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.

A propensity score-matched comparison between Mako robotic arm-assisted system and conventional technique in total hip arthroplasty for patients with osteoarthritis secondary to developmental dysplasia of the hip.

BACKGROUND: The clinical effectiveness of robotic arm-assisted systems remains unclear for total hip arthroplasty (THA) in patients suffering from osteoarthritis secondary to developmental dysplasia of the hip (DDH). METHODS: Patients with DDH who underwent primary THA were included in this study. We conducted a propensity score-matched comparison between THAs using a robotic arm-assisted system (Mako group) versus those using the manual procedure (manual group) to compare the absolute differences in cup placement angles measured using postoperative computed tomography and those planned preoperatively. RESULTS: A total of 217 patients with osteoarthritis due to DDH met the inclusion criteria. Eighty-four patients were matched as the Mako group and 84 as the manual group. The differences were smaller in the Mako group than the manual group in terms of both inclination and anteversion angles (1.1 ± 1.0 versus 4.2 ± 3.1, respectively; 95% CI, 2.4 to 3.8; p < 0.0001, and 1.2 ± 1.1 versus 5.8 ± 4.0, respectively; 95% CI, 3.7 to 5.5; p < 0.0001). CONCLUSIONS: The robotic arm-assisted system may provide more accurate cup placement in THA for DDH.

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.

Long-Term Influence of Atrial Switch Operation on Hemodynamics After the Rastelli Procedure.

The study aimed to investigate the long-term influence of atrial switch on post-Rastelli hemodynamic condition. Of 112 patients with transposition of the great arteries (TGA) or TGA-type double outlet right ventricle, ventricular septal defect (VSD), and pulmonary stenosis (PS) who underwent intra-cardiac repair between 1979 and 2018, 50 patients with levo-TGA underwent atrial switch and Rastelli as an anatomic repair and 62 patients with dextro-TGA underwent Rastelli. Postoperative outcomes were retrospectively compared. The median follow-up durations were 20.1 years (interquartile range: 4.3, 32.4) in the Rastelli group and 15.3 years (7.1, 23.0) in the atrial switch plus Rastelli group (p = 0.19). Sex, age, and weight at operation were similar in both groups. Overall survival rates at 30 years were 69.8% in the Rastelli group and 80.1% in the atrial switch plus Rastelli group (p = 0.18). The atrial switch plus Rastelli group required more frequent catheter interventions (p < 0.001), mainly for caval obstruction (n = 8) and atrial arrhythmia (n = 6). Medication was more frequent in the atrial switch plus Rastelli group (p = 0.009). Exercise capacity was similarly reduced in two groups. Protein-losing enteropathy (PLE) occurred in three long-term survivors in the atrial switch plus Rastelli group (p = 0.07). Concomitantly performed atrial switch operation did not affect long-term survival and exercise capacity after Rastelli procedure. However, the occurrence of PLE, a frequent need for medication, and catheter interventions after atrial switch plus Rastelli may result from atrial switch under the post-Rastelli condition.

Impact of right ventriculotomy for tetralogy of Fallot repair with a pulmonary valve-sparing procedure.

Objectives: The study objectives were to reconfirm the superiority of the pulmonary valve-sparing procedure versus the transannular patch procedure for repair of tetralogy of Fallot and to evaluate the influence of a right ventriculotomy in the pulmonary valve-sparing procedure. Methods: Between 1978 and 2003, 440 patients (aged <10 years) underwent tetralogy of Fallot repair. Of these patients, 242 (55.0%) underwent the transannular patch procedure, 106 (24.1%) underwent the pulmonary valve-sparing procedure without right ventriculotomy, and 92 (20.9%) underwent the pulmonary valve-sparing procedure with right ventriculotomy. End points focused on adverse events and included all-cause mortality, reoperation, catheter intervention, and symptomatic arrhythmias. To compare the outcomes of pulmonary valve sparing with and without right ventriculotomy, inverse probability weighting was applied to adjust for potential confounding factors. Results: The median follow-up period was 20.3 years (interquartile range, 10.7-27.6). In all cohorts, the pulmonary valve-sparing procedure was the independent factor that reduced adverse events after tetralogy of Fallot repair (hazard ratio, 0.47; 95% confidence interval, 0.23-0.94; P = .033). After weighting, there was no difference in overall survival or event-free survival in the pulmonary valve-sparing with and without right ventriculotomy group. However, the pulmonary valve-sparing with right ventriculotomy group exhibited a larger cardiothoracic ratio (beta: 6.01; 95% confidence interval, 2.36-9.66; P = .001), lower medication-free rate (odds ratio, 0.29; 95% confidence interval, 0.098-0.79; P = .019), and higher New York Heart Association functional classification (odds ratio, 2.99; 95% confidence interval, 1.36-6.80; P = .007) at the latest follow-up. Conclusions: Right ventriculotomy for tetralogy of Fallot repair with pulmonary valve-sparing did not increase major adverse events. However, negative impacts on current status cannot be ignored.

