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............................................................................................................................................................................. Thoracic Surgeons Congenital Heart Surgery Database as the first evidence-based score, designed specifically for surgery in ACHD.70

Upon evaluation, the score reached a good predictive power in ACHD, although the corresponding paediatric score performed better in children,71 indicating that in ACHD, individual patients’ comorbidities may play a more important role in determining outcomes. The ACHD mortality score, an expert-based score that accounts for comorbidities, may therefore also be considered for evaluating the risk of cardiac surgery in ACHD.72 Apart from the need for personalized risk assessment, understanding the specific anatomy and haemodynamics, experience with redo surgery, and special requirements in intensive care units are factors that determine short- and long-term outcomes. When ACHD patients are operated on by a congenital cardiac surgeon, outcome is proven to be superior.73 Therefore, the strict recommendation is that all ACHD patients are operated on by a congenital cardiac surgeon in a multidisciplinary environment with expertise in ACHD. This holds true for all ACHD cardiac surgery in the present recommendations except for surgery of uncomplicated bicuspid aortic valve (BAV), heritable thoracic aortic disease (HTAD) such as Marfan syndrome, and secundum-type ASD without anomalous pulmonary venous connection and/or absence of PVD. The multidisciplinary expert environment also favours the growing interest in, and need for, hybrid procedures, where (congenital) cardiac surgeons, vascular surgeons, interventional congenital cardiologists, and electrophysiologists collaborate in technically challenging procedures.

3.4.5 Catheter intervention Catheter-based interventions, either as stand-alone or hybrid procedures, are an appealing alternative to conventional open-heart surgery, obviating the need for redo sternotomy/thoracotomy and cardiopulmonary bypass. The most frequent percutaneous interventions are closure of shunt lesions (in particular secundum-type ASD, rarely VSD, and persistent arterial duct), fistula, or unusual collaterals; balloon dilatation of the pulmonary valve and valved grafts; balloon dilatation and/or stenting of narrowed great vessels [e.g. (re-coarctation of the aorta (CoA) and pulmonary arterial stenosis]; and transcatheter pulmonary valve implantation (TPVI). ACHD diagnosis and, in particular, interventional care should be delivered by people who are trained as CHD caregivers and are part of an ACHD care centre, where individual procedures are reviewed and discussed within a multidisciplinary team.74 It is of particular importance that a programme performing transcatheter interventions in ACHD patients should be co-located with appropriate congenital cardiac services for the management of procedural complications. In many programmes, experienced paediatric interventional cardiologists form an important component of ACHD intervention services, but in others (e.g. those based in a separate paediatric hospital), paediatric interventionalists may be uncomfortable with the interventional management of adults with congenital cardiac problems. Therefore, full collaboration between adult and paediatric cardiology is recommended. Concentration of ACHD catheterization in a limited number of designated centres allows quality assurance as well as co-location of key cardiovascular services.74 Minimum annual numbers of interventions per centre have been proposed in a recent recommendation paper.74 3.4.6 Infective endocarditis The risk of infective endocarditis (IE) in ACHD patients is higher than in the general population, with marked variation between lesions. The 2015 ESC Guidelines on IE maintain the restriction of antibiotic prophylaxis to patients at high risk of IE undergoing at-risk dental procedures.22 High-risk conditions are prosthetic valves, including transcatheter valves, valve repair using a prosthetic ring, previous IE, any cyanotic CHD, and any CHD repaired with prosthetic material up to 6 months after the procedure or lifelong if residual shunt or valvular regurgitation remains.

Non-specific hygiene measures should be applied in all ACHD patients: good oral and cutaneous hygiene, and aseptic measures during healthcare and any invasive procedure. Piercings and tattoos are discouraged and otherwise should be performed in optimal hygienic conditions. All patients need to be educated about symptoms of IE and appropriate behaviour (seeking medical advice, importance of blood cultures before starting antibiotic treatment) at occurrence of such symptoms. Recent studies confirmed the relatively high risk of IE in patients after valve surgery, particularly previous IE and bovine jugular vein conduits.7578 Specific awareness is needed after TPVI using a Melody valve prosthesis.75

3.4.7 Antithrombotic treatment Patients with ACHD are at increased risk of thromboembolic events, but evidence on prevention is limited. In patients with IART or AF, the CHA2DS2-VASc and HAS-BLED scores have proven beneficial in adult patients with acquired heart disease.38 However, as their validity in the CHD population is uncertain, the scores should only be used in combination with individually assessed risks. Traditionally, VKAs are used for thromboembolic prevention, but in general cardiology, NOACs are currently recommended in preference to VKA. Also, in the CHD population, NOACs seem similarly safe and effective, in the absence of mechanical valves or severe mitral valve stenosis.79,80 Anticoagulation therapy is recommended in paroxysmal and persistent AF/IART in patients with moderate or severe CHD, but an individualized approach remains necessary. In patients with mild CHD, the CHA2DS2-VASc and HAS-BLED scores38 should be used as per general recommendations.81 Whether all Fontan patients benefit from anticoagulation is unclear at the moment. Bleeding risk should also be considered and weighed against thrombogenic risk, especially in cyanotic patients. For secondary prevention, anticoagulation is recommended in patients with a thromboembolic event or incidental intracardiac or intravascular thrombus (see also 2017 ESC/ EACTS Guidelines on valvular heart disease and 2018 ESC Guidelines on pregnancy).

3.4.8 Management of cyanotic patients Cyanosis is caused by RL shunt due to an anatomical communication between the systemic and pulmonary circulation at the atrial, ventricular, or arterial level. Cyanotic heart disease comprises a heterogeneous group of lesions with different underlying anatomy and pathophysiology: normal or restricted pulmonary blood flow in the presence of an obstruction across the pulmonary outflow tract or increased pulmonary blood flow in the absence of such an obstruction which, in some defects, may result in development of PAH and Downloaded from academic.oup.com/eurheartj/article/42/6/563/5898606 by guest on 07 October 2026