Prophylactic Pulmonary Artery Banding in Pediatric Dilated Cardiomyopathy: An Additional Therapeutic Option.

Dilated cardiomyopathy (DCM) is the most common childhood cardiomyopathy and is associated with considerable early mortality. Heart transplantation is often the only viable life-saving option. Pulmonary artery banding (PAB) has been recently proposed as a bridge or alternative to transplantation for DCM. In our cohort, PAB was selectively addressed to heritable DCM or DCM with congenital left ventricle aneurysm (CLVA). This study aimed to describe the clinical evolution and left ventricle reverse remodeling (LVRR) over time (6 months and 1 year after surgery). Ten patients with severe DCM received PAB between 2016 and 2021 and underwent clinical and postoperative echocardiography follow-ups. The median age at PAB was <1 year. The in-hospital mortality was zero. Two patients died two months after PAB of end-stage heart failure. The modified Ross class was improved in the eight survivors with DCM and remained stable in the two patients with CLVA. We observed a positive LVRR (LV end-diastolic diameter Z-score: 8.4 ± 3.7 vs. 2.8 ± 3; p < 0.05; LV ejection fraction: 23.8 ± 5.8 to 44.5 ± 13.1 (p < 0.05)). PAB might be useful as part of the armamentarium available in infants and toddlers with severe DCM not sufficiently responding to medical treatment with limited probability of spontaneous recovery.

Long-term results after the réparation à l'étage ventriculaire procedure for transposition of the great arteries and double-outlet right ventricle with pulmonary stenosis.

OBJECTIVES: The purpose of this study is to describe the long-term results of the 'réparation à l'étage ventriculaire' (REV) technique for double-outlet right ventricle and transposition of the great arteries (TGA) with pulmonary stenosis (PS). METHODS: Between 1980 and 2021, 157 patients underwent a REV procedure (median age and weight: 20.8 months and 7.7 kg). The most frequent anatomical presentation was the association between TGA, ventricular septal defect and PS (n = 116, 73.9%). RESULTS: Sixty-seven patients (42.7%) underwent a Rashkind procedure, and 67 patients (42.7%) a prior surgical palliation (including 62 systemic-to-pulmonary artery shunts). Resection of the conal septum and/or ventricular septal defect enlargement was performed in 109 patients (69.4%). Thirteen patients (8.3%) died, including 4 during the first postoperative month and 2 after heart transplant. Overall survival at 40 years was 89.3%. Thirty-seven patients (23.6%) required 68 reinterventions on the right ventricular outflow tract (RVOT), including 49 reoperations, with a median delay of 9 years after the REV (8 months to 27 years). Twenty patients (12.7%) underwent RVOT valvulation (16 surgical and 4 interventional). Freedom from RVOT reintervention and reoperation at 40 years were 60.3% and 62.6%, respectively. Four patients (2.5%) required reoperation for left ventricular outflow tract obstruction, with a median delay of 4.8 years. CONCLUSIONS: The REV procedure is a good alternative for TGA and double-outlet right ventricle with PS patients. Only a quarter of the patients required redo surgery on the RVOT. Reoperations for left ventricular outflow tract obstruction are scarce.

Outcomes of coronary artery obstructions after the arterial switch operation for transposition of the great arteries.

OBJECTIVE: Coronary obstruction is a rare but common complication of the arterial switch operation for transposition of the great arteries. The majority of patients remain asymptomatic and no risk factors allow targeting for reinforced surveillance. We aim to review the natural history of patients diagnosed with coronary obstruction after arterial switch operation for transposition of the great arteries and occurrence of coronary-related outcomes. METHODS: We retrospectively reviewed medical records of the 102 patients diagnosed with coronary obstruction after arterial switch operation for transposition of the great arteries in our institution from 1981 to 2022. Outcomes were anti-ischemic treatment introduction, revascularization (surgical or percutaneous angioplasty), and death; investigations that motivated revascularization were also reviewed. RESULTS: Twenty-eight out of 102 patients presented with myocardial ischemia during the immediate postoperative phase, 31 were diagnosed when symptomatic, and 43 were identified at the presymptomatic stage, according to our screening policy in preschool-aged children. Stenosis-related event occurrence was, respectively, 29 out of 31 and 32 out of 43 in the latter 2 subgroups. Coronary-related mortality reached 10% in patients diagnosed when symptomatic; no patients died in the presymptomatic subgroup. Of the 28 low-risk patients with no signs of ischemia at diagnosis, 10 developed obstruction warranting reintervention during follow-up. Revascularization was motivated by appearance of symptoms in patients with severe stenosis in normal coronary dispositions, and by clinical symptoms or documented silent ischemia in abnormal coronary patterns. CONCLUSIONS: Occurrence of stenosis-related events remains significant in patients after arterial switch operation, underlining the importance of early diagnosis for timely intervention. Initial anatomical evaluation identifies stenotic and at-risk patients; this will require periodical function testing. Follow-up modalities can be tailored to a patient's individual anatomic characteristics.

