Non-invasive assessment of liver abnormalities in pediatric Fontan patients.

Liver abnormalities are well known among long-term survivors of Fontan palliation, which remains the definite surgery for complex congenital heart disease and single ventricle physiology. Pediatric data however are scarce. We assessed the prevalence and degree of liver abnormalities in pediatric Fontan patients through non-invasive investigations suitable for longitudinal follow-up. Thirty-five patients with a median age of 11.8 years (5.2-16.6) and median time since Fontan of 6 years (1.17-13.83) were studied. Each child underwent a blood test, liver Doppler ultrasound (US), and transient elastography (TE). Healthy children were used as controls for TE measurement. AST, ALT, γGT, and direct bilirubin were abnormal in respectively 12 (34%), 5 (14%), 24 (69%), and 7 (20%) patients, while platelet count was decreased in 7 (20%). Splenomegaly was present in 7 (20%) patients. Portal vein mean flow velocity was < 15 cm/s in 19 (54%) patients indicative of portal hypertension. Twenty-two patients (63%) showed inferior vena cava collapsibility index values below 17%, indicating venous congestion. Hepatic artery and superior mesenteric artery resistance index were inversely correlated with time post Fontan (p < 0.05). TE values in Fontan patients were significantly higher than controls, with a median of 12.6 versus 4.6 kPa (p < 0.001) and were already increased shortly after Fontan completion. Conclusion: Liver abnormalities are frequently observed in pediatric Fontan patients. The non-invasive investigations used were not able to confirm liver fibrosis or differentiate hepatic congestion from fibrosis. Based on our findings, we propose a prospective screening protocol with serial measurements of laboratory, (Doppler) US, and TE parameters. What is Known: • Hepatic dysfunction is a well-known consequence of the Fontan circulation. • The natural history of Fontan-associated liver disease in the pediatric age group remains unclear. What is New: • Liver abnormalities are frequently observed in pediatric Fontan patients; however, differentiating liver fibrosis and hepatic congestion with non-invasive investigations remains challenging. Sonographic Doppler measurements may improve our insight in both Fontan-associated liver disease development and the functioning of the Fontan circulation. • A prospective screening protocol is proposed to improve our ability to detect Fontan-associated liver disease early on and understand its natural history.

ACARPA, a rare case of ALCAPA.

An anomalous circumflex coronary artery arising from the right pulmonary artery is very rare. Clinical presentation varies from absence of symptoms to arrhythmia and sudden cardiac death. Since prevalence is very low, no definite therapy has been delineated yet. A 19-year-old patient, previously treated for a vascular ring and mitral valve stenosis, was diagnosed with this anomaly through echocardiography revealing collateral coronary flow, with confirmation of this coronary anomaly on subsequent computed tomography examination. To alleviate the burden for arrhythmia in this young patient, he was successfully treated with surgical reimplantation followed by an uneventful recovery.

QRS Duration During Follow-Up of Tetralogy of Fallot: How Valuable is it? Analysis of ECG Changes in Relation to Pulmonary Valve Implantation.

Long-term results after tetralogy of Fallot (TOF) repair are determined by the extent of right ventricular remodeling to chronic pulmonary regurgitation entailing progressive RV dysfunction and a risk of developing ventricular arrhythmia. Pulmonary valve replacement (PVR) can alleviate this burden. As a predictor of ventricular arrhythmia, QRS duration remains a strong parameter in this decision. We performed a retrospective analysis of all PVR patients between 2005 and 2018, studying the time evolution of electrocardiographic parameters before and after PVR through linear mixed model analysis. 42 TOF patients underwent PVR. The median timespan between primary repair and PVR was 18 years (IQR 13-30). The indication for PVR was primarily based on the association of exercise intolerance (67%) and significant RV dilation on cMRI (median RVEDVi 161 ml/m2 IQR 133-181). Median QRS length was 155 ms (IQR 138-164), 4 (10%) patients had a QRS > 180 ms. QRS duration increased significantly before PVR, but barely showed regression after PVR. Changes of QRS duration after PVR were independent of RV dilation. In conclusion, when the decision for PVR in TOF patients is primarily based on RV volume and/or function threshold, QRS duration > 180 ms is rarely observed. In contrast with the significant increase of QRS duration before PVR, QRS length regression appears to be independent of the extent of RV dilation or QRS > 160 ms. Considering that the decision for PVR is based on mechanical RV characteristics, the utility of serial follow-up of QRS duration in contemporary operated TOF patients becomes questionable in absence of clinical arguments for ventricular arrhythmia.

Determinants of Physical Fitness in Children with Repaired Congenital Heart Disease.

