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This study was intended to determine the impact of extracardiac anomalies on outcomes in patients with functional single ventricle who underwent staged palliation. We reviewed medical records of patients who underwent first-stage palliation at our center between 2001 and 2020. The prevalence and type of extracardiac anomalies were evaluated, and their impact on outcomes during staged palliation was analyzed. Among 602 patients who underwent first-stage palliation, 81 (14%) patients had associated with extracardiac anomalies. They were more frequently associated with prematurity (P =.03) and low birth weight below 2.5 kg (P <.01). Mortality between first-stage palliation and stage II was similar in patients with and without extracardiac anomalies (24.7% vs 17.1%, P =.10). However, mortality between stage II and stage III was significantly higher in patients with extracardiac anomalies compared with those without (22.2% vs 12.5%, P =.02). Mortality after stage III was also higher in patients with extracardiac anomalies compared with those without (4.9% vs 1.5%, P =.04). In the subgroup analysis of 81 patients with extracardiac anomalies, renal anomalies were identified as a significant risk factor for mortality (P =.03, hazard ratio 2.44). The incidence of extracardiac anomalies in this study was 14%, and patients with extracardiac anomalies were highly associated with prematurity and low birth weight. Presence of extracardiac anomalies was associated with higher mortality between stage II and stage III palliation and after stage III phase, but not before stage II. Among extracardiac anomalies, renal anomalies were identified as a risk factor for mortality. [ABSTRACT FROM AUTHOR]

Total anomalous pulmonary venous connection (TAPVC) with a functional single ventricle is a risk factor for mortality during staged palliation. This study aimed to assess TAPVC's impact on staged palliation outcomes. In a total of 602 patients with a functional single ventricle who underwent stage 1 palliation (S1P) at our center between 2001 and 2020, 39 (6.5%) patients were associated with TAPVC. Median age at S1P was 12.0 (interquartile range, 7-21) days with a body weight of 3.1 (interquartile range, 2.8-3.6) kg. Outcomes during staged palliation were compared with the remaining 563 patients without TAPVC. Risk factors for mortality were identified using a Cox proportional hazards regression model. Primary diagnosis in functional single-ventricle patients with TAPVC included hypoplastic left heart syndromes (n = 13), unbalanced atrioventricular septal defects (n = 12) tricuspid atresias (n = 2), double inlet left ventricle (n = 1), and others (n = 11). Types of TAPVC were supracardiac (n = 21), cardiac (n = 10), infracardiac (n = 6), and mixed (n = 2). Pulmonary venous obstruction (PVO) was associated in 19 (49%) patients. S1Ps included Norwood (n = 13), aortopulmonary shunt (n = 21), and pulmonary artery banding (n = 5). Thirty-day mortality after S1P was significantly increased in patients with TAPVC vs without TAPVC (43.6% vs 16.3%; P <.001). After bidirectional cavopulmonary shunt and total cavopulmonary connection procedures, mortality was low in both groups, and no statistically significant differences were found. Correction of TAPVC at the time of S1P was not found to be a significant risk factor in univariable Cox regression analysis. In univariate and multivariate analysis, PVO was identified as an independent risk factor for mortality in patients with TAPVC (P <.001). Overall survival is lower in TAPVC single-ventricle patients than in non-TAPVC patients. Most deaths after S1P were associated with TAPVC, but not after S2P. PVO is a mortality risk factor in TAPVC patients. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES Studies focused on infants with univentricular heart undergoing stage I palliation other than the Norwood procedure remain a topic of great interest. This study evaluated the outcome of infants who underwent systemic to pulmonary shunt (SPS) or pulmonary artery banding (PAB). METHODS Infants who underwent SPS or PAB as stage I palliation between 1994 and 2019 were included. Survival and late systemic ventricular function were evaluated. RESULTS Out of 242 patients, 188 underwent SPS (77.7%) and 54 PAB (22.3%). Main diagnosis included tricuspid atresia, unbalanced atrioventricular septal defects, double inlet left ventricles and single ventricles with other morphology. Thirty-eight patients died before stage II palliation (15.7%). Stage II palliation was performed in 182 patients (75.2%), and mortality between stages II and III was 11 (5.6%). Stage III palliation was performed in 160 (66.1%) patients. Survival at 1, 5 and 15 years after stage I procedure was 81.9, 77.1 and 76.2%, respectively, and similar between both procedures (P  = 0.97). Premature birth [ P  = 0.03, hazard ratio (HR) = 2.1], heterotaxy (P  = 0.006, HR = 2.4) and dominant right ventricle (P  = 0.015, HR = 2.2) were factors associated to mortality. Unbalanced atrioventricular septal defect (P  = 0.005, HR = 4.6) was a factor associated to systemic ventricular dysfunction. CONCLUSIONS In patients with univentricular heart who underwent SPS and PAB as stage I palliation, survival at 15 years was 76%, regardless of th chosen approach. Premature birth, heterotaxy and dominant right ventricle were associated to mortality. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES We sought to identify the prevalence of surgical reintervention on the neo-aorta after Norwood procedure and its impact on long-term outcomes. METHODS We reviewed the medical records of all patients who underwent Norwood procedure. The impacts of surgical neoaortic reintervention on outcomes were analysed in each stage of palliation. RESULTS A total of 335 patients were included in this study. Thirty patients underwent surgical reintervention on the neo-aorta after Norwood procedure. The timing of initial reintervention was before stage II in 13 patients, at stage II in 7, between stage II and stage III in 5, at stage III in 3 and after stage III in 2. A reintervention before stage II was significantly associated with mortality (HR 14.4, 95% confidence interval 6.00–34.6, P < 0.001). In patients who underwent stage II (n = 251), reintervention had no significant impact on mortality. In patients who underwent stage III (n = 188), the previous reintervention was significantly associated with higher mean pulmonary pressure (P = 0.05) and a higher rate of reduced ventricular function (P = 0.002). Greater than mild atrioventricular valve regurgitation was significantly associated with the development of a neoaortic arch stenosis after stage II (P = 0.03). CONCLUSIONS Surgical reinterventions on the neo-aorta were required in each inter-stage phase. A surgical neoaortic reintervention was not related to increased mortality after stage II but significantly associated with a higher rate of reduced ventricular function and elevated mean pulmonary artery pressure. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES We sought to investigate the impact of early postoperative low arterial oxygen saturation on mortality and morbidity after bidirectional cavopulmonary shunt (BCPS). METHODS The medical records of all patients who underwent BCPS between 2013 and 2018 were reviewed. RESULTS A total of 164 patients were included in this study. Forty-seven patients underwent reintervention during hospital stay at median 7 days after BCPS. Before reintervention, 30 patients were intubated or had SpO2 of <75%. All re-interventions for Glenn pathway obstruction and 4 out of 5 venovenous coil embolization resulted in hospital discharge, while high mortality was observed after other re-interventions (atrioventricular valve surgery, thrombolysis, systemic ventricular outflow obstruction relief, extracorporeal membrane oxygenation implantation and diaphragmatic plication). Additional aortopulmonary shunt with pulmonary artery discontinuation was performed in 8 patients who showed severe cyanosis with median SpO2 of 59% under maximal ventilation support. In the univariable Cox regression analysis, the associated factors for mortality before total cavopulmonary connection were reduced ventricular function [hazard ratio (HR) 6.89, 95% confidence interval (CI) 1.76–26.9, P -value 0.006], greater than moderate atrioventricular valve regurgitation (HR 5.89, 95% CI 1.70–20.4, P -value 0.005), SpO2 1 h after extubation (HR 0.87, 95% CI 0.80–0.96, P -value 0.004) and mean pulmonary artery pressure 1 h after extubation (HR 1.14, 95% CI 1.02–1.26, P -value 0.016). CONCLUSIONS After BCPS, unacceptable cyanosis persisted with various aetiologies. Low arterial oxygen saturation within 1 h after extubation is significantly associated with high mortality after BCPS. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES Despite improvements in the surgical management of the hypoplastic left heart syndrome and its variant, the Norwood procedure is still associated with substantial mortality and morbidity and extracorporeal membrane oxygenation support is required in some patients. METHODS We reviewed patients with the Norwood procedure between 2007 and 2019. The primary end point of the study was mortality during extracorporeal membrane oxygenation. Secondary end points included morbidity, bidirectional cavopulmonary shunt and Fontan completion. RESULTS Of the 257 patients in whom the Norwood procedure was performed, mechanical support was required in 41 patients (16%). Indications for extracorporeal membrane oxygenation were low cardiac output (n = 16, 39%), hypoxaemia (n = 12, 29%) and inability to wean from cardiopulmonary bypass (n = 9, 22%). The median age at extracorporeal membrane oxygenation was 10.9 days (interquartile range, 7.9–21.2) and veno-arterial support was required in 37 patients (90.2%). Weaning from extracorporeal membrane oxygenation was achieved in 61% (n = 25). Survival to hospital discharge and 1-year survival was 34.6% (standard deviation: 17.1) and 25.7% (standard deviation: 7), respectively. Bidirectional cavopulmonary shunt was performed in 24% (n = 10) and Fontan completion in 7% (n = 3). Preoperative moderate or greater atrioventricular valve regurgitation was independently associated with mechanical support. Implantation of extracorporeal membrane oxygenation in the paediatric catheter laboratory was identified as an independent risk factor for mortality. CONCLUSIONS Moderate or greater atrioventricular valve regurgitation is an independent risk factor for mechanical support after the Norwood procedure. Mechanical support is associated with substantial in-hospital mortality; however, successful Fontan completion was accomplished in some patients. [ABSTRACT FROM AUTHOR]