Outcomes of aortic valve repair in children stratified by complexity: Which outcome for which lesion?

OBJECTIVE: Aortic valvuloplasty frequency has significantly increased over the past 15 years. Surgical repair varies in complexity depending on valvular lesions. Our aim is to report results on the whole spectrum of aortic valvuloplasty techniques. METHODS: All children who consecutively underwent aortic valvuloplasty for aortic stenosis and/or aortic insufficiency between January 2006 and December 2020 at Necker Sick Children's Hospital (Paris, France) were included in a retrospective cohort study. Aortic valvuloplasty techniques were classified into 3 difficulty levels: (1) simple repair, corresponding to commissurotomy and/or shaving in aortic stenosis (AS) in neonates (group 1) and children >1 month (group 2); (2) intermediate-complexity repair, corresponding to commissuroplasty, leaflet resuspension, and fenestration closure in aortic insufficiency (leaflet prolapse in connective tissue disease, isolated leaflet prolapse and Laubry-Pezzi groups); and (3) complex repair requiring a pericardial patch to restore a functional aortic valve in mixed aortic valve disease (bicuspidization with neocommissure and cusp extension groups). RESULTS: During the study period, 324 children underwent aortic valvuloplasty. Survival and freedom from aortic valve reintervention at 10 years were, respectively, 86.1% and 50.9% in neonates with AS, 95.2% and 71.7% in children >1 month with AS, 93.8% and 79.5% in leaflet prolapse in connective tissue disease, 97.7% and 91.9% in isolated leaflet prolapse, 100% and 88% in those with Laubry-Pezzi syndrome, 97.4% and 84.8% in bicuspidization with neocommissure, and 100% and 54.2% in the cusp extension. CONCLUSIONS: Durability of aortic valvuloplasty techniques is satisfactory and offers the possibility to delay the Ross procedure, regardless of the lesion's complexity.

Outcomes after aortic coarctation repair in neonates weighing less than 2000 g.

BACKGROUND: Preterm birth is common in children with congenital heart disease. However, data on how to manage low-birth-weight infants with aortic coarctation are scarce and outcomes are poorly reported. Surgery is often delayed in these infants because gaining weight is supposed to improve mortality and to reduce the risk for recoarctation. METHODS: All infants weighing less than 2000 g who underwent repair for aortic coarctation at our institution between January 2017 and December 2020 were included in a retrospective study. Baseline characteristics, medical and surgical management, and outcomes, including recoarctation, death, and complications of preterm birth, were analyzed. RESULTS: A total of 15 patients had coarctation repair at a median age of 15 days and at a median weight of 1585 g. Infants with a birth weight <1200 g were operated on later and did not have higher recoarctation rates compared to those with a birth weight >1200 g. The recoarctation rate was 26.6% and one infant died of an extracardiac cause. Concerning prematurity-related complications, we observed 40% of bronchopulmonary dysplasia, 40% of intraventricular hemorrhage, and 27% of retinopathy of prematurity. These complications were more prevalent in children with a birth weight of <1200 g. CONCLUSION: Delaying surgery beyond 15 days to gain weight does not appear to decrease the risk of recoarctation and may be deleterious in low-birth-weight infants who are exposed for a longer period to risk factors of prematurity-related complications.

Resection and double pericardial patch repair of a congenital aneurysm of the mitral-aortic intervalvular fibrosa.

