The symptoms of DORV vary depending on the severity of the defect and associated complications. In many cases, symptoms become apparent soon after birth, as the baby may show signs of poor oxygenation or heart strain.

Common Symptoms of DORV:

• Cyanosis: A bluish tint to the skin, lips, or nails due to low oxygen levels in the blood. This is a key sign of the defect and is often the first symptom noticed.

• Shortness of breath: Difficulty breathing, especially during feeding or crying, due to inadequate oxygen supply.

• Fatigue: Unusual tiredness or weakness, often due to reduced blood flow and oxygenation.

• Poor feeding: Difficulty feeding, poor weight gain, and slow growth are common in babies with DORV, as the heart struggles to pump blood effectively.

• Heart murmur: A characteristic heart sound caused by turbulent blood flow through the abnormal heart valves or openings.

• Increased heart rate: The heart works harder to compensate for insufficient blood flow, leading to a faster than normal heart rate.

• Swelling: In severe cases, fluid may build up in the body (edema), causing swelling in the legs, abdomen, or feet.

As the child grows, more severe complications can arise if the condition is left untreated, including heart failure, arrhythmias, and developmental delays. Timely surgical intervention is crucial to improve the quality of life and overall health of the child.

Diagnosing DORV requires a combination of imaging tests and physical examinations. Since DORV is a congenital condition, it is often detected shortly after birth or during routine pediatric exams.

Diagnostic Tools for DORV:

• Physical Examination: A pediatrician may first notice signs such as cyanosis, heart murmurs, and rapid breathing during a physical exam.

• Echocardiogram (ECHO): This is the primary diagnostic tool used to visualize the heart’s structure and function. An echocardiogram can help identify the abnormal connections between the right ventricle and the great arteries, as well as assess any other heart defects.

• Chest X-ray: This test provides an image of the heart and lungs and can help identify issues such as an enlarged heart or fluid buildup in the lungs, both of which may result from DORV.

• Cardiac MRI: A magnetic resonance imaging scan provides a detailed image of the heart’s anatomy and can be useful in planning surgery or assessing complex cases of DORV.

• Electrocardiogram (ECG): This test measures the electrical activity of the heart and can detect any arrhythmias that might be caused by DORV.

In some cases, a cardiac catheterization may be performed to evaluate the heart’s blood flow and pressures, especially when surgical options are being considered.

The primary treatment for DORV is surgery, and it is usually performed in infancy or early childhood to ensure the best outcomes. The surgery aims to correct the abnormal connections between the ventricles and the great arteries, restoring proper blood flow to the lungs and the rest of the body.

Surgical Treatment Options:

• Anatomical Correction: The most common surgical approach for DORV is an anatomical correction, which involves repositioning the aorta to arise from the left ventricle and creating a new connection for the pulmonary artery. This procedure restores the normal flow of oxygenated and deoxygenated blood.

• Rastelli Procedure: In cases of DORV with a subpulmonary VSD (ventricular septal defect), a technique known as the Rastelli procedure may be used. It involves closing the VSD and rerouting the blood flow using a conduit from the right ventricle to the pulmonary artery.

• Fontan Procedure: For children with more complex forms of DORV, such as those with additional heart defects or uncorrectable defects, the Fontan procedure may be used. This procedure creates a connection between the right atrium and the pulmonary arteries, allowing the heart to pump blood more effectively without the use of the right ventricle.

In some cases, a heart transplant may be considered if the child’s heart is severely damaged and cannot be repaired through surgery. However, this is rare, and most children with DORV can benefit from surgical correction.

Since DORV is a congenital condition, it cannot be prevented. However, there are steps that can be taken during pregnancy to reduce the risk of congenital heart defects:

Prevention and Management Tips:

• Prenatal care: Regular prenatal visits and screening can help detect potential heart defects in the fetus. Fetal echocardiography can be performed to assess the structure of the heart early in pregnancy, especially in high-risk pregnancies.

• Genetic counseling: Families with a history of congenital heart defects may benefit from genetic counseling to understand the risks of passing on heart conditions.

• Healthy lifestyle: Maintaining a healthy lifestyle before and during pregnancy, including proper nutrition, avoiding smoking, and controlling diabetes, can help reduce the risk of congenital defects.

• Timely intervention: Early detection and surgical intervention are critical for improving outcomes. Surgery is typically performed in infancy, though some children may require follow-up procedures or additional surgeries as they grow.

While DORV repair surgery is generally successful, there are some risks and potential complications associated with the procedure, including:

• Surgical risks: As with any surgery, there is a risk of bleeding, infection, or damage to surrounding structures during the procedure.

• Arrhythmias: The repair may disrupt the heart’s electrical system, leading to abnormal heart rhythms (arrhythmias), which may require further treatment.

• Conduit failure: If a conduit is used to redirect blood flow (as in the Rastelli procedure), it may narrow or fail over time, requiring additional surgery or interventions.

• Heart failure: In some cases, the repaired heart may not function as well as expected, leading to heart failure.

• Delayed development: Children with DORV may experience developmental delays due to the early impact of the condition on oxygen delivery to vital organs.

Despite these potential risks, most children with DORV who undergo surgery experience a significant improvement in their quality of life and survival rates.

With successful surgical repair, many individuals with DORV can lead active, fulfilling lives. Adherence to medical advice, regular follow-ups, and a supportive care environment contribute significantly to long-term well-being. Advancements in surgical techniques and postoperative care have markedly improved the prognosis for those affected by this congenital heart defect.​