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.

Autologous skeletal myoblast sheet implantation for pediatric dilated cardiomyopathy: A case report.

BACKGROUND: In children with dilated cardiomyopathy, heart transplantation is the last treatment option. However, new regenerative treatments, such as cell therapy, have attracted scientific attention. We have previously demonstrated the efficacy of autologous skeletal myoblast sheet implantation for treatment of ischemic and dilated cardiomyopathy in adults. Because of the mechanism underlying this cell therapy, a similar effectiveness is expected for patients with pediatric dilated cardiomyopathy. CASE: Herein, we describe the case of a child with dilated cardiomyopathy who underwent an autologous skeletal myoblast sheet implantation, which proved to be safe, and led to sustained maintenance and improvements in cardiac function and clinical status.

Clinical significance of right atrial volume in patients with repaired tetralogy of Fallot.

OBJECTIVES: Atrial arrhythmia and right ventricular (RV) diastolic dysfunction are associated with increased morbidity and mortality in patients with repaired tetralogy of Fallot (TOF). This study was performed to determine whether the preoperative right atrial (RA) volume can predict the development of atrial arrhythmia after pulmonary valve replacement (PVR) in patients with TOF. METHODS: Forty-seven consecutive adult patients with repaired TOF underwent PVR from 2003 to 2018. Seventeen patients showed preoperative atrial arrhythmia and underwent the Maze procedure concomitantly. The preoperative RA volume was evaluated with computed tomography and image analysis software. RESULTS: The median follow-up period after PVR was 6.8 years. Among 17 patients who underwent the Maze procedure, recurrent atrial arrhythmia developed in 8 patients. Among 30 patients without preoperative atrial arrhythmia, novel atrial arrhythmia developed in 2 patients. The RA volume index was a significant predictor according to the multivariate analysis (P = 0.003). The RA volume index showed a significant positive correlation with the RV end-diastolic pressure (P < 0.001). CONCLUSIONS: The RA volume predicts the development of atrial arrhythmia after PVR in patients with repaired TOF. RA volume measurement is strongly recommended in the management of this patient population.

Midterm Outcomes and Frequency of Osteolysis of Total Hip Arthroplasty Using Cementless Modular Stem for Asian Patients.

BACKGROUND: Modular stems are useful for total hip arthroplasty (THA) in anatomically difficult dysplasia. Here, we present mean 6.8-year outcomes of cementless primary THA using S-ROM-A (modified modular stem for Asian patients) femoral prosthesis in anatomically difficult cases. METHODS: Charts of 373 patients (461 hips) undergoing THA (mean age, 58 years) were reviewed for clinical evaluation of modified Merle d'Aubigné-Postel score and Kaplan-Meier survivorship with revision for any reason as the end point. For radiographic analysis, 331 patients (412 hips) followed up for ≥5 years were included. Bearing couples were metal-on-metal (n = 145), metal-on-polyethylene (n = 120), and ceramic-on-polyethylene (n = 147). Radiography and multiplanar computed tomography were performed. RESULTS: No postoperative dislocation or deep infection occurred. Mean modified Merle d'Aubigné-Postel score improved significantly (10.9 points preoperatively, 16.7 points at last follow-up; P < .001). Cumulative 5- and 10-year stem survival rates were 100% and 84%, respectively (95% confidence interval, 75%-93%). All stems were classified as bone ingrown fixation. Osteolysis occurred in metal-on-metal (42.8%) and metal-on-polyethylene (15.8%) groups. Mean time to osteolysis was ~3 years, with no significant difference between 3 groups (P = .264). In logistic regression, lower cup inclination angle was significantly associated with osteolysis (odds ratio, 0.914; 95% confidence interval, 0.84-0.99; P = .029). CONCLUSION: S-ROM-A femoral prosthesis achieved excellent midterm fixation, and the modular system was useful in primary THA with severe deformity. Bearing couples are potentially associated with adverse reactions to metal debris. S-ROM-A with ceramic-on-polyethylene bearing couples may be an option for anatomically difficult THA.

