Mitral valve replacement in children: balancing durability and risk with mechanical and bioprosthetic valves.

OBJECTIVES: To investigate if there is still a place for bioprosthetic mitral valve replacement in children by comparing the prosthetic durability and transplant-free survival after bioprosthetic and mechanical mitral valve replacement. METHODS: We reviewed all mitral valve replacements in children between 1981 and 2020. Bioprosthetic mitral valve replacement cases were individually matched to mechanical mitral valve replacement cases. The incidence rate of a 2nd replacement was calculated using the cumulative incidence function that considered death or transplantation as a competing risk. RESULTS: The median age at implantation was 3.6 years (interquartile range 0.8-7.9) for the bioprosthetic valve cohort (n = 28) and 3 years (interquartile range 1.3-7.8) for the mechanical valve cohort (n = 28). Seven years after bioprosthetic mitral valve replacement, the cumulative incidence of death or transplantation was 17.9% [95% confidence interval (CI) 6.3-34.1] and the cumulative incidence of a 2nd replacement was 63.6% (95% CI 39.9-80.1). Seven years after mechanical mitral valve replacement, the cumulative incidence of death or transplantation was 28.6% (95% CI 13.3-46) and the cumulative incidence of a 2nd replacement was 10.7% (95% CI 2.6-25.5). Fifteen years after mechanical mitral valve replacement, the cumulative incidence of death or transplantation was 33.6% (95% CI 16.2-52.1) and the cumulative incidence of a 2nd replacement was 41.1% (95% CI 18.4-62.7). The cumulative incidence curves for bioprosthetic and mechanical mitral valve replacement were statistically different for a 2nd valve replacement (P < 0.001) but not for death or transplantation (P = 0.33). CONCLUSIONS: There is no difference in transplant-free survival after bioprosthetic and mechanical mitral valve replacement in children. The lifespan of bioprosthetic mitral valves remains limited in children because of structural valve failure due to calcification. After 15 years, 40% of mechanical valves were replaced, primarily because of patient-prosthesis mismatch related to somatic growth.

The patient with congenital heart disease in ambulatory surgery.

The number of patients with congenital heart disease (CHD) undergoing ambulatory surgery is increasing. Deciding whether a CHD patient is suitable for an ambulatory procedure is still challenging. Several factors must be considered, including the type of planned procedure, the complexity of the underlying pathology, the American Society of Anesthesiologists' Physical Status classification of the patient, and other patient-specific factors, including comorbidity, chronic complications of CHD, medication, coagulation disorders, and issues related to the presence of a pacemaker (PM) or cardioverter-defibrillator. Numerous studies reported higher perioperative mortality and morbidity rates in surgical patients with CHD than non-CHD patients. However, most of these studies were conducted in a cohort of hospitalized patients and may not reflect the ambulatory setting. The current review aims to provide the anesthesiologist with an overview and practical recommendations on selecting and managing a CHD patient scheduled for an ambulatory procedure.

Reversal of Right Ventricular Remodeling After Correction of Pulmonary Regurgitation in Tetralogy of Fallot.

In the sheep model with pathophysiologic changes similar to patients with repaired TOF, severe PR leads to fibrotic changes in the RV. Pulmonary valve replacement reverses these fibrotic changes. Early valve replacement led to a quick RV recovery, and in time there was no difference in outcome between early and late valve replacement. These data support the benefit of valve replacement for RV function and suggest that there is a margin in the timing of the surgery. The fibrotic changes correlated well with the circulating biomarker PICP, which can have an added value in the clinical follow-up of patients with repaired TOF.

Preterm Birth Is Associated With Adverse Cardiac Remodeling and Worse Outcomes in Patients With a Functional Single Right Ventricle.

