Double outlet right ventricle

Double outlet right ventricle

Overview

Double outlet right ventricle (DORV) is a complex congenital heart defect where the pulmonary artery and the aorta — the two great vessels — arise from the right ventricle. Instead of connecting to the left ventricle (the heart chamber that normally pumps blood to the body), the aorta connects to the right ventricle (the heart chamber that pumps blood to the lungs). Both the pulmonary artery (which carries oxygen-depleted blood to the lungs) and aorta (which carries oxygenated blood from the heart to the rest of the body) come from the same pumping chamber.

DORV is always accompanied by a ventricular septal defect (VSD). Other conditions that may occur along with the defect are:

  • Coarctation of the aorta
  • Mitral valve problems
  • Pulmonary atresia
  • Pulmonary valve stenosis
  • Right aortic arch
  • Transposition of the great arteries

The cause of double outlet right ventricle is not completely understood.

 

Symptoms

Patients with double outlet right ventricle can present in several ways, all related to their unique individual anatomy. Some will present like a large ventricular septal defect. Symptoms may include:

  • Feeding difficulty and poor weight gain
  • Baby tires easily, especially when feeding
  • Rapid breathing or trouble breathing
  • Rapid heartbeat
  • Enlarged heart
  • Heart murmur
  • Pale coloring
  • Sweating
  • Swollen legs or abdomen
  • Cyanosis (bluish skin), either related to right ventricular outflow obstruction (similar to tetralogy of Fallot) or to a subpulmonary defect (similar to d-transposition of the great arteries)
  • Clubbing (thickening of the nail beds) on toes and fingers (late sign)

Types

There are several types of DORV, differentiated by the location of the VSD compared to where the pulmonary artery and aorta are positioned. The problem’s symptoms and severity depend on the type of DORV. If pulmonary valve stenosis is present, it also affects the condition.

Diagnosis

A pediatrician may recommend any of the following tests:

  • Chest X-rays
  • Cardiac catheterization —  a thin, flexible tube is threaded up into the heart to measure blood pressure and inject dye for special pictures of the heart and arteries
  • Echocardiogram (ultrasound exam of the heart)
  • Magnetic resonance imaging (MRI)

Treatment

Double outlet right ventricle cannot close spontaneously. It requires surgical repair, the type and timing of which depend on:

  • The size and location of the ventricular septal defect
  • The amount, if any, of pulmonary stenosis
  • The anatomic relationship of the great vessels

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