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Left ventricular outflow tract obstruction and aortopathies R Increase the recommendation class from IIa to I in low-flow,

low-gradient AS for intervention. Intervention is indicated in patients with severe low-flow, low-

gradient (mean gradient <40 mmHg) AS with reduced EF and evidence of flow (contractile) reserve excluding pseudosevere AS.

R Lower mean Doppler gradient threshold for LVOTO inter-

vention from 50 to 40 mmHg. In symptomatic patients with valvular, subvalvular, or supra-

valvular AS and mean Doppler gradient >_40 mmHg surgery is recommended.

R Include BNP levels and increased PAP in the indication for

intervention in valvular AS. Intervention should be considered in asymptomatic patients

with normal EF and no exercise test abnormalities (see Section 4.5.1) if the surgical risk is low and one of the following findings is present:  Markedly elevated BNP levels (>3-fold age- and sex-corrected normal range) confirmed by repeated measurements without other explanation.  Severe PH (systolic PAP at rest >60 mmHg confirmed by invasive measurement) without other explanation.

R Confirm pressure gradients by invasive measurement and pre-

fer stenting in coarctation and re-coarctation when technically feasible. Repair of coarctation or re-coarctation (surgically or catheter

based) is indicated in hypertensive patients with an increased non-invasive gradient between upper and lower limbs confirmed by invasive measurement (peak-to-peak >_20 mmHg), with preference for catheter treatment (stenting) when technically feasible.

N Catheter treatment (stenting) of coarctation should be considered in normotensive patients with an increased non-invasive gradient con-

firmed with invasive measurement (peak-to-peak >_20 mmHg) when technically feasible.

N In aortopathies, aortic valve repair using the reimplantation or remodelling with aortic annuloplasty technique is recommended in young

patients with Marfan syndrome or related HTAD with aortic root dilation and tricuspid aortic valves, when performed by experienced surgeons.

N Surgery should be considered in patients with a TGFBR1 or TGFBR2 mutation (including LoeysDietz syndrome) who have aortic root dis-

ease with maximal aortic sinus diameter >_45 mm.

N In Turner syndrome, elective surgery for aneurysms of the aortic root and/or ascending aorta should be considered for women who are >16 years of age, have an ascending ASI >25 mm/m2, and have associated risk factors for aortic dissection. N In Turner syndrome, elective surgery for aneurysms of the aortic root and/or ascending aorta may be considered for women who are

>16 years of age, have an ascending aortic size index >25 mm/m2, and do not have associated risk factors for aortic dissection.

Right ventricular outflow tract obstruction / tetralogy of Fallot /Ebstein R Adjust recommendations for surgical intervention in RVOTO

according to symptoms. If surgical valve replacement is the only option, it is indicated

in patients with severe stenosis who are symptomatic. If surgical valve replacement is the only option in patients with severe stenosis who are asymptomatic, it is indicated in the presence of one or more of the following:  Objective decrease in exercise capacity  Decreasing RV function and/or progressive TR to at least moderate  RVSP >80 mmHg  RL shunting via an ASD or VSD.

R Include preference for catheter intervention for pulmonary

valve implantation in TOF. In patients with no native outflow tract, catheter intervention

(TPVI) should be preferred if anatomically feasible.

R Specify RV dilatation in the setting of pulmonary valve replace-

ment for TOF and for RVOT conduits. Pulmonary valve replacement should be considered in asymp-

tomatic patients with severe PR and/or RVOTO, in the presence of progressive RV dilation to RVESVi >_80 mL/m2, and/or RVEDVi >_160 mL/m2, and/or progression of TR to at least moderate.

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