OBJECTIVE: To assess the effects of preterm birth on cardiac structure and function and transplant-free survival in patients with hypoplastic left heart syndrome and associated anomalies throughout the staged palliation process. STUDY DESIGN: Data from the Single Ventricle Reconstruction trial were used to assess the impact of prematurity on echocardiographic measures at birth, Norwood, Stage II, and 14 months in 549 patients with a single functional right ventricle. Medical history was recorded once a year using medical records or telephone interviews. Cox regression models were applied to analyze transplant-free survival to age 6 years. Causal mediation analysis was performed to estimate the mediating effect of birth weight within this relationship. RESULTS: Of the 549 participants, 64 (11.7%) were born preterm. Preterm-born participants had lower indexed right ventricle end-diastolic volumes at birth but higher volumes than term-born participants by age 14 months. Preterm-born participants had an increased risk of death or heart transplantation from birth to age 6 years, with an almost linear increase in the observed risk as gestational age decreased below 37 weeks. Of the total effect of preterm birth on transplant-free survival, 27.3% (95% CI 2.5-59.0%) was mediated through birth weight. CONCLUSIONS: Preterm birth is associated with adverse right ventricle remodeling and worse transplant-free survival throughout the palliation process, in part independently of low birth weight. Further investigation into this vulnerable group may allow development of strategies that mitigate the impact of prematurity on outcomes in patients with hypoplastic left heart syndrome.

Long-Term Consequences of Acute Kidney Injury After Pediatric Cardiac Surgery: A Systematic Review.

OBJECTIVE: The objective of this study was to evaluate the available data on long-term kidney dysfunction, hypertension, and mortality after cardiac surgery-associated acute kidney injury (AKI) in the pediatric population. STUDY DESIGN: PubMed/MEDLINE, Embase, Scopus, and reference lists of relevant articles were searched for eligible studies published from inception through March 2022. Long-term outcomes after pediatric cardiac surgery complicated by AKI and those without were investigated. RESULTS: We identified 14 studies published between 2013 and 2022 that included a total of 6701 patients (AKI: 1376 patients; no AKI: 5325 patients). These studies used different well-established classifications to define AKI. All the studies suggested that AKI after heart surgery is common in the pediatric patient population and reported a potential link between cardiac surgery-associated AKI and important clinical outcomes. However, only 4 out of 11 studies found a strong association between (absence of recovery from) cardiac surgery-associated AKI and risk of developing chronic kidney disease, and 3 out of 5 studies found a significant increase in mortality rates for pediatric patients who developed AKI after cardiac surgery. Only 1 out of 4 studies found an association between AKI and hypertension at 12 months postoperatively, but found no association at later follow-up times. CONCLUSIONS: Although there is a trend, evidence on the long-term consequences of cardiac surgery-associated AKI in the pediatric population is mixed. Genetic syndromes, preexisting kidney disease, univentricular or cyanotic heart conditions, and/or high-complexity surgery may be more important for the development of kidney dysfunction by adolescence and early adulthood. Regardless, these children may benefit from a long-term kidney follow-up.

Persistent Markers of Kidney Injury in Children Who Developed Acute Kidney Injury After Pediatric Cardiac Surgery: A Prospective Cohort Study.

Background Acute kidney injury (AKI) after pediatric cardiac surgery is common. Longer-term outcomes and the incidence of chronic kidney disease after AKI are not well-known. Methods and Results All eligible children (aged <16 years) who had developed AKI following cardiac surgery at our tertiary referral hospital were prospectively invited for a formal kidney assessment ≈5 years after AKI, including measurements of estimated glomerular filtration rate, proteinuria, α1-microglobulin, blood pressure, and kidney ultrasound. Longer-term follow-up data on kidney function were collected at the latest available visit. Among 571 patients who underwent surgery, AKI occurred in 113 (19.7%) over a 4-year period. Fifteen of these (13.3%) died at a median of 31 days (interquartile range [IQR], 9-57) after surgery. A total of 66 patients participated in the kidney assessment at a median of 4.8 years (IQR, 3.9-5.7) after the index AKI episode. Thirty-nine patients (59.1%) had at least 1 marker of kidney injury, including estimated glomerular filtration rate <90 mL/min per 1.73 m2 in 9 (13.6%), proteinuria in 27 (40.9%), α1-microglobinuria in 5 (7.6%), hypertension in 13 (19.7%), and abnormalities on kidney ultrasound in 9 (13.6%). Stages 1 to 5 chronic kidney disease were present in 18 (27.3%) patients. Patients with CKD were more likely to have an associated syndrome (55.6% versus 20.8%, P=0.015). At 13.1 years (IQR, 11.2-14.0) follow-up, estimated glomerular filtration rate <90 mL/min per 1.73 m² was present in 18 of 49 patients (36.7%), suggesting an average estimated glomerular filtration rate decline rate of -1.81 mL/min per 1.73 m² per year. Conclusions Children who developed AKI after pediatric cardiac surgery showed persistent markers of kidney injury. As chronic kidney disease is a risk factor for cardiovascular comorbidity, long-term kidney follow-up in this population is warranted.

