Search

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.

We analyzed the early and long-term survival after ABO-compatible heart transplantation in children under 3 years of age from 1991 to 2021 at our center. This retrospective and descriptive study aimed to identify serious adverse events associated with mortality after pediatric heart transplantation.46 patients with congenital heart failure (37%) in end-stage heart failure have undergone a pediatric heart transplantation. Primary outcome of interest was survival at follow-up time.Median (IQR) follow-up time (y), age (y), body-weight (kg) and BMI (kg/cmPediatric heart transplantation has acceptable long-term results and is still the best therapeutic option in children with end-stage cardiac failure. Underlying anomalies and single ventricle physiology, age below 3 months had a significant impact on survival.

Background While early outcome after the Norwood operation for hypoplastic left heart syndrome has improved, inter-stage mortality until bidirectional cavopulmonary shunt (BCPS) remains a concern. Our aim was to institute a home monitoring program to (HMP) reduce inter-stage mortality. Methods Among 264 patients who survived Norwood procedure and were discharged before BCPS, 80 patients were included in the HMP and compared to the remaining 184 patients regarding inter-stage mortality. In patients with HMP, events during the inter-stage period were evaluated. Results Inter-stage mortality was 8% (n = 21) and was significantly lower in patients with HMP (2.5%, n = 2), compared to those without (10.3%, n = 19, p = 0.031). Patients with inter-stage mortality had significantly lower birth weight (p

Background: About 5–10% of adults with congenital heart disease (ACHD) will develop pulmonary arterial hypertension (PAH), which is associated with significant mortality. Studies on risk factors for poor outcome in a contemporary cohort of these patients with PAH associated with CHD (PAH-CHD) are rare. Methods: In this retrospective, single-center study, adult patients with the diagnosis PAH-CHD who had at least one contact as an outpatient or inpatient at the German Heart Centre Munich during the period January 2010–September 2019 were included. Patients with PAH without a CHD were excluded. The primary endpoint was all-cause mortality. Results: Altogether, 158 patients (mean age 39.9 ± 15.4 years, female 64.6%) were included in the study. A pre-tricuspid shunt was present in 17.7%, other shunts in 51.3%, PAH associated with complex CHD in 22.8%, and segmental PAH in 8.2%. An NT-proBNP measurement at baseline was available in 95 patients (60.1%). During a median follow-up of 5.37 years [IQR 1.76–8.63], the primary endpoint occurred in 10 patients (6.7%). On univariate analysis, CRP (log) (HR 3.35, 95% CI (1.07–10.48), p = 0.037), NT-proBNP (log) (HR: 7.10, 95% CI: 1.57–32.23, p = 0.011), and uric acid (HR: 1.37, 95% CI: 1.05–1.79, p = 0.020) were predictors of the primary endpoint. On multivariate analysis, only NT-proBNP (log) (HR: 6.91, 95% CI: 1.36–35.02, p = 0.0196) remained as an independent predictor. Conclusion: NT-proBNP is an independent predictor of all-cause mortality in a contemporary cohort of PAH-CHD patients. The role of CRP and uric acid should be further assessed in future studies.

Systemic-to-pulmonary shunt malfunction contributes to morbidity in children with complex congenital heart disease after palliative procedure. Neointimal hyperplasia might play a role in the pathogenesis increasing risk for shunt obstruction. The aim was to evaluate the role of epidermal growth factor receptor (EGFR) and matrix-metalloproteinase 9 (MMP-9) in the formation of neointimal within shunts. Immunohistochemistry was performed with anti-EGFR and anti-MMP-9 on shunts removed at follow-up palliative or corrective procedure. Whole-genome single-nucleotide polymorphisms genotyping was performed on DNA extracted from patients´ blood samples and allele frequencies were compared between the group of patients with shunts displaying severe stenosis (≥ 40% of lumen) and the remaining group. Immunohistochemistry detected EGFR and MMP-9 in 24 of 31 shunts, located mainly in the luminal area. Cross-sectional area of EGFR and MMP-9 measured in median 0.19 mm2 (IQR 0.1–0.3 mm2) and 0.04 mm2 (IQR 0.03–0.09 mm2), respectively, and correlated positively with the area of neointimal measured on histology (r = 0.729, p r = 0.0479, p = 0.018, respectively). There was a trend of inverse correlation between the dose of acetylsalicylic acid and the degree of EGFR, but not MMP-9, expression within neointima. Certain alleles in epidermal growth factor (EGF) and tissue inhibitor of metalloproteinases 1 (TIMP-1) were associated with increased stenosis and neointimal hyperplasia within shunts. EGFR and MMP-9 contribute to neointimal proliferation in SP shunts of children with complex cyanotic heart disease. SP shunts from patients carrying certain risk alleles in the genes encoding for EGF and TIMP-1 displayed increased neointima.

