Learning about the Double Outlet Right Ventricle

Little Olirmathi who is only one year and two months old was born with a complex congenital heart defect. Her association with Genesis Foundation (GF), which is a congenital heart foundation working towards saving these little hearts has been a remarkable journey.

Little Olirmathi battled DORV corrective surgery at 1.2 years old.

Olirmathi is the younger daughter of Gandhimathi and Selvakumar of Vellore district in Tamil Nadu. When she was two months old, she developed pneumonia along with seizures. Her parents took her to a government hospital in Chennai where she was detected with a heart murmur. Once she was admitted and stabilized, her treatment for seizures began. However, later on when an Echo was performed, the resulting diagnosis was Double Outlet Right Ventricle (DORV) with severe Pulmonary Stenosis which meant there was an overriding of the Aorta and the Pulmonary Artery. Her Aorta was originating more from the right ventricle.

After being out on medicines for a year, she was called for a review. After the echo was repeated, the diagnosis was confirmed. Olirmathi required Biventricular Repair (with RV to PA Conduit). In simpler terms it meant she needed an open-heart surgery. One of their relatives in Chennai knew a doctor in a private hospital, but the amount required for the surgery was exorbitant. They returned to Vellore, dejected.

Like every cloud has a silver lining, Genesis Foundation became Olirmathi’s beacon of hope. A paediatrician referred them to Miot Hospital in Chennai and through GF’s CSR partnership with K&S Partners, the required financial assistance for Olirmathi’s operation was successfully provided. Her parents were counselled for the surgery and Olirmathi underwent the corrective surgery with a conduit placed from the Right Ventricle to Pulmonary Artery. Olirmathi is a healthy girl and her condition is quite stable now.

What is the Double Outlet Right Ventricle Heart Defect?

Double Outlet Right Ventricle (DORV) is a rare congenital heart defect in which the pulmonary artery and the aorta, that are the heart’s two major arteries – both connect to the right ventricle. And therein lies the problem, because it is the left ventricle – the chamber which is responsible for pumping oxygen-rich blood to the body

In a normal heart, the pulmonary artery connects to the right ventricle, and the aorta connects to the left ventricle. DORV creates a problem because the right ventricle carries oxygen-poor blood, which then gets circulated in the body. The term DORV was first coined by Dr. Witham in 1957

Difference between a normal heart and a heart with the DORV defect

A Ventricular Septal Defect (VSD) or an abnormal opening (hole) is always associated with DORV. The VSD allows the oxygen-rich blood to pass from the left ventricle to the aorta and pulmonary artery. But even with this added oxygen, the body does not get enough of it, causing the heart to work harder.

Types & Symptoms of DORV

DORV can be classified into 4 types depending on the location of the VSD

• Sub-Aortic Ventricular Septal Defect
• Doubly Committed VSD
• Non-Committed VSD
• Sub Pulmonic VSD

Children can exhibit multiple symptoms of DORV.

Diagnosis of DORV

To understand a child’s condition better, the doctor might order one or more of the following investigations:

• Electrocardiogram (ECG or EKG)
• Echocardiogram (Cardiac Ultrasound)
• Chest x-ray
• Cardiac Catheterization

Treatment of DORV

The treatment of DORV is an open-heart surgery. There are different techniques.

The most common one is called intraventricular tunnel repair. For this surgery, the surgeon creates a tunnel to connect the left ventricle to the aorta. A patch placed within the heart directs left ventricle blood flow to the aorta.

Another treatment modality is total intracardiac repair which is similar to the Tetralogy of Fallot (TOF) repair. This is indicated in DORV, subaortic VSD with severe Pulmonary stenosis.
Moreover, where there is a Sub Pulmonic VSD, Arterial Switch is performed. For this procedure, a tunnel from the VSD to the pulmonary artery is created, connecting the left ventricle to the pulmonary artery.

Long-Term Outcomes for DORV

The long-term outcomes for DORV corrective surgery are good. Usually children can lead a normal life after the total repair. In a few cases, additional interventions might be required in future (5 to 10%).

Other DORV Cases Associated with the Genesis Foundation

A congenital heart foundation, everyone associated with Genesis Foundation works with equal dedication as the doctors. Along with Olirmathi, 11-year-old Devananda Sunil is also a GF star kid, whose faith in life renewed ours. Currently studying in Grade 6, she is a strong and resilient girl who aims to pursue a career in science.

Devananda Sunil who is in Grade 6 had a DORV surgery

Devananda has three sisters and is the second daughter to her parents Renu and Sunil. She is the apple of their eyes. During a routine check-up in school, when her heart murmur was discovered, Sunil was shattered. A painter by profession in Idukki, Kerala, he earned a monthly income of Rs. 5000.

After being referred to Amrita Institute in Kochi for further evaluation, she was diagnosed with a Double Outlet Right Ventricle. In Devananda’s case, there was an overriding of the Aorta and the Pulmonary Artery. The overriding was approximately 60%, which meant she needed an open-heart surgery.

Renu and Sunil’s helplessness to collect sufficient funds led them to the Genesis Foundation, upon recommendation by the Amrita Institute. With help from WPP India’s CSR Foundation, Genesis Foundation covered all the costs incurred for Devananda’s open heart surgery. Her surgery was successful and now she leads her life like any other child fighting for their dreams.

Genesis Foundation has been helping save little hearts with not just the financial aid but making a difference in every child’s life.