Heart failure (HF) is the principal cause of morbidity and mortality in adults with congenital heart disease (ACHD). Robust evidence-based treatment options are lacking. This study aims to evaluate the safety, tolerability, and short-term HF-related effects of sodium-glucose cotransporter 2 inhibitors (SGLT2i) in a real-world ACHD population. All patients with ACHD treated with SGLT2i in 4 European ACHD centers were included in this retrospective study. Data were collected from 1 year before starting SGLT2i to the most recent follow-up. Data on side effects, discontinuation, mortality, and hospitalizations were collected. In total, 174 patients with ACHD were treated with SGLT2i from April 2016 to July 2023. The mean age was 48.7 ± 15.3 years, 72 (41.4%) were female, and 29 (16.7%) had type 2 diabetes mellitus. Ten (5.7%) patients had mild, 75 (43.1%) moderate, and 89 (51.1%) severe congenital heart disease. HF was the most frequent starting indication (n = 162, 93.1%), followed by type 2 diabetes (n = 11, 6.3%) and chronic kidney disease (n = 1, 0.6%). At median follow-up of 7.7 months (Q1-Q3: 3.9-13.2 months), 18 patients (10.3%) reported side effects, 12 (6.9%) permanently discontinued SGLT2i, and 4 (2.3%) died of SGLT2i-unrelated causes. A significant reduction in the HF hospitalization rate was observed from 6 months before to 6 months after starting SGLT2i (relative rate = 0.30; 95% CI: 0.14-0.62; P = 0.001). SGLT2i generally seem safe, well-tolerated, and potentially beneficial in patients with ACHD. SGLT2i was associated with a 3-fold reduction in the 6-month HF hospitalization rate. These results warrant prospective randomized investigation of the potential benefits of SGLT2i for patients with ACHD.

Background: Short-term improvements in quality of life (QOL) have been reported in adult congenital heart disease patients with systemic right ventricle (sRV) failure after treatment with sacubitril/valsartan. This study aimed to evaluate the medium-term QOL changes in sRV failure patients treated with sacubitril/valsartan. Methods: In this single-centre, prospective cohort study, patients with symptomatic sRV failure completed the Netherlands Organisation for Applied Scientific Research/Academic Hospital Leiden Questionnaire for Adult’s Health-Related Quality of Life (TAAQOL) at baseline and after starting treatment with sacubitril/valsartan. The TAAQOL was taken at structured outpatient follow-up moments after 6, 12, 24 and 36 months of treatment. Linear mixed effects models were used to evaluate the medium-term changes in 12 QOL domains. Results: Of 40 sRV failure patients initiated on sacubitril/valsartan, 35 completed the titration phase, and 31 filled in a total of 98 TAAQOL questionnaires (response rate 77.5%). Significant improvements in gross motoric functioning (p=0.008), cognitive function (p=0.002), sleep (p=0.041), social functioning (p<0.001) and daily activities (p=0.001) were observed during follow-up. No significant changes were observed in fine motoric functioning, pain, sexuality, vitality, positive, depressive or aggressive emotions. Of interest, periods with restrictions relating to the COVID-19 pandemic did not significantly influence changes over time in any of the 12 QOL domains. Conclusions: Sacubitril/valsartan treatment was associated with persistent medium-term QOL improvements in gross motoric functioning, cognitive function, sleep, social functioning and daily activities domains in sRV failure patients. Self-perceived QOL of sRV failure patients may be amenable to improvement with sacubitril/valsartan.

OBJECTIVES: Patients with complex congenital heart disease carry an increased risk of damage to retrosternal structures each time they undergo redo sternotomy. The aim of this study was to evaluate the safety and efficacy of neck cannulation for peripheral cardiopulmonary bypass to alleviate the risks in high-risk redo sternotomy patients.METHODS: Children and adults with congenital heart disease undergoing high-risk redo sternotomy were included in this retrospective study. The primary outcome was the safety and efficacy of neck cannulation for cardiopulmonary bypass. The secondary outcome was to assess preoperative risk factors as an indication for neck cannulation. The right common carotid artery and right internal jugular vein were cannulated and full cardiopulmonary bypass was initiated with vacuum-assisted venous drainage. Redo sternotomy was performed on a decompressed heart, and bifrontal regional cerebral oxygen saturation was monitored via near-infrared spectroscopy.RESULTS: In total, 35 patients were included. No mortality, neurological or vascular complications occurred postoperatively. Mean left- and right-sided near-infrared spectroscopy were 70.0% (±10.5) and 64.2% (±12.0), respectively, and the mean difference was 5.7% (±6.9). Main preoperative risk factors were; adherent ascending aorta (45.7%), adherent conduit (40%), severely dilated retrosternal right ventricle (17.1%) and skeletal deformations (14.3%).CONCLUSIONS: Cannulation of the right neck vessels for peripheral cardiopulmonary bypass prior to high-risk redo sternotomy in children and adults with congenital heart disease is a safe and effective strategy. In combination with near-infrared spectroscopy monitoring, adequate cerebral oxygenation can be ensured while the risk of catastrophic haemorrhage is minimized.

Single ventricle patients are at high risk of developing circulatory failure. There is limited evidence for pharmacological treatment. This study assessed the echocardiographic changes in ventricular function during sodium-glucose cotransporter 2 inhibitor (SGLT2i) therapy in patients with single ventricle failure (SVF). SVF patients with a baseline transthoracic echocardiogram within six months before starting SGLT2i and at least one echocardiographic examination within twelve months follow-up were included from a real-world international registry of adult congenital heart disease patients on SGLT2i. Mixed models were used to evaluate longitudinal changes in ventricular function and differences between patients with SVF with ≥ moderately reduced systolic function (SVFrEF) and with ≤ mildly reduced function (SVFpEF). Thirteen patients were included. The median age was 21 [20–42] years, 8 (61.5 %) were female, 10 (76.9 %) had a Fontan circulation, 8 (61.5 %) had SVFrEF, and 5 (38.5 %) SVFpEF at the start of SGLT2i. The mean follow-up was 7.6 ± 3.3 months. End-systolic area decreased significantly in all patients (−1.6 cm² per month, p = 0.007) in the first 100 days. Fractional area change improved in the first 100 days in SVFrEF patients (3.5 %-point per month, p < 0.001), while SVFpEF patients remained stable. There was a significant improvement in the free wall strain in all patients (−0.3 %-point per month, p = 0.036) but not in global longitudinal strain (p = 0.087). Isovolumic acceleration also improved in the first 100 days (0.5 m/s² per month, p = 0.010). Echocardiographic signals of improved ventricular function were observed in the first year of SGLT2i therapy in patients with SVF.