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............................................................................................................................................................................. 7.1.5 Cyanotic patients • Cyanotic patients present with a multisystem disorder and are at risk for both bleeding and thrombotic complications, causing a therapeutic dilemma. • Routine phlebotomies must be avoided as they put patients at risk for iron-deficient anaemia and cerebrovascular complications. Therapeutic phlebotomy is only indicated in the presence of moderate/severe hyperviscosity symptoms. • Cyanotic patients have a very balanced, but fragile, pathophysiology and any intervention puts the patient at high risk; all interventions must therefore be performed in an ACHD expert centre. • Prophylactic measures are the mainstay of care to prevent and avoid complications. 7.2 Specific lesions 7.2.1 Shunt lesions • Treatment decisions require careful evaluation of ventricular volume overload and pulmonary circulation. • In patients with non-invasive signs of elevated PAP, heart catheterization with assessment of PVR is mandatory. • In the presence of PVR >_5 WU, ASD closure should be avoided. VSD and PDA closure may only be considered in selected patients with significant shunt after careful evaluation in an ACHD and PH expert centre. • Device closure is the treatment of choice when technically feasible. 7.2.2 Left ventricular outflow tract obstruction • The strongest indications for surgery remain symptoms and LV dysfunction. • Exercise testing should be performed in patients with severe obstruction who do not report symptoms in order to confirm asymptomatic status. • In congenital valvular AS, associated aortic disease (ascending aortic dilatation and/or CoA) needs to be excluded. 7.2.3 Aortic coarctation • Correct blood pressure measurement (right arm, ambulatory) is essential in the follow-up of patients with CoA. • The decision to (re)intervene depends on blood pressure, gradient, and stenosis morphology. • Stenting is the treatment of choice when technically feasible. 7.2.4 Aortopathies • Lifelong surveillance is essential in all HTAD patients and should include imaging of the entire aorta, as well as assessment of valvular and myocardial function. • The aortic diameter at which surgery should be performed depends on the underlying disease and presence of risk factors. 7.2.5 Right ventricular outflow tract obstruction • RVOTO may be overestimated by the flow velocity across the obstruction, particularly when the narrowing is elongated, or stenosis is present in series (e.g. subvalvular and valvular). Therefore, cross-checking with RV pressure estimated from TR velocity is required. • Catheter intervention is the treatment of choice for patients with non-dysplastic valvular PS (balloon valvuloplasty) and with peripheral PS (often with stent implantation). • The indication for intervention is more restrictive whenever a valve substitute is required because of its long-term implications for complications and requirement for reintervention. 7.2.6 Ebstein anomaly • Timing of surgery remains challenging and this operation should only be performed by surgeons with specific experience in this lesion. • Valve repair is the preferred technique whenever feasible. 7.2.7 Tetralogy of Fallot • Significant PR and/or RVOTO, RV and LV dysfunction, and arrhythmias are common long-term complications. • Possible risk factors associated with any ventricular arrhythmia and SCD in rTOF are QRS duration >180 ms, LV systolic or diastolic dysfunction, RV dysfunction, inducible VT at programmed electrical stimulation, and history of atrial arrhythmia. • The optimal timing for intervention in asymptomatic severe PR remains challenging. Normalization of RV size becomes unlikely when the end diastolic volume index exceeds 160 mL/m2, but this cut-off for reintervention may not correlate with clinical benefit. • TPVI has become the treatment of choice for RVOT reintervention when anatomically feasible. 7.2.8 Transposition of the great arteries • Systemic ventricular failure, secondary systemic AV valve regurgitation, arrhythmia, and baffle stenosis and/or leakage are common long-term complications that need to be addressed after atrial switch operation. • Outcome of morbidity has markedly improved with introduction of the arterial switch operation. Dilatation of the neo-aortic root with or without significant regurgitation of the neo-aortic valve, supravalvular PS, and pulmonary branch stenosis mostly occur during infancy, but may need reintervention during adulthood. • New LV systolic dysfunction and/or arrhythmias after arterial switch require full evaluation, including exclusion of ostial/proximal stenoses of the reimplanted coronary arteries. • Failure of the RV-to-PA conduit (stenosis, regurgitation, or both) is the predominant long-term complication requiring reintervention after the Rastelli operation. 7.2.9 Congenitally corrected transposition of the great arteries • Systemic RV failure, systemic AV valve regurgitation, AV block, and atrial arrhythmia are common late complications. • Systemic AV valve regurgitation is an important driver of late outcome and, when severe, should be addressed before systemic RV function becomes impaired. 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