scholarly journals Ventricular Tachyarrhythmia Risk in Paediatric/Young vs. Adult Brugada Syndrome Patients: A Territory-Wide Study

2021 ◽  
Vol 8 ◽  
Author(s):  
Sharen Lee ◽  
Wing Tak Wong ◽  
Ian Chi Kei Wong ◽  
Chloe Mak ◽  
Ngai Shing Mok ◽  
...  

Introduction: Brugada syndrome (BrS) is a cardiac ion channelopathy with a higher prevalence in Asia compared to the Western populations. The present study compared the differences in clinical and electrocardiographic (ECG) presentation between paediatric/young (≤25 years old) and adult (>25 years) BrS patients.Method: This was a territory-wide retrospective cohort study of consecutive BrS patients presenting to public hospitals in Hong Kong. The primary outcome was spontaneous ventricular tachycardia/ventricular fibrillation (VT/VF).Results: The cohort consists of 550 consecutive patients (median age of initial presentation = 51 ± 23 years; female = 7.3%; follow-up period = 83 ± 80 months), divided into adult (n = 505, mean age of initial presentation = 52 ± 19 years; female = 6.7%; mean follow-up period = 83 ± 80 months) and paediatric/young subgroups (n = 45, mean age of initial presentation = 21 ± 5 years, female = 13.3%, mean follow-up period = 73 ± 83 months). The mean annual VT/VF incidence rate were 17 and 25 cases per 1,000 patient-year, respectively. Multivariate analysis showed that initial presentation of type 1 pattern (HR = 1.80, 95% CI = [1.02, 3.15], p = 0.041), initial asymptomatic presentation (HR = 0.26, 95% CI = [0.07, 0.94], p = 0.040) and increased P-wave axis (HR = 0.98, 95% CI = [0.96, 1.00], p = 0.036) were significant predictors of VT/VF for the adult subgroup. Only initial presentation of VT/VF was predictive (HR = 29.30, 95% CI = [1.75, 492.00], p = 0.019) in the paediatric/young subgroup.Conclusion: Clinical and ECG presentation of BrS vary between the paediatric/young and adult population in BrS. Risk stratification and management strategies for younger patients should take into consideration and adopt an individualised approach.

EP Europace ◽  
2021 ◽  
Vol 23 (Supplement_3) ◽  
Author(s):  
S Lee ◽  
J Zhou ◽  
KHC Li ◽  
KSK Leung ◽  
I Lakhani ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. Introduction  Brugada syndrome (BrS) is an ion channelopathy that predisposes affected patients to spontaneous ventricular tachycardia/fibrillation (VT/VF) and sudden cardiac death (SCD). Despite its greater prevalence in Asia and epidemiological heterogeneity in disease manifestation, the majority of the conducted cohort studies available in current literature are based in Western countries.  Purpose The aim of this study is to examine the clinical and electrocardiographic predictive factors of spontaneous VT/VF for Asian BrS patients. Methods This was a territory-wide retrospective cohort study of patients diagnosed with BrS between 1997 and 2019. The primary outcome was spontaneous VT/VF detected either during hospital admission or by implantable-cardioverter defibrillator (ICD) data. Cox regression was used to identify significant clinical and electrocardiographic risk predictors. Non-linear interactions between variables (latent patterns) were extracted using non-negative matrix factorization (NMF) and used as inputs into the random survival forest (RSF) model. Results This study included 516 consecutive BrS patients (mean age of initial presentation= 50 ± 16 years, male= 92%) with a median follow-up of 86 (interquartile range: 45-118) months. The cohort was divided into subgroups based on initial disease manifestation: asymptomatic (n = 314), syncope (n = 159) or VT/VF (n = 41). Annualized event rates per person-year were 1.70%, 0.05% and 0.01% for the VT/VF, syncope and asymptomatic subgroups, respectively. Multivariate Cox regression analysis revealed initial presentation of VT/VF (HR = 24.0, 95% CI = [1.21, 479] , P= 0.037) and standard deviation of P-wave duration (HR = 1.07, 95% CI = [1.00, 1.13], P = 0.044) were significant predictors. The NMF-RSF showed the best predictive performance compared to RSF and Cox regression models (precision: 0.87 v.s. 0.83 v.s. 0.76, recall: 0.89 v.s. 0.85 v.s. 0.73, F1-score: 0.88 v.s. 0.84 v.s. 0.74). Conclusions This is one of the largest territory-wide cohort studies on BrS and the largest study in Asia published to date, with an extensive median follow-up duration of 7 years. Clinical history, electrocardiographic markers and investigation results provide important information for risk stratification. Machine learning techniques using NMF and RSF significantly improves overall risk stratification performance.


