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OBJECTIVES Our aim was to evaluate the results of tricuspid valve repair (TVr) in patients with hypoplastic left heart syndrome during staged reconstruction, focussing on the timing of the repair and the mechanisms of tricuspid regurgitation (TR). METHODS Records of 44 children with hypoplastic left heart syndrome who underwent a total of 62 tricuspid valve (TV) procedures during staged reconstruction were retrospectively analysed. RESULTS TVr was performed before stage II in 4 (9%) patients, at stage II in 23 (52%) patients, between stages II and III in 3 (7%) patients and at stage III in 14 (32%) patients. The median age at the first TV procedure was 5 months. At surgery, TR emanated commonly from the anteroseptal commissure in 21 (48%) patients. Anterior leaflet prolapse was observed most frequently (n = 23; 52%), followed by septal leaflet restriction (n = 22; 50%), dilated annulus (n = 21; 48%) and cleft anterior leaflet (n = 9; 21%). Surgical techniques included commissuroplasty in 27 (61.4%) patients, leaflet adaptation in 20 (44%) patients, partial annuloplasty in 11 (25%) patients, chordal reconstruction in 10 (23%) patients and cleft closure in 10 (23%) patients. Among all 44 patients, 27 (61%) patients had preoperative grade III TR and 17 (39%) patients had grade IV; postoperatively, there were no patients with grade IV, 25 patients with grade III (57%), 10 patients with grade II (23%) and 6 patients with grade I (14%). Fifteen patients required redo TV surgeries. Reoperation-free survival was 52% at 5 years. Lower weight at initial TVr predicted mortality [hazard ratio (HR) 0.7, P = 0.044] and reoperation (HR 0.8, P = 0.015). TVr before stage II was a risk for both reoperation (HR 5.5, P = 0.042) and TV replacement (HR 36.9, P = 0.013). Among morphological factors, septal leaflet restriction was a risk for reoperation (HR 4.7, P = 0.017) and anterior (HR 4.7, P = 0.037) and posterior (HR 7.3, P = 0.015) leaflet chordal anomaly for TV replacement. CONCLUSIONS Anterior leaflet prolapse and septal leaflet restriction are the main mechanisms of TR in hypoplastic left heart syndrome. Early-onset TR before stage II predicts worse outcome. Refinements to repair techniques in early infancy, especially for septal leaflet restrictions and chordal anomalies, are mandatory to improve outcomes.

OBJECTIVES The aim of this study was to investigate the impact of an early extubation strategy on the outcome following extracardiac total cavopulmonary connection. METHODS From 1999 through 2017, 458 patients underwent extracardiac total cavopulmonary connection; 257 (56%) patients were managed with an early extubation strategy adopted in 2009 (group A). Their outcome was compared with those of 201 (44%) patients treated before 2009 (group B). In group A, the outcome of unstable patients, defined as >75th percentile for volume administered and inotrope scores, was compared with those of stable patients. RESULTS Ventilation time (median: 4 h vs 16 h, P < 0.001), fluid volume administered during the first 24 h (mean: 110 ml/kg vs 164 ml/kg, P = 0.003), chest tube duration (median: 3 days vs 4 days, P = 0.028) and length of intensive care unit stay (median: 6 days vs 7 days, P = 0.001) were less in group A than in group B. The reintubation rate (7% vs 6%, P = 0.547) and early mortality (0.8% vs 1.5%, P = 0.465) were similar between groups. The 80 unstable group A patients received more inotropic support (P < 0.001) and fluid volume (P < 0.001) than stable patients, but the ventilation time (6 h vs 5 h, P = 0.220), the reintubation rate (10% vs 6%, P = 0.283) and the length of intensive care unit stay (7 days vs 6 days, P = 0.590) were similar. In unstable patients, mean arterial pressure before extubation was significantly lower than stable patients (P = 0.001). However, mean arterial pressure in unstable patients increased significantly (P < 0.001) soon after extubation, and became similar to the value in stable patients. CONCLUSIONS Early extubation following extracardiac total cavopulmonary connection improves postoperative haemodynamics and recovery regardless of the initial haemodynamic status.

Hybrid OPCAB/MIDCAB and TAVI prove to be a safe and effective alternative treatment option in selected higher risk patients.

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Background: The Carpentier-Edwards Perimount Magna Ease prosthesis (PME) represents the latest generation of stented bioprostheses used for surgical aortic valve replacement (SAVR). The aim of our study was to evaluate the long-term clinical outcome and hemodynamic performance of the prosthesis with a focus on the incidence and course of structural valve deterioration (SVD) by serial echocardiographic examinations. Methods: SAVR with the PME was performed in 58 consecutive patients between 2007 and 2008.Transthoracic echocardiography was performed preoperatively, at discharge and annually during a 10-year follow-up at the German Heart Center Munich. Results: Mean age at surgery was 62±14 years. At discharge (n=57), the overall mean pressure gradient (MPG) and effective orifice area (EOA) were 15.8±4.1 mmHg and 1.8±0.4 cm 2 , respectively. Moderate patient-prosthesis mismatch (PPM) was present in 18 patients (32%) and severe PPM in 6 patients (11%) at discharge. Ten years following SAVR (n=33), the overall MPG was 16.6±7.3 mmHg and EOA was 1.3±0.4 cm 2 .Thirty-day and late mortality was 2% (n=1) and 21% (n=12), respectively. Survival at 1, 5, and 10 years was 94.7%±3.3%, 91.1%±4.1%, and 77.3%±5.9%, respectively. Freedom from reoperation at 10 years was 88.8%±4.7%. Ten years after PME implantation the cumulative incidence of any SVD, severe SVD, and bioprosthetic valve failure (BVF) was 25%±6%, 14%±5%, and 16%±5%, respectively. Conclusions:The PME shows an excellent hemodynamic performance over the course of 10 years with development of clinically relevant SVD as late as 6 years post implant, and a 10-year incidence of severe SVD of 14%.

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Bilateral superior vena cava (SVC), which occurs following bilateral bidirectional cavopulmonary shunt (BCPS), is an anomaly marked by unique hemodynamics. This study aimed to determine its effects on outcomes after Fontan completion. Among 405 patients who underwent BCPS and total cavopulmonary connection (TCPC) between 1997 and 2017, 40 required a bilateral-BCPS. The dominant SVC prior to TCPC was identified according to the direction of blood flow in the central pulmonary artery, and its relationship to the inferior vena cava (IVC) was classified as a concordant or discordant relationship. Preoperative factors were analyzed to identify the risk factors for specific adverse outcomes. The length of intensive care unit (ICU) stay after TCPC was longer in the 40 patients who underwent bilateral-BCPS than in those who underwent unilateral BCPS (p = 0.024), and the survival rate was lower in the former group than in the latter group (p = 0.004). In the patients who underwent bilateral-BCPS, the dominant SVC was concordant with the IVC in 30 patients and discordant in 10 patients. With regard to whether certain morphological, hemodynamic, and flow dynamics-related variables were risk factors for adverse outcomes following TCPC, a discordant relationship between dominant SVC and IVC was identified as an independent risk factor for both a longer ICU stay (p = 0.037, HR 2.370) and worse survival (p = 0.019, HR 13.880). Therefore, in patients with a bilateral SVC who have previously undergone bilateral-BCPS, a discordant relationship between dominant SVC and IVC might contribute to worse outcomes following TCPC.