Objective This study aimed to determine the longitudinal change of systemic ventricular function and atrioventricular valve regurgitation after total cavopulmonary connection. Methods In 620 patients who underwent total cavopulmonary connection between 1994 and 2021, 4219 longitudinal echocardiographic examinations of systemic ventricular function and atrioventricular valve 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 in 91 patients. Dominant right ventricle was observed in 329 (53%) and dominant left ventricle in 291 (47%). Median age at total cavopulmonary connection was 2.3 (1.8–3.4) years. Transplant-free survival at 5, 10, and 15 years after total cavopulmonary connection was 96.3, 94.7, and 93.6%, respectively in patients with dominant right ventricle and 97.3, 94.6, and 94.6%, respectively in those with dominant left ventricle (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 right ventricle, compared with those with dominant left ventricle (15 years: p = 0.007, 20 years: p = 0.03). Atrioventricular valve regurgitation was more frequent after total cavopulmonary connection in patients with dominant right ventricle compared with those with dominant left ventricle (p Conclusions There was no transplant-free survival difference and no difference in ventricular function between dominant right ventricle and dominant left ventricle for the first 10 years after total cavopulmonary connection. Thereafter, ventricular function in dominant right ventricle was inferior to that in dominant left ventricle. The degree of atrioventricular valve regurgitation was significantly higher in dominant right ventricle, compared with dominant left ventricle, and it was positively associated with ventricular dysfunction, especially in dominant right ventricle.

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%; P0.001). 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 (P0.001). 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.

Objective This study aimed to prove if pulmonary artery size influences survival and if an additional aortopulmonary shunt promotes left pulmonary artery growth after bidirectional cavopulmonary shunt in patients with hypoplastic left heart syndrome. Methods The medical records of patients with hypoplastic left heart syndrome who underwent Norwood procedure and bidirectional cavopulmonary shunt between 2007 and 2020 were reviewed. Right, left, and total (right + left) pulmonary artery indices were calculated according to Nakata and colleagues. Results A total of 158 patients were included in this study. Median age at Norwood and bidirectional cavopulmonary shunt 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 bidirectional cavopulmonary shunt was 90.3% at 1 year, and 86.2% at 2 years. Total, right, and left pulmonary artery indices were 238 (195-316), 136 (101-185), and 102 (75-130) mm2/m2 at the time of bidirectional cavopulmonary shunt, and they were 237 (198-284), 151 (123-186), and 86 (69-108) mm2/m2 at the time of Fontan. Left pulmonary artery index decreased significantly between the time of bidirectional cavopulmonary shunt and Fontan (p Conclusions Preoperative pulmonary artery index did not affect the mortality after bidirectional cavopulmonary shunt. The partial takedown and additional aortopulmonary shunt for failing bidirectional cavopulmonary shunt were unable to improve left pulmonary artery size.

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.

Ventricular assist device implantation presents a possible bridge to heart transplantation for patients with failing Fontan physiology. However, evidence regarding outcome and possible pitfalls associated with the Fontan circulation is still insufficient. We describe the course of a 13-year-old male, who was born with hypoplastic left heart syndrome and underwent HeartMate III implantation due to refractory failure of the systemic right ventricle.

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.

OBJECTIVES We sought to evaluate the predictive power of the Society of Thoracic Surgeons–European Association for Cardio-Thoracic Surgery (STAT) mortality score and the adult congenital heart surgery (ACHS) mortality score for the adults undergoing congenital heart operations entered into the European Congenital Heart Surgeons Association (ECHSA) database. METHODS The data set comprised 17 662 major operations performed between 1997 and 2019, on patients 18 years of age or older, in European centres participating in the ECHSA database. Each operation was assigned a STAT mortality score and category and an ACHS mortality score. Operative mortality was based on the 30-day status and on the status at hospital discharge. The discriminatory power of the STAT and ACHS scores was assessed using the area under the receiver operating characteristic curve (c-index). RESULTS A total of 17 214 (97.46%) operations were assigned ACHS scores. The 3 most frequent primary procedures were closure of the atrial septal defect (19.0%), aortic valve replacement (8.8%) and non-valve-sparing aortic root replacement (6.1%). Operative mortality for ACHS-coded operations was 2.07%. The procedures with the highest mortality were atrial septal defect creation/enlargement (19.0%), lung transplantation (18.8%) and heart transplantation (18.2%). A total of 17 638 (99.86%) operations were assigned a STAT score and category. The operative mortality for STAT-coded operations was 2.27%. The c-index for mortality was 0.720 for the STAT mortality score and 0.701 for the ACHS score. CONCLUSIONS The ACHS mortality score and the STAT mortality score reached similar, moderate predictive power in adult patients undergoing congenital heart surgery in ECHSA database.

