Long-term outcomes of functional single ventricles associated with heterotaxy syndrome†.

OBJECTIVES: The goal of this study was to determine the long-term surgical outcomes of patients with functional single ventricles associated with heterotaxy syndrome, risk factors for mortality and factors associated with Fontan stage completion. METHODS: Overall, 279 patients with a functional single ventricle associated with heterotaxy syndrome who underwent an initial surgical procedure at our institute between 1978 and 2021 were grouped into 4 "eras" based on the surgical year during which the initial procedure was performed: era 1 (1978-1989, n = 71), era 2 (1990-1999, n = 98), era 3 (2000-2009, n = 64) and era 4 (2010-2021, n = 46). Neonatal surgery was more frequent in eras 3 and 4 than in eras 1 and 2. RESULTS: Overall, 228 patients had right atrial isomerism; 120 patients (43.0%) had a total anomalous pulmonary venous connection; and 58 patients (20.8%) underwent an initial procedure as neonates. Overall survival rates at 10, 20 and 30 years after the initial procedure were 47.1%, 40.6% and 36.1%, respectively. Neonatal surgery (P < 0.001), total anomalous pulmonary venous connection repair at the initial procedure (P < 0.001) and early era (P < 0.001) were identified as risk factors for mortality, with the last 2 variables being negatively associated with Fontan stage completion (P < 0.001 for both). CONCLUSIONS: Although era had a favourable effect on survival, total anomalous pulmonary venous connection with intrinsic pulmonary vein obstruction was associated with both mortality and Fontan stage completion. CLINICAL REGISTRATION NUMBER: R19092.

Death, reoperation, and late cardiopulmonary function after truncus repair.

Objective: To identify the late surgical outcomes of truncus arteriosus. Methods: Fifty consecutive patients with truncus arteriosus who underwent surgery between 1978 and 2020 at our institute were enrolled in this retrospective, single institutional cohort study. The primary outcome was death and reoperation. The secondary outcome was late clinical status, including exercise capacity. The peak oxygen uptake was measured by a ramp-like progressive exercise test on a treadmill. Results: Nine patients underwent palliative surgery, which resulted in 2 deaths. Forty-eight patients went on to truncus arteriosus repair, including 17 neonates (35.4%). The median age and body weight at repair were 92.5 days (interquartile range, 10-272 days) and 3.85 kg (interquartile range, 2.9-6.5 kg), respectively. The survival rate at 30 years was 68.5%. Significant truncal valve regurgitation (P = .030) was a risk factor for survival. Survival rates were similar between in the early 25 and late 25 patients (P = .452). The freedom from death or reoperation rate at 15 years was 35.8%. Significant truncal valve regurgitation was a risk factor (P = .001). The mean follow-up period in hospital survivors was 15.4 ± 12 years (maximum, 43 years). The peak oxygen uptake, which was performed in 12 long-term survivors at a median duration from repair of 19.7 years (interquartile range, 16.8-30.9 years), was 70.2% of predicted normal (interquartile range, 64.5%-80.4%). Conclusions: Truncal valve regurgitation was a risk factor for both survival and reoperation, thus improvement of truncal valve surgery is essential for better life prognosis and quality of life. Slightly reduced exercise tolerance was common in long-term survivors.

Patient-Prosthesis Mismatch Associated With Somatic Growth After Mechanical Mitral Valve Replacement in Small Children: Metrics for Reoperation and Outcomes.

