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Background: The progression of atrial fibrosis long after Fontan surgery is unclear. This study aimed to evaluate the degree of atrial fibrosis long after the classic Fontan procedure and to investigate the factors associated with atrial fibrosis., Methods: We obtained atrial free wall specimens resected at Fontan conversion from 43 patients (Fontan group) and studied the degree of atrial fibrosis, along with its association with atrial tachycardia/fibrillation (AT/AF) and other clinical parameters, compared with those of the control group without heart disease (n=6)., Results: The time after the initial Fontan procedure was 19.9 (15.9-25.3) years. Atrial fibrosis (%) was more common in the Fontan group than in the control group [24.3 (20.9-35.0)% vs. 6.2 (5.6-7.5)%, p<0.001]. The severity of atrial fibrosis was mild in 16% (n=7), moderate in 54% (n=23), and severe in 30% (n=13) of cases. Atrial fibrosis (%) was more common in the persistent/permanent AT/AF group than in the no AT/AF (p<0.001) and paroxysmal AT/AF (p<0.001) groups. The maximum atrial diameter on computed tomography (CT) (mm) significantly correlated with atrial fibrosis (%) (r=0.52, p<0.001). The maximum diameter of the right atrium (≥75 mm) on CT was a significant risk factor for severe atrial fibrosis on multivariate logistic analysis (hazard ratio=10.22, 95% confidence interval=1.04-254.8, p=0.04)., Conclusions: Atrial fibrosis was prominent long after classic Fontan surgery, especially in patients with non-paroxysmal AT/AF and dilated right atrium., (Copyright © 2021. Published by Elsevier Ltd.)

