart's structure.

During surgery, doctors might:

• Create a pathway (a tunnel): Connecting the lower left heart chamber (left ventricle) to the main artery (aorta) to improve blood flow.
• Correct the arteries: If the main arteries (aorta and pulmonary artery) are switched, they may be repositioned.
• Patch a hole: Fixing a hole between the lower heart chambers.
• Add a new blood vessel: Inserting a blood vessel to connect the right ventricle to the pulmonary artery to improve blood flow, especially if the pulmonary artery is too narrow.
• Widen the pulmonary artery: Expanding the pulmonary artery to allow better blood flow to the lungs.
• Address other birth defects: Repairing any other heart problems the baby might have.

In some cases, a temporary tube called a shunt is used to help blood flow to the lungs until more extensive surgery can be done. This shunt is usually placed between the aorta and the pulmonary artery and removed later during the corrective heart surgery.

Even after surgery, lifelong checkups are important. Adults with DORV should see a doctor specializing in congenital heart conditions (an adult congenital cardiologist) for regular care. Later in life, further surgery might be needed to fix a leaking or narrowed heart valve. In some cases, medicine might be needed to help the heart chambers function better.

Congenital Heart Defects in Children: Understanding the Diagnosis and Management

Congenital heart defects (CHDs) are heart problems that some babies are born with. A pediatric cardiologist, like Dr. Jonathan Johnson, can help diagnose and manage these conditions. Not all CHDs are the same. Some, like small holes or slightly narrowed heart valves, might only need regular checkups, perhaps every few years, with tests like an echocardiogram. More serious CHDs may require surgery, either through a traditional open-heart procedure or a less invasive method using specialized tools in a cardiac catheterization lab. In very severe cases, a heart transplant might be necessary.

Recognizing the Signs

The symptoms of CHD vary greatly depending on the child's age. Infants often show signs during feeding, such as shortness of breath, trouble breathing, or sweating. Younger children might experience nausea, vomiting, or discomfort with activity. Older children and teenagers may report chest pain, fainting, or rapid heartbeat, especially during exercise. These symptoms, particularly in adolescents, are important red flags for a cardiologist and require immediate attention.

Planning for the Future

After a CHD diagnosis, it's normal to feel overwhelmed. It's crucial to ask your doctor detailed questions during follow-up appointments. Think about the next few years: what procedures or surgeries might be needed? What testing and checkups will be necessary? How will this affect your child's daily activities, sports, and overall well-being? Most importantly, discuss how to help your child live as normal a life as possible.

Surgical Options and Timing

Different types of CHDs may require open-heart surgery or cardiac catheterization. With open-heart surgery, asking about the optimal timing is vital. Some procedures are best performed at specific ages to maximize the child's long-term health. Discuss with your doctor the best time for surgery based on your child's specific condition.

Sports and Activities

Many parents and children ask about participating in sports and activities. Most CHDs allow for participation, but some may limit certain activities. For example, children with particular genetic conditions may need to avoid activities that put excessive strain on their arteries. Your cardiologist will help you understand what activities are safe for your child based on their specific condition.

Heritability and Pregnancy

As children with CHDs grow older, it's important to understand the potential for the condition to be inherited. If a parent has a CHD, there is a small chance their child could inherit a similar or different type of CHD. If a parent with a CHD becomes pregnant, careful monitoring, including additional fetal echocardiograms, is important during the pregnancy. Fortunately, most children with CHDs can have children of their own.

Open Communication

Maintaining open communication between the family, the child, and the cardiologist is essential. CHDs often require long-term care, and your cardiologist will be a partner in your child's health journey. Don't hesitate to ask questions if something isn't clear. Regular communication and open dialogue are key to effectively managing CHDs.

Diagnosing Congenital Heart Defects

Congenital heart defects can be diagnosed during pregnancy or after birth. A routine fetal ultrasound during pregnancy can sometimes reveal signs of certain heart defects.

After birth, a healthcare professional may suspect a CHD if a baby shows signs like:

• Growth delays: The baby may not gain weight or grow as expected.
• Color changes: The baby's lips, tongue, or fingernails might have an unusual color, like bluish tint (cyanosis).

A heart murmur, a sound heard during a physical exam, might also indicate a CHD. Most murmurs are harmless, but some can signal a problem with blood flow through the heart.