Reoperation after pediatric mitral valve replacement (MVR) is inevitable due to patient-prosthesis mismatch (PPM) associated with somatic growth. We analyzed potential metrics for PPM and outcomes of redo MVR for valve upsizing. Between 1999 and 2018, 15 children without obstructive left heart lesions other than mitral stenosis underwent initial MVR with a 16-mm ATS-Advanced Performance valve. We analyzed hemodynamic data from 28 postoperative catheterizations and concomitant echocardiograms. The median age and body weight at initial MVR were 4.9 months (25th, 75th percentile: 3.6, 6.6) and 5.9 kg (5.0, 7.3). Redo MVR was planned when patients had congestive heart failure and postcapillary pulmonary hypertension (PH) due to PPM: systolic pulmonary arterial pressure (SPAP) >35 mm Hg and pulmonary capillary wedge pressure (PCWP) >15 mm Hg on catheterization. Indexed effective orifice area (iEOA) and mean transmitral pressure gradient (TMPG) were strongly correlated with SPAP (r = -0.72, P < 0.001 and r = 0.75, P < 0.001) and PCWP (r = -082, P < 0.001 and r = 0.84, P < 0.001). Cut-off values for detecting postcapillary PH due to PPM were 1.0 cm2/m2 for iEOA and 18 mm Hg for mean TMPG. Nine patients underwent redo MVR for postcapillary PH due to PPM at a median postoperative interval of 10 years (9.2, 11.9). All the patients survived, and PH was improved one year after surgery. iEOA and mean TMPG can be metrics for PPM in children after MVR. Careful follow-up is required to confirm the improvement of preoperatively existing PH after redo MVR for valve upsizing.

Comparison of long-term outcomes of atrial switch with Rastelli and physiological repair using left ventricle-to-pulmonary artery conduit for levo-transposition of the great arteries.

OBJECTIVES: The objectives of this study was to compare the long-term outcomes of anatomic repair using atrial switch with the Rastelli procedure versus physiological repair with left ventricle-to-pulmonary artery conduit for patients with levo-transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction. METHODS: Of patients with levo-transposition of the great arteries who underwent biventricular repair between 1978 and 2001, 31 hospital survivors after anatomic repair of atrial switch and the Rastelli (anatomic group) and 14 hospital survivors after physiological repair with left ventricle-to-pulmonary artery conduit (physiological group) were enrolled. Survival rates, reoperation rates, and most recent conditions were compared. RESULTS: The overall survival rate at 20 years was 79.7% (95% CI, 66.4%-95.6%) in the anatomic group and 85.1% (95% CI, 68.0%-100%) in the physiological group (P = .87). The reoperation rate at 10 years was 19.8% (95% CI, 5.6%-34.0%) in the anatomic group and 52.0% (95% CI, 25.0%-79.1%) in the physiological group (P = .067). Only patients in the physiological group underwent systemic tricuspid valve replacement. The anatomic group showed a better cardiac index at catheterization (2.79 ± 0.75 L/min/m2 vs 2.30 ± 0.54 L/min/m2; P = .035), lower serum brain natriuretic peptide (73 ± 86 pg/mL vs 163 ± 171 pg/mL; P = .024), and better maximal oxygen uptake in the treadmill test (64.1 ± 16.5% vs 52.7 ± 17.8% of predicted normal; P = .036), although the period until most recent catheterization, blood inspection, and treadmill testing were earlier in the anatomic group. CONCLUSIONS: Preservation of the left ventricle as the systemic ventricle using anatomic repair contributes to better cardiopulmonary condition compared with physiological repair.

Short-term outcomes of EXCOR Paediatric implantation.

