Double Outlet Right Ventricle (DORV)
Double Outlet Right Ventricle (DORV) is a congenital heart defect characterized by both the aorta and pulmonary artery arising predominantly from the right ventricle. This condition is often associated with a ventricular septal defect (VSD), which is a hole between the ventricles, and can lead to various clinical manifestations depending on the specific anatomical features present.
Pathophysiology of DORV
DORV presents a spectrum of anatomical variations, primarily influenced by the size and location of the VSD, as well as the presence of any pulmonary stenosis. In this condition, systemic and pulmonary blood flow can mix, resulting in varying degrees of oxygen saturation in the blood. The severity of symptoms often correlates with the degree of obstruction to pulmonary blood flow and the specific arrangement of the great vessels.
- Types of DORV: The great arteries can have different spatial relationships:
- Concordant: Both great arteries arise from the right ventricle.
- Transposition-like: The aorta may be positioned anteriorly to the pulmonary artery.
- Taussig-Bing anomaly: A specific arrangement where oxygenated and deoxygenated blood flows through both great arteries.
Symptoms of DORV
Clinical manifestations of DORV can range from mild to severe, including:
- Cyanosis: A bluish tint to the skin due to low oxygen levels.
- Heart Failure: Symptoms such as fatigue, difficulty breathing, and poor growth in infants.
- Murmurs: Heart murmurs may be detected during physical examinations due to abnormal blood flow.
Diagnosis
Diagnosing DORV typically involves several imaging techniques:
- Echocardiography: This is the primary tool used to visualize heart structure and function.
- Electrocardiogram (ECG): Can indicate right ventricular hypertrophy.
- Chest X-ray: May show cardiomegaly or increased pulmonary vascularity.
- Cardiac Catheterization: Provides detailed information about heart pressures and oxygen levels.
Surgical Management
Surgical intervention is often necessary for patients with DORV. The choice of surgical approach is influenced by:
- The position of the VSD.
- The relationship between arterial outlets.
The goal is to establish unobstructed blood flow from the left ventricle to the aorta while ensuring that the right ventricle communicates freely with the pulmonary trunk. Common surgical procedures include:
- Ventricular Septal Defect Repair: Closing the VSD to prevent mixing of oxygenated and deoxygenated blood.
- Aortic Reconstruction: Connecting the left ventricle directly to the aorta.
Double Outlet Right Ventricle is a complex congenital heart defect that requires careful diagnosis and management. Early intervention through surgical repair can significantly improve outcomes for affected individuals. At Sheikh Shakhbout Medical City (SSMC), advanced diagnostic tools and surgical techniques are employed to provide comprehensive care for patients with DORV, ensuring optimal treatment tailored to each patient's unique anatomical considerations.
