Multidisciplinary Program Targets Adult Congenital Heart Disease

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Innovations in Cardiovascular Medicine & Surgery - Summer 2019

For children born with congenital heart disease, early surgical intervention and ongoing medical management have enabled a 90 percent national survival rate. But with patients and families relying on a pediatric care team, what happens during the transition to adulthood?

Experts at University Hospitals Rainbow Babies & Children’s Hospital, University Hospitals Harrington Heart & Vascular Institute and University Hospitals MacDonald Women’s Hospital have partnered to develop a multidisciplinary program that provides seamless care delivery for this increasing population coping with the lifelong challenges of adult congenital heart disease (ACHD).

“Integrated, standardized care is what is needed to properly address the long-term issues these patients experience,” says Pradeepkumar Charla, MD, Director, Adult Congenital Heart Disease Program, University Hospitals; and Assistant Professor, Case Western Reserve University School of Medicine. “At UH, our collaborative team has a singular vision to advance the care for our ACHD patients.”

Pradeepkumar Charla, MDPradeepkumar Charla, MD

Unique in Ohio, the center’s ACHD clinics bring together specialists from pediatric, adult and maternal health in a central setting where they assess each patient’s natural history and current status, consult to develop a comprehensive care pathway, and further define protocols. Board-certified physicians and advanced practitioners partner in the innovative care model to address both physical and emotional well-being. The team includes:

  • heart surgeons
  • interventional cardiologists
  • advanced cardiac imaging specialists
  • electrophysiologists
  • anesthesiologists
  • heart failure and pulmonary hypertension specialists
  • maternal fetal medicine specialists
  • psychiatry, psychology and social work

The center is in the process of seeking accreditation by the Adult Congenital Heart Association, an advocacy organization striving to set criteria and eliminate care gaps for more than 1.4 million Americans living with ACHD, including an estimated 20,000 additional young adults each year.

RISK STRATIFICATION AND EARLY INTERVENTION

Despite best practices care, ACHD patients with complex disease are at risk for heart failure and may require heart transplant. Statistically, 3.3 percent of adult transplants are ACHD-related. Risk stratification and proactive management can extend the time before transplant. Additionally, early introduction to heart failure specialists can improve outcomes. 

“Our multidisciplinary clinics are connecting patients to the heart transplant team sooner, enabling us to develop relationships that help ease the process,” says Guilherme Oliveira, MD, Director, Advanced Heart Failure & Transplant Center, UH Harrington Heart & Vascular Institute; Lorraine and Bill Dodero Master Clinician in Heart Failure and Transplantation; and Professor, Case Western Reserve University School of Medicine. “Careful selection of appropriate candidates is helping us put patients on the waiting list before they are in crisis or experiencing end-organ failure.”

Heart transplant in ACHD patients is markedly different from that for acquired heart disease because the procedure is dependent on structural repair. “These patients often experience functional decline because of chronic passive pulmonary circulation,” says Robert Schilz, DO, Director, Pulmonary Vascular Disease & Lung Transplantation, University Hospitals Cleveland Medical Center; and Associate Professor, Case Western Reserve University School of Medicine. “Heart transplant in ACHD often requires extensive reconstruction of pulmonary arteries.” Collaborative preplanning ensures that all members of the surgical team are in alignment for the complex procedures. 

SPECIALIZED CARE FOR MOTHERS AND BABIES

Monthly maternal fetal medicine ACHD (MFM-ACHD) clinics provide support for high-risk pregnancy to address physiologic cardiovascular changes during gestation that may be poorly tolerated in women with underlying cardiac disease, placing them at significantly increased risk of morbidity and mortality. 

“Given that approximately two-thirds of heart disease in pregnancy is congenital, our coordinated multidisciplinary clinics are central to reducing risks,” says Justin Ross Lappen, MD, Maternal and Fetal Medicine, UH Cleveland Medical Center; and Assistant Professor, Case Western Reserve University School of Medicine. “Through our collaborative effort in MFM-ACHD clinic, we can safely transition women into and out of pregnancy care and make appropriate plans for a safe delivery. 

“Additionally, because congenital heart disease is genetically linked, we are able to provide screening and in-utero treatment in select cases when indicated,” Dr. Lappen adds.

GLOBAL TRIAL OF EXTERNAL VENTILATION THERAPY 

In addition to maintaining robust clinical practices, physicians on the ACHD team are committed to investigational research. Currently, UH is the site for a global-first feasibility (Phase II) study of the efficacy of ambulatory biphasic cuirass ventilation for individuals with palliated univentricle ACHD. 

The Hayek RTX external pulmonary device is a noninvasive therapy that is showing tremendous potential. The Hayek RTX external pulmonary device is a noninvasive therapy that is showing tremendous potential.
Although Fontan palliation in childhood successfully redirects circulation, lifelong low cardiac output and decreased pulmonary blood flow are inevitable. “In order to help these children survive to adulthood, we have to bypass the need for a second chamber by surgically routing blood directly to the pulmonary artery,” Dr. Charla says. “Over time, pressure in the Fontan circuit builds, and delayed complications such as arrhythmia, cyanosis or heart failure can develop.”

When one or more of these complications occurs, treatment options historically have been limited. Use of a Food and Drug Administration-approved external ventilator (Hayek RTX) is a noninvasive therapy that is showing tremendous potential.

“External negative pressure ventilation has demonstrated efficacy in addressing the underlying pathophysiology,” Dr. Charla says. “We are finding that 10-minute applications of this device can improve cardiac output by 20 percent — that is extremely significant.”

PATIENT-CENTERED CARE FOR TODAY AND TOMORROW

“The data we collect from patients in these dedicated clinics will help us optimize therapy and extend research opportunities,” Dr. Charla says. “We take advantage of every resource to address the physical and emotional impact of ACHD, extend survival rates and improve quality of life for our patients.”

For more information about the UH Adult Congenital Heart Disease Program, call 216-553-1439 or email Dr. Charla at Pradeepkumar.Charla@UHhospitals.org.

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