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4.16.6 Follow-up recommendations As a result of these many complex issues, the care of Fontan patients is one of the major challenges for ACHD practitioners. All Fontan patients should be followed in specialized ACHD centres, usually at least annually, with echocardiography, ECG, blood tests, and exercise testing. Intervals for CMR and hepatic ultrasound (or computed tomography) must be decided on an individual basis. For adults, it appears reasonable to perform a baseline hepatic assessment with magnetic resonance imaging at the first visit to guide the frequency and mode of follow-up based on the degree of pre-existing hepatic changes. In addition, yearly follow-up hepatic assessments including, for example, liver ultrasound and alpha-fetoprotein measurement, should be considered after consultation with local hepatology services. Comprehensive assessment is mandatory for patients with manifestations of the ‘failing Fontan’ complex, with particular care to exclude even minor obstructions to cavopulmonary flow and pulmonary venous return, which may have a major haemodynamic impact.

4.16.7 Additional considerations

• Exercise/sports: after Fontan operation, patients have significantly reduced exercise capacity as part of their circulation. However, moderate symptom-limited aerobic exercise is to be recommended according to current recommendations to improve musculatory strength and quality of life.24 • Pregnancy: patients with a Fontan circulation and any complication should be advised against pregnancy. Successful pregnancy is possible in selected patients, although with significant maternal morbidity, especially heart failure and arrhythmia, but also thromboembolic complications. Therapeutic anticoagulants should be considered, balanced against the risk of bleeding, which is also higher in these patients. Intensive monitoring, including after delivery, is mandatory. There is a high miscarriage rate of 2755% and a high rate of prematurity and intrauterine growth restriction. Whether pregnancy with its volume loading has an adverse effect on the long-term outcome of women with a single ventricle remains to be elucidated. • IE prophylaxis: only recommended in patients with a recent redo Fontan (<6 months), cyanosis, a prosthetic valve, residual patch leak, or prior endocarditis.

4.17 Coronary anomalies

4.17.1 Introduction and background Coronary anomalies include anomalous aortic origin of a coronary artery (AAOCA), anomalous coronary artery from the PA (ACAPA), and coronary fistulae.

4.17.1.1 Anomalous coronary artery from the pulmonary artery Although many congenital coronary anomalies are benign, natural history studies of anomalous left coronary artery from the PA (ALCAPA) document poor outcome if untreated.318 ACAPA results in low oxygen levels in the coronary artery, coronary steal syndrome, and myocardial ischaemia. ALCAPA can present as a silent or symptomatic myocardial infarction, LV dysfunction, VTs, or even SCD. Patients may also primarily present with volume overload due to LR shunt causing heart failure symptoms. However, anomalous right coronary artery from the PA (ARCAPA) has frequently been diagnosed incidentally. Dual coronary system repair, including coronary button transfer with or without an interposition graft, is preferred. A coronary artery bypass graft (CABG) with closure of the ACAPA should be reserved for those in whom coronary transfer is not feasible.

4.17.1.2 Anomalous aortic origin of a coronary artery Natural history studies are lacking regarding untreated patients with AAOCA. The debate about management is ongoing, in particular in patients with an interarterial course of an anomalous coronary artery. Risk assessment for SCD is difficult because of the lack of data. Autopsy series show that most patients are young (<35 years) and die during, or shortly after, exercise. Myocardial fibrosis has been demonstrated, suggesting myocardial ischaemia may play a role. Left coronary artery arising from the opposite (right) sinus is less

Special considerations and recommendations for intervention after Fontan operation

Recommendations Classa Levelb

Sustained atrial arrhythmia with rapid AV conduction is a medical emergency and should be promptly treated with electrical cardioversion. I C Anticoagulation is indicated in the presence, or with a history, of atrial thrombus, atrial arrhythmias, or thromboembolic events. I C It is recommended that women with a Fontan circulation and any complication are counselled against pregnancy. I C Cardiac catheterization is recommended at a low threshold in cases of unexplained oedema, exercise deterioration, new-onset arrhythmia, cyanosis, and haemoptysis. I C In patients with arrhythmias, a proactive approach of electrophysiologic evaluation and ablation (where appropriate) should be considered. IIa C Regular liver imaging (ultrasound, computed tomography, magnetic resonance) should be considered. IIa C Endothelin receptor antagonists and phosphodiesterase-5 inhibitors may be considered in selected patients with elevated pulmonary pressure/resistance in the absence of elevated ventricular end diastolic pressure. IIb C In selected patients with significant cyanosis, device closure of a fenestration may be considered but requires careful evaluation before intervention to exclude induction of systemic venous pressure increase or fall in cardiac output. IIb C

AV = atrioventricular. aClass of recommendation. bLevel of evidence. Downloaded from academic.oup.com/eurheartj/article/42/6/563/5898606 by guest on 07 October 2026