Pediatric Double Outlet Right Ventricle and a Taussig Bing Heart

Normally, there are two arteries that arise out of the heart. Arteries are blood vessels that bring blood away from the heart. These two arteries are called aorta, which brings blood to the body, and the pulmonary artery, which brings blood to the lungs. The aorta usually arises from the left sided ventricle, or pumping chamber. The pulmonary artery usually arises from the right ventricle. Typically, there are no holes between the left and right side of the heart.

In double outlet right ventricle (DORV), the aorta and the pulmonary artery arise off of the right ventricle. The blood gets out of the left side of the heart through a hole between the ventricles, known as a ventricular septal defect (VSD). The symptoms that occur depend on the size and location of where the VSD occurs on the wall between the ventricles. DORV has many possible presentations and varies in its urgency for management. Your child may have critical heart disease that needs an intervention within several hours of being born to not needing any intervention for many months after birth.

A Taussig Bing heart is a type of DORV. In this type of the DORV, the VSD is closer to the valve of the pulmonary artery than the aorta. Because of this fact, the care, associated diagnoses, and surgical treatment is different than other types of DORV. Children with Taussig Bing hearts need comprehensive, personalized care at a high level of care center like Children’s Memorial Hermann Hospital.

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What Other Diagnoses Are Associated With DORV?

• Small aortic arch
• Stenosis of the right ventricle’s pathway out of the heart
• A hole between the right and left collecting chamber, or atrial septal defect
• Genetic abnormalities

Children with DORV, in particular need an individual approach to their care. There are multiple very different variations. As stated previously, symptoms may develop very early within a few hours of life or a patient may never develop symptoms. All children with DORV will eventually need heart surgery. The heart surgery may involve just closing the hole between the two ventricles or may need a much more complex repair including closing the holes inside the heart, repairing a small aortic arch, and switching the full pulmonary artery with the aorta.

The Children’s Heart Institute at Children’s Memorial Hermann Hospital has extensive experience treating infants, children, and adults from across the world who have DORV. Treatment is individualized to the patient ’s specific anatomy, age, size and other associated cardiac conditions.

Our Innovative Approach to DORV

Innovations at Children’s Memorial Hermann Hospital in the treatment DORV include:

• Earlier repair before the onset of symptoms
• All-natural tissue repairs
• Valve-sparing approaches to improve long-term outcomes
• Advanced perfusion strategies
• “Hybrid” collaborations between interventional cardiology and cardiac surgery

At Children’s Heart Institute at Children’s Memorial Hermann Hospital, patients with congenital or acquired heart disorders receive hands-on specialized care 24/7 from a team of affiliated physicians and specialty-trained nurses who aim to deliver the best possible outcomes.

Children’s Memorial Hermann Hospital was named one of the top children's hospitals nationally in Cardiology & Heart Surgery by U.S. News & World Report. In addition, Children’s Heart Institute is among the top congenital heart surgery programs in North America for patient care and outcomes, according to the Fall 2019 Society of Thoracic Surgeons (STS) Congenital Heart Surgery Database Report of 118 STS participating programs.

In collaboration with various subspecialties, the affiliated team provides comprehensive care for newborns, children and adolescents, with the ability to transition into adult congenital cardiac care. Team members have the experience and skills necessary to offer innovative treatment methods and specialized services, including, but not limited to:

• Biventricular repairs and biventricular conversions
• Congenital heart optimization
• Full repairs for complex congenital heart defects in newborns
• Hybrid catheterization and surgical procedures
• Minimally invasive transcatheter pulmonary valve (TPV) therapy
• Minimally invasive repairs
• Treatment for adult congenital heart disease
• Valve repairs and preservation

With the Level IV Neonatal Intensive Care Unit (NICU) and a dedicated Children’s Heart Institute Intensive Care Unit at Children’s Memorial Hermann Hospital, critical heart patients have access to quality, specialized care. By utilizing state-of-the-art techniques, the team at Children’s Heart Institute strives to offer patients with the most complex problems the greatest opportunity for a normal life.

If you have any questions, use the online tool below to help us connect with you. To refer a patient or schedule an appointment, please contact our clinic using the information below.

• Pediatric Cardiology Clinic
  The University of Texas Health Science Center Professional Building
  6410 Fannin, Suite 370
  Houston, TX 77030
  Phone: (713) 486-6755 (Appointment Line)
   
• Pediatric and Congenital Heart Surgery Clinic
  The University of Texas Health Science Center Professional Building
  6410 Fannin, Suite 370
  Houston, TX 77030
  Phone: (713) 500-5746
  CMHH-Heart@memorialhermann.org

The Children’s Heart Institute is a collaboration between the affiliated physicians at McGovern Medical School at UTHealth Houston and Children’s Memorial Hermann Hospital. Typically, patients are seen on an outpatient basis at a UT Physicians clinic with all inpatient procedures performed at Children’s Memorial Hermann Hospital.