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............................................................................................................................................................................. 6 Gaps in evidence

6.1 General aspects

6.1.1 Organization of care and patient evaluation

• Congenital heart defects are arbitrarily classified into lesions of different complexities (mild, moderate, severe; Table 4). The validity of this classification for clinical management and risk stratification remains to be investigated in large registries. • Minimal volumes of patients under care at each ACHD expert centre, and the necessary staff resources for an optimal outcome, need to be defined. • Relevant outcome measures, over and above mortality, need to be determined to measure quality of care. • The role of neurohormones to estimate disease severity and timing of interventions is not yet fully established and needs to be defined.

6.1.2 Heart failure

• The pathophysiology/mechanism of heart failure, especially in those with a systemic RV and Fontan circulation, is incompletely understood and needs further study to find better ways for prevention and treatment. • Indications for application of standard heart failure treatment in both acute and chronic settings need to be better defined. • The prediction and course of heart failure needs to be better defined to improve indications and timing of ventricular assist device/transplantation. • Large-scale collaborative international prospective registries on medical and device therapy in ACHD are needed and support for the set up and practical implementation is required.

6.1.3 Arrhythmia

• Dedicated scoring systems to assess the indication for anticoagulation in the setting of atrial arrhythmias are needed for moderate and complex CHD. • Targeting slow-conducting anatomical isthmuses by catheter ablation has been highly effective to control monomorphic VT in repaired tetralogy of Fallot. Whether catheter mapping can contribute to individualized risk stratification in patients without spontaneous VTs in repaired tetralogy of Fallot and related defects requires further research. • The potential loss of accessibility to slow-conducting anatomical isthmuses by catheter ablation after re-valving rTOF and related defects is of concern. Whether patients without documented VT benefit from preventive ablation before or during re-valving should be studied. • Indications for pacing and CRT in ACHD patients are mainly derived from adults with anatomically normal hearts with ischaemic or dilated cardiomyopathy and are not adapted to the diversity of structural and functional CHD substrates. The selection of CRT candidates, CRT application, and optimal pacing sites for the different CHD substrates requires further research.

6.1.4 Pulmonary arterial hypertension

• The impact of medical PAH therapy on survival of patients with Eisenmenger syndrome requires further study. • The role of upfront combination therapy in PAH-CHD requires further attention. • Limited experience is available for prostacyclin therapy in patients with PAH-CHD and needs further investigation.

6.1.5 Cyanotic patients

• The benefit of routine anticoagulation, in the absence of any strong risk factor for thromboembolic complications (e.g. atrial arrhythmias), is controversial and requires further study.

6.2 Specific lesions

6.2.1 Shunt lesions

• Late outcome after device closure requires further study, particularly of arrhythmias. • The impact of shunt closure on long-term outcome of patients with PAH remains an area of uncertainty; further research is required to better define the thresholds for treatment recommendations.

6.2.2 Left ventricular outflow tract obstruction and coarctation

• The optimal timing of intervention in asymptomatic severe LVOTO requires further study. • Transcatheter aortic valve implantation is rapidly evolving; its role in ACHD needs further refinement. • According to the 2018 ESC Guidelines for the management of arterial hypertension,190 definitions of hypertension in patients with repaired CoA are the same as in general arterial hypertension and patients should be managed according to the general guidelines: evidence for this strategy is lacking and prospective studies are required.

6.2.3 Aortopathies

• Estimation of the risk for aortic dissection and definition of the threshold for prophylactic surgery in HTAD based on diameter alone is suboptimal and calls for a more personalized approach. Whether the type of underlying gene defect is helpful in this stratification requires further study. • Current prophylactic treatment regimens in HTAD patients include beta blockers and ARBs. Either in monotherapy or combined, neither one prevents further growth of the aorta. The search for new and better treatment targets should continue.

6.2.4 Right ventricular outflow tract obstruction

• Criteria for concomitant TV repair at the time of RVOT surgery require refinement. • The identification of patients with low-gradient RVOTO who have severe stenosis and would benefit from intervention requires improvement. • The criteria for identification of RVOTO patients who would benefit from reintervention for residual PR requires further research. • The role of an EP study for risk stratification for SCD is controversial in patients with rTOF and needs further study. Downloaded from academic.oup.com/eurheartj/article/42/6/563/5898606 by guest on 07 October 2026