Mitral-aortic intervalvular fibrosa aneurysms and pseudoaneurysms are rare entities but can lead to different, unpredictable and sometime dramatic complications. We report the case of a young woman presenting a congenital form of this aneurysm. Given the clinical and symptomatological progression, surgical treatment is mandatory. After a transverse aortotomy, we resected the aneurysm. Performing a pulmonary arteriotomy allows adequate control of the left main coronary artery, adjacent to the lesion. We repaired the remaining cavity, not far from the left coronary aortic cusp, with a double patch of bovine pericardium.

Congenital disconnection of the pulmonary arteries.

OBJECTIVES: Disconnected pulmonary artery (PA) is a rare anomaly that can be isolated or associated with complex intracardiac malformations. Early reimplantation of the disconnected PA is recommended to allow growth and satisfactory pulmonary perfusion while preventing collateral artery development. The aim of this study was to describe the characteristics of patients with disconnected PA and, for those who had surgical reimplantation, to determine the incidence, delay and predictive factors of reintervention for reconnected PA stenosis. METHODS: We include patients with the diagnosis of congenitally disconnected PA and surgical repair at our institution. RESULTS: Retrospective observational study of 55 patients with a disconnected PA. Fifty-one underwent surgical correction and were followed up at our institution between 2000 and 2022. Disconnected PAs were observed in isolation in 31% of the cases. The most frequent form was left PA originating from the arterial duct (58%). The reimplantation was done at the median age of 12 days. Anastomotic stenosis was observed during follow-up in 71% of the patients with 75% of them requiring reintervention (55% of the population). The median delay to reintervention was 3.2 years after reimplantation, and >25% of reinterventions on the reimplanted PA occurred within the first postoperative year. We found more reintervention if associated cardiac defect, without significant statistic difference. Weight at re-confluence, presence of ductal tissue at the origin of the PA and prior shunt placement on the disconnected PA were not found to be risk factors for reintervention. DISCUSSION: After surgical reimplantation of PA, >50% of patients required reintervention for PA stenosis. Technical improvements should be sought to reduce the incidence of this complication.

Epicardial vs. transvenous implantable cardioverter defibrillators in children.

AIMS: The implantable cardioverter defibrillator (ICD) has been increasingly used in children. Both epicardial and transvenous approaches are used, with controversy regarding the best option with no specific recommendations. We aimed to compare outcomes associated with epicardial vs. transvenous ICDs in children. METHODS AND RESULTS: Data were analysed from a retrospective study including all patients <18-year-old implanted with an ICD in a tertiary centre from 2003 to 2021. Outcomes were compared between epicardial and transvenous ICDs. A total of 122 children with an ICD (mean age 11.5 ± 3.8 years, 57.4% males) were enrolled, with 84 (64.1%) epicardial ICDs and 38 (29.0%) transvenous ICDs. Early (<30 days) ICD-related complications were reported in 17 (20.2%) patients with an epicardial ICD vs. 0 (0.0%) with a transvenous ICD (P = 0.002). Over a mean follow-up of 4.8 ± 4.0 years, 25 (29.8%) patients with an epicardial ICD and 9 (23.7%) patients with a transvenous ICD experienced at least one late ICD-related complication [hazard ratio (HR) 1.8, 95% confidence interval (CI) 0.8-4.0]. Implantable cardioverter defibrillator lead dysfunction occurred in 19 (22.6%) patients with an epicardial ICD vs. 3 (7.9%) with a transvenous ICD (HR 5.7, 95% CI 1.3-24.5) and was associated with a higher incidence of ICD-related reintervention (HR 3.0, 95% CI 1.3-7.0). After considering potential confounders, especially age and weight at implantation, this association was no longer significant (P = 0.112). The freedom from ICD lead dysfunction was greater in patients with pleural coils than in those with epicardial coils (HR 0.38, 95% CI 0.15-0.96). CONCLUSION: In children, after a consideration of patient characteristics at implantation, the burden of complications and ICD lead dysfunction appears to be similar in patients with epicardial and transvenous devices. Pleural coils seem to be associated with better outcomes than epicardial coils in this population. CLINICAL TRIAL REGISTRATION: NCT05349162.

Bicuspid valve repair outcomes are improved with reduction and stabilization of sinotubular junction and annulus with external annuloplasty.

