p.68 抽出テキスト

............................................................................................................................................................................. 6.2.5 Ebstein anomaly

• The identification of asymptomatic Ebstein patients with severe TR who would benefit from TV surgery requires further improvement. • The identification of Ebstein patients at risk for late lifethreatening arrhythmias needs to be improved.

6.2.6 Tetralogy of Fallot

• Optimal timing of PVRep for asymptomatic patients with significant PR needs to be further improved. • Long-term follow-up studies after TPVI are required to increase knowledge about valve durability, consequences of stent fractures, and occurrence of endocarditis. • The identification of rTOF patients at risk for late life-threatening arrhythmias, who would benefit from ICD implantation as primary prevention, needs to be improved. • The effect of medical treatment on RV dilatation and/or dysfunction in rTOF patients needs to be established.

6.2.7 Transposition of the great arteries

• The potential benefit of classical heart failure medical therapy and biventricular pacing in patients with a systemic RV after an atrial switch procedure requires further study. • Risk stratification for SCD and indications for primary ICD implantation after atrial switch require refinement. • After an arterial switch procedure, the risk of dissection/rupture of neo-aortic root aneurysms requires further study to refine the recommendations for prophylactic surgery. • Long-term follow-up after arterial switch is required to study the risk for development of CAD after reimplantation of the coronary arteries into the neo-aortic root.

6.2.8 Congenitally corrected transposition of the great arteries

• The definition of optimal timing for TV replacement in patients with severe asymptomatic TR requires more data. • The potential benefit of pulmonary banding for the preservation of systemic ventricular function requires further study.

6.2.9 Univentricular heart and Fontan operation

• The role of medication, including pulmonary vasodilators, in Fontan patients is not clear and requires further study. • The effects of pregnancy on long-term maternal outcome must be better established. • Physiological determinants of long-term outcome in Fontan patients, including the role of the lymphatic system, requires more research.

6.2.10 Coronary anomalies

• Identification of adult patients with coronary anomalies (AAOCA, ACAPA) who are at risk for SCD, and for whom surgery provides benefit at adult age, requires further research. 7 Key messages

7.1 General aspects

7.1.1 Organization of care and patient evaluation

• Special structural and organizational healthcare requirements are necessary to meet the needs of ACHD patients. • Multimodality imaging is key for adequate assessment of overall anatomy and ventricular and valvular function, and quantification of blood flow, including perfusion distribution. • Objective exercise testing is an important tool for determining the timing of interventions and reinterventions. • Cardiac catheterization remains key for the assessment of haemodynamics, in particular, PAP and vascular resistance.

7.1.2 Heart failure

• The key treatment for heart failure in ACHD patients remains its prevention by optimizing haemodynamics and heart rhythm. This requires systematic follow-up in specialized centres to facilitate timely intervention. • In a biventricular circulation, standard heart failure treatment can be extrapolated to ACHD patients with a systemic LV and may be applied in patients with a systemic RV, although it remains uncertain whether the known benefits of treating a failing LV can be expected. Pathophysiology of patients with an atrial switch, and especially with a UVH and Fontan palliation, differs markedly from a ‘regular circulation’ and standard heart failure therapy has to be applied cautiously. • Timely referral and consultation with ACHD and heart failure specialists in a centre with a transplant service and ACHD expertise is recommended, especially in those with moderate and severe complexity CHD.

7.1.3 Arrhythmia

• In all patients, evaluation for a reversible cause of an arrhythmia and for new or residual haemodynamic abnormalities should be performed. • Maintenance of sinus rhythm is the aim in most ACHD patients. • For optimal chronic arrhythmia management, referral to a centre with a multidisciplinary team and expertise in ACHD-related arrhythmias is mandatory. • Patients with documented arrhythmias or at high risk for postprocedural arrhythmias considered for percutaneous or surgical (re)interventions should be discussed in a multidisciplinary team with expertise in interventions and invasive treatment of arrhythmias.

7.1.4 Pulmonary arterial hypertension

• PAH in CHD is a progressive disease with poor prognosis. • High suspicion of PAH, and regular assessment for the presence of PAH in patients with shunt lesions, after defect closure is recommended. • Proactive treatment is required in all PAH patients, including those with Eisenmenger syndrome. • Women with CHD and confirmed pre-capillary PH should be counselled against pregnancy. Downloaded from academic.oup.com/eurheartj/article/42/6/563/5898606 by guest on 07 October 2026