Flow Dynamics of Bilateral Superior Cavopulomonary Shunts Influence Outcomes After Fontan Completion.

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.

Impact of Characteristics at Stage-2-Palliation on Outcome Following Fontan Completion.

The optimal timing of stage-2-palliation (S2P) in single left ventricle is not clear. The aim of this study was to identify S2P related factors associated with outcomes after total cavopulmonary connection (TCPC), particularly relative to the dominant systemic ventricle. A total of 405 patients who underwent both S2P and TCPC at our institute between 1997 and 2017 was included. Patients were divided into two groups, dominant right ventricle (RV type, n = 235) and dominant left ventricle (LV type, n = 170). S2P related factors associated with mortality, postoperative ventricular function, and late exercise capacity following TCPC, were analyzed. The median age at S2P was 4 [3-7] and 6 [3-11] months in RV and LV type patients, respectively (p = 0.092). Survival after TCPC was similar in RV and LV type patients (p = 0.280). In those with RV type, risk factors for mortality following TCPC were older age (p < 0.001), heavier weight (p = 0.001), higher PAP (p < 0.001), higher TPG (p = 0.010), and lower SO2 (p = 0.008) at S2P. In those with LV type, no risk factor was identified. Risk factors for postoperative impaired ventricular function were older age and higher weight at S2P in both RV and LV type patients. Older age at S2P was also identified as a risk for inferior peak oxygen uptake (VO2) years after TCPC both in RV and LV type patients. Older age at S2P was associated with higher mortality after Fontan completion only in RV type patients. However, it was associated with postoperative ventricular dysfunction and lower exercise capacity after TCPC in both RV and LV type patients.

Impact of Extracardiac Anomalies on Mortality and Morbidity in Staged Single Ventricle Palliation.

BACKGROUND: This study was intended to determine the impact of extracardiac anomalies on outcomes in patients with functional single ventricle who underwent staged palliation. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.

Impact of Anatomical Sub-types and Shunt Types on Aortopulmonary Collaterals in Hypoplastic Left Heart Syndrome.

This study aims to clarify the relation of development of aortopulmonary collateral arteries (APCs) with anatomical sub-types and the shunt types at Norwood procedure in patients with hypoplastic left heart syndrome (HLHS). A total of 140 patients with HLHS who completed 3 staged palliation between 2003 and 2019 were included. Incidence of APCs and corresponding interventions were examined using angiogram by cardiac catheterization, with respect to the anatomical sub-types and shunt types. Totally, APCs were observed in 87 (62%) of the patients; pre-stage II in 32 (23%), pre-stage III in 64 (46%), and after stage III in 40 (29%). The incidence of APCs before stage II was significantly higher in patients with aortic atresia/mitral atresia (AA/MA) compared with other sub-types (P = 0.022). Patients with right ventricle to pulmonary artery conduit (RVPAC) had a higher incidence of APCs originating from the descending aorta, compared with those with modified Blalock-Taussig shunt (20% vs 2%, P= 0.002). Interventions for APCs were performed in 58 (41%) patients; before stage II in 10 (7%), after stage II in 7 (5%), before stage III in 22 (16%), and after stage III in 32 (23%). Patients with AA/MA had more interventions before stage II (P= 0.019), and patients with aortic stenosis/mitral stenosis (AS/MS) had a lower incidence of interventions after stage III (P= 0.047). More than half of the patients with HLHS developed APCs. Before stage II, patients with AA/MA sub-type had a higher incidence of APCs, and those with RVPAC had significantly more APCs from the descending aorta.

The impact of pulmonary artery size on midterm outcomes after nonfenestrated Fontan operation.

OBJECTIVE: We sought to identify the impact of pulmonary artery size on outcomes after nonfenestrated total cavopulmonary connection. In a subgroup of patients with right-sided bidirectional cavopulmonary shunt, the impact of each branch pulmonary artery size was individually determined. METHODS: The medical records of all patients who underwent nonfenestrated total cavopulmonary connection between 2009 and 2021 were reviewed. The pulmonary artery index was calculated using angiography before the operation. RESULTS: A total of 247 patients were included in this study. A right-sided bidirectional cavopulmonary shunt was performed in 217 patients (88%). Median pulmonary artery index was 162 (133-207) mm2/m2 before total cavopulmonary connection. Chylothorax occurred in 55 patients (22%). Pulmonary artery index was an independent factor for chylothorax (odds ratio, 0.98, 95% confidence interval, 0.97-0.99, P < .001) with a cutoff value of 170 mm2/m2. In a subgroup of patients with right-sided bidirectional cavopulmonary shunt, the left pulmonary artery index was identified as an independent risk factor for longer stay in the intensive care unit (coefficient B -0.02, 95% confidence interval, -0.04 to -0.002, P = .034) and for adverse events (hazard ratio, 0.98, 95% confidence interval, 0.96-0.99, P = .011) with a cutoff value of 56 mm2/m2. CONCLUSIONS: The pulmonary artery index is significantly associated with the occurrence of chylothorax after nonfenestrated total cavopulmonary connection with a cutoff value of 170 mm2/m2. In patients with right-sided bidirectional cavopulmonary shunt, left pulmonary artery index has a significant predictive value for longer stay in the intensive care unit and adverse events with a cutoff value of 56 mm2/m2.