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.

OBJECTIVES With improvements in early survival after the stage I palliation (S1P) Norwood procedure for hypoplastic left heart syndrome (HLHS) and its variants, inter-stage death accounts for an increasing proportion of mortality. Our aim was to identify the risk factors for inter-stage mortality. METHODS The records of 322 neonates with HLHS or a variant who underwent the Norwood procedure at our centre between 2001 and 2019 were retrospectively analysed. RESULTS The diagnoses included 271 neonates with HLHS (84%) and 51 with variants (16%). Aortic atresia was observed in 138 (43%) patients, mitral atresia in 91 (28%), extracardiac anomalies in 42 (13%) and genetic disorder in 14 (4%). The median age and weight of the patients at the S1P Norwood procedure were 9 (interquartile range: 7–12) days and 3.2 (2.9–3.5) kg, respectively. The median cardiopulmonary bypass time was 137 (107–163) min. Modified Blalock–Taussig shunts were used in 159 (49%) and unvalved right ventricle-to-pulmonary artery shunts in 163 (51%) patients. The number of inter-stage deaths was as follows: between S1P and stage II palliation (S2P), 61 including 38 early (30 days) deaths, and between S2P and stage III palliation, 32 deaths. Low birth weight ( CONCLUSIONS The risk factors for inter-stage attrition after the Norwood procedure were different between each stage. Preoperative factors, including birth weight, restrictive atrial septum and extracardiac anomalies, adversely affected the inter-stage mortality.

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 In our study, we sought to analyse the mid-term results after interventional aortic coarctation (CoA) stenting with sequential dilation of the stent. Methods The data of all 218 patients, who are above the age of 6 years and underwent CoA-stent implantation in our hospital, were retrospectively analysed on the rate of re-interventions, complications and arterial hypertension at a follow-up time of 31 months. To avoid any aortic complications, stents were deployed primarily not in full size and a second cardiac catheterisation for further dilatation was scheduled within 6–12 months after the stent implantation. Results The median peak invasive systolic pressure gradient declined significantly from 26.2 mmHg to 2.7 mmHg after stenting. There was one procedure related death due to an aortic rupture after stent implantation. There were in total 33 (15.1%) procedure-related complications including femoral artery complications, stent fracture and stent dislocation (in 9, 9 and 7 patients, respectively). In 85 patients a re-dilatation and in 25 patients a second stent-implantation was necessary at the first re-intervention. The systolic blood pressure declined significantly from 144 mmHg to 131 mmHg after stenting. The number of patients being normotensive changed from 18% before stenting to 78.5% after stenting with adjusted antihypertensive medication. Conclusion Aortic stenting is an effective means for CoA treatment. With sequential dilation of the stent, a very low rate of life-threatening procedural complications and mortality can be achieved. CoA stenting with proper antihypertensive medications results in better control of blood pressure.

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.

Introduction We sought to determine the surgical outcomes of patients with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) enrolled in the European Congenital Heart Surgeons Association (ECHSA) database. Materials and methods From 1999 to 2019, 907 patients with ALCAPA underwent surgical repair and were included in the current study. The primary outcome was in-hospital mortality. Secondary outcomes included frequency and results of concomitant mitral valve surgery and postoperative mechanical circulatory support (MCS). Results The overall in-hospital mortality was 6% (54/907) and was significantly higher in neonates (p = .01), patients with lower body surface area (BSA) (p = .01), and those requiring postoperative MCS (p = .001). Associated mitral valve surgery was performed in 144 patients (15.9%) and was associated with longer cardiopulmonary bypass (CPB) and aortic cross-clamp times (AOX) (p ≤ .0001) but was not significantly related to an increase in in-hospital mortality. Postoperative MCS was required in 66 patients (7.3%). These patients were younger (p ≤ .001), had a lower BSA (p ≤ .001), and required a longer CPB (p ≤ .001) and AOX time (p ≤ .001). Conclusions ALCAPA repair can be achieved successfully, and with low surgical risk. Concomitant mitral valve procedures can be performed without increasing operative mortality. The use of MCS remains a valuable option, especially in younger patients.

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.

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 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.

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 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.