This study sought to determine long-term outcomes of a primary Damus-Kaye-Stansel (DKS) procedure in patients with a functional single ventricle and to compare the results with those of our historical control subjects who underwent pulmonary artery banding before the DKS procedure. The study reviewed the medical records of all patients who underwent the DKS procedure at the German Heart Center of Munich, Germany between December 1994 and December 2019. The DKS procedure was performed as initial palliation in 52 patients (primary DKS group) and as staged palliation after pulmonary artery banding in 24 patients (staged DKS group). The median follow-up period after the DKS procedure was 8.9 years in the primary DKS group and 8.0 years in the staged DKS group. The survival rates at 10 years after the DKS procedure were 89% in the primary DKS group and 68% in the staged DKS group (log-rank P = 0.04). Before total cavopulmonary connection, the pressure gradient through the systemic ventricular outflow tract was significantly lower in the primary DKS group than in the staged DKS group (P <.001). At last follow-up echocardiography, reduced ventricular function was observed in 1 patient in the primary DKS group and in 7 patients in the staged DKS group (P <.001). The degree of neoaortic regurgitation was significantly higher in the staged DKS group than in the primary DKS group (P <.001). A primary DKS procedure in patients with a functional single ventricle and potential systemic ventricular outflow tract obstruction is recommended to obtain favorable long-term survival with preserved ventricular function and competent semilunar valve function. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES This study was performed to determine the clinical and haemodynamic variables associated with early adverse outcomes after the neonatal Norwood procedure. METHODS Patients who underwent the neonatal Norwood procedure between 2001 and 2019 were included. The patient diagnosis, morphological characteristics and haemodynamic parameters were analysed to identify factors associated with length of stay (LOS) in the intensive care unit (ICU) and mortality during the stay. RESULTS A total of 322 patients were depicted. The median age and weight at the Norwood procedure were 9 days and 3.2 kg, respectively. Certain morphological and preoperative parameters, such as birth weight below 2.5 kg, restrictive atrial septal defect, extracardiac anomalies and the diameter of the ascending aorta, were found to be associated with the LOS in the ICU. Analysis using early postoperative haemodynamic variables revealed that systolic arterial pressure, diastolic arterial pressure, serum lactate levels and reduced ventricular function at 2 days postoperatively were associated with the LOS in the ICU. Birth weight <2.5 kg (P  = 0.010), a restrictive atrial septal defect (P  = 0.001) and smaller ascending aorta (P  = 0.039) were associated with death in the ICU. Reduced ventricular function, lower systolic aortic pressure and higher lactate levels at various time points (P  < 0.05) were also associated with ICU deaths. The LOS in the ICU was significantly associated with late mortality (P  < 0.001, Hazard Ratio (HR) = 1.015). CONCLUSIONS The LOS in the ICU after the Norwood procedure was predicted by early postoperative haemodynamic variables, suggesting that good early postoperative haemodynamics determine early recovery. A prolonged stay in the ICU after the Norwood procedure was associated with late mortality. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES A common atrioventricular valve (CAVV) is considered to be a risk factor for early and late deaths in patients with functional single ventricle (FSV). CAVV surgery in patients with FSV is challenging and there is limited knowledge of the outcomes of CAVV repair with univentricular physiology. METHODS We reviewed all CAVV surgical procedures in patients with FSV who underwent univentricular palliation. End points of the study were survival after CAVV surgery and cumulative incidence of reoperation. RESULTS Between 1984 and 2019, 66 children with CAVV and FSV underwent single-ventricle palliation, of whom 45.5% (30/66) required CAVV surgery. Indication for surgery was moderate CAVV regurgitation in 40% (12/30) and severe CAVV regurgitation in 60% (18/30). CAVV repair was performed in 93.3% (28/30) and CAVV replacement in 6.7% (2/30). The median age and weight at surgery were 0.9 years (interquartile range 0.3–1.8) and 6.5 kg (interquartile range 3.9–8.7), respectively. Operative and late mortality were 23.3% and 8.7%, respectively. Survival and cumulative incidence of reoperation at 4 years after CAVV surgery were 68.9% [standard deviation (SD): 8.7] and 35.8% (SD: 9.1), respectively. Fontan completion was achieved in 60% (18/30). Survival at 4 years after birth was 69.7% (SD: 8.5) in 30 patients with CAVV surgery, whereas it was 83% (SD: 6.3) in 36 patients without CAVV surgery (P  = 0.32). CONCLUSIONS CAVV surgery in patients with FSV is associated with substantial mortality and a high incidence of reoperation. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES The ideal shunt for pulmonary blood flow, modified Blalock–Taussig shunt (MBTS) or right ventricular–pulmonary artery conduit (RVPAC) is yet to be determined. This study aimed to evaluate outcomes after the Norwood procedure according to the type of shunt. METHODS A total of 322 neonates with hypoplastic left heart syndrome and related anomalies who underwent the Norwood procedure at our institution between 2001 and 2019 were divided into MBTS and RVPAC groups and the outcomes after the Norwood procedure were compared between the groups with respect to mortality after each staged procedure. RESULTS We identified 322 consequent patients who underwent neonatal Norwood procedure for hypoplastic left heart syndrome (271 patients, 84.2%) and its variant (51 patients, 15.8%). RVPAC was performed in 163 (50.6%) patients and MBTS was performed in 159 (49.4%). There were no differences in the rate of early death (11.0% vs 12.6%, P  = 0.69) or late death (7.4% vs 6.9%, P  = 0.87) between the 2 groups after the Norwood procedure, and no significant difference in the number of patients who reached bidirectional cavopulmonary shunt (77.9% vs 76.1%, P  = 0.69), and there was no difference in mortality after bidirectional cavopulmonary shunt (12.3% vs 7.5%, P  = 0.15) or Fontan completion rate (54.0% vs 52.2%, P  = 0.42) between the 2 groups. Survival at 0.5, 1, 3 and 6 years after the Norwood procedure was 81.0%, 73.8%, 67.9% and 67.0% in patients with RVPAC and 77.1%, 73.3%, 69.1% and 67.9% in patients with MBTS. There was no significant difference in the survival between the 2 groups during the median follow-up of 2.6 (interquartile ranges: 0.3–8.4, maximal 18.8) years (P  = 0.97). CONCLUSIONS In neonates undergoing the Norwood procedure, our available data of maximal 18.8 years follow-up showed no significant difference in early mortality, inter-stage attritions, or overall survival, between MBTS and RVPAC. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES Heterotaxy is a known risk factor for morbidity and mortality in single ventricle palliation. In this study, we examined our experience with this challenging group of patients. METHODS Records of patients born between 2001 and 2019 with heterotaxy, who needed staged single ventricle palliation were retrospectively analysed. RESULTS A total of 53 patients were included in this study. Thirty-five (66%) patients had a right ventricular dominance, common atrioventricular septal defect was present in 37 (70%) patients. Anomalous pulmonary venous drainage was present in 29 (55%) patients. Forty-six (86%) patients underwent first-stage palliation. Forty-one (77.3%) patients received a bidirectional cavopulmonary connection. Thirty-one (58%) patients received total cavopulmonary connection (TCPC). Overall survival rate was 92% at 1 month, 74% at 1 year and 68% at 10 years. Twelve (22.6%) patients died before second palliation stage. Four (10%) patients died before TCPC. No patient died after TCPC. Independent risk factors for mortality in the multivariate COX regression were a presence of restrictive pulmonary blood flow (HR 3.23; 95% CI 1.02–10.2; P  = 0.05) and greater than mild atrioventricular valve regurgitation (HR 3.57; 95% CI 1.27–10.0; P  = 0.02). CONCLUSIONS Mortality and morbidity in patients with single ventricle and heterotaxy are high. Restrictive pulmonary blood flow needing early modulation and greater than mild atrioventricular valve regurgitation at presentation are independent risk factors for mortality. Total anomalous pulmonary venous connection was not identified as a risk factor in the current era. [ABSTRACT FROM AUTHOR]

