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Table 3 Selected revised recommendations (R), new recommendations (N), and new concepts

Arrhythmia N There were no formal recommendations on arrhythmia in the 2010 edition  these have now been included  for details see recom-

mendation tables in Sections 3.4.2 and 4.10. Summary of the most important items:  Emphasizing the importance of understanding the cause, the mechanism of the arrhythmia, and the anatomy of the underlying CHD  Emphasizing the importance of a multidisciplinary approach for optimal arrhythmia treatment prior to, or concomitant with, percutaneous or surgical interventions  Consideration of early catheter ablation as an alternative to long-term medical treatment for symptomatic SVT and VT provided that the procedure is performed in experienced centres  Targeting VT-related anatomical isthmuses in repaired tetralogy of Fallot patients with sustained VTs before percutaneous or surgical reintervention in the RVOT, as these procedures may lead to inaccessibility of the VT substrates  Recognizing the association between bradycardia and IART and the potential benefit of PM implantation.

Eisenmenger sydrome /pulmonary arterial hypertension N It is recommended that patients with CHD and confirmed pre-capillary PH are counselled against pregnancy. N Risk assessment is recommended in all patients with PAH-CHD. N In low- and intermediate-risk patients with repaired simple lesions and pre-capillary PH, initial oral combination therapy or sequential combination therapy is recommended, and high-risk patients should be treated with initial combination therapy including parenteral

prostanoids.

R Emphasize the importance of sequential PAH therapy strategy

in Eisenmenger syndrome and use of 6MWT for the decision to initiate therapy. In Eisenmenger patients with reduced exercise capacity

(6MWT distance <450 m), a treatment strategy with initial endothelin receptor antagonist monotherapy should be considered followed by combination therapy if patients fail to improve.

Shunt lesions N In patients with shunt lesions and non-invasive signs of PAP elevation, invasive measurement of PVR is mandatory. N/R Adjusted recommendations for shunt closure (when Qp:Qs >1.5) according to calculated PVR:

<3 WU: class I for ASD, VSD, and PDA 35 WU: class IIa for ASD, VSD, and PDA >_5 WU but decreasing to <5 WU after targeted PAH treatment: class IIb for ASD (fenestrated closure only) >_5 WU for VSD and PDA (careful individual decision in expert centres); class IIb >_5 WU despite targeted PAH treatment: class III for ASD.

N In patients with ASD and LV disease, it is recommended to perform balloon testing and carefully weigh the benefit of eliminating LR

shunt against the potential negative impact of ASD closure on outcome due to increase in filling pressure (taking closure, fenestrated closure, and no closure into consideration).

N Take older age into account in the decision for surgical ASD

closure. In elderly patients not suitable for device closure, it is recom-

mended to weigh the surgical risk against the potential benefit of ASD closure.

R Transcatheter VSD closure has become an alternative to sur-

gery in selected patients, particularly in residual VSD. Transcatheter VSD closure has become an alternative, partic-

ularly in residual VSDs, VSDs poorly accessible by surgery, and in muscular VSDs centrally located in the interventricular septum.

R Specify the requirement for a congenital cardiac surgeon for

partial AVSD closure. Surgical closure is recommended in patients with significant

RV volume overload and should only be performed by a congenital cardiac surgeon.

R Specify the presence of atrial fibrillation or PH as require-

ments for valve repair in AVSD. In asymptomatic patients with severe left-sided AV valve

regurgitation, preserved LV function (LVESD <45 mm and/or LVEF >60%), high likelihood of successful valve repair, and low surgical risk, intervention should be considered when atrial fibrillation or systolic PAP >50 mmHg is present.

R Specify the option of fenestrated ASD closure. In patients with PVR >_5 WU, fenestrated ASD closure may be

considered when PVR falls below 5 WU after targeted PAH treatment and significant LR shunt is present (Qp:Qs >1.5).

R Include desaturation on exercise as a contraindication for

ASD, VSD, AVSD, and PDA closure. Shunt closure is not recommended in patients with severe

PAH (PVR >_5 WU) presenting with desaturation on exercise.

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