Key result
GDMT in sRV patients linked to fewer adverse events and ~68m improvement in 6MWD.
Why the study?
Adults with congenital heart disease and a systemic right ventricle are at risk of heart failure, prompting evaluation of the effects of implementing guideline-directed medical therapy in this population.
Does guideline-directed medical therapy reduce adverse events and improve functional capacity in adults with congenital heart disease and a failing systemic right ventricle?
Does guideline-directed medical therapy reduce adverse events and improve functional capacity in adults with congenital heart disease and a failing systemic right ventricle?
Implementation of guideline-directed medical therapy, including ARNI and SGLT2 inhibitors, in adults with a failing systemic right ventricle is associated with reduced adverse events, improved exercise capacity, and better ventricular strain.
GDMT was associated with fewer events and better function in systemic right ventricle failure; leaves open need for randomized confirmation.
Introduction Adults with congenital heart disease(ACHD)with a systemic right ventricle(sRV) are at risk of developing heart failure(HF). Last guidelines have recommended a 4pillars-approach, including angiotensin receptor neprilysin inhibitor(ARNI)and sodium-glucose co-transporter2inhibitors(SGLT2i) together with betablockers(BB) and mineralcorticoid receptor antagonists(MRA) for HF with reduced ejection fraction. Aim of this study is to present our experience on the effects of guideline-directed medical therapy(GDMT)implementation in individuals with a failing sRV. Methods We retrospectively reviewed the medical records of all patients with a sRV attending our tertiary ACHD center from January2020 to January2025. A failing sRV was defined using a fractional area change cut-off≤35% measured by echocardiography. Any changes in pharmacological treatment were noted. Patients with univentricular physiology, SBP<90mmHg, and a GFR<25mL/min/1.73m2, as well as those on intermittent inotropic treatment were excluded. Data of any adverse events to the medication, including treatment discontinuation were collected. Baseline data including nt-proBNPvalues, 6-minute walking distance(6MWD) and sRV frewall global longitudinal strain(FWGLS)were compared to those collected at last follow-up. Results From the total population of 92patients with sRV followed at our ACHD unit, 70(76%) patients(mean age 40±12 years, 54%male) showed sRV dysfunction and were considered for GDMT:44(63%) patients with transposition of the great arteries(TGA)following atrial switch repair and 26(37%)with congenitally correctedTGA. Changes in pharmacological treatment are summarized in Figure1.ARNI was prescribed in 67(96%) patients. Forty-five(64%) patients were also started dapagliflozin 10mg daily following a mean of 17±8months from ARNI initiation, while 3patients initiated only SGLT2i and not ARNI. Mean FU was 2.9 ±0.9years. ARNI interruption was required in 5(7%) cases:2 patients stopped temporary the treatment during pregnancy, while 2 ceased ARNI due to hypotension and 1 to epigastrial discomfort. Dapagliflozin was stopped in 1 female patient due to recurrent urinary infections. During follow-up significant reduction in the incidence of adverse events including hospitalization for HF and sustained ventricular tachycardia/ICD therapies compared to three years preceding the treatment initiation(Figure 1). Moreover, GDMT was associated to increased 6MWD from 383.3±106 to 451±118 m;p<0.0001 and improved sRV FWGLS from-15.5 ±5.2 to-17.4±3.2% at last follow-up;p=0.003, despite stable nt-proBNP values 237[137-431] VS 249[139-427]pg/ml at latest follow-up, p=0.8. Conclusion Our experience in implementing GDMT for HF in patients with a sRV demonstrated that a4-pillars strategy is associated to a reduction of adverse events at3-year follow-up. Further prospective studies are required to ascertain whether those medications may also provide a positive impact on the outcome.
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Altobelli et al. (2025) studied this question. Guideline-directed medical therapy in systemic right ventricle patients reduced adverse events and improved 6MWD (by ~68m) and sRV FWGLS at 3-year follow-up.
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