OBJECTIVES: The aim of this study was to review a single institution's experience with EXCOR Paediatric implantation. METHODS: Patients <15 years old who underwent EXCOR implantation as a bridge to transplantation between 2015 and 2021 were enrolled. Major adverse events included death, cerebrovascular event resulting in sequelae, major infection (sepsis or surgical site infection requiring open sternal irrigation or device removal) and device malfunction requiring surgical treatment. RESULTS: Overall median age and weight for all 20 children at implantation were 10.8 (interquartile range, 7.9-33.2) months and 6.3 (4.6-10.2) kg. Ten patients (50%) weighed <5 kg. Primary diagnoses were dilated cardiomyopathy in 13 patients, fulminant myocarditis in 3, restrictive cardiomyopathy in 2 and congenital heart disease in 2. Two patients required biventricular assist support. The median support time was 365 (241-636) days. Six patients (30%) were supported for >20 months. One patient died. Seven patients underwent heart transplant. Heart transplant has not been performed in the last 1.5 years. Five patients were weaned from EXCOR support after native myocardial recovery, including a patient with dilated cardiomyopathy who recovered after 24 months of EXCOR support. Major complication-free survival at 6, 12 and 18 months were 79.3%, 49.6% and 38.6%, respectively. Body weight <5 kg at implantation was a risk factor for decreased major complication-free survival. CONCLUSIONS: Survival during EXCOR Paediatric support was good, but it prolonged the wait time for a heart transplant. The number of major complications increased over time and was not negligible, especially in small children.

Intermediate-term outcomes of deferred Norwood strategy.

OBJECTIVE: The aim of this study was to assess outcomes of the deferred Norwood strategy, i.e. planned Norwood following routine bilateral pulmonary artery banding and ductal stenting or continuous prostaglandin E1 administration. METHODS: Forty-five patients with hypoplastic left heart syndrome and its variants treated with the deferred Norwood strategy between 2012 and 2021 were enrolled. Mid-term outcomes were retrospectively reviewed. The median follow-up period after Norwood in hospital survivors was 4.6 years (interquartile range: 1.9, 6.8). RESULTS: Fourteen patients (31.1%) had no risk factors. The median age and weight at Norwood were 1.8 months (0.9, 3.5) and 3.1 kg (2.7, 3.6). Transplant-free survival at 6 years was 84.5%. Birth weight ≤2.5 kg and systemic atrioventricular valve (SAVV) regurgitation ≥ moderate after birth were not risk factors for mortality; body weight at Norwood ≤2.5 kg, however, was a risk factor [hazard ratio (HR), 11.3; 95% confidence interval (CI), 1.2-11; P = 0.036]. Twenty-two (48.9%) underwent Fontan with no mortalities, and 7 (15.5%) are awaiting Fontan. Freedom from SAVV surgery at 5 years was 53.1%. SAVV regurgitation ≥ moderate after birth was a risk factor for SAVV surgery (HR, 16; 95% CI, 3.6-71; P < 0.001); however, ductal stenting had a protective effect against SAVV surgery (HR, 0.09; 95% CI, 0.01-0.68; P = 0.019). Freedom from both surgical and catheter-based pulmonary artery intervention at 3 years was 27.1. CONCLUSIONS: Although deferred Norwood provided acceptable intermediate-term survival, the Fontan completion rate was unsatisfactory. SAVV surgery and pulmonary artery intervention were frequently required.

Impact of bilateral bidirectional Glenn anastomosis on staged Fontan strategy and Fontan circulation.

OBJECTIVES: The aim of this study was to identify the impact of bilateral bidirectional Glenn (BBDG) anastomosis on staged Fontan strategy and late Fontan circulation. METHODS: Of 267 patients who underwent bidirectional Glenn prior to Fontan completion between 1989 and 2013, 62 patients (23%) who underwent BBDG were enrolled in this study. Age at operation was 0.84 years (25th-75th percentile: 0.58-1.39). Thirty-three patients had heterotaxy syndrome (53%). The mean follow-up period was 12.7 ± 8.1 (max. 30.6) years. RESULTS: The overall survival rate at 15 years was 73%. Although 49 patients (79.0%) went on to Fontan completion, 12 patients (19.4%) died without achieving it. Thrombus formation and poor development in a central pulmonary artery were not observed, but obstruction of the superior vena cava (SVC) occurred in 8 patients (13%), mainly those with right atrial isomerism (P = 0.037). SVC obstruction was not, however, a risk factor for mortality (P = 0.097) or Fontan completion (P = 0.41). The shape of BBDG anastomosis, symmetricity of pulmonary blood flow, impingement of caval blood flow returning from the superior and inferior vena cavae or coexisting interrupted inferior vena cava with azygos or hemi-azygos continuation did not affect late Fontan outcomes, such as overall survival, freedom from protein-losing enteropathy or pulmonary arterio-venous malformation rates. CONCLUSIONS: SVC obstruction after BBDG frequently occurred, mainly in patients with right atrial isomerism; however, its direct impact on prognosis or achieving Fontan completion was not identified. Once Fontan circulation was established, the arrangement of the Fontan pathway did not affect late Fontan outcomes.

