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............................................................................................................................................................................. inconclusive non-invasive assessment or suspected PAH, cardiac catheterization is indicated.

4.12.3.3 Surgical/catheter interventional treatment Indications for intervention are summarized in the Recommendations for interventions in TGA after arterial switch operation table.

RVOTO can be subvalvular, valvular (both rare), or supravalvular (most common). Indications for treatment are similar to those described in section 4.8, but different anatomies may require different approaches.

4.12.4 Rastelli-type operation 4.12.4.1 Clinical presentation after Rastelli-type repair Adults with TGA, VSD, and PS (complex transposition) have often had a Rastelli-type repair. The VSD patch directs blood from the LV to the aorta and the RV is connected to the PA with a valved conduit. Variants of the Rastelli technique, sharing the same principle, are the re ́paration a l’etage ventriculaire and Nikaidoh techniques.

Common complications are: • Stenosis or regurgitation of the valved conduit between the RV and the PA. • LVOTO, i.e. obstruction of the flow from the LV to the aorta. • Residual VSD. • AR. • LV dysfunction. • Arrhythmias, both ventricular and supraventricular. • Endocarditis of the valved conduit. • Death, either sudden (arrhythmia) or due to heart failure. The few long-term outcome studies report 20-year survival <60% and 20-year event-free survival 2030%. Replacement of the conduit between the RV and PA is the most common indication for reoperation. Relief of LVOTO is the second most common, followed by closure of a residual VSD.288 Endocarditis of the valved conduit is relatively common.

Exercise capacity is mildly to substantially diminished. Repeat reintervention, surgical or percutaneous, is the fate of most patients with a Rastelli-type repair.

4.12.4.2 Diagnostic work-up See section 3.3 for general principles. Clinical findings may suggest conduit stenosis, residual VSD, TR, mitral regurgitation, or AR. • Echocardiography: the connection between the posteriorly positioned LV and the anteriorly positioned (due to the TGA) aortic valve, aortic valve function, and aortic root diameters should be assessed. The anatomy and function of the conduit between the RV and the pulmonary trunk must be visualized and assessed with Doppler interrogation. RV pressure assessed with Doppler measurement of TR jet velocity is of particular importance because the Doppler technique often overestimates the pressure gradient across the RVPA conduit. • CMR provides a more robust quantification of LV and RV volumes, aortic diameters, and EF. The RVPA conduit, often difficult to visualize by echocardiogram, and peripheral PAs can be readily seen and measured with CMR. In the presence of a residual VSD, Qp:Qs can be calculated. • Cardiac catheterization may be required for haemodynamic assessment of conduit stenosis. Angiography can be helpful for assessing the level of stenosis and peripheral PA stenosis.

4.12.4.3 Surgical/catheter interventional treatment For indications for treatment of conduit stenosis, see section 4.14. If LR shunting through a residual VSD causes symptoms or substantial LV volume overload, surgical/catheter treatment should be performed (IC recommendation).

4.12.5 Follow-up recommendations (irrespective of type of repair) All patients with TGA, regardless of the type of operation, should be seen at least annually in a specialized ACHD centre, with attention given to the specific issues previously described (see sections 4.12.2.1, 4.12.3.1 and 4.12.4.1).

4.12.6 Additional considerations (irrespective of type of repair)

• Exercise/pregnancy/IE prophylaxis: see sections 3.4.6, 3.5.5 and 3.5.7.

4.13 Congenitally corrected

transposition of the great arteries 4.13.1 Introduction and background ccTGA, or discordant atrio-ventricular and ventriculo-arterial connections, is uncommon. The ventricles are inverted, with the aorta arising anteriorly from the RV (usually on the left side) and the PA arising posteriorly from the LV (usually on the right side). The abnormal connections in ‘double’ discordance may be present in hearts

Recommendations for intervention in transposition of the great arteries after arterial switch operation

Recommendations Classa Levelb

Stenting or surgery (depending on substrate) is recommended for coronary artery stenosis causing ischaemia. I C Neo-aortic root surgery should be considered when the neo-aortic root is >55 mm, providing average adult stature (for neo-aortic valve replacement for severe neo-aortic AR see valvular heart disease guidelines with special considerationsc). IIa C Stenting should be considered for PA branch stenosis, regardless of symptoms, if >50% diameter narrowing and RVSP >50 mmHg and/or related reduced lung perfusion are present. IIa C

AR = aortic regurgitation; ESC = European Society of Cardiology; PA = pulmonary artery; RVSP = right ventricular systolic pressure. aClass of recommendation. bLevel of evidence. cWhen applying the 2017 ESC/EACTS valvular heart disease Guidelines25 for the decision to operate, it has to be taken into account that this is a reoperation and technically more difficult. Downloaded from academic.oup.com/eurheartj/article/42/6/563/5898606 by guest on 07 October 2026