Background: Little is known about the effects of preoperative electrophysiological study (EPS) and catheter ablation (CA) in Fontan surgery candidates with supraventricular tachycardia (SVT)., Objective: The purpose of this study was to investigate the clinical impact of EPS-guided intervention in Fontan surgery candidates with preceding SVT events., Methods: A total of 109 consecutive patients with a history of SVT before Fontan surgery were divided into 3 groups: 44 in whom EPS with CA was attempted (CA group); 21 in whom EPS without CA was attempted (EPS group); and 44 in whom EPS was not performed (N group). The incidence and diagnosis of SVT, acute success rate of CA, and risk factors of peri/postoperative SVT were retrospectively investigated., Results: The total incidence of SVT within 1 year after Fontan surgery was 34% (n = 37), with 91% of cases occurring within 1 month. Among the 71 SVT incidences diagnosed with EPS, 31 were atrioventricular reentrant tachycardias (AVRTs) involving twin atrioventricular nodes, 12 were atrioventricular nodal reentrant tachycardias, 12 were atrial tachycardias, 7 were orthodromic AVRTs via the accessory pathway, 7 were atrial flutters, and 2 were junctional tachycardias. The acute success rate of CA was 91% (48/53). The rate of peri/postoperative atrioventricular reciprocating SVT was significantly lower in the CA group than in the N or EPS group (11% vs 43% or 43%; P <.05). No/unsuccessful CA significantly increased the risk of peri/postoperative SVT in multivariate analysis (odds ratio 4.43; 95% confidence interval 1.69-11.59)., Conclusion: Preoperative CA reduces peri/postoperative SVT occurrence in Fontan surgery candidates at high risk for SVT., (Copyright © 2020 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Objectives: It is controversial whether to stop the fusion at L5 or S1 in adult spinal deformity (ASD) surgery. Our hypothesis is that we can stop long fusion at L5 for selected patients with less severe disability and less complex deformity. Aim was to compare minimum 5-year outcomes between ASD patients with fusion to L5 versus S1., Patients and Methods: Consecutive 40 patients (≥50 years of age) with ASD underwent spinal fusion from lower thoracic spine to L5 or S1 between 2008 and 2011. 33 patients (82.5 %) had a minimum 5-year follow-up. Lower instrumented vertebra (LIV) was L5 in 12 patients (L5 group) and S1 in 21 (S1 group). Clinical and radiographical parameters were compared between L5 and S1 group., Results: There were statistically significant differences between two groups (L5 vs S1) in %male (50 % vs 14 %), %type-N of SRS-Schwab classification (83 % vs 38 %), preop ODI (40.5 vs 56), correction loss of LL (11˚ vs 3˚), final TK (32˚ vs 50˚), correction loss of TK (-1˚ vs 17˚), final improvement of PT (3˚ vs 10˚), final improvement of PI-LL (26˚ vs 39˚), PJK (8% vs 48 %), and revision surgery rate (50 % vs 14 %). Causes of revision surgery in L5 group were distal junctional failure in 5 patients and foraminal stenosis at L5-S1 in 1. All of them underwent additional spinal fusion to the sacrum. Whereas, causes of revision surgery in S1 group were rod fracture in 2 patients and proximal junctional failure in 1., Conclusion: Although fusion to L5 was conducted for selected ASD patients with less severe disability (better ODI) and less complex deformity (type N), 50 % of the patients required additional fusion to the pelvis. Decision making to stop the long fusion at L5 for ASD patients ≥50 years of age should be made with careful considerations., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Background: In 1999, the Japanese Orthopaedic Association decided to develop a new Cervical Myelopathy Evaluation Questionnaire (JOACMEQ). The final version of the JOACMEQ, comprising 24 questions and five domains (cervical spine function (CF); upper extremity function (UF); lower extremity function (LF); bladder function (BF); and quality of life (QOL)), was established after three nationwide investigations. The fourth investigation, reported in this paper, was performed to confirm the responsiveness of the questionnaire., Methods: A total of 137 patients with cervical myelopathy were included in the study. Each patient was interviewed twice using the JOACMEQ before and after treatment. At the second interview, the patients self-rated their condition in five domains for "worse," "somewhat worse," "no change," "somewhat better," or "better," and these scores were defined as the external assessment rating. The difference of the points in five domains between the first and the second interview was calculated against each external assessment. Based on the results, substantial clinical benefit (SCB) thresholds for the JOACMEQ were determined., Results: The statistically significant median values of the acquired points were 17.5 for CF, 16.0 and 21.0 for UF, 27.0 and 20.5 for LF, 13.0 for BF, and 29.0 for QOL. After consideration of the results, the committee decided that an acquired point ≥20 could be interpreted as representing an SCB threshold for the JOACMEQ., Conclusion: We have concluded that a treatment can be judged to be effective for a patient if 1) The patient give all answers for the questions necessary to calculate the functional score of a domain and an increase of ≥20 points is obtained for that score, or 2) The functional score after treatment is > 90 points even if the answer for the unanswered questions was supposed to be the worst possible choice., (Copyright © 2018 The Authors. Published by Elsevier B.V. All rights reserved.)

This study is the first of its kind to utilize longitudinal, nationally representative panel data from the United States to assess the relationship between exposure to air pollution and reports of psychological distress. Using annual-average measures of air pollution in respondents' census blocks of residence we find that over the period 1999-2011 particulate matter 2.5 is significantly associated with increased psychological distress; this association remains even after controlling for a robust set of demographic, socioeconomic, and health-related covariates. This study suggests that public health efforts to reduce the personal and societal costs of mental illness should consider addressing not only individual characteristics and factors in the social environment, but also underexplored facets of the physical environment such as air pollution., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

Background: Validity and reliability of the Japanese Orthopaedic Association Back Pain Evaluation Questionnaire (JOABPEQ) had already been verified as the patients' self-rating assessment of low back pain and lumbar spinal disease and, the present study demonstrated the responsiveness of this measure., Methods: 192 subjects who were determined by medical instructors of the Japanese Society for Spine Surgery and Related Research were analyzed. They had completed a series of treatment and both surveys before and after the treatment. Authors investigated rates of concordance between assessment by physicians and subjective assessment by patients. The mean, standard deviation, minimum, 25th percentile, median, 75th percentile and maximum values for pre-treatment, post-treatment, and acquired points were calculated, and then, we also investigated the trend between subjective assessment by patients and mean acquired points for each JOABPEQ domain and substantial clinical benefit thresholds for the JOABPEQ., Results: Symptom changes as assessed by physicians did not coincide with those by patients, and acquired points in each JOABPEQ domain were significantly increased with improved self-rating by patients. In addition, patients who rated symptom changes as "slightly improved" showed a mean acquired points of ≥20, and those reporting "improved" showed a 25th percentile points of the acquired points of ≥20 approximately., Conclusion: A significant correlation was noted between the self-rating of patients and acquired points JOABPEQ, suggesting that ≥20 acquired points can be interpreted as substantial clinical benefit thresholds for the JOABPEQ., (Copyright © 2017 The Authors. Published by Elsevier B.V. All rights reserved.)

