Stanislav Ovroutski

This study aimed to evaluate the short-/mid-term outcome of patients with complex dextro (d)-/levo (l)-transposition of the great arteries (TGA), ventricular septal defect and left ventricular outflow tract obstructions. A single-centre, retrospective review of all complex dextro-TGA (n = 85) and levo-TGA (n = 22) patients undergoing different surgeries [Arterial switch operation + left ventricular outflow tract obstruction-resection (ASO-R), half-turned truncal switch/Mair (HTTS), Nikaidoh and Rastelli] between May 1990 and September 2022 was performed. Groups were analysed using Kruskal–Wallis test with post hoc pairwise comparison and Kaplan–Meier time-to-event models. A total of 107 patients [ASO-R (n = 20), HTTS (n = 23), Nikaidoh (n = 21), Rastelli (n = 43)] were included, with a median age of 1.0 year (0.5–2.5) and surgical repair median follow-up was 3.8 years (0.3–10.5). Groups did not differ in respect to early postoperative complications/early mortality. Five-year overall survival curves were comparable: ASO-R 78.9% (53.2–91.5), HTTS 75.3% (46.8–89.9), Nikaidoh 85% (60.4–94.9) and Rastelli 83.9% (67.5–92.5), P = 0.9. Highest rates of right ventricular outflow tract (RVOT) reinterventions [33.3% and 32.6% (P = 0.04)] and reoperations [28.6% and 32.6% (P = 0.02)] occurred after Nikaidoh and Rastelli procedures. However, overall freedom from RVOT reinterventions and RVOT reoperations at 5 years did not differ statistically significantly between the groups (ASO-R, HTTS, Nikaidoh and Rastelli): 94.4% (66.6–99.2), 69.1% (25.4–90.5), 67.8% (34–86.9), 64.4% (44.6–78.7), P = 0.2, and 90.0% (65.6–97.4), 91% (50.8–98.7), 65.3% (32.0–85.3) and 67.0% (47.4–80.6), P = 0.3. Surgical repair of complex dextro-/levo-TGA can be performed with satisfying early/mid-term survival. RVOT reinterventions/reoperations were frequent, with highest rates after Nikaidoh and Rastelli procedures. Left ventricular outflow tract obstruction reoperations were rare with zero events after Nikaidoh and HTTS procedures.

Objective: The goal was to report the midterm performance of decellularized equine pericardium used for repair of various congenital heart defects in the paediatric population. Methods: A retrospective review of all patients undergoing patch implants between 2016 and 2020 was performed. Patch quality, surgical handling, haemostasis and early patch-related complications were studied in all patients. Midterm performance was observed in patients with ≥12 months follow-up and an intact patch at discharge (without reoperation/stent implant). Results: A total of 201 patients with a median age of 2.5 years [interquartile range: 0.6-6.5] underwent 207 procedures at 314 implant locations. The patch was used in the following numbers/locations: 171 for pulmonary artery (PA) augmentation, 36 for aortic repair, 35 for septal defect closure, 22 for valvular repair and 50 at other locations. Early/30-day mortality was 6.5%. Early patch-related reoperations/stent implants occurred in 28 locations (8.9%). No patch-related complications were noted except for bleeding in 3 locations (1%). Follow-up for ≥ 12 months was available for 132 patients/200 locations. During a median follow-up of 29.7 months [interquartile range: 20.7-38.3], 53 patch-related reoperations/catheter reinterventions occurred (26.5%), with the majority in the PA position (88.7%, 47/53). Overall 12- and 24-months freedom from patch-related reoperation/catheter reintervention per location was 91.5% (95% confidence interval: 86.7%-94.6%) and 85.2% (95% confidence interval: 78.9%-89.6%), respectively. Conclusion: Decellularized equine pericardium used for repair of various congenital heart defects showed acceptable midterm performance. The range of reoperation/reintervention rates was similar to those observed with other xenogeneic materials in previously reported articles, occurring most frequently after PA augmentation.