Impact of Total Anomalous Pulmonary Venous Connection in Staged Single Ventricle Palliation.

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

Right Ventricular Electromechanical Dyssynchrony and Its Relation to Right Ventricular Remodeling, Dysfunction, and Exercise Capacity in Ebstein Anomaly.

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 VO2 (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.

Impact of hypoxemia and re-interventions on clinical outcomes after bidirectional cavopulmonary shunt.

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.

Aortopulmonary collaterals in single ventricle: incidence, associated factors and clinical significance.

OBJECTIVES: Clinical significance of aortopulmonary collaterals (APCs) in patients with univentricular heart remains controversial. This study aimed to evaluate the incidence and associated factors for APCs and their influence during staged palliation. METHODS: In total, 430 patients who underwent staged palliation by bidirectional Glenn shunt and total cavopulmonary connection between 2003 and 2019 were examined. APCs were determined by angiogram. Incidence and interventions for APCs were analysed. RESULTS: The most frequent diagnosis was hypoplastic left heart syndrome in 146 (34%) patients. The median age at Glenn and Fontan was 4.9 months and 2.1 years, respectively. APCs were observed in 54 (13%) patients at Glenn and in 179 (42%) at Fontan. Closure of APCs was performed before Glenn in 12 (3%) patients, at Glenn in 13 (3%), after Glenn in 8 (2%), before Fontan in 44 (10%), at Fontan in 26 (6%) and after Fontan in 52 (12%). Hypoplastic left heart syndrome (P < 0.01) was highly associated with the development of APCs before Glenn. Lower Nakata-Index and younger age at Glenn shunt were associated with the development of APCs at Fontan procedure. The presence of APCs or intervention for APCs before total cavopulmonary connection did not influence intensive care unit stay or mortality after total cavopulmonary connection. CONCLUSIONS: APCs were most frequently observed before Fontan procedure. Hypoplastic left heart syndrome was highly associated with the development of APCs before Glenn shunt. Lower Nakata-Index and younger age at Glenn shunt were associated with APCs before Fontan procedure.

Risk Factors for Thrombus Formation at Stage 2 Palliation and Its Effect on Long-Term Outcome in Patients With Univentricular Heart.

Thrombus formation is a feared complication following bidirectional cavopulmonary shunt (BCPS). We aimed to investigate the effect of thrombus formation on outcome. BCPS was performed in 525 patients at our center between 1998 and 2018. The impacts of thrombus formation on survival and probability of Fontan completion were analyzed, and risk factors for thrombus formation were examined. Thrombus formation occurred in 30 patients (5.7%). Compared with the remaining 495 patients, there was no significant difference in the median age at BCPS (4.9 vs 4.7 months; P = 0.587). However, unbalanced atrioventricular septal defects (17 vs 5%; P = 0.008) and preoperative ventricular dysfunction (23.3 vs 8%; P = 0.004) were more frequent in patients who developed a thrombus. Thrombolytic therapy was performed in all patients and surgical thrombus removal was required in 13 patients. In-hospital mortality was higher in patients with thrombus (30.0 vs 2.2%; P < 0.001). Of 505 hospital survivors, an estimated survival at 1 year after hospital discharge following BCPS was 84.4% (95% CI, 76.1-92.7%) in patients with thrombus and 96.8% (95% CI, 96.0-97.6%) in those without (P < 0.001). Cumulative incidence of Fontan completion at 3 years after BCPS was 52.8% (95% CI, 30.3-75.2%) in patients with thrombus and 90.1% (95% CI, 87.2-92.9%) in those without (P = 0.004). Higher left atrial pressure (OR = 1.165; P = 0.029) and longer cardiopulmonary bypass time (OR = 1.013, P = 0.001) at BCPS were independent risk factors for thrombus formation after BCPS. Thrombus formation after BCPS poses a significant risk for survival and Fontan completion. Preoperative higher left atrial pressure and longer cardiopulmonary bypass time are significant risk factors.