Biomarkers of acute kidney injury after pediatric cardiac surgery: a meta-analysis of diagnostic test accuracy.

Acute kidney injury (AKI) occurs frequently after cardiac surgery in children. Although current diagnostic criteria rely on serum creatinine and urine output, changes occur only after considerable loss of kidney function. This meta-analysis aimed to synthesize the knowledge on novel biomarkers and compare their ability to predict AKI. PubMed/MEDLINE, Embase, Scopus, and reference lists were searched for relevant studies published by March 2021. Diagnostic accuracy parameters were extracted and analyzed using hierarchical summary receiver operating characteristic (HSROC) method. Pooled estimates of the area under the curve (AUC) were calculated using conventional random-effects meta-analysis. Fifty-six articles investigating 49 biomarkers in 8617 participants fulfilled our eligibility criteria. Data from 37 studies were available for meta-analysis. Of the 10 biomarkers suitable for HSROC analysis, urinary neutrophil gelatinase-associated lipocalin (uNGAL) to creatinine (Cr) ratio yielded the highest diagnostic odds ratio (91.0, 95% CI 90.1-91.9), with a sensitivity of 91.3% (95% CI 91.2-91.3%) and a specificity of 89.7% (95% CI 89.6-89.7%). These results were confirmed in pooled AUC analysis, as uNGAL-to-Cr ratio and uNGAL were the only elaborately studied biomarkers (> 5 observations) with pooled AUCs ≥ 0.800. Liver fatty acid-binding protein (L-FABP), serum cystatin C (sCysC), serum NGAL (sNGAL), and interleukin-18 (IL-18) all had AUCs ≥ 0.700. CONCLUSION: A variety of biomarkers have been proposed as predictors of cardiac surgery-associated AKI in children, of which uNGAL was the most prominent with excellent diagnostic qualities. However, more consolidatory evidence will be required before these novel biomarkers may eventually help realize precision medicine in AKI management. WHAT IS KNOWN: • Acute kidney injury (AKI) occurs in about 30-60% of children undergoing cardiac surgery and is associated with increased in-hospital mortality and adverse short-term outcomes. However, in current clinical practice, AKI definitions and detection often rely on changes in serum creatinine and urine output, which are late and insensitive markers of kidney injury. • Although various novel biomarkers have been studied for the diagnosis of AKI in children after cardiac surgery, it remains unclear how these compare to one another in terms of diagnostic accuracy. WHAT IS NEW: • Pooled analyses suggest that for the diagnosis of AKI in children who underwent cardiac surgery, NGAL is the most accurate among the most frequently studied biomarkers. • A number of other promising biomarkers have been reported, although they will require further research into their diagnostic accuracy and clinical applicability.

Pulmonary Valve Replacement in Tetralogy of Fallot: An Updated Meta-Analysis.