The aim of this study was to determine factors associated with physical fitness (PF) in children who underwent surgery for congenital heart disease (CHD). Sixty-six children (7-14 years) who underwent surgery for ventricular septal defect (n = 19), transposition of great arteries (n = 22), coarctation of aorta (n = 10), and tetralogy of Fallot (n = 15) were included. All children performed PF tests: cardiorespiratory fitness, upper- and lower-limb muscle strength, speed, balance, and flexibility. Cardiac evaluation was done via echocardiography and cardiopulmonary exercise test. Factors related to child's characteristics, child's lifestyle, physical activity motivators/barriers, and parental factors were assessed. Linear regression analyses were conducted. The results showed no significant differences in physical activity (PA) level by CHD type. Boys had better cardiorespiratory fitness (difference = 1.86 ml/kg/min [0.51;3.22]) and were more physically active (difference = 19.40 min/day [8.14;30.66]), while girls had better flexibility (difference = - 3.60 cm [- 7.07;- 0.14]). Physical activity motivators showed an association with four out of six PF components: cardiorespiratory fitness, coefficient = 0.063 [0.01;0.11]; upper-limb muscle strength, coefficient = 0.076 [0.01;0.14]; lower-limb muscle strength, coefficient = 0.598 [0.07;1.13]; and speed, coefficient = 0.03 [0.01;0.05]. Age, sex, and motivators together reached a maximum adjusted R2 = 0.707 for upper-limb strength. Adding other possible determinants did not significantly increase the explained variance. Apart from age and sex as non-modifiable determinants, the main target which might improve fitness would be the introduction of an intervention which increases the motivation to be active.

Right Ventricular Systolic Assessment by Transesophageal Versus Transthoracic Echocardiography: Displacement, Velocity, and Myocardial Deformation.

OBJECTIVE: First, to compare tricuspid annular displacement and velocity in transthoracic and transesophageal echocardiography (TTE, TEE) using conventional angle-dependent technologies. Second, to evaluate both alternative TEE views as well as an alternative technology (speckle tracking) for overcoming proposed differences in TTE and TEE. DESIGN: Prospective, comparative, cross-over study with a randomized order of image acquisition. SETTING: University hospital. PARTICIPANTS: Adults undergoing cardiac surgery. INTERVENTIONS: Postinduction standardized image acquisition and analysis in TTE and TEE by 2 echocardiographers. MEASUREMENTS AND MAIN RESULTS: The authors measured tricuspid annular plane systolic excursion (TAPSE) by M-mode and velocity by tissue Doppler (S') in the apical 4-chamber TTE view and midesophageal 4-chamber TEE view (AP4C, ME4C). They then examined (1) the same measurements in alternative TEE views with proposed better ultrasound angulation; and (2) speckle tracking-based endpoints (TAPSE by speckle tracking, strain, and strain rate). Data were available in 24 of 25 patients. Conventional TAPSE by M-mode and velocity by tissue Doppler (TDI) were underestimated in the ME4C compared with the AP4C reference (mean ± standard deviation: TAPSE: 13.1 ± 3.8 mm v 17.3 ± 4.0 mm; S': 6.7 ± 2.1 cm/s v 9.1 ± 2.2 cm/s; both p < 0.001). Neither a modified deep transgastric view (TAPSE 14.5 ± 4.7 mm, p = 0.017; S' 6.8 ± 1.8 cm/s, p < 0.001) nor a transgastric right ventricular inflow view (TAPSE 12.3 ± 4.0 mm, p = 0.001; S' 6.0 ± 1.3 cm/s, p < 0.001) was similar to the AP4C. Speckle tracking TAPSE was unbiased but with high variability (mean bias = -0.3 mm, 95% limits of agreement = -9.1 to 8.4); strain and strain rate were higher in TEE than for TTE (-17.7 ± 3.6 v -12.6 ± 2.1, p < 0.001; -1.0 ± 0.2/s v -0.7 ± 0.1/s, p < 0.001). CONCLUSIONS: Right ventricular displacement, velocity, and myocardial deformation measured by TEE versus TTE are different. Neither alternative transesophageal echocardiography views nor speckle tracking-based deformation is promising; TAPSE by speckle tracking is unbiased but imprecise.

Analysis of clinical outcome and postoperative organ function effects in a propensity-matched comparison between conventional and minimally invasive mitral valve surgery.

