Double Outlet Right Ventricle (DORV)

What is Double Outlet Right Ventricle (DORV)?

Double Outlet Right Ventricle (DORV) is a congenital heart defect present at birth. In this condition, both the aorta—the body’s main artery—and the pulmonary artery—the artery responsible for carrying blood to the lungs—are incorrectly connected to the right ventricle, the heart’s lower chamber responsible for pumping oxygen-poor blood to the lungs.

In a healthy heart, the aorta connects to the left ventricle, while the pulmonary artery connects to the right ventricle. In babies with DORV, the aorta and pulmonary artery connect partially or completely to the right ventricle. This condition often coexists with a ventricular septal defect (VSD), which is a hole between the two lower chambers of the heart. This hole allows oxygen-rich and oxygen-poor blood to mix, often resulting in insufficient oxygen levels in the bloodstream. As a result, affected infants may exhibit cyanosis, characterized by a bluish or grayish tint to the skin.

Associated Conditions

DORV may occur alongside other congenital heart problems, including additional septal defects, heart valve abnormalities, or issues with blood vessels. The complexity of these associated conditions can vary widely among affected individuals, influencing treatment options and long-term outcomes.

Symptoms and Complications

Symptoms of DORV can manifest shortly after birth and may include:

  • Cyanosis: Bluish or gray skin, lips, or nails due to low oxygen levels.
  • Rapid Breathing: Difficulty in breathing or rapid respiratory rates.
  • Poor Weight Gain: Infants may struggle to gain weight adequately.
  • Fatigue: Unusual tiredness or lethargy, particularly during feeding or activity.

If left untreated, excess blood flow through the pulmonary artery can lead to heart failure, inadequate growth, and other complications. These issues may necessitate immediate medical intervention.

Diagnosis

The diagnosis of DORV typically begins with a thorough clinical evaluation and may include several diagnostic tests:

  1. Echocardiogram: This ultrasound test uses sound waves to create images of the heart’s structure and blood flow, confirming the presence of DORV.

  2. Cardiac CT Scan: This imaging technique provides detailed cross-sectional images of the heart, helping to assess its anatomy.

  3. Cardiac MRI Scan: Using magnetic fields and radio waves, this test generates detailed images of the heart’s structure and function.

  4. Cardiac Catheterization: This invasive procedure involves inserting a catheter into a blood vessel, usually in the groin or wrist, and guiding it to the heart. It allows for pressure measurements and the injection of dye to visualize heart structures.

Treatment Options

Treatment for DORV often requires surgical intervention, with the timing and type of surgery depending on the individual’s specific anatomy and associated conditions:

  • Early Surgery: Some infants may require surgical repair within the first few days of life, while others might undergo surgery several months later.

  • Types of Surgical Procedures:

    • Creation of a Tunnel: Connecting the left ventricle to the aorta via a tunnel through the ventricular septal defect.
    • Correcting Vessel Positions: Realigning the aorta and pulmonary artery if they are reversed.
    • Patching the Ventricular Septal Defect: Closing the hole between the ventricles to prevent blood mixing.
    • Vessel Insertion: Installing a conduit to improve blood flow from the right ventricle to the pulmonary artery if it is underdeveloped.
    • Widening the Pulmonary Artery: This procedure enhances blood flow if the artery is narrowed.

In some cases, a temporary shunt may be placed to facilitate increased blood flow to the lungs, with plans for removal during later surgery.

Long-term Care

Individuals born with DORV require lifelong follow-up care. Regular check-ups with healthcare providers specializing in congenital heart conditions are essential to monitor heart function and detect any complications. Adults with a history of DORV may need further interventions for issues like narrowed or leaking heart valves, and some may require supportive therapies to assist heart function.

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