BACKGROUND: The benefits of pulmonary valve replacement (PVR) for pulmonary insufficiency in patients with repaired tetralogy of Fallot are still incompletely understood, and optimal timing remains challenging. METHODS: We systematically reviewed databases (PubMed/MEDLINE, Embase, Cochrane Central Register of Controlled Trials /Cochrane Controlled Trials Register, ClinicalTrials.gov, Scientific Electronic Library Online, Literatura Latino Americana em Ciências da Saúde, and Google Scholar) and reference lists of relevant articles for studies about PVR in repaired tetralogy of Fallot patients that reported any of the following outcomes: mortality and redo PVR rates, right ventricular (RV) and left ventricular measures, QRS duration, cardiopulmonary exercise test results, or brain natriuretic peptide. In addition to calculating the pooled treatment effects using a random-effects meta-analysis, we evaluated the effect of preoperative measures on PVR outcomes using meta-regressions. RESULTS: Eighty-four studies involving 7544 patients met the eligibility criteria. Pooled mortality at 30 days, 5 years, and 10 years after PVR was 0.87% (63 of 7253 patients, 80 studies), 2.7% (132 of 4952 patients, 37 studies), and 6.2% (510 of 2765 patients, 15 studies), respectively. Pooled 5- and 10-year redo PVR rates were 3.7% (141 of 3755 patients, 23 studies) and 16.8% (172 of 3035 patients, 16 studies), respectively. The results of the previous meta-analysis could be confirmed. In addition, we demonstrated that after PVR (1) QRS duration, cardiopulmonary exercise test results, and RV and left ventricular measures longitudinal strain do not significantly change; (2) brain natriuretic peptide decreases; and (3) greater indexed RV end-diastolic and end-systolic volumes are associated with lower chances of RV volume normalization after PVR. CONCLUSIONS: This updated meta-analysis provides evidence about the benefits of PVR.

Risk factors for acute kidney injury after pediatric cardiac surgery: a meta-analysis.

BACKGROUND: Cardiac surgery-associated acute kidney injury (AKI) is associated with increased morbidity and mortality in both adults and children. OBJECTIVES: This study aimed to identify clinical risk factors for AKI following cardiac surgery in the pediatric population. DATA SOURCES: PubMed/MEDLINE, Embase, Scopus, and reference lists of relevant articles were searched for studies published by August 2020. STUDY ELIGIBILITY CRITERIA: Studies were included if (1) the population consisted of pediatric patients (< 18 years old), (2) patients underwent cardiac surgery, (3) risk factors were compared between patients who developed AKI and those who did not, and (4) studies were prospective or retrospective observational studies or randomized controlled trials. PARTICIPANTS AND INTERVENTIONS: Children undergoing pediatric cardiac surgery. STUDY APPRAISAL AND SYNTHESIS METHODS: Random-effects meta-analysis was performed, comparing potential risk factors between pediatric patients who developed CS-AKI and those who did not. RESULTS: Sixty-one publications including a total of 19,680 participants (AKI: 7257 participants; no AKI: 12,423 participants) were included from studies published between 2008 and 2020. The pooled estimated incidence of AKI was 34.3% (95% confidence interval 30.0-38.8%, I2 = 96.8%). Binary risk factors that were significantly and consistently associated with AKI were the presence of pulmonary hypertension, cyanotic heart disease, univentricular heart, risk adjustment for congenital heart surgery 1 (RACHS-1) score ≥ 3, vasopressor use, cardiopulmonary bypass use, reoperation, and sepsis. Significant continuous risk factors included younger age, lower body weight, lower preoperative creatinine, higher preoperative estimated glomerular filtration rate (eGFR), higher RACHS-1 score, longer surgery time, longer cardiopulmonary bypass time, longer aortic cross-clamp time, and higher red blood cell transfusion volume. LIMITATIONS: Results are limited by heterogeneity and potential residual confounding. CONCLUSIONS AND IMPLICATIONS OF KEY FINDINGS: Our meta-analysis identified clinical risk factors that are associated with AKI in children undergoing cardiac surgery. This might help clinicians anticipate and manage more carefully this population and implement standardized preventive strategies. SYSTEMATIC REVIEW REGISTRATION NUMBER: CRD42021262699. A higher resolution version of the Graphical abstract is available as Supplementary information.

Functional brain maturation and sleep organisation in neonates with congenital heart disease.