Improved Long-term Outcome of Damus-Kaye-Stansel Procedure Without Previous Pulmonary Artery Banding.

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

Outcome after stage 1 palliation in non-hypoplastic left heart syndrome patients as a univentricular palliation.

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.

Pleural and mediastinal effusions after the extracardiac total cavopulmonary connection: Risk factors and impact on outcome.

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.

Surgical reintervention on the neo-aorta after the Norwood operation.

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.

Single-centre outcome of extracorporeal membrane oxygenation after the neonatal Norwood procedure.

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.

Influence of Shunt Type on Survival and Right Heart Function after the Norwood Procedure for Aortic Atresia.

The study objective was to compare the results after Norwood procedure between modified Blalock-Taussig shunt (MBTS) and right ventricle-to-pulmonary artery conduit (RVPAC) according to Sano in patients with hypoplastic left heart syndrome (HLHS) and aortic atresia (AA). A total of 146 neonates with HLHS and AA who underwent the Norwood procedure at our institution between 2001 and 2020 were divided into 2 groups according to shunt type (MBTS or RVPAC). Survival after the Norwood procedure was compared between the groups. Longitudinal right ventricular and tricuspid valve function in each group were evaluated using cubic splines method. RVPAC was performed in 103 patients and MBTS in 43 according to surgeon preference. There were no differences in the 30-day mortality rates (16.5% vs 16.3%, P  = 0.973). Survival at 0.5, 1 and 3 years was 79.6%, 74.6%, and 68.9% in RVPAC and 66.8%, 64.3%, and 58.5% in MBTS (P  =  0.293). Among 23 patients undergoing tricuspid valve procedure, different mechanisms of tricuspid regurgitation were observed between the groups. Longitudinal analysis revealed greater prevalence of late right ventricular dysfunction in RVPAC patients. In 77 patients who completed Fontan procedure, the postoperative N-terminal pro B-type natriuretic peptide value was significantly higher in RVPAC vs MBTS (554 vs 276 ng/L, P  =  0.007). No survival advantage of RVPAC over MBTS was observed in neonates with HLHS and AA undergoing the Norwood procedure. Longitudinal analysis demonstrated a greater prevalence of right ventricular dysfunction and higher N-terminal pro B-type natriuretic peptide values during late follow-up in patients with RVPAC.

Management of failing bidirectional cavopulmonary shunt: Influence of additional systemic-to-pulmonary-artery shunt with classic Glenn physiology.

Objectives: Severe hypoxemia in the early postoperative period after bidirectional cavopulmonary shunt (BCPS) is a critical complication. We aimed to evaluate patients who underwent additional systemic to pulmonary shunt and septation of central pulmonary artery (partial takedown) after BCPS. Methods: The medical records of all patients who underwent BCPS between 2007 and 2020 were reviewed. Patients who underwent partial takedown were extracted and their outcomes were analyzed. Results: Of 441 BCPS patients, 27 patients (6%) required partial takedown. Most frequent diagnosis was hypoplastic left heart syndrome (n = 14; 52%). Additional complicating factors included pulmonary artery hypoplasia (n = 12) and pulmonary venous obstruction (n = 3). Thirteen patients (48%) underwent partial takedown on the same day of BCPS, and all of them survived the procedure. The remaining 14 patients (52%) underwent partial takedown between postoperative 1 to 64 days. The reasons for partial takedown were: postoperative high pulmonary vascular resistance (n = 4), early BCPS (<90 days) with PA hypoplasia (n = 3), mediastinitis/pneumonia (n = 3), pulmonary venous obstruction (n = 2), ventricular dysfunction (n = 1), and recurrent pneumothorax (n = 1). Four patients experienced hospital deaths. Six patients died after discharge, 10 achieved Fontan completion, and 6 were alive and waiting for Fontan. Overall survival after partial takedown was 54% at 3 years. The pulmonary venous obstruction (P = .041) and genetic/extracardiac anomalies (P = .085) were identified as risks for mortality after partial takedown. Conclusions: The partial takedown resulted in a 3-year survival rate of more than 50%. Of these patients, a significant number underwent successful Fontan completion who would exhibit potential early death with conservative treatment.

Clinical and haemodynamic variables associated with intensive care unit length of stay and early adverse outcomes after the Norwood procedure.

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.

Factors Affecting Health-Related Quality of Life After the Arterial Switch Operation.

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.

Common atrioventricular valve surgery in children with functional single ventricle.

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.