Effect of Sodium-Glucose Cotransporter 2 Inhibitors in Adults With Congenital Heart Disease.

BACKGROUND: 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. OBJECTIVES: 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. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.

The potential of sodium-glucose cotransporter 2 inhibitors for the treatment of systemic right ventricular failure in adults with congenital heart disease.

Aims: Given the compelling evidence on the effectiveness of sodium-glucose cotransporter 2 inhibitors (SGLT2i) in the conventional heart failure population, SGLT2i deserve exploration in systemic right ventricular (sRV) failure. The initial experience with dapagliflozin in sRV failure patients is described, with a focus on tolerability and short-term effects on clinical outcomes. Methods and results: Ten patients (70% female, median age 50 years [46.5-52]) with symptomatic sRV failure who received dapagliflozin 10 mg per day on top of optimal medical therapy between 04-2021 and 01-2023 were included. Within 4 weeks, no significant changes in blood pressure, electrolytes, or serum glucose occurred. Creatinine and estimated glomerular filtration rate (eGFR) showed a slight decline (88 ± 17 to 97 ± 23 µmol/L, p = 0.036, and 72 ± 14 vs. 66 ± 16 ml/min/1.73m2, p = 0.020, respectively). At 6 months follow-up (n = 8), median NT-proBNP decreased significantly from 736.6 [589.3-1193.3] to 531.6 [400.8-1018] ng/L (p = 0.012). Creatinine and eGFR recovered to baseline levels. There were no significant changes in echocardiographic systolic sRV or left ventricular function. New York Heart Association class improved significantly in 4 out of 8 patients (p = 0.046), who also showed an improvement in the 6-minute walk test or bicycle exercise test performance. One female patient developed an uncomplicated urinary tract infection. No patients discontinued treatment. Conclusion: Dapagliflozin was well-tolerated in this small cohort of sRV failure patients. While the early results on the reduction of NT-proBNP and clinical outcome parameters are encouraging, large-scale prospective studies are warranted to thoroughly evaluate the effects of SGLT2i in the growing sRV failure population.

Noninvasive detection of soft tissue sarcoma using volatile organic compounds in exhaled breath: a pilot study.

Aim: The aim of this pilot study was to assess whether an electronic nose can detect patients with soft tissue sarcoma (STS) based on volatile organic compound profiles in exhaled breath. Patients & methods: In this cross-sectional pilot study, patients with primary STS and healthy controls, matched on sex and age, were included for breath analysis. Machine learning techniques were used to develop the best-fitting model. Results: Fifty-nine breath samples were collected (29 STS and 30 control) from March 2018 to March 2022. The final model yielded a c-statistic of 0.85 with a sensitivity of 83% and specificity of 60%. Conclusion: This study suggests that exhaled volatile organic compound analysis could serve as a noninvasive diagnostic biomarker for the detection of STS with a good performance.

Diagnosing soft tissue sarcoma (STS) among the large number of benign soft tissue tumors is challenging. There is a serious need for a novel and easy tool that could accurately detect patients with STS. This study aimed to assess how well an easy-to-use electronic nose could differentiate between patients with STS and those without STS based on their exhaled breath. This is the first pilot study to reveal that an electronic nose could serve as a diagnostic tool for the detection of STS with a good performance. Future studies are needed to validate the findings in larger cohorts.

Contemporary Management Strategies of Baffle Leaks in Adults with a Failing Systemic Right Ventricle Late after Atrial Switch: A Case Series and Literature Overview.

Baffle leaks are a frequently encountered and often overlooked complication after the atrial switch procedure for transposition of the great arteries. Baffle leaks are present in up to 50% of non-selected patients, and while they initially may not cause clear symptoms, they can complicate the hemodynamic course and influence the prognosis in this complex patient group. A shunt from the pulmonary venous atrium (PVA) to the systemic venous atrium (SVA) can lead to pulmonary overflow and subpulmonary left ventricular (LV) volume overload, while a shunt from the SVA to the PVA can result in (exercise-associated) cyanosis and paradoxical embolism. We report three cases of baffle leaks in patients with systemic right ventricular (sRV) failure late after the atrial switch procedure. Two symptomatic patients who presented with exercise-associated cyanosis due to SVA to PVA shunting over the baffle leak underwent successful percutaneous baffle leak closure with a septal occluder device. One patient with overt sRV failure and signs of subpulmonary LV volume overload due to PVA to SVA shunting was managed conservatively, as baffle leak closure was expected to lead to an increase in sRV end-diastolic pressure and aggravation of sRV dysfunction. These three cases illustrate the considerations made, challenges faced, and necessity of a patient-tailored approach when addressing baffle leaks.