OBJECTIVE: Neonates with Congenital Heart Disease (CHD) have structural delays in brain development. To evaluate whether functional brain maturation and sleep-wake physiology is also disturbed, the Functional Brain Age (FBA) and sleep organisation on EEG during the neonatal period is investigated. METHODS: We compared 15 neonates with CHD who underwent multichannel EEG with healthy term newborns of the same postmenstrual age, including subgroup analysis for d-Transposition of the Great Arteries (d-TGA) (n = 8). To estimate FBA, a prediction tool using quantitative EEG features as input, was applied. Second, the EEG was automatically classified into the 4 neonatal sleep stages. Neonates with CHD underwent neurodevelopmental testing using the Bayley Scale of Infant Development-III at 24 months. RESULTS: Preoperatively, the FBA was delayed in CHD infants and more so in d-TGA infants. The FBA was positively correlated with motor scores. Sleep organisation was significantly altered in neonates with CHD. The duration of the sleep cycle and the proportion of Active Sleep Stage 1 was decreased, again more marked in the d-TGA infants. Neonates with d-TGA spent less time in High Voltage Slow Wave Sleep and more in Tracé Alternant compared to healthy terms. Both FBA and sleep organisation normalised postoperatively. The duration of High Voltage Slow Wave Sleep remained positively correlated with motor scores in d-TGA infants. INTERPRETATION: Altered early brain function and sleep is present in neonates with CHD. These results are intruiging, as inefficient neonatal sleep has been linked with adverse long-term outcome. Identifying how these rapid alterations in brain function are mitigated through improvements in cerebral oxygenation, surgery, drugs and nutrition may have relevance for clinical practice and outcome.

Tricuspid Valve Intervention at the Time of Pulmonary Valve Replacement in Adults With Congenital Heart Disease: A Systematic Review and Meta-Analysis.

Background Tricuspid regurgitation (TR) is a common finding in adults with congenital heart disease referred for pulmonary valve replacement (PVR). However, indications for combined valve surgery remain controversial. This study aimed to evaluate early results of concomitant tricuspid valve intervention (TVI) at the time of PVR. Methods and Results Observational studies comparing TVI+PVR and isolated PVR were identified by a systematic search of published research. Random-effects meta-analysis was performed, comparing outcomes between the 2 groups. Six studies involving 749 patients (TVI+PVR, 278 patients; PVR, 471 patients) met the eligibility criteria. In the pooled analysis, both TVI+PVR and PVR reduced TR grade, pulmonary regurgitation grade, right ventricular end-diastolic volume, and right ventricular end-systolic volumes. TVI+PVR, but not PVR, was associated with a decrease in tricuspid valve annulus size (mean difference, -6.43 mm, 95% CI, -10.59 to -2.27; P=0.010). Furthermore, TVI+PVR was associated with a larger reduction in TR grade compared with PVR (mean difference, -0.40; 95% CI, -0.75 to -0.05; P=0.031). No evidence could be established for an effect of either treatment on right ventricular ejection fraction or echocardiographic assessment of right ventricular dilatation and dysfunction. There was no evidence for a difference in hospital mortality or reoperation for TR. Conclusions While both strategies are effective in reducing TR and right ventricular volumes, routine TVI+PVR can reduce TR grade to a larger extent than isolated PVR. Further studies are needed to identify the subgroups of patients who might benefit most from combined valve surgery.

Strategies to Prevent Acute Kidney Injury after Pediatric Cardiac Surgery: A Network Meta-Analysis.

BACKGROUND AND OBJECTIVES: AKI is a common complication after pediatric cardiac surgery and has been associated with higher morbidity and mortality. We aimed to compare the efficacy of available pharmacologic and nonpharmacologic strategies to prevent AKI after pediatric cardiac surgery. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: PubMed/MEDLINE, Embase, Cochrane Controlled Trials Register, and reference lists of relevant articles were searched for randomized controlled trials from inception until August 2020. Random effects traditional pairwise, Bayesian network meta-analyses, and trial sequential analyses were performed. RESULTS: Twenty randomized controlled trials including 2339 patients and 11 preventive strategies met the eligibility criteria. No overall significant differences were observed compared with control for corticosteroids, fenoldopam, hydroxyethyl starch, or remote ischemic preconditioning in traditional pairwise meta-analysis. In contrast, trial sequential analysis suggested a 80% relative risk reduction with dexmedetomidine and evidence of <57% relative risk reduction with remote ischemic preconditioning. Nonetheless, the network meta-analysis was unable to demonstrate any significant differences among the examined treatments, including also acetaminophen, aminophylline, levosimendan, milrinone, and normothermic cardiopulmonary bypass. Surface under the cumulative ranking curve probabilities showed that milrinone (76%) was most likely to result in the lowest risk of AKI, followed by dexmedetomidine (70%), levosimendan (70%), aminophylline (59%), normothermic cardiopulmonary bypass (57%), and remote ischemic preconditioning (55%), although all showing important overlap. CONCLUSIONS: Current evidence from randomized controlled trials does not support the efficacy of most strategies to prevent AKI in the pediatric population, apart from limited evidence for dexmedetomidine and remote ischemic preconditioning.