Diagnostic Tests

Several tests can help diagnose a CHD:

• Pulse oximetry: Measures the amount of oxygen in the blood.
• Electrocardiogram (ECG/EKG): Records the heart's electrical activity.
• Echocardiogram: Uses sound waves to create images of the heart, showing blood flow and valve function. A fetal echocardiogram is done during pregnancy.
• Chest X-ray: Shows the size and condition of the heart and lungs.
• Cardiac catheterization: A thin tube is inserted into a blood vessel to get detailed information about blood flow to and from the heart. Some procedures can be done during this test.
• Heart MRI: Creates detailed 3D images of the heart, particularly useful in diagnosing and evaluating CHDs in adolescents and adults.

These tests help doctors understand the specific type of CHD and the best course of treatment.

Treating Children with Congenital Heart Defects

Children born with heart problems, called congenital heart defects, need different kinds of care depending on the problem and how serious it is. Some defects don't cause long-term health issues and may not need any treatment at all. Other defects, like a small hole in the heart, might close up naturally as the child grows older.

However, severe congenital heart defects require treatment soon after diagnosis. This treatment can involve various approaches:

• Medications: These are often used to manage symptoms or complications. They might be used alone or with other treatments. Some common types of medicine include:

  • Water pills (diuretics): These help the body get rid of extra fluid, reducing the strain on the heart.
  • Heart rhythm medications (anti-arrhythmics): These help regulate irregular heartbeats.

• Heart Procedures: These are often necessary for more severe defects.

  • Cardiac Catheterization: This is a procedure where thin, flexible tubes (catheters) are inserted into the heart through a blood vessel, usually in the leg. Doctors use tiny tools threaded through the catheters to fix problems like holes or narrow areas in the heart. Sometimes this can be done in stages over time. This method avoids the need for open-heart surgery in many cases.
  • Heart Surgery: In some cases, open-heart surgery (or less invasive procedures) is needed to repair the heart defect. The type of surgery depends on the specific problem.
  • Heart Transplant: If the defect is too serious to be repaired, a heart transplant might be necessary.
  • Fetal Cardiac Intervention: This is a very specialized procedure performed before a baby is born, to fix a serious heart problem or prevent complications during pregnancy. It's used only in specific, severe cases.

Many children with congenital heart defects need multiple procedures and surgeries throughout their lives. This is why ongoing medical care is essential. Regular check-ups with a cardiologist (a doctor specializing in heart conditions) are crucial. These check-ups might include blood tests and imaging scans to monitor the child's heart health and look for any complications.

Important Note: The type and timing of treatment will vary greatly depending on the specific heart defect and its severity. It's crucial to discuss treatment options with a medical professional.

Expert Insight (Dr. Dearani): Minimally invasive heart surgery techniques, initially developed for adults, are now being used more often in children and teenagers. This is a testament to advancements in medical technology and procedures. Specialized equipment and training are crucial for these procedures and are often not readily available in all children's hospitals.

Living with a congenital heart defect can sometimes require lifestyle adjustments to help keep the heart healthy and prevent future problems.

Staying Active Safely: Children with congenital heart defects may need to adjust their activity levels. Some might need to limit sports or strenuous exercise. However, many children with these conditions can still participate in regular activities. It's crucial to talk to your child's doctor or cardiologist about what kinds of sports and exercise are suitable. They can assess your child's specific condition and recommend appropriate activity levels.

Protecting Against Infections: Certain congenital heart defects can make your child more vulnerable to infections in the heart's lining or valves, a condition called infective endocarditis. To prevent this, your doctor might recommend taking antibiotics before certain medical procedures, like dental work. This is especially important for children with artificial (mechanical) heart valves. Always ask your child's cardiologist if preventive antibiotics are needed.

Finding Support: Sharing experiences with others facing similar challenges can be incredibly helpful. Connecting with support groups can offer comfort, encouragement, and valuable insights. Your child's healthcare team can provide information about support groups in your area.

Managing Stress and Anxiety: Living with a congenital heart defect can sometimes lead to stress and anxiety, both for the child and the family. Talking to a counselor or therapist can provide tools and strategies to manage these feelings effectively. Your healthcare provider can recommend counselors or therapists in your area.