OBJECTIVE: We investigated long-term outcomes of bicuspid aortic valve (BAV) repair, with external annuloplasty, according to aorta phenotype. METHODS: Between 2003 and 2020, all patients with BAV operated on for aortic insufficiency (AI) and/or aneurysm were included. Repairs included isolated AI repair with subvalvular with or without sinotubular junction (STJ) (single or double) annuloplasty, supracoronary aorta replacement (with or without hemiroot remodeling), and root remodeling with external subvalvular ring annuloplasty. RESULTS: Among 343 patients operated, reparability rate was 81.3% (n = 279; age 46 ± 13.3 years). At 10 years (median follow-up: 3.42 years; interquartile range, 1.1, 5.8), survival was 93.9% (n = 8 deaths, similar to general population), cumulative incidence of reoperation was 6.2% (n = 10), AI grade >2 was 5.8% (n = 9), and grade >1 was 23.0% (n = 30). BAV repair stabilizing both the annulus and STJ with annuloplasty, compared with nonstabilized STJ repair (single annuloplasty), had lower incidence of reoperation (2.6% vs 22.5%, P = .0018) and AI grade >2 (1.2% vs 23.6%, P < .001) at 9 years. Initial commissural angle <160° was not a risk factor for reoperation, compared with angle ≥160° if symmetrical repair was achieved (2.7% and 4.1%, respectively, at 6 years, P = .85). Multivariable model showed that absence of STJ stabilization (odds ratio, 6.7; 95% confidence interval, 2.1-20, P = .001) increased recurrent AI, but not initial commissural angle <160° (odds ratio, 1.01; 95% confidence interval, 0.39-2.63, P = .98). Commissures adjusted symmetrically led to lower transvalvular gradient, compared with nonsymmetrical repair (8.7 mm Hg vs 10.2 mm Hg, P = .029). CONCLUSIONS: BAV repair, tailored to aorta phenotype, is associated with excellent durable outcomes if both annulus and STJ are reduced and stabilized with external ring annuloplasty. Commissural angle <160° is not associated with reoperation if symmetrical repair is achieved.

Indications and outcomes of cardiac catheterization following congenital heart surgery in children.

OBJECTIVES: Our goal was to evaluate the indications for postoperative cardiac catheterizations after paediatric cardiac surgeries and their impact on outcomes. METHODS: Non-planned cardiac catheterizations performed after congenital heart surgeries and before discharge between January 2013 and July 2019 were reviewed. Hybrid procedures were excluded. Heart defects, illness course, surgeries and catheter procedures were classified. Indications and findings were comprehensively regrouped. Outcomes were analysed. RESULTS: Cardiac catheterizations were performed on 192 patients (median age 2.3 months, weight 4.2 kg) on median postoperative day 7 (interquartile range, 2-17 days). Patients had defects of great complexity (79.9%), high disease severity index (46.4%), high Aristotle level of surgical complexity (75%) and a high Catheterization RISk Score for Pediatrics category of catheterizations (61%). Catheterizations confirmed 66% of suspected diagnoses. Confirmed diagnoses were more likely to be haemodynamic anomalies than anatomical lesions (81.3% > 53.7%, P < 0.001). Confirmed anatomical lesions were more likely to be residual than new lesions created by surgery (88.5% > 40.4%, P < 0.001). New diagnoses were identified in 36.5% of patients. Catheterization findings led to catheter-based or surgical interventions in 120 (62.5%) patients. Transcatheter interventions were successful (97.7%), immediate (89.5%) and performed across fresh suture lines (27.8%). Repeat catheterizations (76% interventional) were necessary in 25 (13%) patients. A high index of disease severity [odds ratio (OR): 16.26, 95% confidence interval (CI): 3.72-71.17], extracorporeal membrane oxygenation support (OR: 10.35, 95% CI: 2.78-38.56), delayed sternal closure (OR: 4.66, 95% CI: 1.25-17.32) and surgically acquired lesions (OR: 3.70, 95% CI: 1.22-11.16) were significant risk factors of 12-month mortality. CONCLUSIONS: Postoperative cardiac catheterizations answer both anatomical and haemodynamic questions in high-risk patients with complicated courses and guide subsequent treatment with satisfactory outcomes.

Ross procedure or complex aortic valve repair using pericardium in children: A real dilemma.

