scholarly journals Prevalence and Prognostic Significance of Left Ventricular Dysfunction in Patients Presenting Acutely with Atrial Fibrillation

2010 ◽  
Vol 4 ◽  
pp. CMC.S4106 ◽  
Author(s):  
Chin Lin ◽  
Colin Edwards ◽  
Guy P. Armstrong ◽  
Anthony Scott ◽  
Hitesh Patel ◽  
...  

The prevalence and prognostic importance of CM occurring as a consequence of AF is poorly defined. This study investigated the incidence of CM in patients with AF, its clinical features and long-term outcomes. We demonstrated that CM is common in patients presenting acutely with newly diagnosed rapid AF, and carries a worse long-term prognosis. Systolic dysfunction was reversible in an important proportion of patients, suggesting a greater prevalence of rate-related CM in AF than has previously been postulated. This underscores the importance of appropriate rhythm management strategies and repeat imaging studies. Background Atrial fibrillation (AF) may precipitate LV dysfunction, potentially leading to cardiomyopathy (CM). The prevalence and prognostic importance of CM occurring as a consequence of AF is poorly defined. We investigated the incidence of CM in patients with AF, its clinical features and long-term outcomes. Methods We reviewed 292 consecutive patients (average age 72 ± 13yrs) presenting acutely with AF and tachycardia over a 3 year period from June 2004. Clinical details were obtained from medical records. CM was defined as ejection fraction (EF) ≤ 50% on index admission. Results Echo was performed 93% of patients at index admission, and 69 (24%) had CM (average EF% = 37 ± 11), 60 of which were newly diagnosed. Patients with CM had significantly higher presenting heart rate (141 ± 19 vs. 132 ± 23 bpm), larger end-diastolic (5.7 vs. 5.2 cm) and end-systolic (4.5 vs. 3.2 cm) dimensions, and larger left atrial size (4.6 vs. 4.3 cm) ( P < 0.05 for all). They were also statistically more likely ( P < 0.05) to be male, present with breathlessness, have a history of coronary disease, and be treated with digoxin and warfarin. Follow-up echo between 6 and 12 months was performed in 46% of patients with new CM, and average EF rose to 53 ± 12%. At an average follow-up of 2.5 years, there was a significant increase in mortality in CM patients (16% vs. 9.5%, P < 0.05). Conclusion CM is common in patients presenting acutely with newly diagnosed rapid AF, and carries a worse long-term prognosis. Systolic dysfunction was reversible in an important proportion of patients, suggesting a greater prevalence of rate-related CM in AF than has previously been postulated. This underscores the importance of appropriate rhythm management strategies and repeat imaging studies.

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Jeong-Seon Lee ◽  
Joong-Gon Kim ◽  
Soyoung Lee

AbstractChildhood-onset polyarteritis nodosa (PAN) is a rare and systemic necrotising vasculitis in children affecting small- to medium-sized arteries. To date, there have been only a few reports because of its rarity. Thus, we aimed to investigate the clinical manifestations, laboratory findings, treatment, and long-term outcomes in patients with childhood-onset PAN and to evaluate the usefulness of the paediatric vasculitis activity score (PVAS). We retrospectively analysed the data of nine patients with childhood-onset PAN from March 2003 to February 2020. The median ages at symptom onset, diagnosis, and follow-up duration were 7.6 (3–17.5), 7.7 (3.5–17.6), and 7.0 (1.6–16.3) years, respectively. All patients had constitutional symptoms and skin manifestations, while five exhibited Raynaud’s phenomenon. Organ involvement was observed in one patient. The median PVAS at diagnosis was 7 (range: 2–32). Prednisolone was initially used for induction in all patients, and other drugs were added in cases refractory to prednisolone. All patients survived, but three patients with high PVAS at diagnosis experienced irreversible sequelae, including intracranial haemorrhage and digital amputation. In conclusion, early diagnosis and treatment may minimise sequelae in patients with childhood-onset PAN. This study suggests that high PVAS score at diagnosis may be associated with poor prognosis.