Background: Hybrid constructs have been widely used to surgically correct thoracic adolescent idiopathic scoliosis (AIS). To enhance the correction obtained with hybrid constructs, we perform concave rib head resection and convex costovertebral release as posterior release procedures. The objective of the study was to evaluate coronal and sagittal curve correction in patients with adolescent idiopathic scoliosis (AIS) treated with hybrid constructs combined with concave rib head resection and convex transverse process resection as posterior release procedures., Methods: The records of 24 patients with Lenke type 1 or 2 AIS treated with hybrid constructs combined with posterior release procedures were retrospectively reviewed. The mean age at surgery was 14.3 years. The mean follow-up period was 33.0 months (range, 24-60 months). Radiographs were evaluated before surgery, immediately postoperatively, and at latest follow-up., Results: The average preoperative Cobb angle of the main thoracic (MT) curve was 58.1 ± 12.6° (range, 45-88°). The MT curve was corrected to 12.8 ± 9.0° (range, 0-38°) immediately after surgery. At the latest follow-up, the average Cobb angle was 13.6 ± 9.9° (range, 0-44°; correction, 77.5 ± 14.0%). The average loss of coronal correction was 0.8°. The average preoperative flexibility of the MT curve was 54.6 ± 17.4%. The average Cincinnati correction index was 1.53 ± 0.48 at the latest follow-up. The average preoperative thoracic kyphosis (TK) was 13.7 ± 12.0° (range, -12-34°). Immediately after surgery, TK was corrected to 18.6 ± 5.9° (range, 10-29°). At the latest follow-up, TK measured 18.1 ± 6.5° (range, 6-32°)., Conclusions: Hybrid instrumentation combined with concave rib head resection and convex transverse process resection as posterior release procedures achieved satisfactory coronal and sagittal curve correction with little loss of correction at 2-year follow-up., (Copyright © 2017 The Japanese Orthopaedic Association. Published by Elsevier B.V. All rights reserved.)

Background: Coagulation abnormality is associated with a high incidence of intracardiac thrombus (ICT) and systemic thromboembolism in Fontan patients. The biomarker for detecting ICT is currently unknown., Methods: We retrospectively investigated the underlying coagulation abnormality and useful biomarkers to screen for ICT in adult Fontan patients. We measured various biomarkers of blood coagulation, fibrinolysis, and platelet activity in 122 Fontan patients (Fontan group: median age [P25-P75]: 27 [20-34] years) and compared them to those in 50 patients with atrial septal defect (ASD group: 31 [24-40] years)., Results: Regardless of whether the patient had ICT, the Fontan group showed significantly lower levels of antithrombin III, thrombomodulin, and α2-antiplasmin; lower protein C and protein S activities; and significantly higher levels of thrombin-antithrombin complex and α2-plasmin inhibitor complex than the ASD group. Among various biomarkers, D-dimer level measured by using latex immunoassay was significantly higher in the patients with ICT (thrombus group: n=21) than in the patients without ICT (non-thrombus group: n=101). Fifteen (26%) of 57 patients on warfarin achieved prothrombin time international normalized ratios (PT-INRs) of >2. The proportion of patients with PT-INRs of >2 tended to be lower in the thrombus group than in the non-thrombus group (13% vs 31%). Persistent atrial arrhythmia and D-dimer level were significant risk factors associated with ICT formation in the multivariate analysis (persistent atrial arrhythmia: hazard ratio [HR], 6.89; 95% confidence interval [CI], 1.44-34.5; D-dimer: HR, 0.29; 95% CI, 0.13-0.50). Receiver-operating characteristic curve analysis revealed that the appropriate cutoff D-dimer level for screening for ICT was 1.8μg/mL (area under the curve, 0.94), with a negative predictive value of 95%., Conclusions: In the adult Fontan patients, blood coagulation abnormalities existed regardless of the absence of ICT. D-dimer level may be a useful biomarker for screening for ICT in adult Fontan patients., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

