Medical Professional Publications

Treating Patients with End-Stage Lung Disease

(From Pediatric Directions, Issue 39)

In November 2011, surgeons from The Heart Center at Nationwide Children’s Hospital and the Heart-Lung Transplant Program bridged Bethany to lung transplant using ambulatory venovenous extracorporeal membrane oxygenation (VV ECMO). She is one of the youngest patients recorded to have undergone this procedure.

Lung transplant patient Bethany

This historic medical event proved the capability of ambulatory VV ECMO to extend the life of patients, many of whom suffer congenital disorders and are awaiting lung transplantation or heart-lung transplantation. Typically, Bethany and patients like her, would remain on a mechanical ventilator before surgery. Although some patients tolerate the mechanical ventilator, patients with advanced lung disease are at higher risk for complications, such as pneumonia, collapsed lung and bronchopleural fistula.

The Heart Center and Pulmonary Medicine teams provide the special expertise required to treat children and adults with congenital disorders, making the hospital home to one of the largest Adult Congenital Heart Disease programs in the country. The multidisciplinary teams of pulmonary and cardiothoracic experts estimate that traditional mechanical ventilation further adds to lung injury in patients with advanced lung or heart-lung disease. With pediatric expertise in applying ECMO, the teams at Nationwide Children’s have modified the mechanical assist device making components smaller and portable. Following this protocol and using the adapted equipment reduces or avoids exposure to mechanical ventilation, allowing its use when patients become critically ill and cannot breathe independently. In addition, patients on VV ECMO can be rehabilitated while they await a lung transplant, which positively impacts long-term outcomes. Bethany has cystic fibrosis, which is one of the diseases that can benefit from ambulatory VV ECMO.

The Heart-Lung Transplant Program team works in conjunction with The Heart Center, offering combined heart-lung transplantation to patients with advanced cardiopulmonary disease. The program has received funding for multiple, patient-based translational research projects, including a multicenter research study funded by the National Institutes of Health. Also, as an active member of the International Pediatric Lung Transplant Consortium, the center consists of programs for cystic fibrosis, pulmonary vascular disease, interstitial lung disease, neonatal lung disease, comprehensive gastrointestinal evaluation and histopathological analysis. Physicians on this team are now performing aggressive right-heart catheterizations as part of the transplant evaluation process.

Treatment Options for Patients Facing Cardiopulmonary Failure
The Heart Center surgeons utilize a full spectrum of mechanical devices designed for short- to longer-term use. Currently, physicians evaluate each case and work collaboratively to decide the treatment option that will ultimately provide the best outcome for the patient.

  • ECMO – Extracorporeal membrane oxygenation provides short-term support of the heart and the lungs for hours to days.
  • Extracorporeal VAD/PediMag – The extracorporeal ventricular assist device provides short-term support of the heart for days to weeks.
  • Paracorporeal VAD/Berlin Heart – The paracorporeal ventricular assist device provides intermediate- to long-term support for left, right or biventricular support in infants and children. Paracorporeal devices may be preferable  for short- to intermediate-term cardiac support. This could be weeks to months of support, while waiting for cardiac recovery or transplantation.
  • Intracorporeal VAD/HeartMate II – Intracorporeal ventricular assistance is fully implanted within the patient and designed for longer-term use in older children and adults waiting for transplant or without other long-term options.
  • NovaLung/Ambulatory ECMO – The NovaLung supports the patient with isolated lung failure. The artificial lungs “breathe” outside the patient’s body. It also lowers the blood pressure, allowing the heart to recover.

The Heart Center and Pulmonary Medicine teams realize it is imperative to provide as much time as possible to patients on the lung transplant list. If patients are being supported with VV ECMO, they can potentially perform rehabilitation and optimize their nutrition intake. When patients go into surgery, the use of ECMO can be continued or changed to cardiopulmonary bypass. For best outcomes, surgeons and perfusionists utilize cell savers, which collect, clean and recycle a patient’s own blood during surgery.

The care provided to each patient after surgery focuses on allowing the patient’s body and immune system to recover. All respiratory support is removed as quickly as possible, which is often dictated by how the patient’s body responds to the new organ. The goal of delivering the best possible care is accomplished through a systematic approach.

Case Study: Bethany’s Story
Bethany became one of the youngest patients ever bridged to lung transplant with ambulatory VV ECMO, and this was the first time the procedure had been performed at a freestanding children’s hospital. Bethany was diagnosed with cystic fibrosis early in life and her disease quickly progressed, necessitating a lung transplant.

