Advances in Pediatric Cardiology

Dr. Emile A. Bacha

Complex aortic valve disease is one of the ongoing challenges for congenital heart surgeons. While outcomes for the Ross procedure, the current gold standard approach, are very good, it is a major operation involving the removal of the diseased aortic valve for replacement with the patient’s own pulmonary valve. The pulmonary valve then has to be replaced with a cadaveric conduit. Over time, those conduits can deteriorate, calcify or become blocked, requiring either replacement or another intervention.

The NewYork-Presbyterian Congenital Heart Center and Pediatric Heart Valve Program recently expanded the options for children needing aortic valve reconstruction with the introduction of the Ozaki aortic valve reconstruction procedure – aortic valve neo-cuspidization – as an alternative to the Ross procedure or prosthetic valve replacement. The procedure is performed by NewYork-Presbyterian/Columbia University Irving Medical Center pediatric cardiac surgeons at both NewYork-Presbyterian Morgan Stanley Children’s Hospital and NewYork-Presbyterian Komansky Children’s Hospital at NewYork-Presbyterian/Weill Cornell Medical Center.

“We desire to offer the entire range of surgical treatment options for complex congenital aortic valve disease, and we believe the Ozaki has a role for certain children,” says Dr. Emile Bacha, Chief of the Division of Cardiac, Thoracic, and Vascular Surgery at NewYork-Presbyterian/Columbia and Director of Congenital and Pediatric Cardiac Surgery at NewYork-Presbyterian.

The Ozaki procedure for children is one that few hospitals can offer. With the Ozaki approach, the diseased aortic valve leaflets are removed, and a new aortic valve is recreated by sewing the leaflets into the native aortic valve annulus and aortic root, providing a new functioning valve. These leaflets may be created from bovine pericardium or a patient’s own pericardium tissue, which is preferred. The procedure is attractive in children because the new valve that has been created has the potential to expand as the heart grows and still maintain its ability to function as a valve. It also doesn’t preclude doing a Ross down the road or using a mechanical valve.

In addition, according to Dr. David Kalfa, the technique is a less extensive surgery than the Ross procedure because it does not require harvesting the pulmonary valve and coronary reimplantation. “Patients are considered candidates for the Ozaki procedure in two primary situations – when the Ross procedure is not an option or when we know that the Ross procedure won’t bring the best possible outcomes,” says Dr. Kalfa. “For example, patients who present with aortic regurgitation and aortic stenosis, and patients who have rheumatoid or other connective tissue disease would not be recommended for a Ross procedure.”

The Ozaki procedure also may be an important treatment option for patients who have had truncus arteriosus repair and for those who have an unusable pulmonary valve, or when long-term results of the Ross procedure are unreliable due to indication of aortic insufficiency in children with enlarged aortic annulus or for those whose root is not large enough to have a definitive “adult-sized” reinforced Ross.

The Ozaki technique preserves normal contributions of the aortic root to left-sided cardiac function, and the reconstruction has the potential to grow with the child. In addition, it preserves the right ventricular outflow tract and pulmonary valve, avoiding future surgical intervention on the pulmonary valve.

Benefits of the Ozaki Procedure

• preserves normal contributions of the aortic root to left-sided cardiac function
• native leaflets avoid the use of a mechanical or synthetic bioprosthetic valve, which requires lifelong dependence on anticoagulants
• the size and shape of the leaflets provide the valve with the potential to
• expand as the heart grows and still maintain its ability to function as a valve, which is particularly relevant in pediatric cases
• preserves the right ventricular outflow tract and pulmonary valve, avoiding future surgical intervention on the pulmonary valve does not preclude performing a Ross procedure at a later time
• takes less time to perform than the Ross procedure, and patients spend less time on bypass

While the Ozaki approach has been well underway for use in adults for nearly a decade with very promising outcomes, long-term durability in children is unknown. The NewYork-Presbyterian Congenital Heart Center strongly believes that the procedure has a bright future in pediatric cardiac surgery. Collectively, our surgeons have now performed over a dozen Ozaki procedures – a number that continues to increase – with excellent outcomes. They emphasize the importance of entertaining an open discussion with patients and their families regarding all available surgical options for complex aortic valve disease to inform them of the pros and cons of each technique to derive the most optimal outcome.