DOUBLE-OUTLET RIGHT VENTRICLE

TYPES

There are several types of Double-Outlet Right Ventricle (DORV), which are classified based on the position of the ventricular septal defect (VSD) and the relationship of the great arteries. Here are some common types:

1. Subaortic VSD: In this type, the VSD is located just below the aortic valve, and both the aorta and the pulmonary artery arise from the right ventricle. This results in oxygen-rich and oxygen-poor blood mixing in the right ventricle.

2. Subpulmonary VSD: In this type, the VSD is located below the pulmonary valve, and both the aorta and the pulmonary artery arise from the right ventricle. Similar to subaortic VSD, oxygen-rich and oxygen-poor blood mix in the right ventricle.

3. Non-committed VSD: In this type, the VSD is located in the center of the ventricular septum, and both the aorta and the pulmonary artery arise from the right ventricle. The VSD is not specifically associated with either the aortic or pulmonary valve.

4. Doubly-committed VSD: In this type, the VSD is located between the aortic and pulmonary valves. The aorta may either override the VSD or be completely committed to the right ventricle. The pulmonary artery also arises from the right ventricle.

5. Remote VSD: In this type, the VSD is located away from the great arteries, and the aorta and the pulmonary artery arise normally from their respective ventricles. However, there is still an abnormal connection between the right ventricle and one of the great arteries.

SYMPTOMS

The symptoms associated with Double-Outlet Right Ventricle (DORV) can vary depending on the specific anatomy of the defect and the degree of mixing of oxygen-rich and oxygen-poor blood. Some common symptoms of DORV may include:

• Cyanosis: Bluish discoloration of the skin, lips, and nails due to inadequate oxygen supply to the body.
• Rapid breathing or shortness of breath: The heart’s inability to pump oxygenated blood effectively can lead to breathing difficulties.
• Poor feeding and growth: Infants with DORV may experience difficulty in feeding and may have inadequate weight gain.
• Fatigue and weakness: The reduced oxygen supply to the body can cause tiredness and weakness, especially during physical activity.
• Heart murmur: An abnormal sound heard during a physical examination, caused by turbulent blood flow through the abnormal connections in the heart.
• Clubbing of fingers and toes: Over time, the chronic lack of oxygen can cause the fingertips and toes to become rounded and enlarged.
• Recurrent respiratory infections: The mixing of blood can make individuals with DORV more prone to respiratory infections.
• Fainting or dizziness: In some cases, inadequate oxygen supply to the brain can result in episodes of fainting or dizziness.

It’s important to note that the severity and specific symptoms can vary from person to person. Some individuals with DORV may have mild symptoms, while others may experience more severe complications. Prompt medical evaluation and diagnosis are crucial to determine the appropriate treatment plan and management for individuals with DORV. If you or someone you know is experiencing any of these symptoms, it is important to consult a healthcare professional for a proper evaluation.

DIAGNOSIS

The diagnosis of Double-Outlet Right Ventricle (DORV) is typically made through a combination of medical history evaluation, physical examination, and various diagnostic tests. Here are some of the common methods used for diagnosing DORV:

1. Physical examination: A healthcare professional may listen to the heart using a stethoscope to detect any abnormal sounds, such as heart murmurs, which could indicate the presence of DORV.

2. Echocardiography: This is a non-invasive imaging test that uses sound waves to create detailed images of the heart’s structure and function. Echocardiography can help identify the abnormal connections of the great arteries and the position of the ventricular septal defect (VSD) in DORV.

3. Cardiac catheterization: This invasive procedure involves inserting a thin, flexible tube (catheter) into a blood vessel and guiding it to the heart. During cardiac catheterization, measurements of pressures and oxygen levels can be taken, and contrast dye can be injected to visualize the blood flow and identify any abnormalities in the heart’s structure.

4. Magnetic Resonance Imaging (MRI) or Computed Tomography (CT) scan: These imaging techniques can provide detailed cross-sectional images of the heart and help in visualizing the anatomy and connections of the heart in DORV.

5. Electrocardiogram (ECG or EKG): This test records the electrical activity of the heart and can help identify any abnormal heart rhythms or conduction abnormalities that may be associated with DORV.

Once a diagnosis of DORV is confirmed, additional tests may be performed to assess the severity and associated abnormalities, such as the presence of other heart defects or complications.

It’s important to consult with a healthcare professional, such as a pediatric cardiologist or a cardiac specialist, for a proper evaluation and diagnosis of DORV. They will guide you through the diagnostic process and develop an appropriate treatment plan based on the individual case.

TREATMENT

The treatment for Double-Outlet Right Ventricle (DORV) depends on several factors, including the specific anatomy of the defect, the severity of symptoms, and the presence of any associated heart abnormalities. Here are some common treatment options for DORV:

1. Medications: Medications may be prescribed to manage symptoms and improve heart function. These can include medications to reduce fluid buildup, improve heart function, or regulate heart rhythm.
2. Surgery: Most individuals with DORV will require surgical intervention to correct the defect. The specific surgical procedure will depend on the anatomy of the defect and may involve procedures such as:

• Arterial switch operation: This procedure involves switching the positions of the aorta and the pulmonary artery, along with repairing any associated heart defects.
• Rastelli procedure: This surgery is performed when there is an associated ventricular septal defect (VSD). It involves redirecting blood flow from the left ventricle to the aorta, while also patching the VSD.
• Fontan procedure: This procedure is performed in cases where the oxygen-poor blood from the lower body does not adequately reach the lungs. It involves redirecting this blood flow directly to the lungs without passing through the right ventricle.

3. Balloon atrial septostomy: In some cases, a temporary procedure called balloon atrial septostomy may be performed to improve blood flow and oxygenation while awaiting surgical repair. This procedure involves enlarging the atrial septal opening using a balloon catheter.

4. Follow-up care: Regular follow-up visits with a healthcare professional, such as a pediatric cardiologist, are essential to monitor the heart’s function, assess for any complications, and make necessary adjustments to medication or treatment plans.

The treatment plan will be tailored to each individual case, and the healthcare team will work closely with the patient and their family to determine the most appropriate course of action.

It is important to consult with a healthcare professional who specializes in congenital heart defects to discuss the specific treatment options and determine the best approach for managing DORV in each individual case.