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Specialized, Complex Surgical Techniques Added to Heart Program at Children’s

Feb 23, 2018 11:34PM ● By Alyssa McGinnis

Complex cardiothoracic surgical procedures such as “unifocalization” – a single procedure employed to repair a defect known as tetralogy of Fallot with pulmonary atresia and major aortopulmonary collaterals – are being offered in a new program at Children’s Hospital & Medical Center.

The Pulmonary Artery Rehabilitation program is being developed by cardiothoracic surgeon Ali Ibrahimiye, MD, of Children’s Specialty Physicians who is also an assistant professor in the Department of Surgery at UNMC College of Medicine.

Unifocalization is a procedure created by Frank L. Hanley, MD, director of the Children’s Heart Center at the Lucile Packard Children’s Hospital at Stanford University. Dr. Ibrahimiye, who is board certified in General Surgery, Thoracic Surgery and Congenital Cardiac Surgery, completed a fellowship in Congenital Cardiac Surgery there, where he performed surgery alongside Dr. Hanley.

“It is a very tiresome, tedious, eight to 10-hour operation, and children can become very sick afterward,” Dr. Ibrahimiye says. “A surgeon has to do a lot of these operations to learn how to do it well and be comfortable performing it. During my fellowship, we were doing one or two a week.”

Not only has Dr. Ibrahimiye performed the unifocalization operation many times, he has also written about it, documented it in videos, and presented about the surgery at numerous conferences, including the Congenital Heart Surgeons’ Society (CHSS) annual meeting in Chicago in October 2017.

Tetralogy of Fallot with pulmonary atresia is an extreme form of tetralogy characterized by absence of flow from the right ventricle to the pulmonary arteries. The defect actually involves four (tetralogy) heart defects: a large ventricular septal defect (VSD), pulmonary stenosis, right ventricular hypertrophy and an overriding aorta. The blood vessels that should connect the heart to the lungs instead connect the lungs to the aorta, and the heart has a hole in the wall that separates the lower chambers.

With tetralogy of Fallot, not enough blood is able to reach the lungs to get oxygen, and oxygen-poor blood flows to the body. Infants and children who have tetralogy of Fallot have episodes of cyanosis, a bluish tint to the skin, lips and fingernails, due to the lower-than normal level of oxygen in the blood leaving the heart.

With the unifocalization procedure, the misdirected blood vessels are rerouted into a single vessel or the pulmonary artery, which is then attached to the right ventricle through a conduit called a homograft, to restore normal circulation from the lungs to the heart. After this step, the hole in the ventricle wall is repaired.

The comprehensive care these patients receive at Children’s takes into account the variability from child to child. “Our heart team not only performs these complex surgeries, but also any additional procedures that may follow,” Dr. Ibrahimiye says.

Dr. Ibrahimiye says the fact that Children’s is a medium-sized program affords patients and their families a higher level of personalization. “It allows our surgeons to not only operate, but to actively participate in our patients’ care,” he says. “I see my patients twice a day. I round in the morning and round again before I go home. That is not always the case in larger programs.”

Having such a program here in the middle of the country gives referring physicians a unique regional option, Dr. Ibrahimiye says. “They do not need to send their patients to California to address this condition or many other pulmonary artery reconstruction issues,” he says. “That keeps patients and their families closer to home, and saves them the expense and burden of moving for what might be months of hospitalization and care.”