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Kristin Deichler was one of three sisters born with a congenital heart disease — a partial atrioventricular septal defect — commonly called a hole in the heart. Today, she's part of a growing population of adults living with congenital heart disease due to advances in medical, transcatheter, and surgical therapies.
“When I was quite young there were some symptoms,” Ms. Deichler says. “I was supposed to be able to walk and be mobile, but I was lethargic. My mom says that I would try to climb the stairs and after two steps I would have to lie down and take a rest.”
At age 18 months — 30 years ago this year — her defect was successfully repaired by a cardiothoracic surgeon at Children’s Hospital of Pittsburgh of UPMC.
For the next 20 years, the Pittsburgh-area native, like her sisters, followed a regimen of care that included annual visits with Children’s Hospital’s pediatric cardiologists at the old Oakland campus. Other than a functional heart murmur, there were no lingering effects. When she reached adulthood, she neither found an adult cardiologist nor continued to see her pediatric cardiologist. After graduating from Duquesne University, she went on to start her teaching career. And with regard to matters of her heart, she simply became lost to follow-up.
This lack of transition is more typical than not for adults whose congenital heart diseases were identified and addressed as infants or children, according to Stephen Cook, MD, FACC, director of the Adult Congenital Heart Disease Center, a joint program of Children’s Hospital and UPMC’s Heart and Vascular Institute (HVI). That’s a problem when you consider that in the United States today there are an estimated 800,000 to 1 million adults with congenital heart disease, he says. Thanks in part to medical advances, such as surgical and transcatheter therapies, “the number of adults with congenital heart diseases has actually outgrown the number of pediatric congenital heart disease patients.”
Recognizing the special needs of this patient population, the American College of Cardiology and the American Congenital Heart Association recommend that adults with congenital heart disease receive care by specialists in this field like Dr. Cook. In support of these guidelines, pediatric cardiologists at Children’s along with their adult counterparts at HVI this year formed the Adult Congenital Heart Disease (ACHD) Center under Dr. Cook’s direction. An expert in the transition of care of patients with congenital heart diseases, Dr. Cook’s unique skill set includes both adult and pediatric cardiology, internal medicine, and pediatrics. He also serves on national committees to help define best practices for this unique population of patients.
“What we’ve learned over the last several decades is that congenital heart disease patients are at risk of late-onset complications,” Dr. Cook explains. “Frequently these develop in their third or fourth decades of life. Patients are at risk for arrhythmias, stroke, heart failure, and sudden cardiac death. Our job as adult congenital specialists is to prevent these late-onset complications.”
Take Ms. Deichler, who was seen this summer at the ACHD Center after receiving a letter from Children’s informing her of Dr. Cook’s arrival. “I wasn’t experiencing any problems, but I knew that it was in my best interest to follow up, having had this congenital heart disease,” she says.
Dr. Cook’s exam included a comprehensive review of her medical history, an electrocardiogram at Children’s, and a more detailed imaging study via cardiac MRI at UPMC Presbyterian. This final test provided accurate information and insight with regards to her underlying congenital heart defect and moderate residual mitral valve regurgitation, a condition that may require surgical repair in the next several years. Now that the relationship with the ACHD Center has been established, she’s in a better position to manage her condition.
Dr. Cook and his colleagues are prepared to help more patients like Ms. Deichler, who were lost in the transition to adult care.
“Transition is important,” says Dr. Cook. “With surgeries so early in life, patients often think they’re fixed. As adults, they then disappear and are lost to follow-up.
“Much of our focus is on education. It’s just homing in on the fact that you need lifelong care to avoid that host of late-onset complications.”
Those “later in life” issues include arrhythmias resulting from either the underlying congenital heart disease itself or the original surgery. Despite medical therapy, some patients continue to develop “pretty aggressive arrhythmias” as middle age approaches, says Dr. Cook. “They may require ablative therapies to help manage or reduce the burden of arrhythmias in the future.”
Still, some patients require the ablation plus placement of either an ICD (implantable cardioverter-defibrillator) or a pacemaker, he says.
“Lastly, it is not uncommon that patients require device therapy due to abnormalities in the underlying conduction system, such as sinus node dysfunction or sinus bradycardia, to appropriately treat not only complex arrhythmias but to implement anticongestive medications in adult patients with complex congenital heart disease.”
Women with congenital heart disease also have special needs. “Health care practitioners who are knowledgeable in adult congenital heart disease and reproduction should provide clinic-based teaching and counseling for women of child-bearing years,” Dr. Cook says.
During outpatient evaluations for both adolescents and young adults at the ACHD Center, education and educational resources are provided for both contraception and pregnancy. Pre-pregnancy counseling often includes risk-stratification and the need for noninvasive studies to evaluate each young woman prior to pregnancy to provide appropriate counseling.
