As advances in science have led to more sophisticated surgical repair and intervention, adults born with serious heart defects are living longer and better lives.
These survivors are part of a growing group of adult patients who need the expert follow-up care best found at adult congenital heart disease centers.
These patients are an emerging population, says Penny Kardis, RN, MSN, clinical coordinator for the Adult Congenital Heart Clinic and the management coordinator for cardiac rehabilitation at the Inova Heart and Vascular Institute in Falls Church, Va.
We are coming into the first generation of people who have survived into adulthood with these problems.
Currently, about 1 million American adults and 800,000 children live with congenital heart disease, according to the Adult Congenital Heart Association. Improved corrective surgeries, percutaneous procedures, and follow-up care have prolonged survival.
Many adults with congenital defects do not receive adequate follow-up care. A study by physicians at the University of Colorado Health Sciences Center in Denver and 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 without care was 10 years, and the most common reason is patients were told they didnt need follow up. Clinicians now know that is not the case.
Loss to care when the patients reach adulthood equates to a possible decrease in quality of life or lifespan, says Nancy Klein, RN, BSN, a staff nurse in the outpatient cardiology clinic at Children’s National Medical Center in Washington, D.C. These patients need follow-up to monitor changes in their heart function and capacity.
Investigating Better Follow-Up
Children’s National and 10 other 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, known as HEART-ACHD.
The purpose of the study is to research the impact of education, treatment, and research with these patients, Klein says. Also, we want to try to understand why we lose these patients to care.
HEART-ACHD also aims to learn the prevalence of lapses in specialized congenital heart disease care and identify barriers to care. The centers will enroll new patients, who will 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 patients condition, surgeries, and diagnostic tests, such as recent electrocardiograms.
The Effects of Defects
Congenital defects occur in approximately one in 120 births, according to ACHA. The defect may be relatively simple, such as a septal defect, a hole between the right and left sides of the heart that 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.
The surgeries and repairs done many years ago are not the surgeries we are doing now on infants, Kardis says. Repairs are having to be redone. The way surgeries were done in the 60s and 70s, heart preservation was not as good, and they tended to do surgeries when the children were older.
Many patients suffered lung and heart damage as a result, Kardis explains.
Survivors have a greater risk than other people of developing additional heart problems, often because of changing blood flow patterns in the heart. Long-term problems include rhythm disturbances, valve problems, heart failure, endocarditis, and stroke.
Patients might ignore subtle symptoms of a worsening heart condition. 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.
Regular follow-up, having a trend of many data points, allows us to see quickly when there is a change so we can intervene early before a change becomes a problem, Kardis says. There is a point of no return in a lot of these cases.
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 late last year. These guidelines call for coordination of ongoing care through regional centers of excellence with expertise in adult congenital heart disease.
Authors of the guidelines stated a regional and national system of centers of excellence has been demonstrated to improve care for adults with similar chronic severe illness, such as severe heart failure, for which measures of improvement surrounding uniformity of care within a guidelines framework, medical, and surgical outcomes, decreased visits, improved patient quality of life, cost containment, data collection and knowledge dissemination, trials of new therapeutics, and enhanced insurability have been achieved.
Some patients try to stay with their pediatric cardiologist, but that also 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.
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 from their parents for their own health.
What Happens at Heart Centers
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. The team can manage patients medically or recommend additional surgeries or cardiac interventions.
Some of them are having problems because their old surgeries are no longer working, Kardis says. They may have valves that have gotten leaky or tight, or they have baffles and things needing to be replaced.
The nurses often work as part of an interdisciplinary team that also helps patients with anxiety and the psychological issues connected to an uncertain future, along with the financial and insurance issues associated with care.
Regular follow-up can lead to better outcomes, Kardis says. Some of these patients can lead full, productive lives.
Debra Anscombe Wood, RN, is a freelance writer.
To comment, e-mail [email protected]