2021 ◽  
pp. bjophthalmol-2020-317483
Author(s):  
Jonathan El-Khoury ◽  
Majd Mustafa ◽  
Roy Daoud ◽  
Mona Harissi-Dagher

Background/aimsTo evaluate the time needed for patients with Boston type 1 keratoprosthesis (KPro) to reach their best-corrected visual acuity (BCVA) and all contributing factors.MethodsWe retrospectively reviewed 137 consecutive eyes from 118 patients, measured how long they needed to reach their BCVA and looked at factors that might affect this time duration including patient demographics, ocular comorbidities and postoperative complications.ResultsThe mean follow-up was 5.49 years. The median time to BCVA postoperatively was 6 months, with 47% of patients achieving their BCVA by 3 months. The mean best achieved logMAR visual acuity was 0.71, representing a gain of 6 lines on the Snellen visual acuity chart. Postoperative glaucoma, retroprosthetic membrane (RPM) and endophthalmitis prolonged this duration. We found no correlation between the following factors and time to BCVA: gender, age, indication for KPro surgery, primary versus secondary KPro, number of previous penetrating keratoplasties, previous retinal surgery, intraoperative anterior vitrectomy and preoperative glaucoma.ConclusionIn our retrospective cohort, the majority of subjects reached their BCVA between 3 and 6 months after KPro implantation. This duration was significantly prolonged by the development of postoperative glaucoma, RPM and endophthalmitis.


2019 ◽  
Vol 48 (6) ◽  
pp. 1526-1534
Author(s):  
Sarah L. Chen ◽  
David R. Maldonado ◽  
Cammille C. Go ◽  
Cynthia Kyin ◽  
Ajay C. Lall ◽  
...  

Background: There is a plethora of literature on outcomes after hip arthroscopic surgery in the adult population; however, outcomes in the adolescent population have not been as widely reported. Additionally, as adolescents represent a very active population, it is imperative to understand their athletic activity and return to sport after hip arthroscopic surgery. Purpose: To analyze patient-reported outcomes (PROs) after hip arthroscopic surgery in adolescents (aged 10-19 years) and present a return-to-sport analysis in the athletic adolescent subgroup. Study Design: Systematic review; Level of evidence, 4. Methods: The PubMed, Embase, and Cochrane databases were searched according to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines to identify articles that reported PROs after hip arthroscopic surgery in adolescents. The standardized mean difference was calculated to compare the effect size of hip arthroscopic surgery on various PROs. For the athletic subgroup, a return-to-sport summary was also provided. Results: Ten studies, with 618 adolescent hips and a collective study period of December 2004 to February 2015, were included in this systematic review. Across all studies, the mean age was 15.8 years (range, 11.0-19.9 years), and female patients composed approximately 56.7% of the entire cohort. The mean follow-up was 34.5 months (range, 12-120 months). The modified Harris Hip Score (mHHS) was reported in 9 studies, and at latest follow-up, scores were excellent in 4 studies (range, 90-95) and good in the remaining 5 studies (range, 82.1-89.6). All adolescents also showed significant improvement on the Non-Arthritic Hip Score (NAHS), the Hip Outcome Score–Activities of Daily Living (HOS-ADL), the HOS–Sport-Specific Subscale (HOS-SSS), the physical component of the 12-Item Short Form Health Survey (SF-12P), a visual analog scale for pain (VAS), and both versions of the International Hip Outcome Tool (iHOT-12 and iHOT-33) at latest follow-up ( P < .05). Further, mean improvements reported in all studies surpassed reported values of the minimal clinically important difference and patient acceptable symptomatic state for the mHHS, HOS-ADL, HOS-SSS, and iHOT-33. Finally, the collective return-to-sport rate among athletic adolescents was 84.9%. Conclusion: In the setting of labral tears and femoroacetabular impingement, hip arthroscopic surgery can safely be performed in adolescents and leads to significant functional improvement. Furthermore, athletic adolescents return to sport at high levels after hip arthroscopic surgery.