“A multidisciplinary team approach to pregnancy, including cardiology, high-risk obstetrics, and anesthesia is often discussed with the patient,” Dr. Cook explains. Putting it together The ACHD Center is structured so that when patients are referred, they may be seen at either Children’s or UPMC Presbyterian, a partner of the ACHD Center because of the hospital’s recognized leadership in adult cardiology and cardiothoracic surgery and parallel ties with the University of Pittsburgh School of Medicine. For an outpatient visit, it may just be a matter of a patient’s personal preference or convenience.
For imaging services, patients are referred to UPMC Presbyterian’s comprehensive Cardiac Imaging Center (See “Get the Picture”). If intervention or surgery is required, the facility selected will be determined based on its capabilities coupled with the patient’s specific needs, potential comorbidities, and the multidisciplinary team required, says Dr. Cook, who as the “grown-up doctor in the children’s hospital,” serves as the liaison between Children’s and UPMC Presbyterian.
“I can take care of a patient’s congenital heart disease-related issues in adulthood, but I have to look at the patient as a whole, and we do that in collaboration with our colleagues at UPMC Presbyterian,” says Dr. Cook. “As cardiologists, we can manage their arrhythmias, heart failure, valvular disease, and all the late-onset complications related to congenital heart disease. However, we also need to be aware of the complications that develop as aging adults — for example, poorly controlled diabetes that’s going to lead to heart artery disease or atherosclerosis.”
When it comes to treatment and repair, Children’s Cardiac Catheterization Lab is a centerpiece of care for the ACHD Center. Headed by Jacqueline Kreutzer, MD, it offers less invasive approaches to surgery, such as use of catheters for opening valves or vessels.
For example, the conventional approach to treating adults presenting with native aortic coarctation, a narrowing of the aortic arch, or recoarctation, would have been surgery through the chest. However, after evaluation and referral from the ACHD Center, the pediatric interventionists can now apply their skills to adult repairs using covered stents, implanted and then expanded in the aorta by way of a catheter inserted through a vein. The stents are FDA-approved for “compassionate use” only, a process Children’s Hospital understands more than most facilities for this condition.
“The pediatric interventionalist has the greatest experience in this congenital defect regardless of age,” Dr. Cook says. “Although the peripheral vascular interventionalist and adult interventionalist have expertise in the femoral and iliac and coronary arteries respectively, it’s the pediatric interventionalists who are most familiar with this congenital lesion, and the challenges associated with complex arch, for example, transverse arch hypoplasia, and transcatheter interventions that involve lesions in close proximity to the brachiocephalic vessels.”
Dr. Cook relates the experience of a 53-year-old woman who was recently referred to Children’s after a chest x-ray, taken for evaluation of cough and congestion, revealed “rib notching,” the sine qua nonof aortic coarctation.
“She then underwent noninvasive testing after a murmur was discovered on her examination that prompted evaluation by several cardiologists and cardiothoracic surgeons, who provided the option of surgical repair. We provided a transcatheter option for repair of her underlying coarctation.
“Unfortunately, surgical repair can be associated with neurologic complications. Therefore, we presented stent therapy as an alternative to surgery to address the underlying coarctation. This is something we perform routinely at Children’s,” says Dr. Cook.
The notion of adults being cared for in a pediatric hospital may seem unusual, but it’s not without precedent. Even before the establishment of the ACHD Center, the hospital has occasionally taken care of adults — even elderly patients.
“From a patient perspective, there is enough privacy,” says Dr. Cook. “We make it clear that adult patients will not be sharing a room with a teenager or preteen. You’ll have the respect of adult privacy at a children’s facility.”
Other adult-friendly features include:
Even an adult attending the ACHD Center at Children’s Lawrenceville campus as an outpatient isn’t as unusual as one might think. Just ask Kristin Deichler.
“It may sound corny, but it felt like going home to me, even though it’s a new building. Knowing that they had this adult center, I was overjoyed,” she says. “I didn’t feel awkward or out of place.
“It was actually the piece of my care that I was looking for. All of my history’s there, and they’re bringing all their passion, their interest for treating people who have had these defects from birth or a young age, and adding to that the knowledge about what to do for an adult who has these disorders.
“I’ve known about my condition all my life and have had many opportunities to learn. Dr. Cook was really phenomenal about educating me even further. He’s very good at relating what happened to you as an infant, as a toddler, as a young child, and then explaining the considerations for an adult.
“After the visit, I called my mom first and told her how awesome it was. She’s been there for the whole journey for all of us at Children’s Hospital. Then I called my younger sister and said, ‘You need to contact the Adult Congenital Heart Disease Center at Children’s. They have a lot of information, and it’s the right fit for you.’”
For an appointment or consultation with the ACHD Center, call 412-692-5540 or send an email to firstname.lastname@example.org.
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