Double Outlet Right Ventricle

A cardiac abnormality known as double-outlet right ventricle is present from birth. It is therefore a congenital cardiac defect. The lung artery and the body's major artery aren't connected to the heart's typical locations with this illness. The aorta is the name of the body's main artery. The term "pulmonary artery" refers to the lung artery. Occasionally the normal directions of these blood arteries are reversed.

The aorta joins the left bottom heart chamber in a normal heart. The pulmonary artery connects to the right lower heart chamber.

Both the aorta and the pulmonary artery are partially or entirely connected to the right lower heart chamber in infants with a double-outlet right ventricle.

A hole exists between the lower heart chambers in infants who have a double-outlet right ventricle. The ventricles are the name for the lower heart chambers. A ventricular septal defect is a medical term for the hole. Because of the perforation, blood with and without oxygen mingles. It's possible that babies with this problem don't obtain enough oxygen in their blood. They could have grey or blue skin.

Double-outlet right ventricles can develop in conjunction with other congenital cardiac conditions. Other holes in the heart, issues with the heart valves, and blood vessel issues are a few examples of these issues.

A ventricular septal defect (VSD) is also present in all DORV infants. VSD refers to a gap in the septum, which is the wall separating the two ventricles. In the gap and in between the ventricles, blood flows. Pulmonary valve stenosis is the additional related abnormalities that may exist.

DORV is categorized by medical professionals according to where the hole is −

  • DORV and subaortic VSD  The VSD is located just below the aorta.

  • Taussig-Bing DORV with subpulmonary VSD  The VSD is located directly below the pulmonary artery.

  • DORV with a doubly committed VSD  Each of the major arteries has a VSD.

  • DORV with a non-committed (or distant) VSD  Neither the aorta nor the pulmonary artery is in the vicinity.

Double Outlet Right Ventricle: Causes

Scientists don't fully comprehend the origins of DORV. Babies that have issues with their chromosomes, the cell structures that house their DNA, may experience this.

Double Outlet Right Ventricle: Symptoms

DORV symptoms often manifest in the first few days or weeks following delivery. They resemble those of other congenital cardiac conditions and consist of −

  • Cyanosis (blue-ish or purple-ish skin, lips, or nails) (blue-ish or purple-ish skin, lips, or nails).

  • Respiratory difficulties, such as shortness of breath or tachypnea (very rapid breathing).

  • Eating problems or weight gain.

  • Cardiac murmur (extra sound in the heartbeat, which a healthcare provider can hear with a stethoscope).

  • Excessive sweating, such as when eating.

  • Tachycardia (heart rate quicker than 100 beats a minute) (heart rate faster than 100 beats a minute).

  • Unusual drowsiness or looking "out of it."

DORV babies frequently have other congenital issues −

  • Ciliary dysfunction  The microscopic hair-like structures that border the airway have issues.

  • Heterotaxy  Organs in the chest and abdomen are not located where they typically are.

  • Intestinal malrotation  This condition causes an abnormal twisting of the intestines in a neonate.

  • The pulmonary valve, which sits between the right ventricle and the pulmonary artery, is excessively tiny or stiff in those with pulmonary stenosis.

  • Hypoplastic ventricles  One or both ventricles are smaller than usual.

Double Outlet Right Ventricle: Risk Factors

DORV often occurs without a known cause. DORV is more likely to affect infants who have specific genetic disorders, such as trisomy 13, trisomy 18, or DiGeorge syndrome (22q11. 2 deletions).

Double Outlet Right Ventricle: Diagnosis

The diagnosis of the double outlet right ventricle is mainly done based on history and some of the tests may be required for confirmation and to rule out underlying causes

Before a baby is born, doctors can occasionally detect a cardiac abnormality. Fetal echocardiography, a common ultrasound test, could allow them to accomplish this.

If not, DORV is typically discovered in the days or weeks following birth as a result of the baby's symptoms.

Occasionally DORV is identified by a medical professional before a baby is born. In some instances, a baby's birth marks the onset of cardiac problems. In any case, the infant needs a checkup −

  • The medical professional will watch for cyanosis symptoms.

  • The healthcare professional will listen to the heart using a stethoscope.

  • The quantity of oxygen in the blood will be determined by the provider using pulse oximetry (also known as "pulse ox" screening). The examination is easy and painless.

The healthcare professional may then request one or more tests −

  • First imaging examinations  X-rays, MRIs, and CT scans are frequently used to acquire images within the body.

  • An electrocardiogram (ECG or EKG) measures the electrical activity of the heart without causing any discomfort.

  • Echocardiogram (echo An echocardiogram measures sound waves from the chest to produce pictures of the heart without causing any discomfort.

  • Cardiac catheterization (cardiac cath Angiography is another name for cardiac catheterization. To obtain information on heart function, a tiny tube is inserted into a vein and then into the heart.

Double Outlet Right Ventricle: Treatment

The treatment is based on the severity of the symptoms. Your doctor may advise conservative or surgical treatment.

Within the first few days after birth, some infants with a double-outlet right ventricle require surgery to repair their hearts. Some could have surgery as young as a few months.

Surgery of several kinds may be performed. The individual cardiac issues determine the type of operation.

The cardiac surgeon may do any of the following procedures −

  • To link the left ventricle to the aorta, dig a tunnel through the hole in the heart.

  • If the aorta and pulmonary arteries are positioned incorrectly, correct them.

  • The gap between the lower heart chambers should be repaired.

To enhance blood flow to the lungs in some newborn infants, a temporary operation using a tube known as a shunt may be carried out. The baby's aorta and pulmonary artery are connected via a shunt, which is implanted by the heart surgeon. Later in life, a cardiac operation is performed to restore the double-outlet right ventricle, and the shunt is taken out.

A person with a double-outlet right ventricle must undergo lifelong routine medical examinations. Adults should visit a doctor who has experience diagnosing and treating congenital heart defects. A physician of this kind is referred to as an adult congenital cardiologist.

Double Outlet Right Ventricle: Prevention

DORV cannot be stopped in any manner. Its cause is still a mystery to scientists.


A broad spectrum of lesions known as double-outlet right ventricle (DORV) is those in which the aorta and pulmonary artery both partially or entirely emerge from the right ventricle. In 1% to 1.5% of patients with CHD, DORV is present.

Dr. Durgesh Kumar Sinha
Dr. Durgesh Kumar Sinha


Updated on: 28-Apr-2023


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