Background: Patients with congenital heart disease (CHD)—including those after Fontan operation—are encouraged to be physically active.Aim: To prospectively determine the effects of an individually adapted, home-based cycle ergometer endurance training in combination with inspiratory muscle training (IMT) in pediatric and adult Fontan patients. We, herein, report the results of the initial 10-months follow-up (phase 1).Methods: 18 patients (median age 16.5 years; range 10-43 years) completed baseline check-ups, and 4 and 10 months follow-up visits, which each included cardiopulmonary exercise testing (CPET), bodyplethysmography (including measurement of respiratory muscle strength), and a quality of life questionnaire (PedsQL™). The training program consisted of a home-based cycle ergometer endurance training on a “Magbike® AM-5i/3i” (DKN Technology®, Clermont-Ferrand, France) and IMT with a handheld “POWERbreathe® Medic plus” device. Patients performed 90 min of endurance training per week in addition to IMT (30 breaths per day, 6-7 times per week). After the first 4 months, patients underwent additional interval training.Results: After 10 months of training, we observed significant increases in maximum relative workload (W/kg, p = 0.003) and in maximum inspiratory (MIP, p = 0.002) and expiratory (MEP, p = 0.008) pressures. Peak VO₂ values did not increase significantly as compared to baseline (p = 0.12) in the entire cohort (n = 18), but reached statistical significance in a subgroup analysis of teenage/adult patients (n = 14; p = 0.03). Patients' subjective quality of life did not show any significant changes after 10 months of training.Discussion: In Fontan patients, an individually adapted home-based training is safe and associated with improvements in some CPET variables. However, these improvements did not translate into an improved QoL after 10 months. With an unclear, but most likely negative, impact of the COVID-19 pandemic, improvements in QoL may become evident during further follow-up (phase 2 of the study).

Aim: Regular evaluation of physical capacity takes a crucial part in long-term follow-up in patients with congenital heart disease (CHD). This study aims to examine the accuracy of self-estimated exercise capacity compared to objective assessments by cardiopulmonary exercise testing in patients with CHD of various complexity. Methods: We conducted a single centre, cross-sectional study with retrospective analysis on 382 patients aged 8–68 years with various CHD who completed cardiopulmonary exercise tests. Peak oxygen uptake was measured. Additionally, questionnaires covering self-estimation of exercise capacity were completed. Peak oxygen uptake was compared to patient’s self-estimated exercise capacity with focus on differences between complex and non-complex defects. Results: Peak oxygen uptake was 25.5 ± 7.9 ml/minute/kg, corresponding to 75.1 ± 18.8% of age- and sex-specific reference values. Higher values of peak oxygen uptake were seen in patients with higher subjective rating of exercise capacity. However, oxygen uptake in patients rating their exercise capacity as good (mean oxygen uptake 78.5 ± 1.6%) or very good (mean oxygen uptake 84.8 ± 4.8%) was on average still reduced compared to normal. In patients with non-complex cardiac defects, we saw a significant correlation between peak oxygen uptake and self-estimated exercise capacity (spearman-rho −0.30, p < 0.001), whereas in patients with complex cardiac defects, no correlation was found (spearman-rho −0.11, p < 0.255). Conclusion: The mismatch between self-estimated and objectively assessed exercise capacity is most prominent in patients with complex CHD. Registration number at Charité Universitätsmedizin Berlin Ethics Committee: EA2/106/14.

Strict patient selection, short cardiopulmonary bypass (CPB) time and short mechanical ventilation are well-recognized aspects of optimizing the postoperative course after total cavopulmonary connection. In this study, we evaluated the influence of our early postoperative extubation strategy in our population of Fontan patients over the past 2 decades. Early postoperative course was evaluated retrospectively in 211 consecutive patients, who were selected for total cavopulmonary connection in our institution between 1995 and 2015. We analysed postoperative haemodynamic parameters and early outcome after surgery (mortality and length of hospital stay) according to preoperative patient characteristics, duration of CPB and duration of mechanical ventilation. To investigate the influence of mechanical ventilation, the cohort was subdivided into a ‘fast-track’ extubation group (≤6 h ventilation, n = 59) and a prolonged ventilation group (>6 h ventilation, n = 152). In the entire cohort, duration of CPB did not correlate with duration of mechanical ventilation (P = 0.1), and it did not differ between both groups (P = 0.3). Patients in the fast-track group showed significantly better haemodynamics with higher mean arterial pressure and lower mean pulmonary artery pressure at 6, 24 and 48 h postoperatively when compared with the prolonged ventilation group (P = 0.02–0.001). In multivariable analysis, longer mechanical ventilation, but not duration of CPB, was independently associated with length of hospital stay. Early weaning from mechanical ventilation correlates with improved early Fontan haemodynamics, whereas early outcome is unrelated to duration of CPB. This indicates that early extubation may represent a principal strategy for improving early results after total cavopulmonary connection.