Our aim was to evaluate the results of stage 2 palliation by means of bidirectional cavopulmonary shunt (BCPS) and to identify risk factors for failed Fontan completion. Between 1998 and 2018, BCPS was performed on 525 patients with functional single ventricle. Patient demographics, surgical data, and echocardiographic and cardiac catheterization measurements were analyzed, and outcomes after BCPS were evaluated. The median age at BCPS was 4.7 months (interquartile range, 3 to 7.4). Hypoplastic left heart syndrome was the most frequent diagnosis. The median follow-up after BCPS was 3.4 years (interquartile range, 1.5 to 8.7); 407 patients underwent the Fontan procedure, 50 were waiting for Fontan completion, 47 died, 6 were considered not suitable for Fontan completion, and 15 were lost to follow-up. Of the 407 patients who underwent Fontan completion, there were 5 early deaths. Freedom from failed Fontan completion (mortality before, unsuitability for, and early mortality after the Fontan procedure) at 1, 2, and 3 years was 91.9%, 87.3%, and 86.1%, respectively. Risk factor analysis for failed Fontan completion revealed hypoplastic left heart syndrome (hazard ratio [HR] 4.1, P =.001), unbalanced atrioventricular septal defect (HR 10.1, P <.001), higher pulmonary artery pressure (HR 1.1, P =.040), and reduced ventricular function (HR 4.2, P =.001) as risks. Stage 2 palliation can be performed with minimal risk and provides excellent subsequent Fontan completion. Hypoplastic left heart syndrome, unbalanced atrioventricular septal defect, high pulmonary artery pressure, and reduced ventricular function at the time of BCPS were identified as risk factors for failure to successfully complete the Fontan procedure. [Display omitted] [ABSTRACT FROM AUTHOR]

