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Open in new tab Download slide OBJECTIVES This study aimed to determine the longitudinal change of systemic ventricular function and atrioventricular valve (AVV) regurgitation after total cavopulmonary connection (TCPC). METHODS In 620 patients who underwent TCPC between 1994 and 2021, 4219 longitudinal echocardiographic examinations of systemic ventricular function and AVV regurgitation were evaluated retrospectively. RESULTS The most frequent primary diagnosis was hypoplastic left heart syndrome in 172, followed by single ventricle in 131, tricuspid atresia in 95 and double inlet left ventricle (LV) in 91 patients. Dominant right ventricle (RV) was observed in 329 (53%) and dominant LV in 291 (47%). The median age at TCPC was 2.3 (1.8–3.4) years. Transplant-free survival at 5, 10 and 15 years after TCPC was 96.3%, 94.7% and 93.6%, respectively, in patients with dominant RV and 97.3%, 94.6% and 94.6%, respectively, in those with dominant LV (P  = 0.987). Longitudinal analysis of systemic ventricular function was similar in both groups during the first 10 years postoperatively. Thereafter, systemic ventricular function worsened significantly in patients with dominant RV, compared with those with dominant LV (15 years: P  = 0.007, 20 years: P  = 0.03). AVV regurgitation was more frequent after TCPC in patients with dominant RV compared with those with dominant LV (P  < 0.001 at 3 months, 3 years, 5 years, 10 years and 15 years, P  = 0.023 at 20 years). There was a significant correlation between postoperative systemic ventricular dysfunction and AVV regurgitation (P  < 0.001). CONCLUSIONS There were no transplant-free survival difference and no difference in ventricular function between dominant RV and dominant LV for the first 10 years after TCPC. Thereafter, ventricular function in dominant RV was inferior to that in dominant LV. The degree of AVV regurgitation was significantly higher in dominant RV, compared with dominant LV, and it was positively associated with ventricular dysfunction, especially in dominant RV. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES This study aimed to prove if pulmonary artery (PA) size influences survival and if an additional aortopulmonary shunt (APS) promotes left PA growth after bidirectional cavopulmonary shunt (BCPS) in patients with hypoplastic left heart syndrome. METHODS The medical records of patients with hypoplastic left heart syndrome who underwent Norwood procedure and BCPS between 2007 and 2020 were reviewed. Right, left and total (right + left) PA indices were calculated according to Nakata and colleagues. RESULTS A total of 158 patients were included in this study. The median age at Norwood and BCPS was 8 (7–11) days and 3.6 (3.1–4.6) months, respectively. There were 7 hospital deaths and 12 late deaths. Survival after BCPS was 90.3% at 1 year and 86.2% at 2 years. Total, right and left PA indices were 238 (195–316), 136 (101–185) and 102 (75–130) mm2/m2 at the time of BCPS, and they were 237 (198–284), 151 (123–186) and 86 (69–108) mm2/m2 at the time of Fontan. Left PA index decreased significantly between the time of BCPS and Fontan (P  < 0.01). Nine patients needed partial takedown and additional APS due to failing BCPS, but the additional APS did not promote the PA growth significantly. CONCLUSIONS Preoperative PA index did not affect the mortality after BCPS. The partial takedown and additional APS for failing BCPS were unable to improve left PA size. [ABSTRACT FROM AUTHOR]

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]

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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]

Objectives: This study aimed to provide a perspective for the interpretation of exercise capacity (peakVO2) in patients with repaired Tetralogy of Fallot (patients with rTOF) by describing the course of peakVO2 from patients aged 6-63 years.Methods: A retrospective study was performed between September 2001 and December 2016 in the German Heart Centre Munich, Germany, and in the University Medical Centre Groningen, the Netherlands. A total of 1175 cardiopulmonary exercise tests (CPETs) were collected from 586 patients with rTOF, 46% female. Maximal exertion was verified using a respiratory exchange ratio ≥1.00. PeakVO2 was modelled using time-dependent multilevel models for repeated measurements (n=889 in 300 patients), and compared with subject-specific reference values calculated by the models of Bongers et al and Mylius et al. RESULTS: The peakVO2 of patients with rTOF was reduced at all ages. At the age of 6, the peakVO2 was 614 mL/min (70% of predicted (95% CI 67 to 73)). The reduced increase in peakVO2 during adolescence resulted in a significant lower maximum peakVO2 of 1209 mL/min at 25 years (65% predicted, p<0.001). A linear decline after 25 years was observed in patients and references, although patients showed an accelerated decline, with a -0.24% point of predicted (95% CI 0.11 to 0.38) per year without differences between sexes (p=0.263).Conclusions: This study provides a context for peakVO2 across ages in patients with rTOF under contemporary treatment strategies. It showed that the reduction in peakVO2 originates from childhood and declines over time. Sex differences in patients with rTOF were similar to natural existing sex differences. [ABSTRACT FROM AUTHOR]

