The ‘Ten Commandments’ in Adult Congenital Heart Disease Guidelines

Congenital heart disease (CHD) is a lifelong condition and appropriate follow-up in expert hands is a key to achieving a good long-term outcome by recognizing and addressing the specific and highly variable complications timely. All adult congenital heart disease (ACHD) patients should be seen once in a specialist centre, allowing ACHD specialists to determine the most appropriate level of care—exclusive care in a specialist centre, shared care between the centre and general adult cardiac services, or care in non-specialist cardiology clinics with access to specialized care when required—as well as adequate follow-up intervals for each individual patient. Echocardiography remains the first-line imaging modality but cardiovascular magnetic resonance imaging (and computed tomography) is frequently required during the diagnostic work-up particularly for quantification of right ventricular and single ventricle volumes, evaluation of the right ventricular outflow tract, quantification of pulmonary regurgitation, evaluation of pulmonary arteries, pulmonary veins, aorta, collaterals, arteriovenous malformations, intra- and extracardiac masses, myocardial perfusion and scare, and for quantification of flows. Cardiopulmonary exercise testing and measurement of neurohormone levels play an important role in the serial follow-up and the timing of intervention and re-intervention. Cardiac catheterization is mandatory in patients with signs of elevated pulmonary artery pressure to assess vascular resistance but is otherwise mainly reserved for resolution of specific anatomical and physiological questions. The threshold for catheterization should be low in Fontan patients when they develop any problems. There is an emerging role of catheter intervention in ACHD: device closure when anatomically suitable is the first choice for the closure of the atrial septal defect and patent ductus arteriosus. In ventricular septal defect, transcatheter closure is an alternative to surgery particularly in residual defects, defects poorly accessible by surgery, and muscular defects centrally located in the interventricular septum. Balloon valvuloplasty is the first choice in valvular pulmonary stenosis and stenting when technically feasible in coarctation of the aorta and stenosis of other vessels. Transcatheter pulmonary valve implantation is the preferred modality when re-intervention is required in patients with no native right ventricular outflow tract (homografts, bovine jugular vein grafts, and bioprostheses/conduits). Arrhythmias including sudden cardiac death are one of the most frequent complications in the entire spectrum of ACHD, requiring a multidisciplinary approach for optimal treatment considering early catheter ablation and implantable cardioverter defibrillator (ICD) or pacemaker implantation where appropriate. Heart failure is a major cause of death. Any haemodynamic abnormalities potentially causing heart failure that can be addressed by catheter intervention or surgery must be identified and, if possible, treated first before initiating conventional heart failure therapy. Pulmonary hypertension (PH) is an important prognostic factor in patients with CHD, requiring particular attention in pregnancy or prior to cardiac intervention (by surgery or catheter) or other major surgery. This includes the decision of whether to close shunt lesions and when to start targeted medical therapy. Meticulous haemodynamic evaluation with distinction between pre-capillary PH (mean pulmonary artery pressure >20 mmHg, wedge pressure ≤15 mmHg, and a PVR ≥3 WU), isolated post-capillary PH (mean pulmonary artery pressure >20 mmHg, wedge pressure >15 mmHg, and PVR <3 WU) and the combination of both. Pre-pregnancy counselling including genetic counselling is key in women with CHD. Although the majority of ACHD patients tolerate pregnancy well, those at higher risks need to be identified. Pregnancy should definitely be avoided in patients with pulmonary arterial hypertension (PAH), severe left ventricular dysfunction [<30% left ventricular ejection fraction (LVEF), New York Heart Association (NYHA) Class III–IV], systemic right ventricles with moderately or severely decreased function, Fontan patients with any complication, severe symptomatic aortic stenosis, severe mitral stenosis or (re)coarctation, and severe aortic dilation (with thresholds dependent on the underlying disease). Late complications and specific aspects requiring consideration vary largely across the wide spectrum of ACHD emphasizing the need for detailed knowledge and highly specialized care. Conflict of interest: H.B. received speaker fees and congress travel support form Edwards Life Sciences and Actelion.