Impact of Long-Term Support with Berlin Heart EXCOR® in Pediatric Patients with Severe Heart Failure.

Berlin Heart EXCOR® (BHE) ventricular assist device (VAD) (Berlin Heart, Berlin Heart AG, Berlin, Germany) implantation is prevalent in patients with severe heart failure. However, clinical outcomes of pediatric patients on long-term BHE support remain mainly unknown. This study aimed to report our clinical experience with long-term support of pediatric patients with severe heart failure supported by BHE VAD. Clinical outcomes of 11 patients (median age 8.4 months; two male), who underwent LVAD implantation of the Berlin Heart EXCO® (BHE) VAD (Berlin Heart, Berlin Heart AG, Berlin, Germany) between 2013 and 2017 at our institution were reviewed. The median support period was 312 (range 45-661) days and five patients were supported for more than 1 year. The longest support duration was 661 days. No mortality occurred, and six patients were successfully bridged to heart transplantation, while three patients were successfully weaned off the device. Two patients are currently on BHE support while they await heart transplantation. Four patients had cerebral bleeding or infarction, but only one case of persistent neurological deficit occurred. No fatal device-related infection occurred during LVAD support. BHE VAD can provide long-term support for pediatric patients with severe heart failure with acceptable mortality and morbidity rates with long-term support.

Stroke Volume Ratio Predicts Redilatation of the Right Ventricle After Pulmonary Valve Replacement.

BACKGROUND: This study aimed to identify the prognostic value of the preoperative stroke volume ratio (right ventricular stroke volume/left ventricular stroke volume) for redilatation of the right ventricle after pulmonary valve replacement in patients with repaired tetralogy of Fallot. METHODS: From April 2004 to November 2013, 20 patients with repaired tetralogy of Fallot underwent pulmonary valve replacement for pulmonary valve regurgitation and right ventricular dilatation. Serial changes in ventricular volume were examined by cardiac magnetic resonance or computed tomography imaging. The redilatation ratio was calculated for right ventricular end-diastolic and end-systolic volume indices by dividing the increment in right ventricular volume from the first (median, 1.1 years) to the second (median, 3.2 years) evaluations after pulmonary valve replacement by the first evaluation. The relationships between the stroke volume ratio and redilatation ratio were assessed. The degree of right ventricular myocardial fibrosis was examined in 13 patients and compared with the stroke volume ratio. RESULTS: Right ventricular volume (redilatation) significantly increased from a median of 1.1 to 3.2 years after pulmonary valve replacement. Significant positive correlations were detected between the stroke volume ratio and redilatation ratio of the right ventricular end-diastolic (r = 0.50. p = 0.02) and end-systolic volume indices (r = 0.49, p = 0.03). The stroke volume ratio also showed a significant positive correlation with the degree of right ventricular myocardial fibrosis (r = 0.73, p = 0.005). CONCLUSIONS: The preoperative stroke volume ratio can predict redilatation of the right ventricle after pulmonary valve replacement and the degree of right ventricular myocardial fibrosis.

Cognitive function at rest and during exercise following breakfast omission.

It has been suggested that breakfast omission, as opposed to breakfast consumption, has the detrimental effects on cognitive function. However, the effects of acute exercise following breakfast omission on cognitive function are poorly understood, particularly during exercise. The purpose of this study was to examine the interactive effects of breakfast and exercise on cognitive function. Ten participants completed cognitive tasks at rest and during exercise in the breakfast consumption or omission conditions. Blood glucose concentration was measured immediately after each cognitive task. We used cognitive tasks to assess working memory [Spatial Delayed Response (DR) task] and executive function [Go/No-Go (GNG) task]. The participants cycled ergometer for 30 min while keeping their heart rate at 140 beats·min(-1). Accuracy of the GNG task was lower at rest in the breakfast omission condition than that in the breakfast consumption condition (Go trial: P=0.012; No-Go trial: P=0.028). However, exercise improved accuracy of the Go trial in the breakfast omission condition (P=0.013). Reaction time in the Go trial decreased during exercise relative to rest in both conditions (P=0.002), and the degree of decreases in reaction time was not different between conditions (P=0.448). Exercise and breakfast did not affect the accuracy of the Spatial DR task. The present results indicate that breakfast omission impairs executive function, but acute exercise improved executive function even after breakfast omission. It appears that beneficial effects of acute exercise on cognitive function are intact following breakfast omission.