With the emergence of a promising approach to treat Alzheimer disease (AD) targeting the beta-amyloid (Abeta) pathway, it is necessary to establish new diagnostic biomarkers that enable the antemortem diagnosis of AD. Although plasma Abeta has been suggested as a non-invasive biomarker, its significance has been inconclusive. Thus, it is important to improve the diagnostic potential of plasma Abeta. One of the potential approaches is to modify plasma Abeta level using various modulators. In this study, we evaluated the influence of glucometabolic status on plasma Abeta level in two lines of AD transgenic mouse. The present study demonstrated that plasma Abeta level rapidly increased after glucose loading. More importantly, the magnitude of the increase in plasma Abeta was significantly larger in AD transgenic mice than in wild-type littermates. These findings might provide a novel diagnostic tool for AD using the elevation of plasma Abeta level after glucose loading.

Background: The optimal management of atrial tachyarrhythmia (AT) late after Fontan operation has not yet been established., Methods and Results: Of 199 patients who were followed for more than 10 years after Fontan operation, 60 patients in whom late postoperative arrhythmias were observed were the subjects of this study. These arrhythmias were managed with anti-arrhythmic drugs. Twenty-one of 60 patients (35%) did not respond to the drugs and they needed further interventions. Fourteen catheter ablation procedures were performed in nine patients (atrial fibrillation in one patient, AT in eight patients) and the success rate was 44%. Conversion to total cavopulmonary connection (TCPC) was performed in 14 patients and Maze operation was performed at the time of Fontan conversion in 6 patients. Sinus rhythm or pacemaker rhythm was maintained in 7 of 14 (50%) patients postoperatively., Conclusions: Although arrhythmogenic substrates accumulate and tachyarrhythmia becomes frequent over the long-term following Fontan operation, most patients with AT can be managed medically. The success rate of catheter ablation and Maze operation is low but those interventions may be indicated in patients with intractable arrhythmias.

Objectives: Right ventricular function may deteriorate after the atrial switch operation in patients with transposition of the great arteries, but the effect on left ventricular function is unknown. Chronic left ventricular function was evaluated in adult patients after the atrial switch operation for transposition of the great arteries., Methods: Right and left ventricular functions were evaluated using data from cardiac catheterization performed in nine patients older than 17 years who had undergone the atrial switch operation for transposition of the great arteries. The mean age at operation was 19 +/- 16 months and the age at catheterization ranged from 17 to 32 years (mean age 23 +/- 5 years). Echocardiography was performed in all patients to evaluate tricuspid regurgitation. Myocardial perfusion studies using technetium-99 m-tetrofosmin were assessed in five patients., Results: Mean right ventricular end-diastolic volume was slightly increased to 122 +/- 27% of the normal value and mean right ventricular ejection fraction was depressed to 44 +/- 5% of the normal value. Left ventricular ejection fraction ranged from 37% to 63% (mean 50 +/- 7%) and was under 50% in four of the nine (44%) patients. Right ventricular ejection fraction was positively correlated with left ventricular ejection fraction(r = 0.72, p < 0.05). All patients had tricuspid regurgitation, mild in four, moderate in four, and severe in one patient. Left ventricular ejection fraction was lower in patients with moderate or severe tricuspid regurgitation(54 +/- 4%) than in patients with mild tricuspid regurgitation(47 +/- 6%, p < 0.05). Mild or moderate perfusion abnormalities were observed in all patients (five of five) who underwent myocardial perfusion studies. Mean right and left ventricular ejection fractions were 43 +/- 3% and 50 +/- 3%, respectively, in patients who underwent myocardial perfusion study, which were under the normal levels., Conclusions: Left ventricular dysfunction is common in adult patients after the atrial switch operation for transposition of the great arteries. Some right ventricular abnormalities may correlate with the left ventricular dysfunction.