2022 ◽  
Author(s):  
Brian Mendel ◽  
Valerie Dirjayanto ◽  
Radityo Prakoso ◽  
Sisca Siagian

Abstract Background: Brugada Syndrome (BrS) and arrhythmogenic right ventricle dysplasia (ARVD) are rare cardiomyopathies predisposing to sudden cardiac death (SCD). Comprehending the electrocardiographic features of these cardiomyopathies are crucial especially in emergency settings.Case presentation: A 25-year old medical student presented with no complaints, but had episodes of syncope, chest pain, and palpitations of unknown origin 10 years ago. The initial assessment showed stable hemodynamics. During examination, the ECG demonstrated incomplete right bundle branch block, Brugada-type 1 pattern, with signs of Epsilon wave. The following year, assessment of the ECG was repeated and findings were found suggestive of Brugada syndrome, although his echocardiography showed no structural abnormality. According to ESC guidelines, asymptomatic Brugada patients should undergo electrophysiology study.Conclusion: Careful follow-up with electrophysiology study is recommended for this patient in order to identify the likelihood of true Brugada and suitability for radiofrequency ablation or implantation of implantable cardioverter defibrillator (ICD).


2020 ◽  
Author(s):  
Xiaoqin Yang ◽  
Kaushal Desai ◽  
Neha Agrawal ◽  
Kirti Mirchandani ◽  
Sagnik Chatterjee ◽  
...  

Abstract Background: Neurofibromatosis type 1 (NF1)-related plexiform neurofibromas (PN) can cause substantial morbidity by disfigurement and compression of vital structures. Real-world data on the burden and cost of disease among pediatric patients with NF1 and PN is limited. The objectives of this study were to describe the characteristics, treatment patterns, healthcare resource use (HCRU), and costs of these patients.Results: A total of 383 patients were included in the retrospective analysis of patients aged ≤18 with at least 1 ICD-10-CM diagnosis code for both NF1 and PN enrolled in the MarketScan® Multistate Medicaid database from October 1, 2014 to December 31, 2017. The mean follow-up was 448 days. The mean age was 11.4 years and 52.0% of patients were male. Most patients were diagnosed by a specialist (63.5%). During the follow-up period, pain medications were used by 58.5% of patients, 25.1% were treated with chemotherapy, 7.1% received surgery for PN, 1.6% received MEK inhibitors, and 0.8% received radiation. Mean per patient per year inpatient, outpatient, emergency room, pharmacy, and other visits were 1.4, 17.3, 1.6, 13.6, and 25.8, respectively. Mean ±SD (median) total per patient per year healthcare costs (2018 USD) were $17,275 ±$61,903 ($2,889), with total medical costs of $14,628 ±$56,203 ($2,334) and pharmacy costs of $2,646 ±$13,303 ($26). Inpatient costs were the largest drivers of medical cost, with a mean per patient per year cost of $6,739.Conclusions: This study showed that many pediatric patients diagnosed with NF1 and PN were treated with supportive care only, highlighting a substantial unmet medical need. This study also highlights the considerable economic burden among patients with NF1 and PN.


2011 ◽  
Vol 19 (3) ◽  
pp. 346-349 ◽  
Author(s):  
Kee Leong Ong ◽  
Arjandas Mahadev

Purpose. To review the outcome of 9 adolescents treated with 2 to 3 cannulated cancellous screws for type-1 capitellum fractures. Methods. Records of 8 boys and one girl aged 12 to 14 (mean, 14) years with type-1 capitellum fractures were reviewed. The mechanism of injury was a fall on the flexed elbow. No patient had associated injuries or neurovascular compromise. In 2 patients, the fracture was minimally displaced and treated with a cast. The remaining 7 patients with displaced fractures underwent open reduction and internal fixation using 4.0-mm cannulated partially threaded cancellous screws inserted in a posterior-to-anterior direction. In one patient, a Kirschner wire was added to fix a small comminuted fragment. Elbow pain, range of motion, stability, and function were evaluated using the Mayo Elbow Performance Index. Results. The mean follow-up period was 7 (range, 2–18) months. The mean Mayo Elbow Performance Index score was 100, indicating excellent outcome. No patient developed avascular necrosis or heterotrophic ossification. All patients achieved anatomic bone union. All implants were removed after a mean of 4.7 (range, 2–7) months. Conclusion. The fixation of type-1 capitellum fractures with 2 to 3 cannulated cancellous screws inserted posteroanteriorly achieved excellent functional outcome.