Indication of Takeuchi technique for patients with anomalous origin of the left coronary artery from the pulmonary artery.

BACKGROUND: Intrapulmonary tunnel repair, called the Takeuchi technique, is a unique procedure for repairing anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). Since 1986, we have clearly defined the indication for the Takeuchi technique based on the location of the left coronary artery (LCA) orifice. METHODS AND RESULTS: From 1986 to 2011, 19 consecutive patients with ALCAPA underwent surgical repair; the dual-coronary system was reconstructed in 16 of these patients with either Takeuchi (n=9: 1 male; median age 14.7 years; median weight 42.7 kg) or a translocation procedure (n=7, 3 males; 0.4 years old, 5.6 kg). Takeuchi was performed in patients whose LCA arose far from the aorta (middle of posterior facing sinus in 3 patients, left side of posterior facing sinus in 2, non-facing sinus in 4). The mean postoperative follow-up period was 7.4±6.1 years in the Takeuchi group and 9.3±8.5 years in the Translocation group. Actuarial survival rate at 10 years was 87.5% and 71.4%, respectively, and the freedom from either reoperation or re-intervention rate at 10 years was 67.7% and 85.7%, respectively. All the patients who died had a preoperative left ventricular ejection fraction≤30%. CONCLUSIONS: The long-term outcome of the Takeuchi technique was acceptable. Although late reoperation and/or re-intervention cannot be disregarded, Takeuchi can be recommended when the LCA arises far from the aorta.

Surgical outcome of two difficult cases with predominant proximal pulmonary artery lesions of chronic thromboembolic pulmonary hypertension.

Pulmonary endarterectomy (PEA) for chronic thromboembolic pulmonary hypertension (CTEPH) is still challenging. The outcome of patients with proximal pulmonary artery disease is generally better than that of a distal lesion. However, we experienced poor results in two difficult cases having predominant proximal lesions even after effective PEA. Both of them had a long-time history of CTEPH and preoperative condition was critical. Although relatively large amount of thickened intima with massive thrombi were extracted from the proximal pulmonary arteries, they required postoperative percutaneous cardiopulmonary support due to residual pulmonary hypertension. Both of them finally died from pulmonary bleeding and adult respiratory distress syndrome.

Osteopontin deficiency impairs wear debris-induced osteolysis via regulation of cytokine secretion from murine macrophages.

OBJECTIVE: To investigate the molecular mechanisms underlying particle-induced osteolysis, we focused on osteopontin (OPN), a cytokine and cell-attachment protein that is associated with macrophage chemoattractant and osteoclast activation. METHODS: We compared OPN protein levels in human periprosthetic osteolysis tissues with those in osteoarthritis (OA) synovial tissues. To investigate the functions of OPN during particle-induced osteolysis in vivo, titanium particles were implanted onto the calvaria of OPN-deficient mice and their wild-type (WT) littermates. Mice were killed on day 10 and evaluated immunohistologically. The effects of OPN deficiency on the secretion of inflammatory cytokines were examined using cultured bone marrow-derived macrophages (BMMs). BMMs from OPN-deficient and WT mice were cultured with titanium particles for 12 hours, and the concentrations of inflammatory cytokines in the conditioned media were measured by enzyme-linked immunosorbent assay. RESULTS: Expression of OPN protein was enhanced in human periprosthetic osteolysis tissues as compared with OA synovial tissues. In the particle-induced model of osteolysis of the calvaria, bone resorption was significantly suppressed by OPN deficiency via inhibition of osteoclastogenesis, whereas an inflammatory reaction was observed regardless of the genotype. Results of immunostaining indicated that OPN protein was highly expressed in the membrane and bone surface at the area of bone resorption in WT mice. When BMMs were exposed to titanium particles, the concentration of proinflammatory cytokines, such as tumor necrosis factor alpha, interleukin-1alpha (IL-1alpha), IL-1beta, and IL-6, as well as chemotactic factors, such as monocyte chemoattractant protein 1 and macrophage inflammatory protein 1alpha, in the conditioned medium were significantly reduced by OPN deficiency. Whereas phagocytic activity of BMMs was not attenuated by OPN deficiency, phagocytosis-mediated NF-kappaB activation was impaired in OPN-deficient BMMs. These data indicated that OPN was implicated in the development of particle-induced osteolysis via the orchestration of pro-/antiinflammatory cytokines secreted from macrophages. CONCLUSION: OPN plays critical roles in wear debris-induced osteolysis, suggesting that OPN is a candidate therapeutic target for periprosthetic osteolysis.