The reduced left ventricular stroke volume does not fully recover after pulmonary valve replacement in patients with repaired tetralogy of Fallot.

OBJECTIVES: The present study was conducted to investigate the decrease in left ventricular stroke volume index (LVSVI) that is caused by pulmonary regurgitation-induced right heart dysfunction and its clinical implications before and after pulmonary valve replacement (PVR). METHODS: Between January 2010 and December 2019, 30 adults who underwent surgical PVR for chronic pulmonary regurgitation with right ventricular dilation late after tetralogy of Fallot (TOF) repair were included. All patients were evaluated using cardiac magnetic resonance before PVR. The median interval from TOF repair to PVR was 29 [25th, 75th percentile: 25, 37] years. The median pulmonary regurgitation fraction and right ventricular end-diastolic volume index were 56 [48, 66] % and 203 [187, 239] ml/m2. Twenty-three patients (76.7%) were re-evaluated 1 year after PVR. RESULTS: Before PVR, the median LVSVI was 40 [35, 46] ml/beat/m2. A lower LVSVI was associated with a longer interval from TOF repair to PVR (r = -0.40, P = 0.029) and a lower right ventricular ejection fraction (r = 0.52, P = 0.004). A lower LVSVI was not associated with a higher right ventricular end-diastolic volume index. LVSVI remained unchanged after PVR. The patients were subdivided into Normal-stroke volume index (SVI) and Subnormal-SVI groups using the preoperative LVSVI cut-off value of 35 mL/beat/m2. Compared with the Normal-SVI group, the Subnormal-SVI group had a higher incidence of ablation therapy before PVR (4.7 vs 2.3 patient-years, P = 0.044). After PVR, LVSVI in the Subnormal-SVI group was still lower (40 [34, 42] vs 44 [42, 47] ml/beat/m2, P = 0.038) despite the right ventricular end-diastolic volume index normalization. There was no difference in the clinical event incidence between the 2 groups during the follow-up period. Brain natriuretic peptide level in the Subnormal-SVI group was higher within 3 years after PVR (P = 0.046). CONCLUSIONS: Reduced left ventricular stroke volume did not fully recover after PVR. PVR for patients with repaired TOF should be performed before the left ventricular stroke volume begins to decrease.

Renal replacement therapy with a cytokine-adsorbing hemofilter after neonatal open-heart surgery.

Two neonates with right atrial isomerism, single right ventricle, common atrioventricular valve regurgitation, and obstructive extra-cardiac total anomalous pulmonary venous connection underwent emergent total anomalous pulmonary venous connection repair combined with adjustment of pulmonary blood flow soon after birth. After the operation, both patients developed serious capillary leak syndrome, acute kidney injury, and lethal lactic acidosis with hemodynamic instability. Continuous renal replacement therapy was initiated with a cytokine-adsorbing hemofilter of polymethyl methacrylate membrane and a double lumen dialysis catheter inserted directly into the atrium. Elevated serum lactate levels were successfully decreased, and blood pressure was immediately increased.

Long-term Outcomes of Extracardiac Total Cavopulmonary Connection for Apicocaval Juxtaposition.