BACKGROUND: Minimally invasive mitral valve (MV) surgery (MIVT) is increasingly performed with excellent clinical outcome, despite longer procedural times. This study analyzes clinical outcomes and effects on secondary organ functions in a propensity-matched comparison with conventional MV surgery. METHODS AND RESULTS: Out of 439 patients undergoing MV surgery from January 2005 to May 2017, 233 patients were included after propensity-matching: 90 sternotomy patients and 143 MIVT patients. Endpoints focused on survival, quality of MV repair, and organ function effects through analysis of biomarkers and functional parameters. Regardless of longer cardiopulmonary bypass (sternotomy: 101(IQR33) min-MIVT:143(IQR45) min, p < .001) and cardioplegic arrest times(sternotomy: 64(IQR25) min-MIVT:90(IQR34) min, p < .001), no differences in survival nor complication rate were found. Effect on renal function(creatinine, p = .751 - ureum, p = .538 - glomerular filtration, p = .848), myocardial damage by troponine I level (sternotomy:1.8 ± 3.9 ng/ml - MIVT:1.2 ± 1.3 ng/ml, p = .438) and prolonged ventilatory support >24 h (sternotomy:5.5% - MIVT:8.4%, p = .417) were comparable. The systemic inflammatory reaction by postoperative C-reactive protein count was markedly lower for MIVT(p < .001). Increased rhadomyolysis was found after MIVT surgery, based on a significant elevation of creatinine-kinase levels(sternotomy: 431 ± 237 U/L - MIVT: 701 ± 595 U/L, p < .001). CONCLUSION: Despite an inherent learning curve, minimally invasive MV surgery guarantees a clinical outcome and MV repair quality, at least non-inferior to those of MV surgery via sternotomy. Notwithstanding longer cardiopulmonary bypass and cardiac arrest times, the impact on secondary organ function is negligible, except for a lower systemic inflammatory response. The postoperative increase of CK-enzymes suggestive for enhanced rhabdomyolysis needs to be accounted for when procedural times tend to exceed the critical time threshold for severe limb ischemia.

Multi-centre evaluation of a comprehensive preimplantation genetic test through haplotyping-by-sequencing.

STUDY QUESTION: Can reduced representation genome sequencing offer an alternative to single nucleotide polymorphism (SNP) arrays as a generic and genome-wide approach for comprehensive preimplantation genetic testing for monogenic disorders (PGT-M), aneuploidy (PGT-A) and structural rearrangements (PGT-SR) in human embryo biopsy samples? SUMMARY ANSWER: Reduced representation genome sequencing, with OnePGT, offers a generic, next-generation sequencing-based approach for automated haplotyping and copy-number assessment, both combined or independently, in human single blastomere and trophectoderm samples. WHAT IS KNOWN ALREADY: Genome-wide haplotyping strategies, such as karyomapping and haplarithmisis, have paved the way for comprehensive PGT, i.e. leveraging PGT-M, PGT-A and PGT-SR in a single workflow. These methods are based upon SNP array technology. STUDY DESIGN, SIZE, DURATION: This multi-centre verification study evaluated the concordance of PGT results for a total of 225 embryos, including 189 originally tested for a monogenic disorder and 36 tested for a translocation. Concordance for whole chromosome aneuploidies was also evaluated where whole genome copy-number reference data were available. Data analysts were kept blind to the results from the reference PGT method. PARTICIPANTS/MATERIALS, SETTING, METHODS: Leftover blastomere/trophectoderm whole genome amplified (WGA) material was used, or secondary trophectoderm biopsies were WGA. A reduced representation library from WGA DNA together with bulk DNA from phasing references was processed across two study sites with the Agilent OnePGT solution. Libraries were sequenced on an Illumina NextSeq500 system, and data were analysed with Agilent Alissa OnePGT software. The embedded PGT-M pipeline utilises the principles of haplarithmisis to deduce haplotype inheritance whereas both the PGT-A and PGT-SR pipelines are based upon read-count analysis in order to evaluate embryonic ploidy. Concordance analysis was performed for both analysis strategies against the reference PGT method. MAIN RESULTS AND THE ROLE OF CHANCE: PGT-M analysis was performed on 189 samples. For nine samples, the data quality was too poor to analyse further, and for 20 samples, no result could be obtained mainly due to biological limitations of the haplotyping approach, such as co-localisation of meiotic crossover events and nullisomy for the chromosome of interest. For the remaining 160 samples, 100% concordance was obtained between OnePGT and the reference PGT-M method. Equally for PGT-SR, 100% concordance for all 36 embryos tested was demonstrated. Moreover, with embryos originally analysed for PGT-M or PGT-SR for which genome-wide copy-number reference data were available, 100% concordance was shown for whole chromosome copy-number calls (PGT-A). LIMITATIONS, REASONS FOR CAUTION: Inherent to haplotyping methodologies, processing of additional family members is still required. Biological limitations caused inconclusive results in 10% of cases. WIDER IMPLICATIONS OF THE FINDINGS: Employment of OnePGT for PGT-M, PGT-SR, PGT-A or combined as comprehensive PGT offers a scalable platform, which is inherently generic and thereby, eliminates the need for family-specific design and optimisation. It can be considered as both an improvement and complement to the current methodologies for PGT. STUDY FUNDING/COMPETING INTEREST(S): Agilent Technologies, the KU Leuven (C1/018 to J.R.V. and T.V.) and the Horizon 2020 WIDENLIFE (692065 to J.R.V. and T.V). H.M. is supported by the Research Foundation Flanders (FWO, 11A7119N). M.Z.E, J.R.V. and T.V. are co-inventors on patent applications: ZL910050-PCT/EP2011/060211- WO/2011/157846 'Methods for haplotyping single cells' and ZL913096-PCT/EP2014/068315 'Haplotyping and copy-number typing using polymorphic variant allelic frequencies'. T.V. and J.R.V. are co-inventors on patent application: ZL912076-PCT/EP2013/070858 'High-throughput genotyping by sequencing'. Haplarithmisis ('Haplotyping and copy-number typing using polymorphic variant allelic frequencies') has been licensed to Agilent Technologies. The following patents are pending for OnePGT: US2016275239, AU2014345516, CA2928013, CN105874081, EP3066213 and WO2015067796. OnePGT is a registered trademark. D.L., J.T. and R.L.R. report personal fees during the conduct of the study and outside the submitted work from Agilent Technologies. S.H. and K.O.F. report personal fees and other during the conduct of the study and outside the submitted work from Agilent Technologies. J.A. reports personal fees and other during the conduct of the study from Agilent Technologies and personal fees from Agilent Technologies and UZ Leuven outside the submitted work. B.D. reports grants from IWT/VLAIO, personal fees during the conduct of the study from Agilent Technologies and personal fees and other outside the submitted work from Agilent Technologies. In addition, B.D. has a patent 20160275239 - Genetic Analysis Method pending. The remaining authors have no conflicts of interest.

