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15 Key messages

(1) Patients with HF are classified based on their LVEF. Those with a LVEF between 41% and 49% are defined as ‘mildly reduced LVEF’ (HFmrEF). (2) Measurement of NPs and echocardiography have key roles in the diagnosis of HF. (3) ACE-I or ARNI, beta-blockers, MRA, and SGLT2 inhibitors are recommended as cornerstone therapies for patients with HFrEF. (4) ICDs are recommended in selected patients with HFrEF of an ischaemic aetiology and should be considered in those with a nonischaemic aetiology. (5) CRT-P/D is recommended in those patients with HFrEF, in sinus rhythm, with a LBBB >_150 ms and should be considered in those with a LBBB >_130149 ms or non-LBBB >_150 ms. (6) Advanced HF strategies (heart transplantation/MCS) may be appropriate in selected patients. (7) ACE-I/ARNI, beta-blockers, and MRA may be considered in patients with HFmrEF. (8) The diagnosis of HFpEF requires objective evidence of cardiac structural, or functional abnormalities as well as elevated plasma NP concentrations consistent with the presence of LV diastolic dysfunction and raised LV filling pressures. A diastolic stress test is recommended when these markers are equivocal. (9) To date, no treatment has been shown to reduce mortality and morbidity in patients with HFpEF. (10) It is recommended that all patients with HF be enrolled in a multidisciplinary HF-MP. (11) Exercise is recommended for all patients who are able, to improve exercise capacity and QOL, and reduce HF hospitalization. (12) Patients with advanced HF refractory to medical/device therapy and who do not have absolute contraindications should be referred for consideration of heart transplantation. MCS should also be considered as BTT or DT in selected patients. (13) Four major clinical presentations of acute HF may occur: ADHF, acute pulmonary oedema, RV failure, and cardiogenic shock. (14) Treatment of acute HF is based on diuretics for congestion, inotropes, and short- term MCS for peripheral hypoperfusion. (15) Patients hospitalized for HF should be carefully evaluated to exclude persistent signs of congestion. Oral treatment should be optimized before discharge. (16) In addition to oral anticoagulation, a strategy of rhythm control including catheter ablation should be considered in patients whose symptoms and/or cardiac dysfunction are associated with AF. (17) SAVR or TAVI, as advised by the Heart Team, are recommended in patients with symptomatic severe aortic valve stenosis. (18) Patients with isolated significant SMR and COAPT criteria should be considered for percutaneous edge-to-edge repair, whereas those with SMR and CAD, who need revascularization, should be considered for surgery. (19) It is recommended that patients with type II diabetes are treated with SGLT2 inhibitors. (20) Patients should be periodically screened for anaemia and iron deficiency and i.v. iron supplementation with ferric carboxymaltose should be considered in symptomatic patients with LVEF <45% and iron deficiency, and in patients recently hospitalized for HF and with LVEF <_50% and iron deficiency.

Table 36 Treatment of adult congenital heart disease and heart failure in specialized centres

ACHD patients with chronic HF should be referred to specialized centres. Specific guidelines for medical treatment of chronic HF in ACHD are lacking, and practitioners should follow the current guidelines for medical treatment of HF. It remains unknown whether the long-term use of neurohormonal modulators affects clinical outcomes and prognosis in ACHD. Sacubitril/valsartan may decrease morbidity,991993 however, no recommendation can be made at this moment based on the retrospective or anecdotical nature of these observations. Co-morbidities in HF such as diabetes mellitus, AF, CSA, iron deficiency, and cachexia should be treated according to specific recommendations reported in this document. In a biventricular circulation, patients with an impaired systemic LV should be treated with conventional HF therapy; this may also be considered in symptomatic patients with a failing systemic right ventricle. Diuretics are recommended to control symptoms of fluid retention. Treatment of symptomatic patients with a failing single ventricle in a Fontan circulation, or in the case of a persistent right-to-left shunt, should always be carefully initiated, taking the labile balance of ventricular preload and systemic afterload into account. CRT may be a therapeutic option in ACHD patients with HF, but evidence in this specific setting is still lacking. Efficacy of CRT will depend on the underlying structural and functional substrate, such as anatomy of the systemic ventricle (left, right, or functionally single), presence and degree of structural systemic AV valve regurgitation, primary myocardial disease or scarring, and type of electrical conduction delay.988 Treatment of acute HF in ACHD patients should be in an expert centre, with proper knowledge of inotropes, the availability of extracorporeal membrane oxygenation, and advanced bridging techniques.988,994 Timely evaluation for transplantation by ACHD HF specialists in a transplant centre with ACHD expertise is recommended. Ventricular assist devices can bridge patients to transplantation, or in a subgroup of patients, may be an option as destination therapy. ACHD = adult congenital heart disease; AF = atrial fibrillation; AV = atrioventricular; CRT = cardiac resynchronization therapy; CSA = central sleep apnoea; HF = heart failure; LV = left ventricular. ESC 2021 Downloaded from academic.oup.com/eurheartj/article/42/36/3599/6358045 by guest on 07 October 2026