ESC adult congenital heart disease guideline stresses lifelong management
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The European Society of Cardiology released a new guideline on adult congenital heart disease emphasizing lifelong follow-up and endorsing catheter-based interventions when possible.
“One of the key messages of this document is ... that congenital heart disease is a lifelong disease,” Helmut Baumgartner, MD, professor of cardiology and adult congenital heart disease and director of the Department of Cardiology III: Adult Congenital and Valvular Heart Disease at University Hospital Muenster in Germany, who is co-chairperson of the guideline task force, said during a live session at the virtual ESC Congress. “We are in the happy situation that most of our patients with congenital heart disease now survive into adulthood, but most of them are not cured and require lifelong follow-up in order to recognize any complications timely and deal with them adequately.”
Various catheter-based interventions received recommendations from the task force, including a class I endorsement of transcatheter pulmonary valve replacement in the non-native outflow tract.
“This surprised some surgeons, but the reason we did this is not that we see evidence that transcatheter intervention is superior to surgery in this situation, but that we have to keep in mind that we operate on these young patients and this would not be their first surgery,” Baumgartner said during the live session. “When we do this procedure, we have to preprogram the next intervention, because this biological valve will not last forever. We are looking at quite a number of surgeries that are required over a lifetime and having the chance to do a transcatheter intervention reduces that number. These patients of course need to be carefully evaluated because we need to consider factors such as the size of the outflow tract and the coronary arteries. This has to be done in specialized hands.”
In patients with right ventricular outflow tract obstruction, “the indication for intervention is more restrictive when a valve substitute is required because of long-term risks such as endocarditis and long-term reintervention for prosthetic valve failure,” task force member Barbara J.M. Mulder, MD, PhD, professor of cardiology at Amsterdam University Medical Center, said during a presentation. “Catheter intervention is the treatment of choice for patients with nondysplastic valvular or pulmonary stenosis, for peripheral pulmonary stenosis and for pulmonary valve replacement in [Tetralogy of] Fallot.”
The task force recommended that patients with congenital heart disease and confirmed precapillary pulmonary hypertension should now be counseled against pregnancy, and that doctors need to have a risk assessment plan for this population, task force member Markus Schwerzmann, MD, clinician scientist and director of the Center for Adult Congenital Heart Disease at University Hospital Inselspital, University of Bern in Switzerland, said in a presentation.
Also new, he said, is that “in low- and intermediate-risk patients with repaired simple lesions and precapillary pulmonary hypertension, initial oral combination therapy or sequential oral combination therapy is recommended, while high-risk patients should be treated with initial combination therapy including parenteral prostanoids.”
A key message of the guideline, Julie De Backer, MD, PhD, associate professor of cardiology and medical genetics at Ghent University Hospital in Belgium, and co-chairperson of the task force, said during the live session, is that patients with adult congenital heart disease “are all different, and they require an individualized approach. There are no two patients who can be compared to each other. They need individualized management led by or in consultation with an expert center.”
The live session also featured presentations from Werner Budts, MD, PhD, adult congenital cardiologist at University Hospitals Leuven, Belgium, and professor of pediatric cardiology at Catholic University Leuven, on management of LV outflow tract obstructions and aortopathies; Folkert Meijboom, MD, PhD, professor of pediatric cardiology at University Medical Centre Utrecht, Netherlands, on transposition of the great arteries; and Erwin Oechslin, MD, director of the adult congenital heart disease program and Bitove Family Professor of Adult Congenital Heart Disease, University Health Network / Peter Munk Cardiac Centre, Toronto, and professor of medicine at the University of Toronto, on uncorrected univentricular hearts and Fontan circulation.
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Yuli Y. Kim, MD
The 2020 ESC Guidelines on the Management of Adult Congenital Heart Disease are a welcome update to the 2010 guidelines that not only build on prior recommendations for specific lesions but redefine the landscape and refine the approach to caring for this population. The guidelines follow the updated U.S. guidelines published in 2019 (Stout KK, et al. Circulation. 2019;doi:10.1161/CIR.0000000000000603) and provide a summary of the latest evidence.
One immediately notable difference is the replacement of the term “Grown-Up Congenital Heart Disease” in the title with “Adult Congenital Heart Disease” (ACHD), which is consistent with international nomenclature. Importantly, the authors emphasize framing the condition as one that is chronic and lifelong, beginning at birth. With improved life expectancy, comorbidities associated with aging and end-of-life care, including advance care planning, are highlighted.
Arrhythmias comprise a large component of the morbidity associated with ACHD and are a challenging component to the care of this population, requiring collaboration with electrophysiologists with expertise in congenital heart disease. The relationship between electrical issues and structural substrate is notable in some of the management strategies, reflecting high level of complexity in management. The authors provide risk estimates for various type of arrhythmias with specific lesions in table form and the accompanying text provides details on management which are invaluable for the general cardiologist or ACHD specialist to know.
Another challenging comorbidity associated with ACHD is pulmonary hypertension. A vast amount of research on this field emanates from Europe, so it is fitting that the recommendations include specifics that are not found in the U.S. guidelines and are distinct from the pulmonary hypertension guidelines (Galiè N, et al. Eur Heart J. 2015;doi:10.1093/eurheartj/ehv317). Reflecting the heterogeneity in etiology and physiology, the authors provide definitions of pulmonary hypertension subtypes in the context of congenital heart disease and provide recommendations for pulmonary hypertension-directed therapy and treatment in a variety of conditions including shunt lesions, single ventricle physiology and Eisenmenger syndrome.
Catheter-based interventions are prominent in this version of the guidelines, reflecting the advent of newer, less invasive options. Subheadings for invasive treatment strategies now read “surgical/catheter interventional treatment,” a departure from 2010. A notable difference in the recommendations compared to U.S. guidelines is the stronger language on transcatheter pulmonary valve implantation in non-native right ventricular outflow tracts (i.e., conduits) in which it “should be preferred” over surgery if anatomically feasible.
One notable absence in the guidelines is the prevalence of psychosocial distress, a known morbidity in ACHD that deserves mention and a section in its own right. Aside from this, 2020 ESC guidelines for ACHD are an excellent update with broad reflection on the ever-changing aspects of management of this growing complex population.
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Baumgartner H, et al. 2020 ESC guidelines on adult congenital heart disease. Presented at: European Society of Cardiology Congress; Aug. 29-Sept. 1, 2020 (virtual meeting).
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