A Novel Speckle-Tracking Based Method for Quantifying Tricuspid Annular Velocities in TEE.

OBJECTIVES: A novel speckle-tracking-based option for measuring tricuspid annular velocities in the midesophageal 4-chamber view (ME4C) was compared with velocities measured by tissue Doppler in the apical-4 chamber view (AP4C). Because this method was based on a modified speckle-tracking-based measurement of tricuspid annular plane systolic excursion (TAPSE), the authors also compared TAPSE by speckle tracking in the ME4C with TAPSE by M-mode in the AP4C. The authors hypothesized that velocities measured by speckle tracking in transesophageal echocardiography (TEE) would be similar, correlate, and agree with those measured by tissue Doppler in transthoracic echocardiography (TTE). DESIGN: Prospective diagnostic study with randomization of the order of post-induction echocardiography views by TTE (AP4C) and TEE (ME4C). Images were both acquired and analyzed by 2 echocardiographers independently. The primary outcome was S'; secondary outcomes were E', A', and TAPSE. SETTING: Single university hospital. PARTICIPANTS: Consecutive adult patients undergoing cardiac surgery (mainly coronary artery bypass grafting). INTERVENTIONS: None. MAIN RESULTS: Complete data was available in 24 of 25 patients. For the primary outcome, S' measured by speckle tracking in the ME4C correlated and agreed with S' measured by tissue Doppler in the AP4C (S'STE = 0.87STDI + 0.60, p < 0.001, r = 0.78; mean bias -0.6 cm/s, 95% limits of agreement (LoA) -3.5 to 2.4 cm/s). Similarly results were found for E', but not A' (E'STE = 0.69E'TDI + 2.37, p < 0.001, r = 0.71; mean bias 0.1 cm/s, 95%LoA -2.5 to 2.8cm/s; A'STE = 0.15A'TDI + 11.17, p = 0.629). TAPSE measurements by the authors' modified speckle-tracking-based technique were similar to TAPSE by M-mode (18.2 ± 5.5 mm and 17.1 ± 3.9 mm, respectively). CONCLUSIONS: Tricuspid annular velocities (S'STE, E'STE) determined by speckle tracking in TEE seem to be promising surrogates for velocities measured in TTE. This may be important for perioperative assessment of the right ventricle.

Population pharmacokinetics of cefazolin before, during and after cardiopulmonary bypass to optimize dosing regimens for children undergoing cardiac surgery.

Objectives: The objective of this study was to characterize cefazolin serum pharmacokinetics in children before, during and after cardiopulmonary bypass (CPB), in order to derive an evidence-based dosing regimen. Patients and methods: This study included children who received cefazolin before surgical incision, before cessation of CPB and after surgery. Blood samples of total and unbound cefazolin concentrations were collected before, during and after CPB. The cefazolin concentration-time profiles were analysed using population pharmacokinetic modelling and predictors for interindividual variability in pharmacokinetic parameters were investigated. Subsequently, optimized dosing regimens were developed using stochastic simulations. Clinicaltrials.gov: NCT02749981. Results: A total of 494 total and unbound cefazolin concentrations obtained from 56 children (aged 6 days to 15 years) were included. A two-compartment model with first-order elimination plus an additional compartment for the effect of CPB best described the data. Clearance (1.56 L/h), central volume (1.93 L) and peripheral volume (2.39 L) were allometrically scaled by body weight. The estimated glomerular filtration rate (eGFR) was identified as a covariate on clearance and the serum albumin concentration was associated with maximum protein binding capacity. Our simulations showed that an additional bolus dose at the start of CPB improves the PTA in typical patients from 59% to >94%. Prolonged surgery and preserved renal function (i.e. drop in eGFR <25%) had a negative impact on PTA. Conclusions: We propose an optimized dosing regimen for cefazolin during cardiac surgery in paediatric patients to avoid treatment failure due to inadequate antibiotic prophylaxis.