Open in new tab Download slide Open in new tab Download slide OBJECTIVES The present study was aiming to determine whether high mean pulmonary artery pressure before bidirectional cavopulmonary shunt is a risk factor for late adverse events in patients with low pulmonary artery pressure before total cavopulmonary connection (TCPC). METHODS We retrospectively reviewed the medical records of all patients undergoing both bidirectional cavopulmonary shunt and TCPC with available cardiac catheterization data. RESULTS A total of 316 patients were included in this study. The patients were divided into 4 groups according to mean pulmonary pressure: those with pre-Glenn <16 mmHg and pre-Fontan <10 mmHg (Group LL, n  = 124), those with pre-Glenn ≥16 mmHg and pre-Fontan <10 mmHg (Group HL, n  = 61), those with pre-Glenn <16 mmHg and pre-Fontan ≥10 mmHg (Group LH, n  = 66) and those with pre-Glenn ≥16 mmHg and pre-Fontan ≥10 mmHg (Group HH, n  = 65). Group HL showed significantly higher rate of adverse events after TCPC than Group LL (P  = 0.02). In univariate linear analysis, a history of atrial septectomy at stage 1 palliation was associated with low pre-Glenn mean pulmonary artery pressure (Coefficient B −1.38, 95% confidence interval −2.53 to −0.24; P  = 0.02), while pulmonary artery banding was a significant risk factor for elevated pre-Fontan mean pulmonary artery pressure (Coefficient B 1.68, 95% confidence interval 0.81 to 2.56, P  < 0.001). CONCLUSIONS High mean pulmonary artery pressure before bidirectional cavopulmoary shunt (≥16mmHg) remains a significant risk factor for adverse events after TCPC even though mean pulmonary artery pressure decreased below 10 mmHg before TCPC. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

OBJECTIVES: Our aim was to evaluate outcomes following a total cavopulmonary connection (TCPC) in patients with preoperatively impaired ventricular function (VF). METHODS: Of 483 consecutive TCPC patients, 44 (9.1%) had impaired VF (ejection fraction <50%, Group A), and 439 patients had normal VF (ejection fraction >_50%, Group B). We compared the clinical outcomes between the groups. RESULTS: The median age at TCPC was 2.8 (interquartile range 1.9-8.3) years in Group A and 2.3 (1.8-3.5) years in Group B (P = 0.025). An atrioventricular valve (AVV) operation prior to (38.6 vs 27.1%, P < 0.001) and concomitant with (31.8 vs 12.1%, P < 0.001) the TCPC was performed more frequently in Group A. The median intensive care unit stay (7.0 vs 7.0 days, P = 0.737) and 30-day survival (97.7 vs 98.4%, P = 0.737) were not significantly different between groups. Freedom from death, transplantation (P = 0.115) and catheter intervention (P = 0.603) showed no difference between groups. However, freedom from cardiac reoperation was significantly lower in Group A (P < 0.001). VF was resolved in 22 of the 39 (56.4%) survivors in Group A. The recovered patients had a lower incidence of AVV reoperation (0 vs 6, P = 0.002) and pacemaker rhythm (0 vs 5, P = 0.006). CONCLUSIONS: In patients planned for TCPC, impaired VF is often associated with AVV regurgitation. TCPC can be performed with low risk and comparable clinical results except for cardiac reoperation in patients with impaired VF when compared to patients with normal VF. Following TCPC, VF recovers in half of the survivors. A competent AVV and sinus rhythm are prerequisites for recovery. [ABSTRACT FROM AUTHOR]

