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............................................................................................... 3.4.3 Pulmonary hypertension 3.4.3.1 Introduction and classification PH is an important prognostic factor in patients with CHD,42 requiring particular attention in pregnancy43 or prior to reparative cardiac or other major surgery. Until recently, PH was defined by an increase in invasively measured mean PAP >_25 mmHg at rest.44 While this has now been reduced to >20 mmHg,45 classification as pre-capillary PH  also called PAH  additionally requires an increase in PVR >_3 Wood units (WU) (Table 8).45 This increase in resistance in this group is due to an obstructive pulmonary vasculopathy which is driven by genetic background, modifier genes, vascular shear stress, and environmental triggers.44 It is important to separate PAH associated with CHD (PAH-CHD, in group 1 of the PH classification44) from conditions with an LV filling pressure >15 mmHg (post-capillary PH due to passive transmission of LV filling pressures in groups 2 and 5 of the PH classification),44 because of the futility of PAH-targeted treatments in post-capillary PH. Clinical subtypes of PAH-CHD are PAH with congenital systemic-pulmonary shunts, Eisenmenger syndrome, repaired defects, and PAH that appears coincidental with CHD, frequently with small defects. Fontan circulation is another condition with pulmonary vascular disease (PVD) and sometimes elevated PVR. An increase in PAP in these patients is, however, more frequently caused post-capillary (increase in ventricular filling pressure and/or AV valve regurgitation). In complex CHD, PAH may be restricted to certain segments of the pulmonary vascular bed (segmental PAH).46 This is most frequently encountered in complex pulmonary atresia with VSD.

Although PAH-CHD can occur in males and females of any age, the disease is more frequently encountered in females, and increases with biological age and age at defect closure.47 The sex bias disappears in PAH-CHD after defect repair.48 More recently, a nationwide CHD

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 Catheter ablation should be considered for symptomatic, monomorphic sustained VT in patients for whom medical therapy is not desired, provided that the procedure is performed in experienced centres. IIa C Implantable cardiac defibrillator 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 ICD implantation should be considered in adults with CHD with biventricular physiology and a systemic LV presenting with symptomatic heart failure (NYHA II/III) and EF <_35% despite >_ 3 months of optimal medical treatment, provided they are expected to survive substantially longer than one year with good functional status.c IIa C ICD implantation should be considered in patients with CHD and unexplained syncope and suspicion for arrhythmia aetiology and either advanced ventricular dysfunction or inducible VT/VF at programmed electrical stimulation. IIa C ICD implantation should be considered in selected TOF patients with multiple risk factors for SCD, including LV dysfunction, non-sustained, symptomatic VT, QRS duration >_180 ms, extensive RV scarring on CMR, or inducible VT at programmed electrical stimulation. IIa C ICD implantation may be considered in patients with advanced single or systemic RV dysfunction (EF systemic RV <35%) in the presence of additional risk factors.d IIb C Pacemaker PM implantation should be considered in ACHD patients with bradycardia-tachycardia syndrome to prevent IART, if ablation fails or is not possible. IIa C PM implantation should be considered for patients with severe CHD and sinus or IIa C

Continued junctional bradycardia (daytime heart rate <40

beats per minute or pauses >3 s). PM implantation should be considered for patients with CHD and compromised haemodynamics due to sinus bradycardia or loss of AV synchrony. IIa C PM implantation may be considered for patients with moderate CHD and sinus or junctional bradycardia (daytime heart rate <40 beats per minute or pauses >3 s). IIb C

ACHD = adult congenital heart disease; ASD = atrial septal defect; AT = atrial tachycardia; AV = atrioventricular; AVNRT = atrioventricular node reentrant tachycardia; AVRT = atrioventricular reentrant tachycardia; CHD = congenital heart disease; CMR = cardiovascular magnetic resonance; EF = ejection fraction; EP = electrophysiology/electrophysiological; IART = intraatrial reentrant tachycardia; ICD = implantable cardioverter defibrillator; LV = left ventricle/ventricular; NYHA = New York Heart Association; PM = pacemaker; RV = right ventricle/ventricular; SCD = sudden cardiac death; SVT = supraventricular tachycardia; TGA = transposition of the great arteries; TOF = tetralogy of Fallot; VF = ventricular fibrillation; VT = ventricular tachycardia. aClass of recommendation. bLevel of evidence. cConsidering the broad spectrum of ACHD with LV pathologies that may differ from acquired heart diseases, the potentially higher risk of ICD-related complications in ACHD, and the paucity of data on the benefit of ICDs for primary prevention of SCD in ACHD, a personalized approach seems appropriate. dData are sparse and risk factors may be lesion specific, including non-sustained VT, NYHA II/III, severe AV valve regurgitation, and wide QRS >_140 ms (TGA). Downloaded from academic.oup.com/eurheartj/article/42/6/563/5898606 by guest on 07 October 2026