Objective: Patients with tetralogy of Fallot (ToF) have limited pulmonary blood flow before corrective surgery and ongoing dysfunction of the pulmonary valve and right ventricle throughout life leading to lower exercise capacity and lung volumes in many patients. Inhalation training can increase lung volumes, improve pulmonary blood flow and consequently exercise capacity. This study tests whether home-based daily breathing training improves exercise capacity and lung volumes.Methods: From February 2017 to November 2018, 60 patients (14.7±4.8 years, 39% female) underwent spirometry (forced vital capacity (FVC); forced expiratory volume in 1 s (FEV1)), cardiopulmonary exercise testing (peak oxygen uptake (peak [Formula: see text]O2)) and breathing excursion measurement. They were randomised into immediate breathing exercise or control group (CG) and re-examined after 6 months. The CG started their training afterwards and were re-examined after further 6 months. Patients trained with an inspiratory volume-oriented breathing device and were encouraged to exercise daily. The primary endpoint of this study was the change in peak [Formula: see text]O2. Results are expressed as mean±SEM (multiple imputations).Results: In the first 6 months (intention to treat analysis), the training group showed a more favourable change in peak [Formula: see text]O2 (Δ0.5±0.6 vs -2.3±0.9 mL/min/kg, p=0.011), FVC (Δ0.18±0.03 vs 0.08±0.03 L, p=0.036) and FEV1 (Δ0.14±0.03 vs -0.00±0.04 L, p=0.007). Including the delayed training data from the CG (n=54), this change in peak [Formula: see text]O2 correlated with self-reported weekly training days (r=0.282, p=0.039).Conclusions: Daily inspiratory volume-oriented breathing training increases dynamic lung volumes and slows down deconditioning in peak [Formula: see text]O2 in young patients with repaired ToF. [ABSTRACT FROM AUTHOR]

Objective: This study compared objectively measured and self-reported physical activity (PA) in adults with congenital heart disease (ACHD) to a healthy reference cohort (RC). Patients and Methods: From May 2017 to August 2020, 211 ACHD (39.9 ± 9.7 years, 101 female) and 141 healthy adults (35.9 ± 14.7 years, 76 female) participated in a wearable-based and self-reported PA assessment. Moderate-to-vigorous PA (MVPA) and the step count were recorded with the Garmin vivofit® 3 device for 7 consecutive days. Additionally, subjects were asked to report the number of days they are active for ≥30 min throughout the week. Results: Only 33 (17%) ACHD and 36 (26%) healthy controls (p = 0.030) accumulated the World Health Organization's (WHO) recommendation of 150 min MVPA per week. ACHD were less active per week (ACHD: 40.0 [0.0; 101.0] min. MVPA vs. RC: 75.0 [22.5; 152.5] min. MVPA, p = 0.002) and walked fewer daily steps (ACHD: 8,246 [6,505; 10,434] vs. RC: 9,413 [7,621; 11,654], p = 0.001) than healthy controls. Especially, patients with moderate (p = 0.030), complex (p < 0.001), or surgically corrected (p = 0.008) congenital heart disease accumulated significantly less MVPA than healthy peers throughout the week. A large majority of 72% of ACHD and 58% of the RC overestimated their weekly active days by more than one day. Conclusions: ACHD walked quite a few steps daily but lacked intensity. ACHD was less active than healthy controls and failed to reach international recommendations. They therefore need encouragement toward more intense movement to improve the exercise capacity and lower cardiovascular risk. Self-reported PA showed no agreement to the objectively measured PA. [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]