New ESC Guidelines on Adult Congenital Heart Disease

Marlene Busko

September 24, 2020

The 2020 European Society for Cardiology (ESC) guidelines for the management of adult congenital heart disease (ACHD) provide new concepts and important revisions to the previous guidelines, issued a decade ago.

New evidence, "particularly on percutaneous interventional techniques and risk stratification with regard to timing of surgery and catheter intervention, as well as medical treatment," made a revision of the document necessary, the authors say.

The guidelines were published online August 29 in the European Heart Journal before being presented at ESC Congress 2020. They are endorsed by the Association for European Paediatric and Congenital Cardiology (AEPC) and the International Society for Adult Congenital Heart Disease (ISACHD).

Cochairs of the writing task force, Helmut Baumgartner, MD, and Julie De Backer, MD, PhD, as well as task force member Barbara J.M. Mulder, MD, discussed the guidelines in an ESC congress session they moderated, in which speakers presented updates for managing patients with five of the many types of lesions covered in-depth in the document.

At the close, Mulder, from Amsterdam University Medical Center, suggested that the comoderators each choose a key message from the guidelines.

Baumgartner, head of adult congenital and valvular heart disease at the University Hospital of Münster, Germany, said: "I would pick the increasing importance of catheter intervention, keeping in mind that this is something that requires careful evaluation, prior to the intervention, for the selection of the patients, hemodynamics, and careful planning."

"My key message would be that ACHD patients are all different," said De Backer, a cardiologist and clinical geneticist at Ghent University Hospital, Belgium. "There are no two patients who can be compared to each other, and they need a very individualized approach and management conducted or led by an expert center, or at least in consultation with an expert center."

Mulder said her key takeaway is "that the threshold for intervention is higher in patients who need a valve replacement and in patients who are asymptomatic — easy to remember, and a clue for all the guidelines for the various defects."

Need for Lifelong Care, Expert Medical Attention

The guidelines now use the term "adult" CHD instead of "grown-up" CHD, in accordance with international literature, and they now classify ACHD as mild, moderate, or severe disease, based on complexity.

"One of the key messages in this document still is — and we actually used this for our central figure in the printed version — that congenital heart disease is a lifelong disease," Baumgartner said.

Most ACHD patients "should be considered as having a lifelong disease, requiring individualized, structured, and appropriate management and follow-up," De Backer added in an email to theheart.org | Medscape Cardiology.

Importantly, more than 90% of children born with CHD will currently reach adulthood, but nearly none of those can be considered "cured."

The significant growth of the ACHD patient population over the past decade, she said, "has led to the general consensus that dedicated expert centers are required to guide management for these patients."

The document recommends that when a patient is transitioning from pediatric to adult cardiology, he or she should be seen in a specialized center at least once, Baumgartner emphasized, and "during this visit, it should be decided how the proper care should then be and how often the patients need to be seen in specialized centers, and how often they can be taken care of in a general cardiology setting."

The document spells out that an ACHD expert center requires 13 types of specialists, including at least two adult/pediatric cardiologists with ACHD certification, other cardiology/pulmonary experts, and a clinical geneticist, psychologist, social worker, and last, a palliative care team.

De Backer noted that the guidelines stress that all ACHD patients need to be seen by an expert in ACHD if they have heart failure (HF), arrhythmia, pulmonary arterial hypertension, cyanosis, or pregnancy.

Expert care is especially important in the setting of a systemic right ventricle or a Fontan circulation, where conventional HF treatment guidelines do not apply, she added.

In arrhythmia, underlying hemodynamic lesions and previous corrections need to be taken into account, and in supraventricular tachycardia, electrophysiological evaluation and ablation should be considered early to prevent lifelong medical treatment.

Pulmonary arterial hypertension related to CHD has a poor prognosis and requires proactive treatment, and cyanosis is a multisystemic disorder, and prevention of complications is very important.

With increased life expectancy, more women reach reproductive age, and "preconception counseling is recommended for women and men to discuss the risk of the defect in offspring and the option of fetal screening," she said.

With regard to sports, De Backer said, "all adults with congenital heart disease should be encouraged to exercise, taking into account the nature of the underlying defect and their own abilities."

Growing Role of Cardiac MRI, Catheter Interventions

Echocardiography remains the first-line imaging modality for diagnosis, said Baumgartner, but cardiovascular MRI and CT have gained importance.

"Catheter-based hemodynamic evaluation is very important in the setting of shunts (for evaluation of pulmonary vascular resistance) or aortic (re)coarctation (for calculation of the pressure gradient) or Fontan circulation (evaluation of pressures and residual lesions)," said De Baker.

There is a greater emerging role for catheter-based intervention, Baumgartner told theheart.org | Medscape Cardiology.

The guidelines recommend transcatheter closure of ventricular septal defects instead of surgery in selected patients, which is new, he said.

In addition, transcatheter pulmonary valve implantation is the preferred modality when re-intervention is required in patients with a no-native right ventricular outflow tract.

And balloon valvuloplasty is the first choice in valvular pulmonary stenosis and stenting, when it is technically feasible, in coarctation of the aorta and stenosis of other vessels.

He stressed, however, that "catheter-based treatment should be performed by specialists in ACHD working within a multidisciplinary team."

Recommendations for Arrhythmia

There were no formal recommendations on arrhythmia in the 2010 guidelines, but they are now included.

"When addressing arrhythmias in ACHD patients, we need to take into account the complexity of the underlying disease, as well as underlying hemodynamic issues that may be treated together, even prior to treating the arrhythmia. You always have to take the whole picture into account," said De Backer.

"Important to bear in mind is that patients with supraventricular tachyarrhythmias may benefit from earlier referral or earlier ablation treatment, as opposed to lifelong medical treatment or to reduce the duration of medical treatment," she noted.

The document now also provides more specific and adjusted recommendations for pulmonary arterial hypertension treatment.

"We have incorporated bicuspid aortic disease, Turner syndrome, and other heritable thoracic aortic diseases in the aortopathy section, and a new section on coronary artery anomalies had been included," said De Backer.

And, Mulder said, "we added a chapter on indications for aortic surgery — not only for patients with Marfan syndrome, but also for patients with other aortic diseases, like Loeys-Dietz and Turner syndrome and bicuspid aortic disease.

"We also added a nice chapter on coronary anomalies, with recommendations for optimal imaging and diagnosis, for risk assessment, and of course for indications for intervention," she added.

Long-term Outcomes

During the ESC congress session, task force members zeroed in on five types of treatment and procedures — pulmonary hypertension and shunt lesions; left ventricular outflow tract obstruction and aortopathies; right ventricular outflow tract obstruction and aortopathies; transposition of the great arteries, and uncorrected univentricular heart and Fontan — but the document covers many more specific lesion types in great detail.

It also has sections on diagnosis and treatment in general, and key messages, and gaps in evidence to guide future research. The information is also summarized in practical tables, including many with recommendations for interventions for ACHD patients with specific lesions.

"We do hope that these new guidelines will provide helpful guidance for the care of this challenging patient group and help to improve long-term outcomes," said Baumgartner.

The disclosure forms of all experts involved in the development of these guidelines are available on the ESC website.

Eur Heart J. Published online August 29, 2020. Full text

European Society of Cardiology (ESC) Congress 2020. Presented September 1, 2020.

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