Long-term prognosis of patients withJ-wave syndrome

Heart ◽  
2019 ◽  
Vol 106 (4) ◽  
pp. 299-306
Author(s):  
Tsukasa Kamakura ◽  
Tetsuji Shinohara ◽  
Kenji Yodogawa ◽  
Nobuyuki Murakoshi ◽  
Hiroshi Morita ◽  
...  

ObjectiveLimited data are currently available regarding the long-term prognosis of patients with J-wave syndrome (JWS). The aim of this study was to investigate the long-term prognosis of patients with JWS and identify predictors of the recurrence of ventricular fibrillation (VF).MethodsThis was a multicentre retrospective study (seven Japanese hospitals) involving 134 patients with JWS (Brugada syndrome (BrS): 85; early repolarisation syndrome (ERS): 49) treated with an implantable cardioverter defibrillator. All patients had a history of VF. All patients with ERS underwent drug provocation testing with standard and high intercostal ECG recordings to rule out BrS. The impact of global J waves (type 1 ECG or anterior J waves and inferolateral J waves in two or more leads) on the prognosis was evaluated.ResultsDuring the 91±66 months of the follow-up period, 52 (39%) patients (BrS: 37; ERS: 15) experienced recurrence of VF. Patients with BrS and ERS with global J waves showed a significantly higher incidence of VF recurrence than those without (BrS: log-rank, p=0.014; ERS: log-rank, p=0.0009). The presence of global J waves was a predictor of VF recurrence in patients with JWS (HR: 2.16, 95% CI 1.21 to 3.91, p=0.0095), while previously reported high-risk electrocardiographic parameters (high-amplitude J waves ≥0.2 mV and J waves associated with a horizontal or descending ST segment) were not predictive of VF recurrence.ConclusionsThis multicentre long-term study showed that the presence of global J waves was associated with a higher incidence of VF recurrence in patients with JWS.

Author(s):  
Martin Geyer ◽  
Karsten Keller ◽  
Kevin Bachmann ◽  
Sonja Born ◽  
Alexander R. Tamm ◽  
...  

Abstract Background Concomitant tricuspid regurgitation (TR) is a common finding in mitral regurgitation (MR). Transcatheter repair (TMVR) is a favorable treatment option in patients at elevated surgical risk. To date, evidence on long-term prognosis and the prognostic impact of TR after TMVR is limited. Methods Long-term survival data of patients undergoing isolated edge-to-edge repair from June 2010 to March 2018 (combinations with other forms of TMVR or tricuspid valve therapy excluded) were analyzed in a retrospective monocentric study. TR severity was categorized and the impact of TR on survival was analysed. Results Overall, 606 patients [46.5% female, 56.4% functional MR (FMR)] were enrolled in this study. TR at baseline was categorized severe/medium/mild/no or trace in 23.2/34.3/36.3/6.3% of the cases. At 30-day follow-up, improvement of at least one TR-grade was documented in 34.9%. Severe TR at baseline was identified as predictor of 1-year survival [65.2% vs. 77.0%, p = 0.030; HR for death 1.68 (95% CI 1.12–2.54), p = 0.013] and in FMR-patients also regarding long-term prognosis [adjusted HR for long-term mortality 1.57 (95% CI 1.00–2.45), p = 0.049]. Missing post-interventional reduction of TR severity was predictive for poor prognosis, especially in the FMR-subgroup [1-year survival: 92.9% vs. 78.3%, p = 0.025; HR for death at 1-year follow-up 3.31 (95% CI 1.15–9.58), p = 0.027]. While BNP levels decreased in both subgroups, TR reduction was associated with improved symptomatic benefit (NYHA-class-reduction 78.6 vs. 65.9%, p = 0.021). Conclusion In this large study, both, severe TR at baseline as well as missing secondary reduction were predictive for impaired long-term prognosis, especially in patients with FMR etiology. TR reduction was associated with increased symptomatic benefit. Graphic abstract


Genes ◽  
2021 ◽  
Vol 12 (12) ◽  
pp. 1853
Author(s):  
Brian G. Ballios ◽  
Emily M. Place ◽  
Luis Martinez-Velazquez ◽  
Eric A. Pierce ◽  
Jason I. Comander ◽  
...  