OBJECTIVE: Difficult to repair aortic valve lesions, requiring the use of a valve substitute, remain controversial in the face of the Ross procedure, despite undeniable technical advances. This study was undertaken to compare midterm outcomes of children treated using the Ross procedure or aortic valvuloplasty for complex aortic valve lesions. METHODS: Between January 2006 and December 2017, 126 patients aged younger than 18 years were treated for complex aortic stenosis and/or aortic insufficiency and were included in this retrospective study. Only aortic valve lesions requiring repair with an autologous or heterologous pericardial patch were considered complex lesions. Propensity score framework analyses were used to compare outcomes of the Ross and aortic valvuloplasty groups while controlling for confounders. RESULTS: Among the 126 patients with complex aortic valve lesions, propensity score matching selected 34 unique pairs of patients with similar characteristics. Survival (aortic valvuloplasty, 94.1%; Ross, 91%; P = .89), freedom from overall reintervention (aortic valvuloplasty, 50.1%; Ross, 69%; P = .32), and freedom from infective endocarditis at 8 years (aortic valvuloplasty, 100%; Ross, 85.9%; P = .21) were similar. However, freedom from reintervention in the left ventricular outflow tract at 8 years was lower after aortic valvuloplasty than after the Ross procedure (50.1% vs 100%, respectively; P = .001). CONCLUSIONS: Aortic valvuloplasty and the Ross procedure yielded similar 8-year outcomes regarding death, reoperation, and infective endocarditis although aortic valvuloplasty tended to be associated with fewer cases of infective endocarditis. Aortic valvuloplasty using a pericardial patch can be chosen as a first-line strategy for treating complex aortic valve lesions and might offer the possibility of a later Ross procedure.

Late Pediatric Mechanical Thrombectomy for Embolic Stroke as Bridge Reinforcement From LVAD to Heart Transplantation.

Although the left ventricular assist device is an important bridge to heart transplantation for patients with end-stage heart failure, it can also be a source of embolic stroke. We present a case of late intracranial mechanical thrombectomy performed for embolic stroke beyond the recommended 6 h, thus allowing for heart transplantation 4 days after intracranial mechanical thrombectomy. (Level of Difficulty: Advanced.).

Outcomes after common arterial trunk repair: Impact of the surgical technique.

OBJECTIVES: We compared the risk of mortality and reintervention after common arterial trunk (CAT) repair for different surgical techniques, in particular the reconstruction of the right ventricle outflow tract with left atrial appendage (LAA) without a monocusp. METHODS: The study population comprised 125 patients with repaired CAT who were followed-up at our institution between 2000 and 2018. Statistical analysis included Cox proportional hazard models. RESULTS: Median follow-up was 10.6 years. The 10-year survival rate was 88.2% (95% confidence interval [CI], 80.6-92.4) with the poorest outcome for CAT type IV (64.3%; 95% CI, 36.8-82.3; P < .01). In multivariable analysis, coronary anomalies (hazard ratio [HR], 11.63 [3.84-35.29], P < .001) and CAT with interrupted aortic arch (HR, 6.50 [2.10-20.16], P = .001) were substantial and independent risk factors for mortality. Initial repair with LAA was not associated with an increased risk of mortality (HR, 0.37 [0.11-1.24], P = .11). The median age at reintervention was 3.6 years [7.3 days-13.1 years]. At 10 years, freedom from reintervention was greater in the group with LAA repair compared with the valved conduit group, 73.3% (95% CI, 41.3-89.4) versus 17.2% (95% CI, 9.2-27.4) (P < .001), respectively. Using a valved conduit for repair (HR, 4.79 [2.45-9.39], P < .001), truncal valve insufficiency (HR, 2.92 [1.62-5.26], P < .001) and DiGeorge syndrome (HR, 2.01 [1.15-3.51], P = .01) were independent and clinically important risk factors for reintervention. CONCLUSIONS: For the repair of CAT, the LAA technique for right ventricle outflow tract reconstruction was associated with comparable survival and greater freedom from reintervention than the use of a valved conduit.

Three-dimensional geometry of coronary arteries after arterial switch operation for transposition of the great arteries and late coronary events.

