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.............................................................................. The most challenging questions are TR, RV dysfunction, and when to implant a TV and/or an ICD. In contrast to the situation in the paediatric age group, in which double switch is an established treatment option in case of systemic RV failure, this approach is very rarely successful in adults.

Systemic AV valve (tricuspid) regurgitation is frequently the focus of surgical treatment. Repair is rarely feasible and, as a rule, valve replacement is the treatment of choice. Pre-operative systemic RVEF <_40%, PAP >50 mmHg, AF, and New York Heart Association (NYHA) class III to IV are associated with late mortality.291

4.13.6 Follow-up recommendations Patients with ccTGA need lifelong follow-up in a specialized ACHD centre at annual intervals, particularly because of conduction disturbances and subaortic ventricular and subaortic AV valve dysfunction. For arrhythmias, see section 3.4.2.

4.13.7 Additional considerations

• Exercise/sports: symptomatic patients with ccTGA and preserved RVEF should avoid high-intensity sports and preferably not do more than moderate-static and moderate-intensity sports. Patients with significant associated lesions and/or decreased subaortic RV function should be restricted to lowstatic and low-intensity sports. • Pregnancy: risk depends on functional status, ventricular function, systemic AV valve function, presence of arrhythmias (especially AV block), and associated lesions (see section 3.5.7).

4.14 Right ventricular to pulmonary

artery conduit 4.14.1 Introduction and background Conduits establish the continuity between the RV and the PA in complex defects when the native outflow tract is not amenable to reconstruction, including pulmonary atresia, common arterial trunk, TOF, absent pulmonary valve syndrome, Rastelli procedure, and Ross operation. Types of conduits include valved [pulmonary or aortic homograft, bioprosthetic valves, bovine jugular vein conduits (Contegra)] and non-valved conduits. There is no ideal conduit. Limited durability implicates early reoperation. Predictors for conduit failure are sterilization/preservation process, smaller conduit, conduit type, younger age at implantation, PA stenosis, and diagnosis of transposition.269,292,293 Freedom from reoperation for conduit failure at 20 years was reported at 32% and 40%.269,292 Complications include outgrowth, progressive obstruction with and without regurgitation, endocarditis, and aneurysms or pseudoaneurysms. Clinical presentation may include exertional dyspnoea, palpitations, syncope, and SCD.

4.14.2 Diagnostic work-up See section 3.3 for general principles. Clinical findings may include a precordial thrill, prominent A wave of the jugular veins, and systolic murmur. Conduit calcification may be seen on chest X-ray. • Echocardiography is the first-line diagnostic tool providing size, shape, and function of both ventricles, PR, TR, and associated lesions. Gradients across the conduit may be difficult to measure and unreliable. RV pressure derived from TR velocity should be used to assess conduit stenoses. • CMR is used to quantify conduit stenosis and/or regurgitation, RV volumes and mass, and to assess PAs. • CMR/CCT is helpful for coronary artery anatomy and proximity of the RV/conduit, and other structures to the retro sternum. • Catheterization with haemodynamic assessment is always required if intervention is considered. Angiography provides information on the level of stenosis, peripheral PA stenoses, and coronary anatomy (anomalies/abnormal course).

4.14.3 Surgical/catheter interventional treatment Balloon dilation/stent implantation have been reported to be safe and to prolong the lifespan of failing conduits.294,295 Percutaneous pulmonary valve implantation has now become the treatment of choice for dysfunctional valves, if technically feasible. Current exclusions for TPVI include occluded systemic veins, active infection, unsuitable outflow tract morphology, and unfavourable coronary anatomy (compression by the expanded implant). Surgery is preferred when additional interventions are considered (tricuspid annuloplasty). Longitudinal data are more important for timing of reintervention than single measurements.

Recommendations for intervention in right ventricular to pulmonary artery conduits

Recommendations Classa Levelb

Symptomatic patients with RVSP >60 mmHg (may be lower in case of reduced flow) and/or severe PRc should undergo intervention with preference for catheter intervention (TPVI) if anatomically feasible. I C Asymptomatic patients with severe RVOTO and/ or severe PR should be considered for intervention, preferably catheter intervention (TPVI) if anatomically feasible, when at least one of the

following criteria is present: • Decrease in objective exercise capacity

(CPET). • Progressive RV dilation to RVESVi >_80 mL/m2, and/or RVEDVi >_160 mL/m2, and/

or progression of TR to at least moderate. • Progressive RV systolic dysfunction. • RVSP >80 mmHg. IIa C CPET = cardiopulmonary exercise testing; CMR = cardiovascular magnetic resonance; PR = pulmonary regurgitation; RV = right ventricle/ventricular; RVEDVi = right ventricular end diastolic volume indexed; RVESVi = right ventricular end systolic volume indexed; RVOTO = right ventricular outflow tract obstruction; RVSP = right ventricular systolic pressure; TPVI = transcatheter pulmonary valve implantation; TR = tricuspid regurgitation. aClass of recommendation. bLevel of evidence. cRegurgitant fraction by CMR >3040%. Downloaded from academic.oup.com/eurheartj/article/42/6/563/5898606 by guest on 07 October 2026