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.............................................. ... 7.2.10 Univentricular heart and Fontan operation • Although quality of life is well preserved in many Fontan patients, all require regular intensive follow-up at an ACHD expert centre as they are at risk of developing multiple severe complications including arrhythmia, heart failure, hepatic disease, and proteinlosing enteropathy. • Low pulmonary artery pressure is mandatory for a good functioning Fontan circulation and a low threshold for invasive haemodynamic assessment is recommended when dysfunction is suspected, or complications occur. • Arrhythmias are poorly tolerated and require immediate action. • Pregnancy is feasible in selected patients with well-functioning Fontan circulation but there is a high risk of miscarriage and pregnancy should be managed in an ACHD expert centre. • Surveillance for liver problems is mandatory in all Fontan patients. 7.2.11 Coronary anomalies • CCT is the preferred technique for the evaluation of high-risk anatomy including features such as an intramural course and orifice anomalies (slit-like orifice, acute-angle take-off, orifice >1 cm above the sinotubular junction). • Assessment of stress-induced ischaemia, by means of advanced imaging modalities with physical stress, is the key for decision making. • In patients with coronary fistulae, the presence of symptoms, complications, and a significant shunt are the main indications for percutaneous or surgical closure. 8 ‘What to do’ and ‘what not to do’ messages from the Guidelines Recommendations Classa Levelb Treatment of arrhythmias in adult congenital heart disease In patients with moderate and severe CHD complexity (Table 4) and documented arrhythmias, referral to a centre with a multidisciplinary team and expertise in ACHD patients and ACHD-related arrhythmia is indicated. I C In CHD patients with documented arrhythmias or at high risk for post-procedural arrhythmias (e.g. ASD closure at older age) considered for percutaneous or surgical (re)interventions, referral to a centre with a multidisciplinary team with expertise in these interventions and in invasive treatment of arrhythmias is indicated. I C In mild CHD, catheter ablation is recommended over long-term medical therapy for symptomatic, sustained recurrent SVT (AVNRT, AVRT, AT, and IART), or if SVT is potentially related to SCD (Table 7). I C Catheter ablation is indicated as adjunctive therapy to ICDs in patients who present with recurrent monomorphic VT, incessant VT, or electrical storm not manageable by medical therapy or ICD reprogramming. I C ICD implantation is indicated in adults with CHD who are survivors of an aborted cardiac arrest due to VF or haemodynamically untolerated VT after evaluation to define the cause of the event and exclusion of reversible causes. I C ICD implantation is indicated in adults with CHD and sustained VT after haemodynamic evaluation and repair when indicated. EP evaluation is required to identify patients in whom catheter ablation or surgical ablation may be beneficial as adjunctive treatment or in whom it may offer a reasonable alternative. I C Treatment of pulmonary arterial hypertension associated with congenital heart disease It is recommended that patients with CHD and confirmed pre-capillary PH are counselled against pregnancy. I C Risk assessment is recommended in all patients with PAH-CHD. I C In low- and intermediate-risk patients with repaired simple lesions and pre-capillary PH, initial oral combination therapy or sequential combination therapy is recommended and high-risk patients should be treated with initial combination therapy including parenteral prostanoids. I A Atrial septal defect (native and residual) In patients with evidence of RV volume overload and no PAH (no non-invasive signs of PAP elevation or invasive confirmation of PVR <3 WU in case of such signs) or LV disease, ASD closure is recommended regardless of symptoms. I B Device closure is recommended as the method of choice for secundum ASD closure when technically suitable. I C In elderly patients not suitable for device closure, it is recommended to carefully weigh the surgical risk against the potential benefit of ASD closure. I C In patients with non-invasive signs of PAP elevation, invasive measurement of PVR is mandatory. I C In patients with LV disease, it is recommended to perform balloon testing and carefully weigh the benefit of eliminating LR shunt against the potential negative impact of ASD closure on outcome due to an increase in filling pressure (taking closure, fenestrated closure, and no closure into consideration). I C ASD closure is not recommended in patients with Eisenmenger physiology, patients with PAH and PVR >_5 WU despite targeted PAH treatment, or desaturation on exercise. III C Continued Downloaded from academic.oup.com/eurheartj/article/42/6/563/5898606 by guest on 07 October 2026