Physical Fitness and Metabolic Syndrome in Children with Repaired Congenital Heart Disease Compared with Healthy Children.

OBJECTIVE: To determine whether children who underwent surgery for congenital heart disease (CHD) are as fit as their peers. STUDY DESIGN: We studied 66 children (6-14 years) who underwent surgery for ventricular septal defect (n = 19), coarctation of aorta (n = 10), tetralogy of Fallot (n = 15), and transposition of great arteries (n = 22); and 520 healthy children (6-12 years). All children performed physical fitness tests: cardiorespiratory fitness, muscular strength, balance, flexibility, and speed. Metabolic score was assessed through z-score standardization using 4 components: waist circumference, blood pressure, blood lipids, and insulin resistance. Assessment also included self-reported and accelerometer-measured physical activity. Linear regression analyses with group (CHD vs control) as a predictor were adjusted for age, body mass index, physical activity, and parental education. RESULTS: Measured physical activity level, body mass index, cardiorespiratory fitness, flexibility, and total metabolic score did not differ between children with CHD and controls, whereas reported physical activity was greater in the CHD group than control group. Boys with CHD were less strong in upper muscular strength, speed, and balance, whereas girls with CHD were better in lower muscular strength and worse in balance. High-density lipoprotein was greater in boys and girls with CHD, whereas boys with CHD showed unhealthier glucose homeostasis. CONCLUSION: Appropriate physical fitness was achieved in children after surgery for CHD, especially in girls. Consequently, children with CHD were not at increased total metabolic risk. Lifestyle counseling should be part of every patient interaction.

Early and late outcomes after surgical management of congenital vascular rings.

Persistent respiratory or feeding problems in children may be associated with a congenital vascular ring. Surgical management is fairly standardized, but long-term outcomes are not well described. This study aims to investigate clinical presentation, surgical treatment, and risk factors for early mortality and late outcome. Our database revealed 62 surgically treated vascular ring patients between 1993 and 2014. Double aortic arch was the most common diagnosis (53%). Median age at operation was 1 year. Symptoms were mainly respiratory (89%) and feeding problems (32%). Median extubation time and hospital stay were 4 h and 5 days. Mean follow-up was 7.8 ± 5.8 years. Early mortality was 8% and was related to anatomical diagnosis, concomitant anomalies, and a need for preoperative intubation. Freedom from residual symptoms at 1 and 6 months was 63 and 82%, respectively. Freedom from inhalation therapy at the last follow-up was 82% and was influenced by a type of vascular ring and preoperative ventilation. Dysphagia symptoms always disappeared. CONCLUSION: Surgical relief of tracheoesophageal compression is commonly effective in vascular ring anomalies. Respiratory symptoms necessitating chronic inhalation therapy only persist in a minority of children. Patients with double aortic arch are at increased risk to remain symptomatic, particularly with infectious exacerbations.

Outcome after prenatal and postnatal diagnosis of complex congenital heart defects and the influence of genetic anomalies.

OBJECTIVE: Determine prenatal detection rate, mortality and association with genetic abnormalities in patients with severe CHD. METHOD: Single center retrospective study in patients with severe CHD diagnosed prenatally or postnatally (2006 to 2014). RESULTS: A total of 567 patients were included, 176 (31%) after prenatal diagnosis, with large differences in prenatal detection rate among CHD types. Coarctation (24%), tetralogy of Fallot (21%) and univentricular heart (19%) were the most prevalent CHD. Overall mortality rate was 30% with important contributions of prenatal mortality including termination of pregnancy (40%) and postnatal compassionate care (15%). In the group requiring surgery, mortality rate was 12%. Genetic testing was available in 70%. A genetic cause was present in 140/394 patients tested (36%; 25% in the total group). Mortality was higher in the group with abnormal genetic testing compared with those with normal or no genetic testing (57/141 vs 112/423; p = 0,002). CONCLUSION: Only one third of severe CHD are detected; overall mortality remains high (30%) with major contributions of termination of pregnancy and compassionate care. A genetic cause was found in 36% and was associated with a decreased survival. Counseling must include the possibility of associated genetic pathology and its impact on survival. © 2017 John Wiley & Sons, Ltd.

