Surgeons conduct rare pediatric double Lung-Heart transplant on a 12-year old girl

A team of surgeons at the University of Maryland Children's Hospital (UMCH) performed a rare bilateral lung-heart transplant on a 12-year-old girl Lindsey Le, a normal tween from Severn, Md. She has been the focus of intense medical treatment and care involving more than 20 physician specialists and dozens of healthcare professionals, which culminated in this unusual lifesaving procedure.

Heart-lung transplantation (cardiopulmonary transplantation) is the simultaneous surgical replacement of the heart and lungs in patients with end-stage cardiac and pulmonary disease. Causes of end-stage cardiopulmonary failure that necessitate cardiopulmonary transplantation range from congenital cardiac disease to idiopathic causes and include the following:

During the summer, while visiting family in Vietnam, she became increasingly short of breath and her feet became swollen and purple. Upon returning from her trip, she collapsed and had to be rushed to the emergency department. She quickly was admitted to the pediatric intensive care unit (PICU) at UMCH, where the cardiology team diagnosed her with severe pulmonary hypertension.

Lindsey had bilateral lung-heart transplant surgery in December 2018 during the holidays. The operation was performed by Dr. Griffith, Dr. Deatrick and Sunjay Kaushal, professor of surgery at UMSOM, director of pediatric and adult congenital cardiac surgery and co-director of the children's heart program at UMCH.

"Heart-lung transplants were performed more often on children in the 1980s and 1990s, but cases have dropped significantly in recent years," says Aldo Iacono, MD, the Hamish S. and Christine C. Osborne Distinguished Professor in Advanced Pulmonary Care and professor of medicine at the University of Maryland School of Medicine. Between the years 1988 and 2013, 188 pediatric heart-lung transplants were performed. Doctors have found that the heart can recover once a patient gets new lungs, but there are certain cases in children where heart-lung transplantation is favorable. "In Lindsey's case, her heart size was extreme and its pumping chambers were too weak," says Dr. Iacono, who is also the medical director of the lung transplant program at the University of Maryland Medical Center (UMMC). Even if she obtained new lungs, her heart likely would not recover.

"When Lindsey came here, her heart was literally the size of a basketball," says Bartley Griffith, MD, the Thomas E., and Alice Marie Hales Distinguished Professor in Transplantation and professor of surgery at UMSOM, and director of the cardiac and lung transplant program at UMMC. The cause of Lindsey's illness was a mystery. Her heart was swollen in its fight to push blood through the lungs."This was not an acute process that happened in Vietnam. She's probably had the condition for years," says Dr Griffith. Lindsey needed a double lung-heart transplant in order to save her life.

While UMMC is a nationally renowned transplant centre with highly skilled surgeons who regularly perform life-saving heart-lung transplants on adult patients, the team hadn't done this particular operation on someone so young. Lindsey represented a unique challenge, and she brought out the best in everyone. "It was truly a team effort to get a little girl with no hope of surviving with her original lungs and heart to a tween who is leaving the hospital, excited to return to school and cannot wait to participate in sports," says Carissa Baker-Smith, MD, MS, MPH, assistant professor of pediatrics at UMSOM and pediatric cardiologist at UMCH.

While waiting for a transplant, Lindsey was placed on a type of life support called extracorporeal membrane oxygenation (ECMO). The life support system uses a pump to circulate blood through an artificial lung back into the bloodstream. The heart-lung bypass support gives the heart and lungs a rest so that the patient will be more comfortable. Lindsey remained on ECMO for four months until her transplant surgery.

"Just a few years ago, we wouldn't keep a person on ECMO for more than a few days," says K. Barry Deatrick, MD, assistant professor of surgery at UMSOM, a pediatric cardiac surgeon and director of the life support program at UMMC. While on ECMO, Lindsey was up and walking. "We believe that mobility helps patients' outcomes. Our specialized physical therapy team makes it possible for patients like Lindsey to safely get out of bed and walk around," says Dr. Deatrick.

After surgery, Lindsey went back to the PICU to recover. She was visited daily by more than 20 providers of different areas of medicine including anesthesiology, critical care, infectious disease, pulmonology, cardiology, nursing, physical therapy, and respiratory therapy. When she was finally discharged in February, her care team lined the hallway of the PICU to cheer her on and wish her well. A blue sky and warm sunshine greeted Lindsey as she exited the hospital. She hadn't been outside in six months.