OBJECTIVE: Using 3-dimensional (3D) modeling to predict late coronary events after the arterial switch operation (ASO) for transposition of the great arteries (TGA). METHOD: We reviewed 100 coronary computed tomography scans performed after ASO randomly selected from free-from-coronary-event patients and 21 coronary computed tomography scans from patients who had a coronary event later than 3 years after ASO. Using 3D modeling software, we defined and measured 6 geometric criteria for each coronary artery: Clockwise position of coronary ostium, First centimeter angle defined as the angle between of the coronary artery ostium and the first centimeter of the vessel, Minimal 3D angle between the coronary first centimeter and the aortic wall, ostium height defined as the distance between the ostium and the aortic valve, distance between the coronary ostium and the pulmonary artery, and distance between the coronary first centimeter and the pulmonary artery. RESULTS: None of the right ostium geometric parameters were associated with coronary events. Four out of 6 criteria of left coronary artery geometry were associated to coronary events: Clockwise position of the left ostium >67° (P < .001), First centimeter angle >62° (P < .01), minimal 3D angle <39° (P = .003), distance between the coronary ostium and the pulmonary artery <1 mm/mm (P = .03). The association of first centimeter angle >62° and minimal angle in 3D <39° had a 88% sensitivity and a 81% specificity to predict coronary events (receiver operator characteristics curve, 0.847; 95% confidence interval, 0.745-0.949; P < .001). CONCLUSIONS: The acquired geometric characteristics of the transferred left coronary artery are associated with coronary events. Imaging coronary arteries after ASO might be useful to select patients at higher risk of coronary events and to tailor surveillance.

A case series of transcatheter Potts Shunt creation in a pediatric population affected with refractory pulmonary artery hypertension: focus on the role of ECMO.

PURPOSE: Patients with suprasystemic idiopathic pulmonary hypertension (S-PAH) have a poor prognosis. Therapeutic options are limited. Reverse Potts shunt creation modifies physiology transforming patients with PAH into Eisenmenger physiology with a better outcome. Percutaneous transcatheter stent secured aortopulmonary connection (transcatheter Potts Shunt, TPS) is a feasible very high-risk procedural option in such patients. We report our experience with patients undergoing TPS at our institution requiring extracorporeal membrane oxygenation (ECMO) support. METHODS: A prospective observational study of patients with drug-refractory PAH, worsening NYHA class, and right ventricular failure undergoing TPS. Two patients required rescue ECMO for cardiac arrest during the procedure. Subsequently, "standby ECMO" was available in all the following cases and elective support was provided in patients with extremely poor conditions. RESULTS: Ten pediatric patients, underwent TPS at our institution. Two patients were rescued by ECMO after cardiac arrest during the shunt creation. This occurred as a result of the acute loading of the left ventricle (LV) after retrograde aortic arch filling through the Potts shunt. Following this, another two patients underwent elective ECMO after the uneventful induction of anesthesia. They all died postoperatively despite a successful TPS procedure. The causes of death were not related to the use of ECMO, but the complication of severe PAH. Six patients with successful TPS did not require ECMO and survived. CONCLUSIONS: TPS is a pioneering procedure offering the opportunity to treat high-risk idiopathic drug-refractory PAH patients. Acute LV failure is a complication of TPS in patients with S-PAH. Elective ECMO, an option to avoid circulatory arrest and acute profound hypoxia secondary to exclusive right-to left shunt systemic perfusion by Potts shunt and LV dysfunction with resulting pulmonary edema, may be used at the early stage of the learning curve, but it does not influence the prognosis of these patients which remains poor.

Longitudinal Analysis of Fetal Ventricular Rate for Risk Stratification in Immune Congenital Heart Block.