Current outcomes of the bi-directional cavopulmonary anastomosis in single ventricle patients: analysis of risk factors for morbidity and mortality, and suitability for Fontan completion.

OBJECTIVES: The bi-directional cavopulmonary anastomosis forms an essential staging procedure for univentricular hearts. This review aims to identify risk factors for morbidity, mortality, and suitability for Fontan completion. METHODS: A total of 114 patients undergoing cavopulmonary anastomosis between 1992 and 2012 were reviewed to assess primary - mortality and survival to Fontan completion - and secondary outcome endpoints - re-intubation, new drain, and ICU stay. Median age and weight were 8 months and 6.9 kg, respectively. In 83% of patients, 1-3 interventions had preceded. Norwood-type procedures became more prevalent over time. RESULTS: Extubation occurred after a median of 4 hours, median ICU stay was 2 days; 10 patients (8.8%) needed re-intubation and 18 received a new drain. Higher central venous pressure and transpulmonary gradient were risk factors for new drain insertion (p<0.01). Higher pre-operative pulmonary pressure correlated with increased inotropic support and prolonged intubation (p=0.01). Need for re-intubation was significantly affected by younger age at operation (p=0.01). Hospital and pre-Fontan mortality were 11.4 and 5.3%, respectively. Operative mortality was independently affected by younger age (p=0.013), lower weight (p=0.02), longer bypass time (p=0.04), and re-intubation (p=0.004). Interstage mortality was mainly influenced by moderate ventricular function (p=0.03); 82% of survivors underwent or are candidates for Fontan completion. CONCLUSION: The cavopulmonary anastomosis remains associated with adverse outcomes. Age at operation decreases with rising prevalence of complex univentricular hearts. Considering the important impact of re-intubation on hospital mortality, peri-operative management should focus on optimising cardio-respiratory status. Once this selection step is taken, successful Fontan completion can be expected, provided that ventricular function is maintained.

Neurodevelopment and behavior after transcatheter versus surgical closure of secundum type atrial septal defect.

OBJECTIVE: To assess the neuropsychological and behavioral profiles of school-aged children treated for atrial septal defect, secundum type (ASD-II) with open-heart surgery or catheterization. STUDY DESIGN: Patients (n = 48; mean age, 9 years, 3 months) and a matched healthy group (mean age, 9 years, 2 months) were evaluated with a shortened intelligence scale (Wechsler Intelligence Scale for Children, third edition, Dutch version) and a developmental neuropsychological test battery (Developmental Neuropsychological Assessment, second edition, Dutch version). Parents completed behavioral checklists (Achenbach Child Behavior Checklist for Children aged 6-18). Hospitalization variables were retrieved from medical files for studying associations with long-term neurodevelopment. RESULTS: Compared with the healthy matched controls, patients treated for ASD-II had significantly lower scores on subtasks underlying such Developmental Neuropsychological Assessment, second edition, Dutch version domains as Attention and Executive Functioning, Language, Working Memory, Sensorimotor Functioning, Social Cognition, and Visuospatial Information Processing. Only subtle differences, mainly in Visuospatial Information Processing, were found between the surgical repair and transcatheter repair groups. Socioeconomic status, longer hospital stay, and larger defect size were associated with neurocognitive outcome measures. Parents of patients reported more thought problems, posttraumatic stress problems, and lower school performance compared with parents of healthy peers. CONCLUSION: After treatment for ASD-II, children display a range of neuropsychologic difficulties that may increase their risk for learning problems and academic underachievement. Differences related to treatment were not found. Our results suggest that neurodevelopmental and behavioral follow-up at school age is warranted in this group.

Right coronary sinus aneurysm repair.

In this case report, we describe the surgical treatment of a right coronary sinus aneurysm. A 69-year-old male patient was screened because of palpitations. He was finally diagnosed with an aneurysm of the sinus of Valsalva of the right coronary cusp. According to current aortic guidelines, surgical reconstruction was proposed. The patient underwent a cardiac operation through a median sternotomy under routine cardiopulmonary bypass. After aortic cross-clamping, the aorta was opened and the connection between the aorta and the aneurysm was clearly visualized, underneath the ostium of the right coronary artery. After excision of the right coronary button and the remaining right coronary sinus wall, this sinus was reconstructed with a Dacron graft, with subsequent coronary reimplantation. The postoperative course was uneventful. The patient was discharged on postoperative day 7. A complete sinus reconstruction was preferred over local patching of the defect because of the proximity of the aneurysm sac to the right coronary artery and the fragile, thin aortic tissue just underneath the coronary ostium.

First report from the European registry for anomalous aortic origin of coronary artery (EURO-AAOCA).