2020 ◽  
Vol 2020 ◽  
pp. 1-8
Author(s):  
Marco Rispoli ◽  
Maria Cristina Savastano ◽  
Bruno Lumbroso ◽  
Lisa Toto ◽  
Luca Di Antonio

Purpose. To evaluate structural changes in response to antivascular endothelial growth factor (anti-VEGF) treatment in patients with long-term type 1 choroidal neovascularization (CNV) by optical coherence tomography (OCT) and OCT angiography (OCTA). Method. This is a longitudinal study that involved a total of 51 eyes with type 1 CNV (35 female and 16 male eyes). Structural OCT and OCTA were performed on all the subjects. AngioVue OCTA (XR Avanti, Optovue, Inc., Fremont, CA) was used to obtain qualitative and quantitative information. All eyes were treated with an anti-VEGF ProReNata (PRN) approach and were followed for a mean of 38.9 months (SD±7.22). Best-corrected visual acuity (BCVA) was assessed at each follow-up timepoint. Results. We observed two kinds of possible evolution of type 1 CNV: “positive evolution,” including stabilization in 20% of patients and chronicity in 35%, and “negative evolution,” in which fibrosis was shown in 18% of patients, chorioretinal atrophy in 25%, and hemorrhage or RPE tears in 2%. The mean BCVA at baseline was 33.67±15.85 ETDRS letters; after 1 and 2 years, it was 31.61±18.04 and 31.18±18.58 ETDRS letters, respectively. The mean BCVA at the end of follow-up was 25.27±20 ETDRS letters. The difference between the values at baseline and at the end of follow-up was not statistically significant (P=0.06, r2=0.10). Conclusions. This study describes an in vivo structural long-term evolution of type 1 CNV by OCT and OCTA. Different possible CNV outcomes were observed. This study suggests that new retinal imaging techniques could be useful tools for assessing the potential retinal changes in the evolution of type 1 CNV to develop personalized medicine. Further studies using OCTA in the long term are needed to better understand why similarly treated type 1 CNV cases evolve differently and produce different results.


2019 ◽  
Vol 10 ◽  
pp. 204201881882220 ◽  
Author(s):  
Zohar Landau ◽  
Galit Kowen-Sandbank ◽  
Daniela Jakubowicz ◽  
Asnat Raziel ◽  
Nasser Sakran ◽  
...  

Objective: We examined short and long-term outcomes of bariatric surgery in patients with obesity and type 1 diabetes mellitus (T1DM). Methods: We reviewed the records of all adults insured by Maccabi Healthcare Services during 2010 -2015, with body mass index (BMI) ⩾30 kg/m2 and T1DM; and compared weight reduction and glucose control according to the performance of bariatric surgery. BMI and glycated hemoglobin (HbA1c) levels were extracted for baseline and every 6 months, for a mean 3.5 years. Results: Of 52 patients, 26(50%) underwent bariatric surgery. Those who underwent surgery were more often female and with a longer duration of diabetes. Immediately postoperative, 4(15%) developed diabetic ketoacidosis, while 6(23%) experienced severe hypoglycemic episodes. The mean BMI decreased among surgery patients: from 39.5±4.4 to 30.1±5.0 kg/m2 ( p < 0.0001); and increased among those who did not undergo surgery: from 33.6±3.9 to 35.1±4.4 kg/m2 ( p = 0.49). The mean HbA1c level decreased during the first 6 months postoperative: from 8.5±0.9% to 7.9±0.9%; however, at the end of follow-up, was similar to baseline, 8.6±2.0% (p = 0.87). For patients who did not undergo surgery, the mean HbA1c increased from 7.9±1.9% to 8.6±1.5% ( p = 0.09). Conclusions: Among individuals with obesity and T1DM, weight loss was successful after bariatric surgery, but glucose control did not improve. The postoperative risks of diabetic ketoacidosis and severe hypoglycemic episodes should be considering when performing bariatric surgery in this population.