Sector and pericentral are two rare, regional forms of retinitis pigmentosa (RP). While usually defined as stable or only very slowly progressing, the available literature to support this claim is limited. Additionally, few studies have analyzed the spectrum of disease within a particular genotype. We identified all cases (9 patients) with an autosomal dominant Rhodopsin variant previously associated with sector RP (RHO c.316G > A, p.Gly106Arg) at our institution. Clinical histories were reviewed, and testing included visual fields, multimodal imaging, and electroretinography. Patients demonstrated a broad phenotypic spectrum that spanned regional phenotypes from sector-like to pericentral RP, as well as generalized disease. We also present evidence of significant intrafamilial variability in regional phenotypes. Finally, we present the longest-reported follow-up for a patient with RHO-associated sector-like RP, showing progression from sectoral to pericentral disease over three decades. In the absence of comorbid macular disease, the long-term prognosis for central visual acuity is good. However, we found that significant progression of RHO p.Gly106Arg disease can occur over protracted periods, with impact on peripheral vision. Longitudinal widefield imaging and periodic ERG reassessment are likely to aid in monitoring disease progression.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
M.A Baturova ◽  
M.M Demidova ◽  
J Carlson ◽  
D Erlinge ◽  
P.G Platonov

Abstract Introduction New onset AF is a known complication in patients with acute ST-segment elevation myocardial infarction (STEMI). However, whether new-onset AF affects the long-term prognosis to the same extent as pre-existing AF is not fully clarified and prescription of oral anticoagulants (OAC) in patients with new-onset AF remains a matter of debates. Purpose We aimed to assess the impact of new-onset AF in STEMI patients undergoing primary percutaneous intervention (PCI) on outcome during long-term follow-up in comparison with pre-existing AF and to evaluate effect of OAC therapy in patients with new-onset AF on survival. Methods Study sample comprised of 2277 consecutive patients with STEMI admitted to a tertiary care hospital for primary PCI from 2007 to 2010 (age 66±12 years, 70% male). AF prior to STEMI was documented by record linkage with the Swedish National Patient Register and review of ECGs obtained from the digital archive containing ECGs recorded in the hospital catchment area since 1988. SWEDEHEART registry was used as the source of information regarding clinical characteristics and events during index admission, including new-onset AF and OAC at discharge. All-cause mortality was assessed using the Swedish Cause-of-Death Register 8 years after discharge. Results AF prior to STEMI was documented in 177 patients (8%). Among patients without pre-existing AF (n=2100), new-onset AF was identified in 151 patients (7%). Patients with new-onset AF were older than those without AF history (74±9 vs 65±12 years, p<0.001), but did not differ in regard to other clinical characteristics. Among 2149 STEMI survivors discharged alive, 523 (24%) died during 8 years of follow-up. OAC was prescribed at discharge in 45 (32%) patients with new onset AF and in 49 (31%) patients with pre-existing AF, p=0.901. In a univariate analysis, both new-onset AF (HR 2.18, 95% CI 1.70–2.81, p<0.001) and pre-existing AF (HR 2.80, 95% CI 2.25–3.48, p<0.001) were associated with all-cause mortality, Figure 1. After adjustment for age, gender, cardiac failure, diabetes, BMI and smoking history, new-onset AF remained an independent predictor of all-cause mortality (HR 1.40, 95% CI 1.02–1.92, p=0.037). OAC prescribed at discharge in patients with new-onset AF was not significantly associated with survival (univariate HR 0.86, 95% CI 0.50–1.50, p=0.599). Conclusion New-onset AF developed during hospital admission with STEMI is common and independently predicts all-cause mortality during long-term follow-up after STEMI with risk estimates similar to pre-existing AF. The effect of OAC on survival in patients with new-onset AF is inconclusive as only one third of them received OAC therapy at discharge. Kaplan-Meier survival curve Funding Acknowledgement Type of funding source: None