Different surgical techniques for the treatment of complex transposition of the great arteries (TGA) with ventricular septal defect and left ventricular outflow tract obstruction (LVOTO) have been developed, in particular the Rastelli operation, the réparation à l’étage ventriculaire procedure and the Nikaidoh procedure. The hitherto published results of the Nikaidoh procedure and its modifications compare favourably with those of other techniques; however, experience with the Nikaidoh procedure is still limited. Here, we report our institutions' early and mid-term results with modifications of the Nikaidoh procedure. Twenty-one patients who underwent a modified Nikaidoh procedure between 2006 and 2012 at our institution, either as aortic root translocation (n = 17) or as en bloc rotation of the arterial trunk (n = 4), were studied retrospectively. There were 2 early and 1 mid-term deaths. The follow-up continued for a median of 2.3 years (range 0.3–6.4 years). During the follow-up, the performance of the reconstructed left ventricular outflow tract (LVOT) remained excellent: no reobstruction and no aortic valve regurgitation classified as more than mild were observed. Left ventricular function was well preserved. In 4 patients, a significant reoccurring right ventricular outflow tract obstruction due to conduit failure was observed; so far, two reoperations with conduit replacement have been necessary. The mean right ventricular outflow tract peak gradient was 24 ± 7.2 mmHg at the last follow-up in the remaining patients. No reobstruction of the right ventricular outflow tract occurred in patients with preserved pulmonary valve tissue after en bloc rotation. The aortic translocation procedure is a valuable surgical option for patients with complex (TGA) with ventricular septal defect and LVOTO. The mid-term results document excellent performance of the reconstructed LVOT. Modifications of the Nikaidoh procedure that preserve pulmonary valve tissue may further reduce the need for right ventricular outflow tract reoperation.

The use of modified extracardiac Fontan operation (ECFO) for total cavo-pulmonary connection allows cardiopulmonary bypass (CPB) to be avoided and seems to improve early postoperative results. We evaluated our experience with the off-pump technique for ECFO. Since 2009, the last 17 consecutive patients of 137 (median age 3.2 years, median weight 14.5 kg) in whom no intracardiac surgery was necessary underwent ECFO without CPB. The non-fenestrated graft was connected end-to-side to the pulmonary artery without bypass; subsequently temporary passive inferior vena cava (IVC)-to-atrial bypass was used for the anastomosis between IVC and graft. The perioperative and postoperative course was compared between consecutive paediatric patients operated on using the CPB vs off-pump technique. There was no mortality in the off-pump group, with a total early mortality of 3.0%. Overall operation time for the Fontan operation using the off-pump technique was significantly reduced (160 vs 200 min, P < 0.001). The median Fontan pressure 24 and 48 h postoperatively was significantly lower in the off-pump group (P = 0.002/0.042). Duration of mechanical ventilation (9 vs 14 h, P = 0.016), pleural effusions (4 vs 8 days, P < 0.001) as well as the median intensive care unit (2 vs 4 days, P = 0.013) and hospital stay (median 10 vs 15 days, P < 0.001) was significantly shorter in patients who underwent the off-pump Fontan operation. The necessity of blood transfusions was significantly reduced with the off-pump in comparison with the on-pump technique (14 of 17 vs 34 of 84 patients, P = 0.003). The ECFO without CPB is an established low-risk surgical procedure that improves the early postoperative course and significantly reduces the use of blood products and the duration of pleural effusions in selected patients.