BACKGROUND: The long-term outcomes of a total cavopulmonary connection (TCPC) with an extracardiac conduit (ECC) for patients with apicocaval juxtaposition (ACJ) remain unclear. METHODS: A total of 38 patients with ACJ who underwent TCPC with ECC between 1998 and 2014 were enrolled in this study. For 19 patients with a superior vena cava - inferior vena cava contralateral position, a long-curved route rounding the opposite side of the apex was selected (CC group). For 11 patients with a superior vena cava-inferior vena cava ipsilateral position, a long-curved route was principally selected (IC group); however, a short, straight route was selected for 8 patients because there was sufficient space behind the ventricular apex (IS group). RESULTS: Follow-up was completed in all patients, with a mean follow-up duration of 13.2 ± 4.9 years. The angles of the caudal conduit anastomosis site measured from the frontal view of cineangiography had significantly straightened in the CC group from 1 year to 15 years (P < .05) and in the IC group from 1 year to 10 years (P < .05). There were 2 late mortalities and 6 reoperations during follow-up. Overall survival and freedom from reoperation rates at 15 years were 95% and 82%, respectively. There were no conduit-related or route-related complications such as death, reoperations, pulmonary venous obstructions, conduit obstructions, or pulmonary arteriovenous malformations in any of the groups. CONCLUSIONS: Even though chronologic geometric changes of curved ECCs were observed, TCPC with ECC for patients with ACJ can be safely applied without conduit- or route-related complications in long-term follow-up.

Interstage management of pulmonary blood flow after the Norwood procedure with right ventricle-to-pulmonary artery conduit.

OBJECTIVES: Our goal was to assess the efficacy of managing pulmonary blood flow from the Norwood procedure with a right ventricle-to-pulmonary artery (RV-PA) conduit until stage 2 palliation (S2P). METHODS: Among 48 consecutive patients undergoing the Norwood procedure between 2008 and 2018, 40 (83.3%) patients who survived to discharge were included in this study. The primary diagnosis was hypoplastic left heart syndrome in 28 (70%) patients and hypoplastic left heart syndrome variant in 12 (30%) patients. All patients received bilateral pulmonary artery banding. The median age and weight at the time of the Norwood procedure were 41 (25th-75th percentiles: 27-89) days and 3.2 (2.7-3.9) kg, respectively. In keeping with institutional strategy, S2P was undertaken when body weight exceeded 5.0 kg, and normal gross motor development was confirmed. RESULTS: The RV-PA conduit was clipped in 28 (70%) patients during the perioperative period of the Norwood procedure, then partial unclipping was performed in 8 (20%) patients and full unclipping was performed in 20 (50%) patients. Before S2P, the median pulmonary-to-systemic blood flow ratio was 1.0 (0.7-1.3). The median age and weight at the time of S2P were 10.7 (9.0-12.9) months and 6.3 (5.5-7.1) kg, respectively. The survival rate 5 years after Norwood discharge was 85.3%. Pre-S2P pulmonary-to-systemic blood flow ratio was linearly correlated with greater interstage changes in systemic atrioventricular valve regurgitation (R2 = 0.223, P = 0.004). CONCLUSIONS: Interstage management of pulmonary blood flow by RV-PA conduit clipping and gradual unclipping provided good interstage outcomes. The median pulmonary-to-systemic blood flow ratio could be controlled to 1.0 at pre-S2P catheter examination.

Serial changes of tricuspid regurgitation after anatomic repair for congenitally corrected transposition.