2021 ◽  
Vol 42 (Supplement_1) ◽  
Author(s):  
S Seitler ◽  
N Al-Sakini ◽  
A Lacerna ◽  
C Flick ◽  
C West ◽  
...  

Abstract Background/Introduction Complete Atrio-Ventricular septal defect (AVSD) is a complex congenital cardiac disease, characterised by malformation at the atrio-ventricular junction and AV valve abnormalities. Long- term outcome data is limited. Purpose We sought to describe the long-term outcomes of adults with repaired complete AVSD in a tertiary adult congenital heart centre. Methods We retrospectively recruited patients with complete AVSD who underwent surgical repair between 1973 and 2001 in our centre. All clinical and echocardiographic data were collected and analysed for evidence of atrio-ventricular valve (AVV) deterioration, or cardiac dysfunction. We also assessed for the requirement for further surgical intervention throughout the follow up period. Results A total of 345 patients with AVSD were identified, partial AVSD 211, unrepaired 82. Fifty-two with repaired complete AVSD formed study group, female 36 (69%) and male 16 (31%), Trisomy 21 (23, 44%). Mean age at initial repair was 44 months (median 12, IQR 31.5). Mean follow up was 25.3 years (Median 24, IQR 9.75). Clinical status: At the latest follow-up, most patient were asymptomatic with NYHA I-II (n=46) Majority (n=36, 69%) required only initial repair and no further intervention. Further surgeries were performed in 16 patients including AVV repair (n=9) and LVOTO relief (n=3). Permanent pacemaker insertion needed in 6 (12%), all for heart block following valve repair. 7 patients (14%) had documented arrythmia, 4 (8%) requiring ablations and only 1 had endocarditis. Echo findings: AV Valve dysfunction was more commonly regurgitant rather than stenotic. More than moderate AVV regurgitation was present in 83% of patients, Left AVV (n=25, 48%), Right AVV (n=18, 35%) compared to 4% stenosis (LAVV n=1, RAVV n=1). Ventricular outflow tract obstruction was present in 8% of patients, RVOT obstruction (2, one native, one secondary to prior banding), LVOT obstruction (2, both native, one requiring surgical intervention). Right ventricular systolic dysfunction was present in 6% of patients (n=3), with mean TAPSE 14.1mm (SD± 3.1mm). Left ventricular dysfunction was present in 4% (n=2), mean LVEF 58.9% (SD±7.1%) and mean LV EDVi 55.06mL/m2 (SD±13.2 mL/m2). Only one patient had significant pulmonary hypertension (Mean PAP 48 mmHg). Conclusion Long-term outcomes of surgically repaired AVSD are highly favourable. Left AVV regurgitation is the most common residual lesion requiring further surgical intervention. Ventricular outflow tract obstruction was much less common, as was ventricular systolic dysfunction. Complete heart block was associated with surgical repair and arrhythmias were potential late complications. FUNDunding Acknowledgement Type of funding sources: None.


2019 ◽  
Vol 7 (1) ◽  
pp. e633 ◽  
Author(s):  
Xiaolu Xu ◽  
Qiang Lu ◽  
Yan Huang ◽  
Siyuan Fan ◽  
Lixin Zhou ◽  
...  

ObjectiveTo describe the detailed clinical characteristics, immunotherapy, and long-term outcomes of patients with anti-NMDA receptor (NMDAR) encephalitis in China.MethodsA single-center, prospective study. Patients who met the diagnostic criteria were enrolled from 2011 to 2017 and followed up. The clinical features, treatment, and long-term outcomes were collected prospectively. Factors affecting the long-term prognosis were analyzed.ResultsThe study included 220 patients. The most common clinical presentations were psychosis (82.7%) and seizures (80.9%). Of the patients, 19.5% had an underlying neoplasm; of which ovarian teratoma was 100% of tumors in females and only one male had lung cancer. Most patients (99.5%) received first-line therapy (glucocorticoids, IV immunoglobulin, or plasmapheresis alone or combined), and only 7.3% received second-line immunotherapy (rituximab, cyclophosphamide alone, or combined). Long-term immunotherapy (mycophenolate mofetil or azathioprine >1 year) was administered to 53.2% of patients. During the first 12 months, 207 (94.1%) patients experienced improvement, and 5 (2.3%) died, whereas 38 (17.3%) experienced relapses. At 12-month follow-up, 92.7% had favorable clinical outcomes (modified Rankin Scale score ≤2).ConclusionsPatients in China present with psychosis and seizure frequently but have a low percentage of underlying neoplasms. Re-enforced first-line immunotherapy is effective in managing anti-NMDAR encephalitis in the acute phase. Although relapse is relatively common, with combined first-line and long-term immunotherapy, most patients reached favorable outcomes.


