Babies born with serious congenital heart defects are living into adulthood thanks to surgical repairs and other interventions. But after pediatric care ends, these patients lack adequate follow-up care at adult congenital heart disease centers.
“We want all of our patients to live long, productive, happy lives doing what they want to do and not making their congenital heart defect their focus in life,” says Mary Wilkie, RN, APN, MS, CCNS, a clinical nurse specialist at the Center for Congenital and Structural Heart Disease at Rush University Medical Center in Chicago.
About 1 million American adults and 800,000 children live with congenital heart diseases, according to the Adult Congenital Heart Association. Only recently, with improved corrective surgeries, have youth with a congenital heart defect lived to adulthood. So many adults with congenital defects do not receive adequate care.
A study by physicians at the University of Colorado Health Sciences Center in Denver, which was reported in the March 2008 International Journal of Cardiology, found 63% of the 158 patients seeking care at its adult congenital heart clinic from 2002 to 2005, had left pediatric care two or more years before seeking adult cardiac follow up. The median duration was 10 years, and the most common reason for not seeking care is they were told they didn’t need follow up, which clinicians now know is not the case.
“Being fixed doesn’t mean you are back to normal,” says Karen Kasinger, RN, MSN, CPNP, a nurse practitioner at the University of Chicago Medical Center. “Certain palliations can last for years, some for decades, but the possibility of having problems in the future is always there.”
Congenital defects occur in about 1-in-120 births, according to the ACHA. The defect may be relatively simple, such as a septal defect, which is correctable with a patch. But babies born with a transposition of the great arteries or a single ventricle require more complex repairs or multiple surgeries.
Better Follow-UpMary Wilkie, RN
Eleven U.S. centers are participating in the two-year National Institutes of Health-funded Health Education and Access Research Trial in Adult Congenital Heart Disease study. ACHA and the Alliance of Adult Research in Congenital Cardiology, a multicenter research group, hope the research will lead to better care for these individuals.
“The survey is to determine more specifically the reasons these folks are lost to follow up,” says Mary Krolikowski, APN, clinical resource nurse at the Herma Heart Center Adult Congenital Heart Disease Program at the Medical College of Wisconsin in Milwaukee, one of the study sites. “The cardiology community believes being lost to follow up may put them at risk of more serious heart disease down the road.”
University of Colorado researchers reported patients with a lapse in medical care have a greater likelihood of developing secondary cardiac abnormalities and an urgent need for intervention.
HEART-ACHD also aims to pinpoint the prevalence of lapses in specialized congenital heart disease care, identify barriers to care, assess whether a targeted educational intervention is successful in improving patients’ self-care, and work to engage them in future research studies.
The centers will enroll new patients who complete a survey. Nurses will educate patients about their conditions and complete the ACHA personal health passport, a mini-booklet with a removable emergency wallet card and information about the patient’s condition, surgeries and diagnostic tests, such as recent ECGs and complete blood counts. These patients’ baselines may differ from the average patient.
Guidelines for Improved Care
The American College of Cardiology and the American Heart Association issued practice guidelines on the management of adults with congenital heart disease in November 2008 that call for coordination of ongoing care through regional centers of excellence with expertise in adult congenital heart disease.
Some patients try to stay with their pediatric cardiologist, but that presents challenges. These patients require the skills of a clinical team with experience in adult comorbidities, such as diabetes or hypertension, and can offer guidance about employment, pregnancy, and physical activity.
“They are not pediatric patients any more,” Wilkie says. “Yet the type of defect they have is not a defect an adult cardiologist would be familiar with.”
The centers work closely with pediatric providers to ensure a smooth transition of care as the patient reaches adulthood. Patients must learn to assume responsibility for their own health.
Frequently, patients present to the centers when they start to develop problems, such as fatigue, exercise intolerance, chest pain, shortness of breath, or palpitations. Clinicians at the specialized centers obtain past medical records and work up patients to determine their current condition, evaluate blood flow through the heart, and identify structures created or remnants of the original congenital condition.
“We want to make sure the heart continues to work properly to meet the demands of the body,” Wilkie says. “We want to make sure the ventricles are functioning properly and the vessels are patent.”
The team may manage the patients medically or recommend additional surgeries or cardiac interventions. For instance, baffles may narrow over time and need reopening and a stent placement.
“If they received a valve when they were young, they would outgrow the valve, or it would wear out over time,” says Linda Hellstedt, RN, APN, a cardiac nurse at the Bluhm Cardiovascular Institute at Northwestern Memorial Hospital in Chicago. “And they would need [other] operations over their lifetime.”
The nurses often work as part of an interdisciplinary team that also helps patients with anxiety and the psychological issues of dealing with an uncertain future and the financial issues associated with care.
“People are living much further out,” Kasinger says. “Even the very most complex heart disease can be palliated, and [patients will] live into their 20s, 30s, and 40s before they have to worry about heart transplant, which is the next step. With heart disease that is not that complex, many people live very long, fulfilling, active lives after being treated.”