Pneumologia ◽  
2019 ◽  
Vol 68 (3) ◽  
pp. 138-143
Author(s):  
Oxana Munteanu ◽  
Dumitru Chesov ◽  
Doina Rusu ◽  
Irina Volosciuc ◽  
Victor Botnaru

Abstract Pulmonary sequelae related to tuberculosis (TB) are among the major causes of bronchiectasis in Eastern Europe. The role of bacterial colonisation in the pathogenesis of bronchiectasis has been continuously studied over the last decades, less understood remains the impact of fungal infection, alone or in association with bacterial. Although the data on the development of chronic pulmonary aspergillosis (CPA) secondary to TB are scarce, recent evidence suggests a higher prevalence of CPA in patients with a past history of pulmonary TB than it was previously estimated. We present a case of natural evolution of CPA, with a radiological follow-up, in a patient with post-tuberculous bronchiectasis.


2009 ◽  
Vol 16 (2) ◽  
pp. 262-267 ◽  
Author(s):  
HEATHER G. BELANGER ◽  
ERIC SPIEGEL ◽  
RODNEY D. VANDERPLOEG

AbstractDebate continues about the long-term neuropsychological impact of multiple mild traumatic brain injuries (MTBI). A meta-analysis of the relevant literature was conducted to determine the impact of having a history of more than one self-reported MTBI (versus just one MTBI) across seven cognitive domains, as well as symptom complaints. The analysis was based on 8 studies, all conducted with athletes, involving 614 cases of multiple MTBI and 926 control cases of a single MTBI. The overall effect of multiple MTBI on neuropsychological functioning was minimal and not significant (d = 0.06). However, follow-up analyses revealed that multiple self-reported MTBI was associated with poorer performance on measures of delayed memory and executive functioning. The implications and limitations of these findings are discussed. (JINS, 2010, 16, 262–267.)


2019 ◽  
Vol 40 (27) ◽  
pp. 2206-2214 ◽  
Author(s):  
Annelieke H J Petrus ◽  
Olaf M Dekkers ◽  
Laurens F Tops ◽  
Eva Timmer ◽  
Robert J M Klautz ◽  
...  

Abstract Aims Recurrent mitral regurgitation (MR) has been reported after mitral valve repair for functional MR. However, the impact of recurrent MR on long-term survival remains poorly defined. In the present study, mortality-adjusted recurrent MR rates, the clinical impact of recurrent MR and its determinants were studied in patients after mitral valve repair with revascularization for functional MR in the setting of ischaemic heart disease. Methods and results Long-term clinical and echocardiographic outcome was evaluated in 261 consecutive patients after restrictive mitral annuloplasty and revascularization for moderate to severe functional MR, between 2000 and 2014. The cumulative incidence of recurrent MR ≥ Grade 2, assessed by competing risk analysis, was 9.6 ± 1.8% at 1-year, 20.3 ± 2.5% at 5-year, and 27.6 ± 2.9% at 10-year follow-up. Cumulative survival was 85.8% [95% confidence interval (CI) 81.0–90.0] at 1-year, 67.3% (95% CI 61.1–72.6%) at 5-year, and 46.1% (95% CI 39.4–52.6%) at 10-year follow-up. Age, preoperative New York Heart Association Class III or IV, a history of renal failure, and recurrence of MR expressed as a time-dependent variable [HR 3.28 (1.87–5.75), P < 0.001], were independently associated with an increased mortality risk. Female gender, a history of ST-elevation myocardial infarction, a preoperative QRS duration ≥120 ms, a higher preoperative MR grade, and a higher indexed left ventricular end-systolic volume were independently associated with an increased likelihood of recurrent MR. Conclusion Mitral valve repair for functional ischaemic MR resulted in a low incidence of recurrent MR with favourable clinical outcome up to 10 years after surgery. Presence of recurrent MR at any moment after surgery proved to be independently associated with an increased risk for mortality.


Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Bhuvnesh Aggarwal ◽  
Gautam Shah ◽  
Mandeep S Randhawa ◽  
A M Lincoff ◽  
Stephen G Ellis ◽  
...  