OBJECTIVES: Anomalous aortic origin of a coronary artery (AAOCA) is a group of rare congenital heart defects with various clinical presentations. The lifetime-risk of an individual living with AAOCA is unknown, and data from multicentre registries are urgently needed to adapt current recommendations and guide optimal patient management. The European AAOCA Registry (EURO-AAOCA) aims to assess differences with regard to AAOCA management between centres. METHODS: EURO-AAOCA is a prospective, multicentre registry including 13 European centres. Herein, we evaluated differences in clinical presentations and management, treatment decisions and surgical outcomes across centres from January 2019 to June 2023. RESULTS: A total of 262 AAOCA patients were included, with a median age of 33 years (12-53) with a bimodal distribution. One hundred thirty-nine (53.1%) were symptomatic, whereas chest pain (n = 74, 53.2%) was the most common complaint, followed by syncope (n = 21, 15.1%). Seven (5%) patients presented with a myocardial infarction, 2 (1.4%) with aborted sudden cardiac death. Right-AAOCA was most frequent (150, 57.5%), followed by left-AAOCA in 51 (19.5%), and circumflex AAOCA in 20 (7.7%). There were significant differences regarding diagnostics between age groups and across centres. Seventy-four (28.2%) patients underwent surgery with no operative deaths; minor postoperative complications occurred in 10 (3.8%) cases. CONCLUSIONS: Currently, no uniform agreement exists among European centres with regard to diagnostic protocols and clinical management for AAOCA variants. Although surgery is a safe procedure in AAOCA, future longitudinal outcome data will hopefully shed light on how to best decide towards optimal selection of patients undergoing revascularization versus conservative treatment.

Society of cardiovascular anesthesiologists: the effect of blood pressure regulation during aortic coarctation repair on brain, kidney, and muscle oxygen saturation measured by near-infrared spectroscopy: a randomized, clinical trial.

BACKGROUND: In this study, we compared the effects of 3 frequently used arterial blood pressure-regulating agents on brain (rScO2), renal (SrO2), and muscle (SmO2) oxygen saturation, during aortic coarctation repair in children. Based on the reported adverse effect of sodium nitroprusside (SNP) on left-sided rScO2 during aortic coarctation repair, we tested the hypothesis that the alterations in left rScO2 occurring with SNP would not be present with sevoflurane and nitroglycerin (NTG). Additionally, we explored the effects of blood pressure regulation with SNP, NTG, or sevoflurane on right-sided rScO2, SrO2, and SmO2. METHODS: Children with isolated aortic coarctation undergoing surgical repair through a left thoracotomy without the use of cardiopulmonary bypass were considered eligible for the study. During aortic cross-clamping, control of mean arterial blood pressure (MAP) was conducted according to randomization by the use of SNP, NTG, or sevoflurane to obtain a mean target right brachial blood pressure of 120% to 150% of the MAP value before cross-clamping. Bilateral rScO2, SrO2, and SmO2 were recorded continuously with near-infrared spectroscopy. As a primary end point, the maximal relative change in left-sided rScO2 in response to aortic cross-clamping was compared among treatment groups. RESULTS: Ten patients per group were included. No significant difference among treatment groups was observed in maximal relative change in left-sided rScO2 (SNP versus sevoflurane: mean difference -0.7%, 99% confidence interval [CI] -31% to 29%, P = 1.0; SNP versus NTG: mean difference -1.8%, 99% CI -32% to 28%, P = 1.0; sevoflurane versus NTG: mean difference -1.1%, 99% CI -31% to 29%, P = 1.0). Additional analyses also detected no difference between groups in right rScO2 (P = 0.4). Compared with NTG, treatment with SNP resulted in a significantly larger (-64% ± 17% vs -34% ± 25%, P = 0.01) and faster (-9 ± 4 %·min(-1) vs -4 ± 3 %·min(-1), P = 0.004) decrease in SmO2. Right-sided rScO2 and MAP showed a poor correlation for NTG (r = -0.2, P = 0.93), whereas borderline for sevoflurane (r = 0.44, P = 0.09) and SNP (r = 0.56, P = 0.04). CONCLUSIONS: The mean differences in left-sided rScO2 among the patients treated with SNP, NTG, or sevoflurane for proximal hypertension during aortic cross-clamping were no more than 32%. Additional analysis demonstrated a low MAP-rScO2 dependence with the use of NTG. Because NTG also resulted in a smaller and slower decrease of oxygen saturation in peripheral tissues, our data suggest that its use might be preferable for proximal blood pressure control during surgical procedures involving aortic cross-clamping.

Implementation of a structured information transfer checklist improves postoperative data transfer after congenital cardiac surgery.