In-Hospital Outcomes of Acute Kidney Injury After Pediatric Cardiac Surgery: A Meta-Analysis.

Background: Cardiac surgery-associated acute kidney injury (CS-AKI) is associated with increased morbidity and mortality in both adults and children. This study aimed to investigate the in-hospital outcomes of CS-AKI in the pediatric population. Methods: PubMed/MEDLINE, Embase, Scopus, and reference lists of relevant articles were searched for studies published by August 2020. Random-effects meta-analysis was performed, comparing in-hospital outcomes between patients who developed CS-AKI and those who did not. Results: Fifty-eight publications between 2008 and 2020 consisting of 18,334 participants (AKI: 5,780; no AKI: 12,554) were included. Higher rates of in-hospital mortality (odds ratio [OR] 7.22, 95% confidence interval [CI] 5.27-9.88), need for renal replacement therapy (RRT) (OR 18.8, 95% CI 11.7-30.5), and cardiac arrhythmias (OR 2.67, 95% 1.86-4.80) were observed in patients with CS-AKI. Furthermore, patients with AKI had longer ventilation times (mean difference [MD] 1.76 days, 95% CI 1.05-2.47), pediatric intensive care unit (PICU) length of stay (MD 3.31, 95% CI 2.52-4.10), and hospital length of stay (MD 5.00, 95% CI 3.34-6.67). Conclusions: CS-AKI in the pediatric population is associated with a higher risk of mortality, cardiac arrhythmias and need for RRT, as well as greater mechanical ventilation time, PICU and hospital length of stay. These results might help improve the clinical care protocols prior to cardiac surgery to minimize the disease burden of CS-AKI in children. Furthermore, etiology-specific approaches to AKI are warranted, as outcomes are likely impacted by the underlying cause.

Critical Coarctation of the Aorta in Selective Fetal Growth Restriction and the Role of Coronary Stent Implantation.

INTRODUCTION: Monochorionic twins are at increased risk of congenital heart defects (CHDs). Up to 26% have a birth weight <1,500 g, a CHD requiring neonatal surgery, therefore, poses particular challenges. OBJECTIVE: The aim of the study was to describe pregnancy characteristics, perinatal management, and outcome of monochorionic twins diagnosed with critical coarctation of the aorta (CoA). METHODS: We included monochorionic twins diagnosed with critical CoA (2010-2019) at 2 tertiary referral centers, and we systematically reviewed the literature regarding CoA in monochorionic twins. RESULTS: Seven neonates were included. All were the smaller twin of pregnancies complicated by selective fetal growth restriction. The median gestational age at birth was 32 weeks (28-34). Birth weight of affected twins ranged as 670-1,800 g. One neonate underwent coarctectomy at the age of 1 month (2,330 g). Six underwent stent implantation, performed between day 8 and 40, followed by definitive coarctectomy between 4 and 9 months in 4. All 7 developed normally, except for 1 child with neurodevelopmental delay. Three co-twins had pulmonary stenosis, of whom 1 required balloon valvuloplasty. The literature review revealed 10 cases of CoA, all in the smaller twin. Six cases detected in the first weeks after birth were treated with prostaglandins alone, by repeated transcatheter angioplasty or by surgical repair, with good outcome in 2 out of 6. CONCLUSIONS: CoA specifically affects the smaller twin of growth discordant monochorionic twin pairs. Stent implantation is a feasible bridging therapy to surgery in these low birth weight neonates.

Outcome of arterial switch operation for transposition of the great arteries. A 35-year follow-up study.