Background: A significant proportion of patients presenting with ST segment elevation myocardial infarction (STEMI) have newly diagnosed diabetes mellitus (DM). Hypothesis: Our aim was to identify patients with previously undiagnosed DM and compare their outcomes to those with known DM and without DM after STEMI. Methods: Consecutive patients undergoing primary PCI for STEMI at our center between Jan 2005 - Dec 2012 were included. Routinely performed admission Glycated hemoglobin (HbA1c) was utilized to identify patients with previously undiagnosed DM (HbA1c ≥ 6.5 and no history of DM or diabetes therapy). Patients were compared for in-hospital and long-term mortality based on follow up data from our institutional PCI registry. Results: 1,734 consecutive patients underwent primary PCI for STEMI and follow up data was available for 1,566 (90%) patients. Mean age was 60 years and 67.3% were males. A quarter of the patients (24.3%, n = 382) had prior history of DM and 8% (n=95) of the remainder had undiagnosed DM. Median follow up was 35 months. Mortality was comparable in patients with known DM and newly diagnosed DM both in hospital (11.2% vs. 12.5%, p=0.87) and at long term follow up (Figure 1, 2). Mortality was significantly worse with both groups when compared with patients with no DM (In-hospital mortality 5.6%; p<0.001 for both groups). Conclusions: One in twelve patients presenting with STEMI have previously undiagnosed DM. Cardiologists have a unique opportunity for identification and initiation of diabetic therapy in this vulnerable population. Patients with newly diagnosed DM have similar short and long-term outcomes when compared with patients with a prior history of DM.


2017 ◽  
Vol 75 (2) ◽  
pp. 90-97 ◽  
Author(s):  
Emma Lise Thorlund Jakobsen ◽  
Karin Biering ◽  
Anette Kærgaard ◽  
Annett Dalbøge ◽  
Johan Hviid Andersen

ObjectivesThe long-term prognosis for neck-shoulder pain and disorders and the impact of shoulder exposure among former sewing machine operators were investigated in a 14-year follow-up study.MethodsInformation on neck-shoulder pain and disorders was collected by questionnaire and clinical examination at baseline in 243 female sewing machine operators and by questionnaire 14 years later. During follow-up, information on comorbidity and job exposures was obtained from registers and by linking register-based D-ISCO 88 codes with a job exposure matrix. Logistic regression analyses were performed to examine associations between neck-shoulder pain and disorders at baseline and neck-shoulder pain and physical functioning at follow-up.ResultsWe found an association between neck-shoulder disorders at baseline and neck-shoulder pain at follow-up (OR 5.9;95% CI 1.9 to 17.7), and between neck-shoulder pain at baseline and neck-shoulder pain at follow-up (OR 8.2;95% CI 3.5 to 19.2). Associations between neck-shoulder disorders and pain at baseline and limited physical functioning at follow-up had ORs of 5.0 (95% CI 1.5 to 16.1) and 2.2 (95% CI 1.1 to 4.6), respectively. In women still working in 2008, the association between neck-shoulder pain in 1994 and in 2008 seemed to be stronger for those in jobs with high job shoulder exposure.ConclusionsThe results suggest a long-term adverse prognosis for neck-shoulder pain. High job shoulder exposure can worsen this prognosis for those who continue working. This knowledge could influence the counselling given to similar workers and emphasises the need to prevent neck-shoulder pain.


2015 ◽  
Vol 172 (5) ◽  
pp. 561-569 ◽  
Author(s):  
A M M Daubenbüchel ◽  
A Hoffmann ◽  
U Gebhardt ◽  
M Warmuth-Metz ◽  
A S Sterkenburg ◽  
...  