OBJECTIVES: The aim of this study was to reveal the serial changes in tricuspid regurgitation (TR) after anatomic repair for congenitally corrected transposition of great arteries. METHODS: Between 1995 and 2018, 48 patients underwent anatomic repair (atrial/arterial switch in 14 patients, atrial switch and Rastelli in 34 patients). The mean age and weight of the patients during anatomic repair was 33 (interquartile range 21.8-62.1) months and 12 (10.3-16.3) kg. The preoperative TR was less than mild in 15 patients (31.3%), mild-to-moderate in 29 patients (60.4%) and more-than-moderate in 4 patients (8.3%). Ebsteinoid dysplasia of the tricuspid valve (TV) was observed in 7 patients (14.6%). During the study period, no patient underwent TV surgery or bidirectional Glenn anastomosis at the time of anatomic repair. RESULTS: There was 1 in-hospital death and 1 late death. The follow-up was completed by other surviving patients, with a median follow-up period of 12.1 years (5.9-18.1). The overall survival, reoperation-free survival and freedom from permanent pacemaker implantation rate at 15 years were 94.3%, 74.3% and 81.5%, respectively. The mean TR grade was 2.0 (1.0-2.6) preoperatively, 2.0 (1.0-2.0) at 1 year, 2.0 (2.0-2.0) at 5 years and 2.0 (2.0-2.0) at 10 years after anatomic repair. A Cox proportional hazards model showed that association of Ebsteinoid dysplasia of the TV, type of anatomic repair and previous pulmonary artery banding did not affect freedom from death or the more-than-moderate TR rate. There were 2 patients who underwent TV surgery after the anatomic repair for severe TR; TV repair was successfully done for 1 patient, the other required semi-closure of TV and one and one-half ventricle conversion. CONCLUSIONS: TR remained subclinical or improved in the majority of patients after anatomic repair without TV repair. However, there were a few patients whose TR progressed to severe or massive, then required TV surgery after anatomic repair. Although exposure was difficult, TR was sometimes repairable following atrial switch, otherwise, one and one-half ventricle repair conversion would be the choice of treatment.

Long-term therapeutic effect of Fontan conversion with an extracardiac conduit.

OBJECTIVES: The aim of this study was to identify the long-term therapeutic effect of total cavopulmonary connection (TCPC) conversion with an extracardiac conduit. METHODS: Between 1991 and 2014, 36 patients underwent TCPC conversion with an extracardiac conduit. Half of these patients were diagnosed with tricuspid atresia or its variant. The left ventricle was dominant in 26 patients (72.2%). Median age at conversion and interval from initial Fontan operation to conversion were 24.1 years (interquartile range 18.9-29.2) and 17.8 years (15.4-20.9), respectively. Surgical cryoablation was concomitantly performed in 32 patients (88.9%). Cardiac catheter examination was performed preoperatively (36 patients, 100%) and at 1 year (31 patients, 86%), 5 years (25 patients, 69%) and 10 years (13 patients, 36%) after TCPC conversion. Symptom-limited treadmill exercise with expired gas analysis was performed preoperatively (32 patients, 88.9%) and at 1 year (27 patients, 75.0%), 5 years (20 patients, 55.6%) and 10 years (12 patients, 33.3%) after conversion. RESULTS: All patients received follow-up; the mean follow-up period was 8.2 ± 4.8 years. Actuarial survival rate, protein-losing enteropathy-free survival rate and rate of survival with sinus rhythm maintenance at 10 years were 79.2%, 67.8% and 48.5%, respectively. The survival curve declined steeply when the duration of Fontan circulation exceeded 25 years. New cases of protein-losing enteropathy developed postoperatively in 2 patients. Permanent pacemakers were implanted in 12 patients (33%), but atrial tachyarrhythmia was not sustained in any of the remaining patients. Pulmonary arterial pressure (11.0 ± 3.1 to 9.5 ± 3.6 mmHg, P = 0.003), pulmonary vascular resistance (2.1 ± 0.7 to 1.3 ± 0.5 WU/m2, P < 0.0001) and cardiac index (2.0 ± 0.3 to 2.9 ± 0.6 l/min/m2, P < 0.0001) significantly improved from preoperative evaluation to 1 year after the conversion, and these improvements were maintained during the entire follow-up period. Peak oxygen uptake remained unchanged from the preoperative evaluation (49.7 ± 11.5% predicted) to 1 year (52.5 ± 12.0%), 5 years (56.2 ± 9.6%) and 10 years (51.2 ± 9.4%) after conversion (P = 0.19). CONCLUSIONS: Owing to its anti-arrhythmic effect and Fontan pathway recruitment effect, TCPC conversion with an extracardiac conduit prevented the natural decline of exercise tolerance that is seen in classic Fontan patients.