Background The adult congenital heart surgery (ACHS) score was derived from The Society of Thoracic Surgeons Congenital Heart Surgery Database. The score was validated with data for 1,603 operations and reached a good predictive power. We sought to evaluate its predictive power for 1,654 operations performed in two European centers. Methods Data of all consecutive patients aged 18 years or more who underwent surgery for congenital heart disease between 2004 and 2013 at center 1 (n = 830) and between 2005 and 2016 at center 2 (n = 824) were collected. Mortality was defined as hospital mortality or mortality within 30 days after surgery. The discriminatory power of the ACHS score was assessed using the area under the receiver-operating characteristics curve (c-index). Results During the examined 13-year period, 1,639 operations of 43 different procedural groups were eligible for scoring. The most frequent procedures were closure of atrial septal defect (n = 175, 10.7%), repair of partial anomalous pulmonary venous connection (n = 117, 7.1%), and aortic valve replacement (n = 112, 6.8%). Hospital mortality was 3.1%. The procedures with the highest mortality were heart transplantation (3 of 11, 27.3%), mitral valve replacement (9 of 39, 23.1%), and systemic venous stenosis repair (2 of 9, 22.2%). The c-index for the ACHS mortality score was 0.760 (0.750 in center 1 and 0.772 in center 2). Conclusions The ACHS score reached similar, good predictive power in two different centers. The score is a useful tool to analyze surgical outcomes and to support individual decision making. [ABSTRACT FROM AUTHOR]

OBJECTIVES: Our aim was to evaluate whether early timing of total cavopulmonary connection (TCPC) affects postoperative outcomes. METHODS: Of 460 consecutive TCPC patients, 51 (11.1%) underwent TCPC <18 months of age (group A), and 409 patients >18 months of age (group B). We compared the clinical outcomes and exercise capacity between groups. RESULTS: Median age at TCPC was 1.4 (interquartile ranges: 1.3–1.5) years in group A and 2.5 (1.9–4.5) years in group B. Duration of intensive care unit stay (6 vs 7 days), hospital stay (20 vs 20 days), and 30-day survival (100 vs 98%) was not significantly different between groups. Estimated survival (95.3 vs 92.1%), freedom from reoperation (93.7 vs 86.3%), freedom from catheter intervention (60.1 vs 77.0%), and freedom from protein losing enteropathy (97.6 vs 93.8%) at 10 years was not significantly different between groups. At last follow-up, no patient in group A but 13 patients in group B exhibited reduced ventricular function (P = 0.035). Exercise-capacity testing showed that both peak oxygen uptake (36.4 vs 28.6 ml/kg/min; P = 0.026) and its percentage of predicted value (82.9 vs 70.0%; P = 0.004) were significantly higher in group A (n = 6, mean postoperative period: 8.9 years) than in group B (n = 119, mean postoperative period: 8.7 years). CONCLUSIONS: Fontan completion for TCPC can be performed at an early age with a low perioperative risk and good intermediate results. Earlier unloading of the systemic ventricle and earlier elimination of chronic cyanosis by staged cavopulmonary connection might be advantageous for preservation of long-term ventricular function. [ABSTRACT FROM AUTHOR]

OBJECTIVES: This study aims to evaluate the clinical outcome following total cavopulmonary connection (TCPC) and to identify factors affecting early and late outcome. METHODS: Between May 1994 and March 2015, 434 patients underwent TCPC with 50 lateral tunnels and 374 extracardiac conduits. The clinical outcome, exercise capacity and liver examination results were retrospectively reviewed. RESULTS: Thirty-day survival was 98.2%, and the estimated survival rate at 15 years was 92.3%. Freedom from tachyarrhythmia at 15 years was 91.0%. Other late morbidities included bradyarrhythmia in 17, protein-losing enteropathy (PLE) in 15, thromboembolism in 3 and plastic bronchitis in 3 patients. At last follow-up, normal systemic ventricular function (ejection fraction >50%) was observed in 88.2%. Atrioventricular valve (AVV) regurgitation was mild or less in 90% of patients with systemic left ventricle, in 63% of those with systemic right ventricle and 58% of the patients with unbalanced atrioventricular septal defect or common inlet ventricles. Cardiopulmonary exercise capacity showed impaired peak oxygen uptake (71% of normal) in a sub-group of 120 patients at a mean of 9 years postoperatively. Biochemistry of 338 patients at last follow-up revealed a gamma-glutamyl transferase value beyond normal in 90 patients (26%), with a positive correlation between the level and the time after the initial operation (P < 0.01). Pre-TCPC high transpulmonary gradient emerged as a predictor for delayed hospital recovery (P = 0.002), late mortality (P = 0.016) and reoperation (P = 0.015) in multivariable analysis. CONCLUSIONS: Contemporary TCPC can be performed with low risk and provides excellent survival in the long-term. Classic morbidities of the original Fontan procedure, such as Fontan pathway revision, tachyarrhythmia and thromboembolism seem mitigated. However, exercise limitations, PLE and liver dysfunction remain an issue. AVV insufficiency and ventricular dysfunction are still a concern. [ABSTRACT FROM AUTHOR]