OBJECTIVES: To assess the perinatal risks of immune complete congenital heart block (iCCHB) based on the longitudinal analysis of fetal heart rate. METHODS: Retrospective analysis of a cohort of grade III congenital heart block diagnosed in utero, in the absence of associated cardiac defect, with positive maternal serum antibodies. Longitudinal measurements of the fetal heart rate were used to estimate the average slope of ventricular rate as a function of gestational age. We then defined the following prognostic stratification based on longitudinal follow-up observations: the high-rate (HR) group included cases for which all prenatal ventricular rate measurements were above the age-specific mean of our population of iCCHB and the low-rate (LR) group included those with at least one observation below the mean during follow-up. The 2 groups were compared to analyze the potential relationship between prenatal ventricular rate and adverse neonatal outcome defined by in utero or perinatal death, neonatal heart rate <50 bpm, or hemodynamic failure requiring emergency pacing. RESULTS: Forty-four cases were studied. Overall, the average heart rate significantly decreased during gestation from 65 bpm at 20 weeks to 55 bpm at 38 weeks. The HR and LR groups included 18 (41%) and 26 (59%) cases, respectively. Adverse perinatal outcome occurred in 1/18 (6%) and 22/26 (85%) cases in the HR and LR groups, respectively (p < 0.001). In the HR group, 33% of cases remained nonpaced at >6 months. The positive predictive values and negative predictive values for adverse perinatal outcome in the LR group were 85% (22/26) and 94% (17/18), respectively (100 and 80% <30 weeks and 88 and 78% at ≥30 weeks). CONCLUSIONS: The prognostic classification we developed based on longitudinal heart rate assessment may be used in the late 2nd or early 3rd trimester to identify iCCHB cases at high risk of adverse perinatal outcome. This prognostic stratification should help refine counseling and perinatal management earlier in pregnancy instead of waiting for late gestation or predelivery assessment.

Anomalous aortic origin of coronary arteries: an alternative to the unroofing strategy.

OBJECTIVES: Anomalous aortic origin of a coronary artery (AAOCA) is the second leading cause of sudden death in children and young adults. The most threatening anatomy is an interarterial and an intramural course, both probably involved in ischaemic phenomena and sudden death. The treatment of interarterial AAOCA remains controversial. Most of the published studies describe the results of the unroofing technique. Our study aims to evaluate the results of a different surgical approach. METHODS: From 2005 to 2019, 61 patients were operated on for an interarterial AAOCA (median age 14.7 years). Forty patients had a right AAOCA, and 21 patients had a left AAOCA including 5 patients with intraseptal course. Seventy percent of patients were symptomatic. Five patients had an aborted sudden cardiac death. Two surgical techniques were used: an 'anatomical' repair for 35 patients (15 left and 22 right AAOCA) or a coronary translocation with creation of a neo-ostia in 19 patients (1 left and 18 right AAOCA). The 5 left AAOCA patients with an intra-septal course required a complete release of the coronary artery from the septum. RESULTS: There was no early or late postoperative death. Three patients had an acute postoperative ischaemic event. Two patients required immediate angioplasty and stenting: 1 patient (7 years) with a hypoplastic right AAOCA and 1 patient (66 years) for inadequate tailoring after septal release. The third patient required an immediate surgical revision (H-2) for left AAOCA thrombosis at the level of the pericardial patch with full myocardial recovery at discharge. During follow-up, 1 patient with right AAOCA translocation and chronic chest pain required subsequent stenting and finally a coronary artery bypass grafting 2 years after initial surgery. One patient who had an asymptomatic mild right coronary stenosis 1 year after anatomical repair was successfully treated by angioplasty alone. All patients but 1 who underwent coronary translocation are totally asymptomatic. All patients with anatomical repair or septal release are free from ischaemic symptoms. CONCLUSIONS: Anatomical repair might provide a better protective option for these patients. Unlike unroofing, it treats the entire intramural segment, relocates the ostium at the appropriate sinus level and corrects any acute take-off angle.

Ascending aorta and aortic root replacement (with or without valve sparing) in early childhood: surgical strategies and long-term outcomes.

OBJECTIVES: Aortic root and ascending aorta replacements (AARs) are rarely required in the paediatric population. We report here a series of AAR performed in young children using different surgical techniques. METHODS: Between 1995 and 2017, 32 children under the age of 10 years (median age 5.4 years) underwent AAR procedures at our institution. Twenty-two (69%) had a connective tissue disease (infantile Marfan syndrome or Loeys-Dietz syndrome). We performed 11 AAR using a composite graft with a mechanical prosthesis and 21 valve-sparing procedures (10 Yacoub operations and 11 David operations). Median follow-up for operative survivors was 7.7 years (interquartile range 4.2-12.8 years). RESULTS: The cardiac-related early mortality rate was 6%. Patient survival was 91% at both 1 and 10 years. Eleven survivors (38%), all with a status of post-valve-sparing procedure, required an aortic root reintervention with an aortic valve replacement after a median interval of 4.2 years. Interestingly, only patients with infantile Marfan syndrome tended to be associated with risk of reoperation. CONCLUSIONS: Aortic root and AARs are safe in young children whatever the surgical procedure. Aortic valve-sparing procedures show good long-term results except in children with infantile Marfan syndrome whose ineluctable aortic annulus dilatation or aortic valve regurgitation requires reintervention after a short period.

