At the forefront with hybrid approach for complex congenital heart disease in high-risk neonate

Babies born with complex congenital heart disease often can have associated issues such as extreme prematurity, low birth weight, abnormal genes and significant congenital disorders other than the heart.

Babies born with complex congenital heart disease often can have associated issues such as extreme prematurity, low birth weight, abnormal genes and significant congenital disorders other than the heart. These associated issues can significantly increase the risk of their heart surgery.

An example is a baby who recently received care at Norton Children’s Hospital. He was born at 28 weeks, weighing around 1.2 kilograms, had genetic abnormalities and was diagnosed to have a very complex heart condition with interrupted aortic arch, small aortic valve and a ventricular septal defect.

Traditionally, the choices were either to wait until the baby grows older and weighs more — and that strategy is often unsuccessful as the baby likely would go into heart failure and develop complications prior to surgery — or to perform early full repair that is very high risk due to his prematurity, low weight and other ongoing conditions.

A third — hybrid — alternative

Fortunately, we recently have adopted the hybrid approach that provides these babies with a third alternative that can be associated with superior results. This hybrid approach involves placing small bands on the pulmonary artery branches to control the amount of blood that goes to the lungs (as too much can lead to heart failure). In addition, placement of a stent in the ductus arteriosus keeps it open to maintain the blood going to the body beyond the obstructed aorta. This stent allows discontinuation of prostaglandin (the intravenous medication we use to keep the ductus open). The medication can be associated with many complications with prolonged use, including needing to keep the babies on the ventilator.

While the hybrid approach isn’t a permanent fix, it is a much safer alternative that stabilizes the circulation and allows delay of the full repair to a later stage after the baby has grown and other clinical conditions have improved. One benefit of this hybrid approach is that the baby gets to go home after the first procedure, rather than remaining in the hospital. Going home means fewer days in the hospital, lower costs and less disruption in the lives of the families.

When the full repair is done a few months later, the surgery can be performed more efficiently and accurately. The baby is larger and healthier and can tolerate this complex operation much better, all leading to lower risk and faster recovery.

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This baby underwent the second operation at 7 months, and he weighed around 7 kilograms at that time. He underwent full repair, in addition to removal of the bands and stent. He had a quick recovery and is still doing great at home. We have used this hybrid approach since then to treat multiple babies similarly born with complex heart defects and aortic arch obstruction and who had many other medical issues that would have made full repair on them very high risk.

Among the first to adopt hybrid approach for the extremely small and high risk

Previously, this hybrid approach has been used to stabilize babies born with single ventricle who are listed for heart transplantation as they awaited donor hearts. It also has been used as an alternative to the Norwood procedure in babies born with hypoplastic left heart syndrome.

At Norton Children’s Heart Institute, affiliated with the UofL School of Medicine, we still offer one-stage full repair in babies with similar complex heart defects who are not extremely premature or small, and consider one-stage full repair the standard of care. Our outcomes with these complex operations are superior. Therefore, we reserve the hybrid approach only to those who are extremely small and have other associated issues that make them at an exceptional high risk for operation. This hybrid approach definitely has improved the outcomes for these babies.

Norton Children’s Heart Institute is among the first North American centers that has adopted this hybrid strategy to treat high-risk, low birthweight preemies needing complicated surgery that leads to full biventricular repair. The utility of this approach is becoming recognized, and it’s likely that more centers will start adopting this strategy. We recently have been invited to present our experience with this hybrid approach for the American Association for Thoracic Surgery and have been asked to write it up for the Seminars of Thoracic and Cardiovascular Surgery: Pediatric Cardiac Surgery Annual, which will be published in the spring.

At Norton Children’s Heart Institute, we’re proud to be able to offer the hybrid approach to our patients and their families. It is one more tool we have at our disposal for the treatment of complex, sick and small neonates. It’s an approach with far better chance of success, lower risk and fewer complications than the traditional alternatives.

Bahaaldin Alsoufi, M.D., is chief of pediatric cardiothoracic surgery and co-director of Norton Children’s Heart Institute.

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