Background After the Fontan-Björk modification for tricuspid atresia, some patients show pulsatile systolic pulmonary flow. We compared the hemodynamic findings and the clinical presentation of patients with and without pulsatile systolic flow after atrioventricular connection. Methods According to the pulmonary flow pattern by pulsed-wave Doppler assessment of transthoracic echocardiography, 41 patients after atrioventricular connection were divided into two groups: patients who showed dominant pulsatile systolic pulmonary flow (group P, n = 11), and patients who did not (group N, n = 30). Results Mean follow-up time was 27.8 ± 4.7 years in group P and 25.3 ± 3.8 years in group N ( p = 0.1). Patients in group P had significantly less frequently catheter ablation procedures for tachyarrhythmia (9% versus 50%, p = 0.03). No patient in group P had had cardiac decompensation, whereas 7 patients (23%) in group N had had an episode of cardiac decompensation ( p = 0.08). Cardiopulmonary exercise testing revealed that patients in group P showed higher oxygen uptake compared with patients in group N (25.0 ± 7.3 versus 19.6 ± 6.0 mL · kg −1 · min −1 , p = 0.03). Patients in group P showed higher systolic pulmonary artery pressure (21.3 ± 8.4 versus 16.8 ± 4.5 mm Hg, p = 0.05), higher right ventricular end-diastolic volume index (88.6 ± 30.2 versus 50.3 ± 28.5 mL · L −1 · m −2 , p = 0.03), and higher right ventricle to left ventricle ratio of end-diastolic volume index (1.4 ± 0.6 to 0.7 ± 0.3, p = 0.01). Conclusions Patients with pulsatile systolic flow in the pulmonary artery had better hemodynamic and better exercise performance compared with patients without pulsatile systolic flow after atrioventricular connection. A sufficient volume and function of the right ventricle is a prerequisite to create pulsatile systolic flow. [ABSTRACT FROM AUTHOR]

Objectives Patients with a single ventricle infrequently undergo total cavopulmonary connection as preadolescents, adolescents, or adults. The purpose of this study was to clarify the characteristics of this cohort and to analyze the factors influencing outcomes. Methods Between 1994 and 2015, 50 of 460 patients underwent total cavopulmonary connection as preadolescents, adolescents, or adults (group A). The patients' characteristics and operative results were compared with those of the remaining 410 patients aged less than 9 years who underwent total cavopulmonary connection (group B). Post–total cavopulmonary connection echocardiogram reports (n = 4862) were used to evaluate longitudinal ventricular function, and ejection fraction was characterized using nonlinear mixed-effects models and compared between groups. Results The median follow-up time was 10.3 (2.8-15.5) years. The differences between groups in 30-day mortality ( P = .20), intensive care unit stay ( P = .20), and incidence of prolonged effusion ( P = .08) were not significant. The estimated survival at 15 years was lower in group A (86.5%) than in group B (94.0%, P = .04) patients. Long-term systemic ventricular ejection fraction, analyzed with linear mixed-effect models, was significantly reduced in group A than in group B patients ( P < .001). At a median postoperative period of 8.4 (7.1-10.5) years, the peak oxygen uptake as measured by exercise capacity testing was lower in group A than in group B patients, respectively (22.3 ± 6.5 [n = 25] vs 30.6 ± 8.1 [n = 100] mL/kg/min, P < .001). Conclusions The total cavopulmonary connection procedure was performed in preadolescent, adolescent, and adult patients with no significant difference in 30-day or hospital mortality compared with those in young children. However, long-term survival and ventricular performance were reduced in this older cohort. [ABSTRACT FROM AUTHOR]

Objective The study objective was to determine the mechanisms of atrioventricular valve regurgitation in single-ventricle physiology and their influence on outcomes after total cavopulmonary connection. Methods Among 460 patients who underwent a total cavopulmonary connection, 101 (22%) had atrioventricular valve surgery before or coincident with total cavopulmonary connection. Results Atrioventricular valve morphology showed 2 separated in 33 patients, mitral in 11 patients, tricuspid in 41 patients, and common in 16 patients. Patients with a tricuspid and a common atrioventricular valve underwent atrioventricular valve surgery frequently, 27% and 36%, respectively. Atrioventricular valve regurgitation was due to 1 or more of the following mechanisms: dysplastic leaflet (62), prolapse (53), annular dilation (27), cleft (22), and chordal anomaly (14). Structural anomalies were observed in 89 patients (88%). The procedure was atrioventricular valve repair in 81 patients, atrioventricular valve closure in 16 patients, and atrioventricular valve replacement in 4 patients. Among 81 patients who underwent initial repair, repeat repair was required in 20 patients, atrioventricular valve replacement was required in 7 patients, and atrioventricular valve closure was required in 3 patients. Among patients undergoing atrioventricular valve surgery, overall survival after total cavopulmonary connection (88% vs 95% at 15 years, P = .01), freedom from atrioventricular valve reoperation after total cavopulmonary connection (75% vs 99% at 15 years, P < .01), and grade of atrioventricular valve regurgitation at a median follow-up of 6.6 years ( P < .01) were worse than in those who did not require atrioventricular valve surgery. Conclusions Atrioventricular valve regurgitation in univentricular heart is more frequently associated with a tricuspid or a common atrioventricular valve, and structural anomalies are the primary cause. Significant atrioventricular valve regurgitation requiring surgery influences survival after total cavopulmonary connection, especially when atrioventricular valve replacement was needed. Surgical management based on mechanisms of regurgitation is mandatory. [ABSTRACT FROM AUTHOR]