Application of modified bicaval technique for pediatric heart transplant with oversized donor heart.

Whereas bicaval technique is an effective surgical method, standard bicaval technique for younger age and donor/recipient caval mismatch was reported to have a risk of superior vena caval obstruction. Between 2016 and 2019, three patients with dilated cardiomyopathy aged 10 years or younger underwent orthotropic heart transplantation with modified bicaval technique at our institute. Donor/recipient body weight and height ratios were 2.36, 0.77, and 2.61 and 1.37, 0.94, and 1.51, respectively. All patients were preoperatively supported by a left ventricular assist device: Excor Pediatric in two patients and Jarvik 2000 in one. Duration of LVAD support was 180, 238, and 220 days. One patient required revision of pulmonary anastomosis during the operation; accordingly, the chest was closed 3 days later. There was no mortality. Caval obstructions were not observed. Three months after the operation, tricuspid regurgitation was mild in two patients and trivial in one.

Midterm results and risk factors of functional single ventricles with extracardiac total anomalous pulmonary venous connection.

OBJECTIVES: To evaluate the clinical outcomes of surgical repair of an extracardiac total anomalous pulmonary venous connection (TAPVC) in a functional single-ventricle (f-SV) strategy. METHODS: This was a retrospective analysis of 48 consecutive cases of extracardiac TAPVC repair, from 1998 to 2015. Demographic and clinical variables were as follows: median age, 24 (range 0-744) days; median weight, 3.1 (range 2.0-9.6) kg; type of TAPVC-supracardiac, 21 patients, infracardiac, 8, and mixed, 19; right atrial isomerism, 45 patients; pulmonary atresia, 24 patients; and obstructed TAPVC, 30 patients. Concomitant procedures included systemic-to-pulmonary shunting in 15 patients, pulmonary artery banding in 12, ventricle-to-pulmonary artery shunting in 2, a Norwood procedure in one, a bidirectional Glenn procedure in 16, and a Fontan procedure in 1. RESULTS: The 1- and 5-year cumulative survival rates were 66.0% and 58.0%, respectively. Of the 28 survivors, 22 (78.6%) underwent Fontan completion and 4 (14.3%) a bidirectional Glenn procedure, and 1 (3.6%) was awaiting a bidirectional Glenn procedure. Recurrent pulmonary venous stenosis (PVS) was observed in 17 patients, with a 1- and 5-year rates of freedom from recurrent PVS 59.8% and 53.5%, respectively. Of the 20 post-operative deaths, only 4 were PVS related. Only pre-operative pulmonary atresia was identified as an independent risk factor of mortality after TAPVC repair. CONCLUSIONS: The midterm surgical outcomes of f-SV with extracardiac TAPVC were acceptable. Moreover, among survivors, Fontan completion can be sufficiently expected. Further improvement, with the development of a comprehensive treatment strategy, is required for this patient group.

Pediatric Case of Staphylococcus lugdunensis-Induced Infective Endocarditis at Bovine Jugular Vein.

A 2-year-old boy underwent repeat right ventricular outflow tract reconstruction (re-RVOTR) with a bovine jugular vein (BJV) graft. He presented with high fever on postoperative day 6. Blood and drainage effusion cultures were all positive for Staphylococcus lugdunensis and vancomycin was prescribed. Echocardiography showed vegetation at the BJV. Re-re-RVOTR was performed 51 days after re-RVOTR. In the operation, vegetation was adhered to the right-sided leaflet and three leaflets were degenerated. After complete BJV graft resection, an expanded polytetrafluoroethylene conduit with trileaflets was implanted. There was no sign of recurrent infection 8 months after the surgery.

Novel Left Atrioventricular Valvuloplasty for Atrioventricular Septal Defect.