2020 ◽  
Vol 38 (17) ◽  
pp. 1928-1937 ◽  
Author(s):  
Nisha S. Joseph ◽  
Jonathan L. Kaufman ◽  
Madhav V. Dhodapkar ◽  
Craig C. Hofmeister ◽  
Dhwani K. Almaula ◽  
...  

PURPOSE The combination of lenalidomide, bortezomib, and dexamethasone (RVD) is a highly effective and convenient induction regimen for both transplantation-eligible and -ineligible patients with myeloma. Here, we present the largest cohort of patients consecutively treated with RVD induction therapy followed by risk-adapted maintenance therapy with the longest follow-up and important information on long-term outcomes. PATIENTS AND METHODS We describe 1,000 consecutive patients with newly diagnosed myeloma treated with RVD induction therapy from January 2007 until August 2016. Demographic and clinical characteristics and outcomes data were obtained from our institutional review board–approved myeloma database. Responses and progression were evaluated per International Myeloma Working Group Uniform Response Criteria. RESULTS The overall response rate was 97.1% after induction therapy and 98.5% after transplantation, with 89.9% of patients achieving a very good partial response (VGPR) or better and 33.3% achieving stringent complete response after transplantation at a median follow-up time of 67 months. The estimated median progression-free survival time was 65 months (95% CI, 58.7 to 71.3 months) for the entire cohort, 40.3 months (95% CI, 33.5 to 47 months) for high-risk patients, and 76.5 months (95% CI, 66.9 to 86.2 months) for standard-risk patients. The median overall survival (OS) time for the entire cohort was 126.6 months (95% CI, 113.3 to 139.8 months). The median OS for high-risk patients was 78.2 months (95% CI, 62.2 to 94.2 months), whereas it has not been reached for standard-risk patients. Five-year OS rates for high-risk and standard-risk patients were 57% and 81%, respectively, and the 10-year OS rates were 29% and 58%, respectively. CONCLUSION RVD is an induction regimen that delivers high response rates (VGPR or better) in close to 90% of patients after transplantation, and risk-adapted maintenance can deliver unprecedented long-term outcomes. This study includes the largest cohort of patients treated with RVD reported to date with long follow-up and demonstrates the ability of 3-drug induction regimens in patients with newly diagnosed multiple myeloma to result in a substantial survival benefit.


2006 ◽  
Vol 105 (1) ◽  
pp. 136-141 ◽  
Author(s):  
Takayuki Hara ◽  
Nobutaka Kawahara ◽  
Koji Tsuboi ◽  
Junji Shibahara ◽  
Tetsuo Ushiku ◽  
...  

✓ Skull base chordomas containing a sarcomatous component are extremely rare. Here the authors report two new cases in which a recurrent tumor with a sarcomatous component appeared after the patient had undergone charged-particle radiotherapy. Histological examinations performed in Case 1 revealed some retention of epithelial features in the sarcomatous component, whereas no such regions were observed in Case 2. Both patients had rapidly deteriorating clinical courses and died within 6 months after diagnosis of the recurrent tumor. The authors discuss the significance of the histological subtypes of these tumors for long-term prognosis and their pathogenetic mechanisms in relation to radiotherapy. Although these sarcomatous transformations are rare in conventional chordomas, a careful histological examination and thorough follow-up imaging studies are crucial when treating patients with such lesions.


Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 52-53
Author(s):  
Elzbieta Szczepanek ◽  
Ositadima Chukwu ◽  
Magdalena Kaminska ◽  
Hubert Wysoglad ◽  
Agnieszka Cenda ◽  
...  

Introduction Imatinib, approved as first-line treatment for patients with newly diagnosed chronic myeloid leukemia (CML) by FDA approximately 20 years ago, revolutionized the treatment of this disease. The life expectancy of newly diagnosed patients with CML has been approaching that of the global population. Second generation TKI (2GTKI) administered as a frontline therapy can induce deeper and faster molecular responses in a higher percentage of patients, however, the overall survival is comparable to that achieved with imatinib. To investigate the outcomes of long-lasting therapy with imatinib administered as initial therapy, we analyzed patients with CML who received imatinib as initial therapy at our institution starting from 2001. Methods We retrospectively analyzed long term outcomes of 267 patients treated with imatinib 400 mg at the Department of Hematology, Jagiellonian University Medical College, Cracow, Poland from 2001. Data from medical records were collected and statistical analysis was performed using R software (R version 4.0.2). Results The median age was 53.5 (16 to 88 years), 129 of patients (pts) (48.31%) were female. At the time of this analysis, 99 pts (37.08 %) remained on imatinib with the median dose at last follow-up (FU) 400 mg. The mean initial dose of imatinib was 410.4 mg/d. Imatinib dose was increased in 53 pts (19.85%), up to 800mg and up to 600 mg in 3 pts (1.13%), and in 49 pts (18.35%) respectively. The mean maximal dose was 465.2 mg. At baseline 124 pts (46.44%) had comorbidities: 79 pts (29.59%) vascular/cardiac, 11 pts (4.12%) renal, and 101 pts (37.33%) other comorbidities. 15 patients (5.63%) had prior malignancies, newly diagnosed malignancies occurred among 11 (4.12%) pts on imatinib. The median follow-up time was 11.37 years (range from 2 months to 19.5 years). 168 pts (62.92%) discontinued imatinib permanently, the median time to imatinib discontinuation was 2.02 years. Among them, 123 pts (71.93 %) switched imatinib to 2GTKI- 79 pts (29.59%) to dasatinib, 68 pts (25.47%) to nilotinib, and 14 pts (5.24%) to bosutinib. During the following treatment 87 pts (32,58%) received one 2GTKI, 33 pts (12.36%) two, and 3 pts (1.12%) more than two 2GTKIs. The main reasons for imatinib therapy discontinuation were intolerance (87 pts, 32.58%) and disease progression (90 pts, 33.71%). The median time to the imatinib discontinuation due to its intolerance was 2 years. Adverse events (AEs) during imatinib therapy were as follows: cardiac/vascular AEs in 22 pts (8.24%), renal in 42 pts (15.73%), hematologic in 43 pts (16.10%), and other in 189 pts (70.79%). Overall, 28 patients died (10.5%), 7 pts (2.2%) transformed to blast phase, 9 pts (3.37%) underwent allo-HSCT. Estimated OS for patients that remained on imatinib for the whole observation period for 15 and 18 years was: 80.2%, and 64.1% respectively (Figure 1). Median follow-up time for patients who continued imatinib was 7.91 years. Intention to treat (ITT) analysis available for 99 pts (37.08%) revealed ITT responses at three months, one, five, ten and fifteen years: 50.52%, 77.4%, 86.25%, 90.28%, 100% for CCyR, 32.99%, 58.07%, 80%, 86.11%, 100% for MMR, 11.34%, 20.44%, 63.75%, 63.89%, 90% for MR4, 2.06%, 12.91%, 35%, 38.89%, 70% for MR4.5, 2.06%, 7.53%, 26.25%, 33.33%, 50% for CMR (undetectable transcripts with ≥100,000 copies ABL) (Table 1). The overall best response rates (at any time) for these 99 pts was 4.04% for MCyr, 5.05% for CCyR, 11.11% for MMR, 14.14% for MR4, 9.09% for MR4.5, 49.49% for CMR. Conclusion The analysis of long-term therapy with imatinib showed that the efficacy of imatinib persisted over time and that long-term administration of imatinib was associated with low rate of late toxic effects. Disclosures Sacha: Novartis: Consultancy, Honoraria, Speakers Bureau; Pfizer: Consultancy, Honoraria, Speakers Bureau; Adamed: Consultancy, Honoraria; Incyte: Consultancy, Honoraria, Speakers Bureau; Bristol-Myers Squibb Company: Consultancy, Honoraria, Speakers Bureau.