Background: Abnormal atrioventricular and intraventricular electrical conduction and dysfunction of the functional right ventricle (fRV) are common in Ebstein anomaly (EA). However, fRV mechanical dyssynchrony and its relation to fRV function are poorly characterized. We evaluated fRV mechanical dyssynchrony in EA patients in relation to fRV remodeling, dysfunction, and exercise intolerance., Methods: We retrospectively analyzed data from nonoperated EA patients and age-matched controls who underwent echocardiography, cardiovascular magnetic resonance imaging, and cardiopulmonary exercise testing to quantify right ventricular (RV) remodeling, dysfunction, and exercise capacity. The relation of these to fRV dyssynchrony was retrospectively investigated. Right ventricular mechanical dyssynchrony was defined by early fRV septal activation (right-sided septal flash), RV lateral wall prestretch/late contraction, postsystolic shortening, and intra-RV delay using two-dimensional strain echocardiography. The SD of time to peak shortening among the fRV segments was calculated as a parameter of mechanical dispersion., Results: Thirty-five EA patients (10 of whom were <18 years of age) and 35 age-matched controls were studied. Ebstein anomaly patients had worse RV function and increased intra-RV dyssynchrony versus controls. Nineteen of 35 (54%) EA patients had early septal activation with simultaneous stretch and consequent late activation and postsystolic shortening of RV lateral segments. Intra-fRV mechanical delay correlated with fRV end-diastolic volume index (r = 0.43, P < .05) and fRV end-systolic volume index (r = 0.63, P < .001). The fRV ejection fraction was lower in EA with versus without right-sided septal flash (44.9 ± 11.0 vs 54.2 ± 8.2, P = .012). The fRV mechanical dispersion correlated with the percentage of predicted peak VO 2 (r = -0.35, P < .05)., Conclusions: In EA, fRV mechanical dyssynchrony is associated with fRV remodeling, dysfunction, and impaired exercise capacity. Mechanical dyssynchrony as a therapeutic target in selected EA patients warrants further study., (Copyright © 2023 American Society of Echocardiography. Published by Elsevier Inc. All rights reserved.)

Objectives: Studies focused on infants with univentricular heart undergoing stage I palliation other than the Norwood procedure remain a topic of great interest. This study evaluated the outcome of infants who underwent systemic to pulmonary shunt (SPS) or pulmonary artery banding (PAB)., Methods: Infants who underwent SPS or PAB as stage I palliation between 1994 and 2019 were included. Survival and late systemic ventricular function were evaluated., Results: Out of 242 patients, 188 underwent SPS (77.7%) and 54 PAB (22.3%). Main diagnosis included tricuspid atresia, unbalanced atrioventricular septal defects, double inlet left ventricles and single ventricles with other morphology. Thirty-eight patients died before stage II palliation (15.7%). Stage II palliation was performed in 182 patients (75.2%), and mortality between stages II and III was 11 (5.6%). Stage III palliation was performed in 160 (66.1%) patients. Survival at 1, 5 and 15 years after stage I procedure was 81.9, 77.1 and 76.2%, respectively, and similar between both procedures (P = 0.97). Premature birth [P = 0.03, hazard ratio (HR) = 2.1], heterotaxy (P = 0.006, HR = 2.4) and dominant right ventricle (P = 0.015, HR = 2.2) were factors associated to mortality. Unbalanced atrioventricular septal defect (P = 0.005, HR = 4.6) was a factor associated to systemic ventricular dysfunction., Conclusions: In patients with univentricular heart who underwent SPS and PAB as stage I palliation, survival at 15 years was 76%, regardless of th chosen approach. Premature birth, heterotaxy and dominant right ventricle were associated to mortality., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Background: This study investigated the volume and duration of pleural and mediastinal effusions following extracardiac total cavopulmonary connection, as well as preoperative risk factors and their impact on outcome., Materials and Methods: A total of 210 patients who underwent extracardiac total cavopulmonary connection at our center between 2012 and 2020 were included in this study. Postoperative daily amount of pleural and mediastinal drainage were collected and factors influencing duration and amount of effusions were analyzed. The impact of effusions on adverse events was analyzed., Results: Median age at extracardiac total cavopulmonary connection was 2.2 (interquartile range, 1.8-2.7) years with median weight of 11.6 (10.7-13.0) kg. Overall duration of drainage after extracardiac total cavopulmonary connection was 9 (6-17) days. The total volume of mediastinal, right pleural, and left pleural drainage was 18.8 (11.9-36.7), 64.4 (27.4-125.9), and 13.6 (0.0-53.5) mL/kg, respectively. Hypoplastic left heart syndrome ( p = 0.004) and end-diastolic pressure ( p = 0.044) were associated with high volume of drainages, and hypoplastic left heart syndrome ( p = 0.007), presence of aortopulmonary collaterals ( p = 0.002), and high end-diastolic pressure ( p = 0.023) were associated with long duration of drainages. Dextrocardia was associated with higher volume ( p < 0.001) and longer duration ( p = 0.006) of left pleural drainage. Duration of drainage was associated with adverse events following extracardiac total cavopulmonary connection ( p = 0.015)., Conclusion: Volume and duration of pleural and mediastinal effusions following extracardiac total cavopulmonary connection were related with hypoplastic left heart syndrome, aortopulmonary collaterals, and end-diastolic pressure. The duration of drainage for effusions was a risk factor for adverse events after total cavopulmonary connection., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Heinisch, Metz, Staehler, Mayr, Vodiskar, Strbad, Ruf, Ewert, Hager, Hörer and Ono.)

Background: Long-term morbidity and mortality outcomes of the arterial switch operation (ASO) in patients with transposition of the great arteries and Taussig-Bing anomaly are excellent. With an increasing number of patients reaching adolescence and adulthood, more attention is directed toward quality of life. Our study aimed to determine the health-related quality of life (hrQoL) outcomes in patients after the ASO and identify factors influencing their hrQoL., Methods: In this cross-sectional study, hrQoL of patients after ASO was assessed with the German version of the Short Form-36 (SF-36) and the potential association of specified clinical factors was analyzed. Patients of at least 14 years of age who underwent ASO in our institution from 1983 were considered eligible., Results: Of the 355 questionnaires sent to eligible patients, 261 (73%) were available for analysis. Compared to the reference population, patients who had undergone ASO had a significantly higher score in all subscales of the SF-36 except for vitality ( P < .01). Patients with an implanted pacemaker ( P = .002), patients who required at least one reoperation ( P < .001), and patients currently taking cardiac medication ( P < .004) or oral anticoagulation ( P = .036) had lower physical component scores compared to patients without these factors., Conclusions: Patients' self-assessed and self-reported hrQoL after ASO (using German version of the Short Form 36) is very good. In this population, hrQoL is influenced by reoperation, the need for a pacemaker, and current cardiac medication or anticoagulant use. The development of strategies designed to mitigate or minimize the requirements for, and/or impact of these factors may lead to better hrQoL in this patient population.