BACKGROUND: During one hospital stay, a patient can be cared for by five different units. With patient transfer from one unit to another, it is of prime importance to convey a complete picture of the patient's situation to minimise the risk of medical errors and to provide optimal patient care. OBJECTIVE(S): This study was designed to test the hypothesis that the implementation of a standardised checklist used during verbal patient handover could improve postoperative data transfer after congenital cardiac surgery. DESIGN: Prospective, pre/postinterventional clinical study. SETTING: Cardiac centre of a university hospital. PATIENTS: Forty-eight patients younger than 16 years undergoing heart surgery. INTERVENTIONS: A standardised checklist was developed containing all data that, according to the investigators, should be communicated during the handover of a paediatric cardiac surgery patient from the operating room to the ICU. MAIN OUTCOME MEASURES: Data transfer during the postoperative handover before and after implementation of the checklist was evaluated. Duration of handover, number of interruptions, number of irrelevant data and number of confusing pieces of information were noted. Assessment of the handover process by ICU medical and nursing staff was quantified. RESULTS: After implementation of the information transfer checklist, the overall data transfer increased from 48 to 73% (P < 0.001). The duration of data transfer decreased from a median (range) of 6 (2 to 16) to 4 min (2 to 19) (P = 0.04). The overall handover assessment by the intensive care nursing staff improved significantly after implementation of the checklist. CONCLUSION: Implementation of an information transfer checklist in postoperative paediatric cardiac surgery patients resulted in a more complete transfer of information, with a decrease in the handover duration.

Neurobehavioural functioning in school-aged children with a corrected septal heart defect.

OBJECTIVE: This cross-sectional study assesses neurobehavioural consequences after surgical treatment for acyanotic congenital heart defect at the age of 5 to 12 years. METHODS AND RESULTS: Fifteen school-aged children who underwent surgical intervention for a septal heart defect were examined with the short form Wechsler Intelligence Scale for Children-third Edition, Dutch Version (WISC-III-NL), and a neuropsychological assessment with the Nepsy. Performances were compared to a matched healthy control group. Parents completed the Child Behaviour Checklist and were interviewed to rate the child's cognitive and emotional functioning. Children older than eight years filled out a self-perception questionnaire. Analyses revealed significant differences between the study groups on several cognitive and behavioural domains. Estimated intelligence scores were in the average range. Domains of attention and fine motor skills, and the subtest level of language abilities, elicited significant group differences, with less favourable outcomes for the patient group. Parents of patients reported more withdrawn behaviour, social difficulties, thought problems, attentional shortcomings and lower competence for activities. These parents also indicated poor problem solving skills in everyday activities. CONCLUSIONS: Surgical patients displayed subtle neuropsychological difficulties concerning language and fine motor skills. Behavioural difficulties were common. Future research should address risk factors for adverse neurobehavioural manifestations in this patient group.

Failure of Tricuspid Annuloplasty for Functional Tricuspid Regurgitation: Impact of Patient's Body Size.

Tricuspid repair is recommended for significant functional tricuspid regurgitation (FTR) or tricuspid annulus (TA) dilation, based on TA >40 mm or >21 mm/m². The concordance between both TA dimensions related to the patient's body size has not been investigated. Patients who underwent rigid ring tricuspid annuloplasty for FTR between 2009 and 2017 were included. Assuming equality between both TA diameter criteria, patients were divided per body surface area (BSA): group 1 = BSA ≤1.9 m² and group 2 = BSA >1.9 m². The primary outcome was TR recurrence at 5 years. Tricuspid annuloplasty was performed in 186 patients (group 1: 130 patients [69.9%]; group 2: 56 patients [30.1%]). Group 1 comprised more female (70.8% to 23.2%, p <0.001) and older patients (77.1 ± 9.3 years; 74.2 ± 8.2 years, p = 0.048). Group 1 had a smaller absolute TA diameter (group 1: 45.3 ± 5.2 mm; group 2: 48.2 ± 5.6 mm, p <0.001), whereas the indexed TA size was inversely higher (group 1: 26.3 ± 3.4 mm/m²; group 2: 24.2 ± 2.7 mm/m², p <0.001). The tenting height was comparable (group 1: 7.8 ± 3.0 mm; group 2: 8.0 ± 2.7 mm, p = 0.714). The median ring size was 30 (interquartile range 28 to 32) and 32 (interquartile range 30 to 34) for groups 1 and 2, respectively (p <0.001). TR recurrence at 5 years was noticed in 20.2% and 6.5% of group 1 and 2 (p = 0.035). Indexed TA diameter (hazard ratio 1.43, 95% confidence interval 1.10 to 1.87, p = 0.008) and tenting height (hazard ratio 5.52, 95% confidence interval 1.87 to 14.57, p = 0.002) were independent predictors of TR recurrence. In conclusion, when the absolute TA diameter is used as the primary criterion, smaller patients are at a higher risk for TR recurrence by having a proportionally larger TA at the time of repair. An individualized approach guided by patient's body size might be more appropriate to indicate FTR correction to adjust for the annuloplasty sizing method.