An infant boy with 21 trisomy was diagnosed with complete atrioventricular septal defect-Rastelli type A with parachute left atrioventricular valve and absent left mural leaflet. Biventricular repair was difficult in infancy because the left ventricular volume and left atrioventricular valve annulus were too small. After 2 pulmonary artery bandings, the left atrioventricular valve annulus increased to 85% of normal mitral valve, and the left ventricular end-diastolic volume increased to 98% of normal. We report a successful instance of complete atrioventricular septal defect that achieved biventricular repair via novel left atrioventricular valvuloplasty for parachute left atrioventricular valve and absent left mural leaflet.

Siglec-H is a microglia-specific marker that discriminates microglia from CNS-associated macrophages and CNS-infiltrating monocytes.

Several types of myeloid cell are resident in the CNS. In the steady state, microglia are present in the CNS parenchyma, whereas macrophages reside in boundary regions of the CNS, such as perivascular spaces, the meninges and choroid plexus. In addition, monocytes infiltrate into the CNS parenchyma from circulation upon blood-brain barrier breakdown after CNS injury and inflammation. Although several markers, such as CD11b and ionized calcium-binding adapter molecule 1 (Iba1), are frequently used as microglial markers, they are also expressed by other types of myeloid cell and microglia-specific markers were not defined until recently. Previous transcriptome analyses of isolated microglia identified a transmembrane lectin, sialic acid-binding immunoglobulin-like lectin H (Siglec-H), as a molecular signature for microglia; however, this was not confirmed by histological studies in the nervous system and the reliability of Siglec-H as a microglial marker remained unclear. Here, we demonstrate that Siglec-H is an authentic marker for microglia in mice by immunohistochemistry using a Siglec-H-specific antibody. Siglec-H was expressed by parenchymal microglia from developmental stages to adulthood, and the expression was maintained in activated microglia under injury or inflammatory condition. However, Siglec-H expression was absent from CNS-associated macrophages and CNS-infiltrating monocytes, except for a minor subset of cells. We also show that the Siglech gene locus is a feasible site for specific targeting of microglia in the nervous system. In conclusion, Siglec-H is a reliable marker for microglia that will allow histological identification of microglia and microglia-specific gene manipulation in the nervous system.

Long-term outcome of patients with right atrial isomerism after common atrioventricular valve plasty.

OBJECTIVES: To review long-term outcomes of patients with right atrial (RA) isomerism who underwent common atrioventricular valve (CAVV) plasty. METHODS: We retrospectively analysed 59 patients with RA isomerism operated on between January 2004 and April 2016. We divided patients into those with CAVV plasty (CAVV plasty (+), 29 patients) and without CAVV plasty (CAVV plasty (-), 30), and we compared the outcome between the groups. We further divided patients into those with CAVV plasty before bidirectional cavopulmonary shunt (BCPS) operation (group before BCPS, 13 patients) or CAVV plasty with or after BCPS (group with or after BCPS, 16), and we compared the outcome between these groups. We reviewed the outcomes of 7 neonatal patients who underwent CAVV plasty. RESULTS: Kaplan-Meier estimated survival rates at 10 years were 70 ± 10% and 69 ± 9% in the CAVV plasty (+) and CAVV plasty (-) groups, respectively ( P = 0.45). Kaplan-Meier estimated survival rates at 10 years were 47 ± 17% and 85 ± 10% in the group before BCPS and group with or after BCPS, respectively ( P = 0.01). Among 7 neonates in the group before BCPS, 4 are alive; Kaplan-Meier estimated survival rates at 1 year and 5 years were 60 ± 20% and 30 ± 24%, respectively. CONCLUSIONS: Patients who underwent CAVV plasty with or after BCPS had good outcomes; the outcome of patients with CAVV plasty was the same as that of those without CAVV plasty. Treatment for patients who require CAVV plasty before BCPS, especially neonates, is challenging.