BACKGROUND: Arterial switch operation (ASO) is today the first-choice surgical treatment for patients with transposition of the great arteries. Long-term outcome data still remain scarce. Moreover, the course of these patients is not uneventful. Therefore, we wanted to evaluate long-term outcome and determine on which variables to focus during follow-up. METHODS: Clinical records of 318 patients who underwent ASO between October 1981 and July 2018 were reviewed. Perioperative, post-operative, and interventional data were collected to determine mortality and the need for re-intervention. Descriptive statistics and Kaplan-Meier survival analysis were performed. RESULTS: Mean follow-up time was 11.1 SD 8.5 years (range 0-35) with a mean age of 12.5 SD 9.0 years (range 0-37) at latest follow-up. In-hospital mortality was 7.5% and overall survival 90.9% for a maximum follow-up time of 35 years. Causes of early mortality were cardiogenic shock, severe pulmonary hypertension, septic shock and multiple organ failure. Causes of late mortality were cardiogenic shock, severe pulmonary hypertension, pacemaker lead fracture and fire death. Re-intervention free survival at 5-year was 91.6%, at 10-year 90.7%, at 20-year 79.2%. For all survivors, the most frequent sequelae after ASO were pulmonary artery stenosis (80.9%), of which 13.5% needed an intervention. The threshold for intervening on lesions at the level of the pulmonary artery bifurcation was higher and the percutaneous re-intervention rate was higher for non-bifurcation lesions. CONCLUSIONS: Despite a relatively high peri-operative mortality, TGA patients have an excellent overall long-term survival. However, a large proportion of patients requires re-interventions, mainly for pulmonary artery stenosis.

Right ventricular outflow tract prestenting with AndraStent XXL before percutaneous pulmonary valve implantation.

BACKGROUND: The indications for percutaneous pulmonary valve implantation (PPVI) have been extended to include large dysfunctional right ventricular outflow tracts (RVOTs). Prestenting of the RVOT is commonly performed before PPVI in order to ensure a stable landing zone. The AndraStent XXL (AndraMed GmbH, Reutlingen, Germany), a cobalt-chromium stent with semi-open cell design, has unique mechanical properties in this indication but is no longer available in France. AIMS: To assess the efficiency of AndraStent XXL before PPVI. METHODS: In this retrospective multicentre cohort study, 86 AndraStents XXL were implanted in 77 patients in 6 centres. RESULTS: PPVI was indicated mainly for pulmonary regurgitation (75.3%) in native or patched RVOT (88.3%). The stents were manually mounted on balloon catheters and delivered through sheaths using a conventional femoral approach. PPVI was performed successfully in 97.4% of patients after successful prestenting, generally during the same procedure (77.9%). There were no deaths associated with stent implantation, and four patients experienced five complications, mainly stent embolization, including one requiring surgery. Neither stent fracture nor dysfunction were observed in any patient during a mean follow-up of 19.2±8.7months. Stent analysis showed an excellent maximal stent expansion (97.1%) regardless of balloon size. A 22.3%±3.4 stent shortening with a 30mm balloon was observed. CONCLUSIONS: Implantation of large cobalt-chromium AndraStent XXL stents is efficient for prestenting before PPVI.

Can ductus arteriosus morphology influence technique/outcome of stent treatment?