ObjectivePediatric patients with sellar masses such as craniopharyngioma (CP) or cyst of Rathke's pouch (CRP) frequently suffer disease- and treatment-related sequelae. We analyzed the impact and prognostic relevance of initial hydrocephalus (HY) and hypothalamic involvement (HI) on long-term survival and functional capacity (FC) in children with CP or CRP.Subjects and methodsUsing retrospective analysis of patient records, presence of initial HY or HI was assessed in 177 pediatric patients (163 CP and 14 CRP). Twenty-year overall survival (OS) and progression-free survival (PFS), FC, and BMI were analyzed with regard to initial HY, degree of resection, or HI.ResultsOf the 177 patients, 105 patients (103/163 CP and 2/14 CRP) presented with initial HY and 96 presented with HI. HY at diagnosis was associated (P=0.000) with papilledema, neurological deficits, and higher BMI at diagnosis and during follow-up. OS, PFS, and FC were not affected by HY at initial diagnosis. HI at diagnosis (96/177) had major negative impact on long-term prognosis. Sellar masses with HI were associated with lower OS (0.84±0.04; P=0.021), lower FC (P=0.003), and higher BMI at diagnosis and last follow-up (P=0.000) when compared with sellar masses without HI (OS: 0.94±0.05). PFS was not affected by HI or degree of resection.ConclusionsInitial HY has no impact on outcome in patients with sellar masses. OS and FC are impaired in survivors presenting with initial HI. PFS is not affected by HY, HI, or degree of resection. Accordingly, gross-total resection is not recommended in sellar masses with initial HI to prevent further hypothalamic damage.


Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 1287-1287
Author(s):  
Mario Arpinati ◽  
Marilina Amabile ◽  
maria Teresa Bochicchio ◽  
Angela Poerio ◽  
Giuseppe Bandini ◽  
...  

Abstract Abstract 1287 Background: Monitoring of minimal residual disease through RT-PCR analysis of bcr-abl transcripts allows early detection of CML relapse after allogeneic HSCT. However, the introduction of more sensitive techniques, such as quantitative PCR, may result in decreased specificity, leading to false positive results. Patients and methods: In this study we reviewed the results of molecular analysis of bcr-abl transcripts in all patients with p210+ CML who underwent allogeneic HSCT from 1983 through 2007. Q-PCR analysis was started in 2002. Out of 189 patients, 87 patients had available Q-PCR data; of these, 63 patients with at least three separate Q-PCR data were included in the study. Median time to the 1st Q-PCR analysis was 2196 days (35-7823). Median age was 36 years (13-56), 62%/38% received transplant from a related/unrelated donor, 62% with BM. 32% were in accelerated phase (AP). Patients with at least one positive Q-PCR value (measured as a ratio of bcr-abl to abl of > 0.001) were classified as Major Molecular Remission (MMR) patients. Each event was defined as one or more consecutive positive results. Results: 60/63 patients are alive after a median follow up of 3693 days (898-9405). 6 have relapsed 2142 (1419-3746) days after transplant. 52 (83%) patients had at least one positive result (28 with a value of >0.01, 24 with a value of <0.01), whereas 11 (17%) had persistent undetectable transcripts. In MMR patients, 94 events occurred. 29 patients had only one event, while 6 had >3 events. In 10 patients, the event occurred within 1 year after transplant, whereas in 28 it occurred after >5 years. 6/52 MMR patients relapsed, as compared to 0/11 with persistent undetectable transcripts (p=0.19). Relapse did not correlate with the Q-PCR value, the number of events or the time to the event. Finally, of 46 MMR patients who did not relapse, 35 had undetectable transcripts at last follow up. Positive Q-PCR had low specificity (19%) and positive predictive value (12%) in predicting relapse after allogeneic HSC transplantation. Conclusion: Our data confirm that the detection of low levels of bcr-abl transcripts (as based on Q-PCR) has a poor accuracy in predicting relapse, and it should not be considered as the sole indication to start treatment. It appears that fluctuation of bcr-abl transcript levels is common as late as > 10 yrs after transplant, possibly suggesting the long term persistence of CML stem cells. Disclosures: Rosti: Novartis: Consultancy, Honoraria; BMS: Consultancy, Honoraria. Martinelli:Novartis: Consultancy, Honoraria; BMS: Consultancy, Honoraria; Pfizer: Consultancy.


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