Cone reconstruction for Ebstein anomaly: Late biventricular function and possible remodeling.

OBJECTIVES: To evaluate late-term tricuspid valve competence and biventricular function following cone reconstruction for Ebstein anomaly, and to explore biventricular remodeling. METHODS: Consecutive adult and pediatric patients who underwent cone reconstruction from 2009 to 2019 were reviewed for inclusion in this retrospective cardiac magnetic resonance imaging study. Tricuspid valve competence was assessed with tricuspid regurgitation fraction. Biventricular systolic function was assessed by ejection fraction, cardiac index, indexed stroke volume, and indexed aortic and pulmonary artery beat volume. Biventricular remodeling was assessed by planimetered areas (right atrium, functional right ventricle, left heart), and indexed end-diastolic and end-systolic ventricular volumes. Paired t tests or Wilcoxon signed-rank tests were used for analyses. RESULTS: Of 58 included patients, 50 underwent cardiac magnetic resonance imaging. Twelve patients had both preoperative and late postoperative cardiac magnetic resonance imaging with a median follow-up of 5.11 years (interquartile range, 3.12-6.07 years). Focusing on these, tricuspid regurgitation fraction decreased (from 69% to 10%; P = .014), right ventricle ejection fraction remained stable, and antegrade pulmonary artery beat volume increased (from 26.7 to 41.6 mL/beat/m2; P = .037). The left ventricle stroke volume (from 30.4 to 44.1 mL/m2; P = .015) and antegrade aortic beat volume (from 28.5 to 41.1 mL/beat/m2; P = .014) also increased, and the left ventricle stroke volume improved progressively with time since surgery (P = .048). Whereas the right atrium area decreased (P = .004), the functional right ventricle and left heart area increased (cm2, P = .021 and P = .004). Right ventricle volumes showed a tendency to normalize and left ventricle indexed end-diastolic volume increased (from 50 to 69 mL/m2; P = .03) over time. CONCLUSIONS: Cone valve integrity was sustained. Biventricular function improved progressively during follow-up, and there are positive signs of biventricular remodeling late after cone reconstruction.

Neck cannulation for bypass in redo sternotomy in children and adults with congenital heart disease.

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.

Grown-up Congenital Heart Surgery in 1093 Consecutive Cases: A "Hidden" Burden of Early Outcome.

BACKGROUND: Surgery in grown-ups with congenital heart disease (GUCH) is characterized by complex anatomy, comorbidities, reoperations, and technical challenges. Although 30-day postoperative mortality is low, this measure might be insufficient to reflect adverse outcome monitoring. Our study aimed to establish whether prolonged intensive care unit (ICU) stay (≥7 days) and 6-month mortality were more clinically meaningful measures than 30-day mortality and to identify predictors of adverse outcome. METHODS: All consecutive GUCH patients from 1998 to 2015 were identified. Perioperative characteristics, diagnoses, and postoperative data were collected retrospectively. Predictors of 30-day and 6-month mortality and prolonged ICU stay were determined with logistic regression. Era effect was tested for quality assurances by dividing the cohort into 4 time intervals. RESULTS: Within 17 years, 1093 consecutive cardiac surgical procedures were identified in 1026 GUCH patients. During the study period, 30-day mortality improved significantly, with an overall 30-day mortality of 1.5%; 6-month mortality and prolonged ICU stay were 2.4% and 6.7%, respectively. Despite a decreased number of preoperative patients in New York Heart Association Functional Classification III or higher, prolonged ICU stay increased over the eras. Predictors of adverse outcome were New York Heart Association class III or higher, preoperative renal failure, disease of great complexity, preoperative ventilator support, cardiopulmonary bypass time, and concomitant procedures. CONCLUSIONS: In the current era of low 30-day mortality, extended 6-month mortality and prolonged ICU stay reporting may be more realistic measures of adverse outcomes for counseling GUCH patients at risk.

Aortic Coarctation/Arch Hypoplasia Repair: How Small Is Too Small.

Aortic coarctation/arch hypoplasia is a relatively common congenital heart disease that leads to severe cardiovascular complications if left untreated. During the modern era, the mortality of the primary surgical repair is very low but the long-term issues, such as recurrent coarctation/arch reobstruction and hypertension, are still significant challenges. The former is related to the surgical repair performed particularly in the management of the smallish distal aortic arch, and for the latter, despite the "successful" repair of the aortic coarctation, the intrinsic vascular anomaly remains a significant long-term morbidity.