The American College of Cardiology and the American Heart Association have issued updated guidelines for managing congenital heart disease in adults.
The guidelines carry a clear directive, says Jonathan Cramer, MD, Nebraska Medicine and Children’s Nebraska pediatric and adult congenital heart disease (ACHD) cardiologist.
“Any patient born with congenital heart disease – many of whom have had a surgery, a cath, an intervention – needs to see an ACHD expert at least once,” he says. “And the vast majority of ACHD patients need to be followed throughout their life at an ACHD center of excellence.”
Nebraska Medicine and Children’s Nebraska provide exactly that – one of 60 accredited ACHD centers of excellence in the U.S. and the only one in the region. The program also features the region’s only board-certified congenital cardiologists and cardiothoracic surgeons.
“As a Nebraska native, I’m so proud that my team and I are able to offer these services to patients and cardiovascular specialists in the region,” Dr. Cramer says. “We provide immediate care locally – ACHD wait times elsewhere can be months to a year.”
PCPs are a critical link
Adult congenital heart disease patients often fall through the cracks during their teens, 20s and 30s when they feel well – and may not even remember having heart surgery as a child.
“Many patients, because their surgery happened so long ago, have little to no recollection of it at a conscious level,” says Nebraska Medicine and Children’s Nebraska cardiothoracic surgeon Ali Ibrahimiye, MD. “When asked if they had a surgery, a significant portion would say no.”
Primary care providers play a crucial role in identifying patients who may have slipped through the cracks and connecting them with the specialized care they need.
“If a provider sees a scar on the chest, they need to be the advocate and say, ‘Let me help you get in to see a congenital cardiologist, and you guys can decide from there,’” Dr. Cramer says.
He recommends referring any patient with:
- A known history of congenital heart disease.
- A midline sternotomy or lateral thoracotomy scar without a known history.
- A history of cardiac catheterization as a child.
- New arrhythmias or palpitations in a patient with past or unknown congenital heart history.
- Exercise intolerance, shortness of breath or signs of heart failure in a patient with past or unknown congenital heart history.
- Unexplained significant right heart dilation.
The single biggest risk: Lost to follow-up
Dr. Cramer says the transition from pediatric to adult care is where most patients disappear.
“There is a period of time where many patients feel fantastic – they’re also teenagers and young adults, and they don’t have time for their doctor’s visits,” he says. “Simply asking, ‘When was the last time you saw your congenital cardiologist?’ goes a long way. Unfortunately, we see people back when bad things happen – they’re in the ER, or they’re pregnant. We would love to see people routinely.”
Why general cardiology often isn’t enough
Adults with congenital heart disease often require a level of subspecialty training that falls outside the scope of most general adult cardiology and cardiothoracic surgery practices.
“Many adult cardiovascular providers, because of training pathway deficits nationally, don’t have a lot of comfort in congenital cardiology, and that’s understandable,” Dr. Cramer says.
For many years, congenital heart disease was not part of standard adult training pathways or was highly limited, Dr. Cramer explains. For that reason, pediatric cardiologists would follow patients well into adulthood.
“As patients aged and encountered adult issues, it became increasingly difficult, and led to other problems,” he says. “Patients would find themselves stuck between worlds and often just give up care – especially if they felt okay.”
To address this issue, board certification in congenital cardiology began to be offered in 2015 for both pediatric and adult cardiologists. Additionally, the Adult Congenital Heart Association (www.achaheart.org) has offered a center of excellence designation since 2017.
Comprehensive care
Accredited ACHD programs offer a level of comprehensive care that general cardiology practices are not inherently structured to provide – including comprehensive cardiology care, social work, mental health support and vocational counseling.
High-risk pregnancies are amongst the most urgent examples of why continuous ACHD follow-up matters.
“All ACHD centers have an established pregnancy program, and at any given time, we’ve got 20 to 30 pregnant women with significant congenital heart disease,” Dr. Cramer says. “It offers a unique challenge for congenital heart disease physiology.”
Refer today
The Nebraska Medicine and Children’s Nebraska Adult Congenital Heart Disease program includes the region’s only board-certified congenital cardiologists and CT surgeons and offers a full spectrum of care from routine clinic visits to heart transplantation.
To refer a patient or learn more, call the Adult Congenital Heart Disease at 402.955.4350.