Background: A Konno operation with a mechanical prosthesis may be applied in patients with complex left ventricular outflow tract obstruction to avoid further operations. We reviewed our 20-year experience with the Konno operation., Methods: All patients who underwent the Konno operation between 1996 and 2015 were evaluated. Study end points were survival and reoperations., Results: Twenty-one consecutive patients were included. The median age at operation was 12 years (5 months to 34 years). Twenty (95%) patients had prior interventions for left-sided heart lesions. Additional mitral valve disease was present in 17 (81%) patients. The preoperative mean pressure gradient over the left ventricular outflow tract was 50 ± 25 mm Hg. The median size of implanted valve prostheses was 21 mm (16-25 mm). Concomitant procedures for left-sided heart lesions were performed in six patients, including two mitral valve replacements. There were two hospital mortalities (9.5%) and four late mortalities (19%). Overall survival was 85% ± 7.8% and 72% ± 11% at 5 and 10 years, respectively. In two patients, mitral valve replacement was performed during the same hospital admission. During a mean follow-up time of 7.6 ± 4.8 years, two patients required late reoperations, one for mitral valve replacement and one for heart transplantation. Freedom from late reoperation at 10 years was 89% ± 7.4%., Conclusions: The Konno operation can be considered as a definitive option with a low probability of reoperation on the left ventricular outflow tract in patients with complex left ventricular heart disease. Subsequent operations focus on the treatment of additional mitral valve disease, which remains the cause of mortality and morbidity.

Objectives: This study aims to evaluate the clinical outcome following total cavopulmonary connection (TCPC) and to identify factors affecting early and late outcome., Methods: Between May 1994 and March 2015, 434 patients underwent TCPC with 50 lateral tunnels and 374 extracardiac conduits. The clinical outcome, exercise capacity and liver examination results were retrospectively reviewed., Results: Thirty-day survival was 98.2%, and the estimated survival rate at 15 years was 92.3%. Freedom from tachyarrhythmia at 15 years was 91.0%. Other late morbidities included bradyarrhythmia in 17, protein-losing enteropathy (PLE) in 15, thromboembolism in 3 and plastic bronchitis in 3 patients. At last follow-up, normal systemic ventricular function (ejection fraction >50%) was observed in 88.2%. Atrioventricular valve (AVV) regurgitation was mild or less in 90% of patients with systemic left ventricle, in 63% of those with systemic right ventricle and 58% of the patients with unbalanced atrioventricular septal defect or common inlet ventricles. Cardiopulmonary exercise capacity showed impaired peak oxygen uptake (71% of normal) in a sub-group of 120 patients at a mean of 9 years postoperatively. Biochemistry of 338 patients at last follow-up revealed a gamma-glutamyl transferase value beyond normal in 90 patients (26%), with a positive correlation between the level and the time after the initial operation (P < 0.01). Pre-TCPC high transpulmonary gradient emerged as a predictor for delayed hospital recovery (P = 0.002), late mortality (P = 0.016) and reoperation (P = 0.015) in multivariable analysis., Conclusions: Contemporary TCPC can be performed with low risk and provides excellent survival in the long-term. Classic morbidities of the original Fontan procedure, such as Fontan pathway revision, tachyarrhythmia and thromboembolism seem mitigated. However, exercise limitations, PLE and liver dysfunction remain an issue. AVV insufficiency and ventricular dysfunction are still a concern., (© The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: Currently, there are few specific risk stratification models available to predict mortality following congenital heart surgery in adults. We sought to evaluate whether the predictive power of the common pediatric scores is applicable for adults. In addition, we evaluated a new grown-ups with congenital heart disease (GUCH) score specifically designed for adults undergoing congenital heart surgery., Methods and Results: Data of all consecutive patients aged 18 years or more, who underwent surgery for congenital heart disease (CHD) between 2004 and 2013 at our institution, were collected. We evaluated the Aristotle Basic Complexity (ABC), the Aristotle Comprehensive Complexity (ACC), the Risk Adjustment in Congenital Heart Surgery (RACHS-1), and the Society of Thoracic Surgeons (STS)-European Association for Cardiothoracic Surgery (EACTS) scores. The proposed GUCH score consists of the STS-EACTS score, the procedure-dependent and -independent factors of the ACC score, and age. The discriminatory power of the scores was assessed using the area under the receiver-operating characteristics curve (c-index). A total of 830 operations were evaluated. Hospital mortality was 2.9%. C-indexes were 0.67, 0.80, 0.62, 0.78, and 0.84 for the ABC, ACC, RACHS-1, STS-EACTS, and GUCH mortality scores, respectively., Conclusion: The evidence-based EACTS-STS score outperforms the expert-based ABC score. The expert-based ACC score is superior to the evidence-based EACTS-STS score since comorbidities are considered. Our proposed GUCH score outperforms all other scores since it integrates the advantages of the evidence-based EACTS-STS score for procedures and the expert-based ACC score for comorbidities. Evidence-based scores for adults with CHD should include comorbidities and patient ages., (© The Author(s) 2016.)