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. Electronic supplementary material The online version of this article (10.1007/s00246-020-02318-x) contains supplementary material, which is available to authorized users.

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.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.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) mmThe pulmonary artery index is significantly associated with the occurrence of chylothorax after nonfenestrated total cavopulmonary connection with a cutoff value of 170 mm

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 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.

Background Right ventricular outflow tract (RVOT) stenosis after repair of tetralogy of Fallot has been linked with favorable right ventricular remodeling but adverse outcomes. The aim of our study was to assess the hemodynamic impact and prognostic relevance of right ventricular pressure load in this population. Methods and Results A total of 296 patients with repaired tetralogy of Fallot (mean age, 17.8±7.9 years) were included in a prospective cardiovascular magnetic resonance multicenter study. Myocardial strain was quantified by feature tracking technique at study entry. Follow‐up, including the need for pulmonary valve replacement, was assessed. The combined end point consisted of ventricular tachycardia and cardiac death. A higher echocardiographic RVOT peak gradient was significantly associated with smaller right ventricular volumes and less pulmonary regurgitation, but lower biventricular longitudinal strain. During a follow‐up of 10.1 (0.1–12.9) years, the primary end point was reached in 19 of 296 patients (cardiac death, n=6; sustained ventricular tachycardia, n=2; and nonsustained ventricular tachycardia, n=11). A higher RVOT gradient was associated with the combined outcome (hazard ratio [HR], 1.03; 95% CI, 1.00–1.06; P =0.026), and a cutoff gradient of ≥25 mm Hg was predictive for cardiovascular events (HR, 3.69; 95% CI, 1.47–9.27; P =0.005). In patients with pulmonary regurgitation ≥25%, a mild residual RVOT gradient (15–30 mm Hg) was not associated with a lower risk for pulmonary valve replacement. Conclusions Higher RVOT gradients were associated with less pulmonary regurgitation and smaller right ventricular dimensions but were related to reduced biventricular strain and emerged as univariate predictors of adverse events. Mild residual pressure gradients did not protect from pulmonary valve replacement. These results may have implications for the indication for RVOT reintervention in this population.

OBJECTIVES Congenital tricuspid valve dysplasia (TVD) is a rare cardiac disease, often discussed in the same way as Ebstein’s anomaly. Though the morphology of TVD is different, the clinical appearance is similar. The indication for surgical treatment depends on the morphology of the tricuspid valve (TV) and the clinical presentation. METHODS We reviewed all patients below 18 years of age who underwent TV repair between 2005 and 2019 and identified 9 with a TVD. The diagnosis was verified with the operative notes and patients were excluded if the septal leaflet was displaced as in Ebstein’s anomaly. The aim of the study was to describe the morphology of the TV and analyse the surgical possibilities and their results. RESULTS The median age at surgery was 2.3 years (5 days to 14 years), 3 patients were neonates with a duct-dependent pulmonary circulation. Morphology of the TV showed a tethering of at least 1 leaflet in all patients, a restrictive septal leaflet in 8, a large anterior leaflet in 7 and a small posterior leaflet in 4. Surgical techniques included an annuloplasty in 7 patients, a leaflet procedure in 6 patients, an edge-to-edge stitch in 5 patients and a primary Starnes procedure in 1 neonate. During a median follow-up time of 2.8 years (5 months to 15 years), 1 valve replacement and 1 conversion to univentricular palliation were necessary. CONCLUSIONS Unequal leaflet size and tethering are the most common features of TVD. A variety of surgical techniques is available to achieve good results beyond the neonatal period.

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.

Covered stents perform similar to surgically implanted conduits, although the stents work inside of vessels. We present a computed tomography (CT)-based workflow for the implantation of covered stents as extravascular conduits.We selected three different use cases: 1. Connecting a left-sided partially anomalous drainage of a pulmonary vein to the left atrium. 2. Bypassing an outgrown Dacron conduit in aortic recoarctation. 3. Re-directing hepatic venous blood to the left lung in a Fontan patient with heterotaxy, connecting the innominate vein to the right pulmonary artery like a right-sided cavopulmonary connection. By postprocessing and analyzing CT scans for planning and by the use of long needles under biplane fluoroscopy for the realization of the procedure, we projected and performed the exit of a long needle out of a vessel, the re-entering of a target vessel, and the bridging of the extravascular distance by implantation of covered stents.In all three cases, the covered stents were placed successfully, connecting vessels of 15-50 mm distance from each other with very good hemodynamic results. In one case, two stents were placed consecutively, overlapping each other to accomplish an exact fitting at the connection sites to the native vessels.