2008 ◽  
Vol 18 (6) ◽  
pp. 581-585 ◽  
Author(s):  
Ebru Y. Imamoglu ◽  
Funda Oztunc ◽  
Ayse G. Eroglu ◽  
Hasan Onal ◽  
Alper Guzeltas

AbstractObjectiveWe aimed, in this study, to compare dispersion of the p wave in patients with type 1 diabetes to nondiabetic control subjects, and to investigate the relationship between the dispersion of the p wave and cardiac autonomic dysfunction in diabetic children.MethodsWe enrolled 49 patients with type 1 diabetes, and 32 age- and sex-matched healthy subjects, measuring the Valsalva ratio, resting heart rate, and orthostatic hypotension in all. The duration of the p wave was measured manually on a high-resolution computer screen. Dispersion, defined as the difference between maximum and minimum durations of the p waves, was also measured in the 12-lead electrocardiogram before and after the Valsalva maneuver.ResultsThe mean age of the patients and their controls were 14.2 ± 4.8 years, and 12.7 ± 4.5 years, respectively. The mean duration of diabetes had been 6.2 ± 4.6 years. Maximal and minimal values for the duration of the p wave were significantly decreased in the diabetic children, with the dispersion itself significantly increased. Values for the dispersion in the diabetic subjects were similar before and after the Valsalva maneuver, whereas dispersion was found significantly increased after this maneuver in the controls. The differences in the Valsalva ratio, resting heart rate, and orthostatic hypotension between the groups, on the other hand, were not found to be statistically significant.ConclusionThe noted increase in the dispersion of the p wave in diabetic children reveals the onset of cardiac electrophysiological heterogeneity before it is possible to detect parasympathetic and sympathetic dysfunction with other tests.


2017 ◽  
Vol 89 (10) ◽  
pp. 17-21 ◽  
Author(s):  
L L Bolotskaya ◽  
E G Bessmertnaya ◽  
M V Shestakova ◽  
M Sh Shamkhalova ◽  
L V Nikankina ◽  
...  

Aim. To assess the time course of changes in the level of glycated hemoglobin (HbA1c) for 20 years after the onset of type 1 diabetes mellitus (T1DM) and to compare its correlation with the development of microvascular complications, such as diabetic retinopathy (DR) and diabetic nephropathy (DN). Subjects and methods. A total of 187 children with new-onset T1DM were registered in Moscow in 1994. During the 20-year follow-up study, these patients underwent regular check-ups at the Endocrinology Research Center, Ministry of Health of the Russian Federation, which included assessment of physical data, HbA1c 2-4 times a year, biochemical blood and albuminuria tests (once per year), and ophthalmologic examination (twice a year). A total of 155 people fully completed the 20-years follow-up study. Results. During the 20-year follow-up period after the onset of T1DM, 86 of the 155 patients developed microvascular complications: DR and DN in 86 (55.5%) and 24 (15.5%) cases, respectively; while DR concurrent with DN were noted in 20 patients. By the time of their last visit, 69 (44.5%) patients had no evidence suggesting the presence of microvascular complications. The level of HbA1c at the onset of the disease in patients who later developed the complications was higher than in those without complications (10.2±0.6 and 8.5±0.2%, respectively (p = 0.003). The statistically significant differences in HbA1c levels between the groups persisted during subsequent 15 years of follow-up, averaging 9.2±1.5, 9.7±0.9, and 8.1±0.7% after 5, 10, and 15 years, respectively, in the complication group and 7.1±0.3, 8.1±0.4, and 7.2±0.2% in the non-complication group (p < 0.01). Over the last 5 years of the follow-up, the mean HbA1c level between the groups was not significantly different, which at the end of the 20-year follow-up period was 7.8±0.3 and 7.4±0.6%, respectively (p > 0.05). The mean duration of T1DM, in which DR developed, was 9.6±6.2, 11.0±2.0, and 13.6±4.6 years for the non-proliferative, pre-proliferative, and proliferative stages, respectively. That of T1DM, in which DN developed, was 11.8±0.6 years for microalbuminuria and 16.1±1.3 years for macroalbuminuria. Conclusion. The 20-year clinical follow-up of patients who had fallen ill with T1DM in childhood showed that diabetic microangiopathies developed with the long-term preservation of poor blood glucose control (BGC) starting at the onset of the disease. At the same time, the complications progressed to more severe stages, despite a clear trend toward better BGC. This may be suggestive of the negative metabolic memory phenomenon, which necessitates stable BGC, starting at the onset of the disease, for the prevention of microvascular complications.


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