Nakata index above 1500 mm2/m2 predicts death in absent pulmonary valve syndrome.

OBJECTIVES: Absent pulmonary valve syndrome is a rare congenital heart disease with severe airway compression due to dilatation of the pulmonary arteries (PAs). We investigated risk factors for death and prolonged mechanical ventilation (>7 days) and a threshold PA size for these outcomes. METHODS: This retrospective 2-centre cohort study included 68 patients with complete repair between January 1996 and December 2015. RESULTS: Median age at repair was 3.9 months (1.3-8.7 months), and median weight was 5 kg (4-7 kg). The mortality rate before hospital discharge was 12%, and the mortality rate at last follow-up was 19%. In multivariable analysis, risk factors for death were higher Nakata index [hazard ratio (HR) 1.001, 95% confidence interval (CI) 1.001-1.002; P < 0.001] and lower SpO2 (HR 1.06, 95% CI 1.02-1.09; P = 0.002). The accuracy of the Nakata index to predict death was excellent (area under the curve at 6 months: 0.92; P = 0.010). A Nakata index above 1500 mm2/m2 predicted mortality at 6 months with a sensitivity of 98% and a specificity of 82%. Twenty-five patients (37%) had prolonged mechanical ventilation. The only multivariable risk factor for prolonged ventilation was lower weight at repair (odds ratio 2.9, 95% CI 1.3-6.7; P = 0.008). Neither PA plasty nor the LeCompte manoeuvre had a protective effect on mortality or prolonged ventilation. A Nakata index above 1500 mm2/m2 remained a risk factor for mortality (P = 0.022) in patients who had a PA plasty or the LeCompte manoeuvre. CONCLUSIONS: In patients with absent pulmonary valve syndrome, the Nakata index predicts mortality with a cut-off of 1500 mm2/m2. Lower weight at repair is the only multivariable risk factor for prolonged ventilation. Neither PA plasty nor the LeCompte manoeuvre had a protective effect on these outcomes.

Single-Shot Cold Histidine-Tryptophan-Ketoglutarate Cardioplegia for Long Aortic Cross-Clamping Durations in Neonates.

OBJECTIVE: More than 30% of European pediatric cardiac surgery centers use single-dose cold histidine-tryptophan-ketoglutarate cardioplegia (Custodiol; Dr Franz Köhler Chemie GmbH, Bensheim, Germany). In neonates with transposition of the great arteries, arterial switch surgery (ASO) implies aortic division, and it is unknown whether repeated ostial cannulation causes intimal insult and affects long-term results, and therefore, single-dose Custodiol is appealing. The present study investigated the association among myocardial no-flow duration, postoperative troponins, and postoperative outcomes in neonates undergoing ASO with Custodiol cardioplegia. DESIGN: Retrospective analysis of the association among myocardial no-flow duration, postoperative troponin release (concentration magnitude × measurement duration within 48 h), and outcomes using stratification according to coronary anatomy and attending surgeon. SETTING: Single-institutional, tertiary pediatric cardiac surgery unit of a university hospital. PARTICIPANTS: The study comprised 101 neonates undergoing ASO. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The mean age of patients was 6.1 ± 5.4 days, the cardiopulmonary bypass duration was 108.7 ± 54.1 minutes, the temperature during cross-clamping was 31.1°C ± 1.7°C, the duration of mechanical ventilation was 4 (3-6) days, the length of intensive care unit stay was 7 (5-8) days, delayed sternal closure occurred in 32 (31.7%) patients, and no patients died. The myocardial no-flow duration averaged 62.3 ± 14.6 minutes and was linked with both troponin release (p = 0.04) and low cardiac output syndrome, as assessed by the requirement for delayed sternal closure (p = 0.03), regardless of cardiopulmonary bypass duration and temperature. Eighty-two percent of the patients with myocardial no-flow duration >74 minutes necessitated delayed sternal closure. CONCLUSIONS: Single-dose Custodiol may be inadequate for prolonged cross-clamping durations without myocardial perfusion in neonates.