INTRODUCTION: Results and outcomes of ductus arteriosus stenting vary widely. The aim of this study was to determine whether ductus morphology is associated with different procedural outcome. METHODS: Over an 18-year period, 123 patients presented with ductal dependent pulmonary blood flow. Results were retrospectively assessed based on radiographic anatomic features of the ductus arteriosus: Group 1: "straight" ductus arteriosus, typically seen in patients with Pulmonary atresia with intact septum (PA-IVS), Group 2: "intermediate" ductus arteriosus as seen in severe pulmonary stenosis (PS)-single ventricle, Group 3: "vertical" ductus arteriosus typically seen in patients with pulmonary atresia-ventricular septal defect, Group 4: ductus arteriosus arising from the aorta to a single lung, Group 5: ductus arteriosus arising from the innominate/subclavian artery to a single lung, Group 6: ductus arteriosus from innominate/subclavian artery to both lungs. RESULTS: Ductal stenting (DS) was attempted in 98 patients with 99 ducts. Successful stenting was possible in 83 patients. Success of DS was significantly different among the groups (p = .04, F = 5.41). Groups 1, 4, and 5 were "easy" with good success while Groups 2, 3, and 6 were complex and demanding. There were two deaths (after 5 and 7 days, respectively) that could be ascribed to DS. Elective re-interventions were performed in 34 ductuses (40%). Fifty three percent (n = 44/83) of successful ductus stents proceeded to further surgery and 20 ducts closed spontaneously in asymptomatic patients over time. CONCLUSIONS: Ductus arteriosus morphology influences technique and determines complexity, safety, and final outcome of ductus arteriosus stenting.

Outcome of the Glenn procedure as definitive palliation in single ventricle patients.

OBJECTIVES: In selected single ventricle patients, a Glenn procedure (SV-Glenn) may be considered as definitive palliation. Either the patient is unsuited to progress to a Fontan circulation or a SV-Glenn circulation is preferred. This study aimed at describing the clinical course, and long-term mortality/morbidity of SV-Glenn patients. METHODS: All SV-Glenn patients followed at the University Hospitals Leuven before May 2018 were included. Patients who underwent, or were awaiting, TCPC completion and those who underwent a Glenn in the setting of a biventricular circulation one-and-a-half repair (OAHR), were excluded. RESULTS: Of 65 Glenn-only patients identified, 21 (32%) had OAHR, whereas 44 (68%) were SV-Glenn patients. Of SV-Glenn patients, 19 died within 6 months after the Glenn procedure. Of 25 SV-Glenn survivors, median age at Glenn was 6.3 (IQR 1.2-29.7) years. Eight were unsuited for TCPC completion; in 17 SV-Glenn was preferred over TCPC completion. Over a median follow-up time of 11 (IQR 3-18) years after the Glenn procedure, 5 (20%) patients died. At latest follow-up 10 (40%) had heart failure, 5 (20%) had atrial and 4 (16%) ventricular arrhythmias, 2 (8%) a thromboembolic event, 7 (28%) required pacemaker implantation, and 2 (8%) had infective endocarditis but none developed cirrhosis or protein-losing enteropathy. Mean saturation at latest follow-up was 87 ± 7%. CONCLUSION: SV-Glenn patients represent a unique and heterogeneous patient population. Outcome was reasonable, although comorbidities, such as heart failure and arrhythmias were not uncommon. In SV-Glenn patients, 'classic' complications related to Fontan physiology, such as cirrhosis and protein-losing enteropathy, were absent.

Percutaneous obliteration of the right ventricle to avoid coronary damage by sinusoids in patients with pulmonary atresia intact ventricular septum during staged single ventricle palliation.

BACKGROUND AND AIMS: Suprasystemic pressure waves can damage the coronary arteries resulting in myocardial ischemia and excess early mortality. We aimed to reduce the coronary pressure wave through the sinusoids by abolishing RV volume with percutaneous devices. METHODS AND RESULTS: Four patients with PA-IVS and coronary sinusoids from the hypertensive rudimentary RV were evaluated at a median age 26.6 months (range: 2.7-51.7). Right ventricle coronary dependent flow to the left ventricular myocardium was excluded. All four patients had dual perfusion with competitive flow from the RV through the sinusoids to the coronary arteries. Devices used were: Amplatzer vascular plug II of 10-16 mm; 27 coils (diameter 5-15 mm) in the oldest patient. Right ventricular angiography after cavity obliteration showed no more significant coronary perfusion through the sinusoids. There were no complications or deaths. Only minor and transient changes in the levels of troponin were observed. Coronary angiography at pre-Fontan evaluation showed no progress of coronary abnormalities in two patients. CONCLUSION: In selected patients with functionally single left ventricle, obliteration of the hypertensive RV cavity by percutaneous devices is safe and abolishes the systolic pressure wave in coronary sinusoids. When performed early, this may halt coronary damage and avoid excess mortality.