In 2011, Bethany was admitted to Nationwide Children’s for gastrointestinal complications of her disease. During admission, she developed infectious complications that eventually led to the development of acute respiratory failure. Due to the severity of her lung disease, The Heart Center and Pulmonary Medicine teams implemented VV ECMO to avoid lung injury from mechanical ventilation. She tolerated the VV ECMO without complications and successfully underwent bilateral lung transplantation eight days later. Today, Bethany is home and doing well.

View an interview with Dr. Hayes regarding Nationwide Children’s Lung and Heart-Lung Programs.

Author Bios:

Don Hayes, MD, MS, MEdDon Hayes, MD, MS, MEd, medical director of the Lung and Heart-Lung Transplant Programs at Nationwide Children’s, completed his medical training at the University of Kentucky College of Medicine graduating with honors. He pursued his combined internal medicine and pediatrics residency at East Carolina University Brody School of Medicine. He then completed three fellowships at the University of Wisconsin School of Medicine and Public Health with a concentration in advanced lung disease and lung transplantation during his adult pulmonary training. Dr. Hayes holds two master’s degrees – one in clinical and translational science and one in medical education.

Dr. Hayes is a nationally recognized physician, named a 2011 Top Physician and recent recipient of the American Medical Association Doctor William Beaumont Award in Medicine. He had published in more than 60 peer-reviews journals and has served as both an international and national speaker at symposia. His focus and experience is in bridging patients with severely advanced lung disease to lung transplant with artificial technology, such as extracorporeal membrane oxygenation (ECMO).

Reference and recent publication by author.
Hayes D Jr, Kukreja J, Tobias JD, Ballard HO, Hoopes CW. Ambulatory venovenous extracorporeal respiratory support as a bridge for cystic fibrosis patients to emergent lung transplantation. J Cyst Fibros 2012;11(1):40-5.

Mark Galantowicz, MD, FACSMark Galantowicz, MD, FACS, is the chief of Cardiothoracic Surgery and co-director of The Heart Center at Nationwide Children’s. He is professor of Surgery at the Ohio State University College of Medicine and is the recipient of the Murray D. Lincoln Endowed Chair in Cardiothoracic Surgery. Dr. Galantowicz received his medical degree from Cornell University. He completed his residency at Columbia-Presbyterian Medical Center, New York. He is board certified in congenital heart surgery, thoracic surgery and surgery.

Timothy M. Hoffman, MD, FACCTimothy M. Hoffman, MD, FACC, is medical director of the Heart Transplant and Heart Failure Program in The Heart Center at Nationwide Children’s. He is associate professor of Pediatrics, Division of Cardiology, at the Ohio State University College of Medicine. Dr. Hoffman received his medical degree from West Virginia University. He completed his residency at West Virginia University Medical Center and received fellowship training at Children’s Hospital of Philadelphia. He is board certified in pediatric cardiology and pediatrics.

Steve Kirkby, MDSteve Kirkby, MD, is the clinical director of the Lung and Heart-Lung Transplant Programs at Nationwide Children’s and an assistant clinical professor of Internal Medicine and Pediatrics at the Ohio State University College of Medicine. Dr. Kirkby graduated from Penn State University College of Medicine. He completed his residency training in internal medicine and pediatrics at The Ohio State University Wexner Medical Center and Nationwide Children’s. He continued to complete training fellowships in adult pulmonary/critical care medicine and pediatric pulmonary medicine.

He is board certified in internal medicine, pediatrics, adult pulmonary medicine, critical care medicine and pediatric pulmonary medicine. Dr. Kirkby’s main clinical interests include care of adults and adolescents with cystic fibrosis, treatment of unusual pulmonary infections, lung transplantations, asthma and critical care medicine. His research interests are centered on mechanisms of disease progression in cystic fibrosis and clinical outcomes in cystic fibrosis and lung transplantation.

Patrick I. McConnell, MDPatrick I. McConnell, MD, is an attending surgeon in the Department of Cardiothoracic Surgery at The Heart Center at Nationwide Children’s. He is assistant professor of Surgery at the Ohio State University College of Medicine. Dr. McConnell received his medical degree from University of Nebraska Medical Center and completed Surgery residency at the University of Utah. He completed fellowships at New York Medical College, Oregon Health & Science University, Ohio State’s Wexner Medical Center and Nationwide Children’s. Dr. McConnell’s clinical activities include congenital cardiothoracic surgery to include adult congenital heart disease and pediatric mechanical assist devices. His primary research interests involve the use of mechanical assist devices and associated therapies targeting heart recovery. He is board certified in surgery and thoracic surgery.

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