2016 ◽  
Vol 2016 ◽  
pp. 1-5 ◽  
Author(s):  
Peng Yun ◽  
Ai-ming Du ◽  
Xue-jun Chen ◽  
Jing-cheng Liu ◽  
Hu Xiao

Objective. To investigate the effect of acarbose therapy on the long-term prognosis of patients with acute coronary syndromes (ACS) complicating newly diagnosed impaired glucose tolerance (IGT).Methodology. 135 patients hospitalized for ACS who had been newly diagnosed with IGT were randomly assigned to acarbose group (150 mg/day,n=67) or control group (no acarbose,n=68). All cases in each group were given the same elementary treatment. Mean follow-up was 2.3 years. The incidence of major adverse cardiovascular event (MACE) and carotid intima-middle thickness (CIMT) were statistically analyzed.Results. During the mean follow-up of 2.3 years, the risk of recurrent MACE in acarbose group was decreased significantly compared with that in control group (26.67% versus 46.88%,P<0.05); at the same time, thickening of the CIMT was significantly slower than the control group ((1.28 ± 0.42) mm versus (1.51 ± 0.64) mm,P<0.05).Conclusions. Acarbose can effectively reduce the risk of MACE in ACS patients with newly diagnosed IGT, simultaneously retarding the progression of carotid intima-media thickness.


Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 335-335 ◽  
Author(s):  
Jorge Cortes ◽  
Michele Baccarani ◽  
François Guilhot ◽  
Brian J. Druker ◽  
Susan Branford ◽  
...  

Abstract Background: IM 400 mg/d is the standard of care for pts with newly diagnosed CML-CP. Previous reports suggest the rate of major molecular response (MMR), defined as BCR-ABL/control gene (BAC) ratio of ≤ 0.1% on the International Scale, predicts for a benefit in long-term outcomes. Phase 2 trials demonstrated that IM 800 mg/d as initial treatment of CML-CP decreases the time to MMR and increases the depth of molecular response (MR), and may therefore improve long-term outcomes. Methods: TOPS is a prospective, open-label, randomized (2:1) Phase 3 trial that compared IM 800 mg/d to 400 mg/d in CML-CP. Pts were stratified by Sokal risk score. The primary endpoint is MMR rate at 12 months (mo) and secondary endpoints include: rates of complete hematological response, complete cytogenetic response (CCyR), time to CCyR and MMR, progression to accelerated phase (AP) or blast crisis (BC), eventfree survival (EFS), overall survival (OS), IM dose-intensity, pharmacokinetics, and safety. Rates were compared by Fisher’s exact test and time to event outcomes by logrank test. Results: 476 pts were enrolled (800 mg/d, n=319; 400 mg/d, n=157) at 103 sites in 19 countries between 6/05 and 12/06. Median age at diagnosis was 47 yrs, and 24% of pts had high Sokal risk score. Significantly more pts receiving IM 800 mg/d achieved MMR at 3 mo and 6 mo, but not at 12 mo when compared with 400 mg/d (Table 1). Time to MMR was faster in the 800 mg/d arm compared to 400 mg/d; P = .0038. Table 1: MMR rate (%) over time according to randomized dose of IM MMR rate (%) Intent-to-treat (ITT) population Evaluable pts (with polymerase chain reaction assessment) 400 mg (N = 157) 800 mg (N = 319) P-value 400 mg %, (n) 800 mg %, (n) P-value Month 3 3 12 .0011 4 (137) 14 (283) .0011 Month 6 17 34 .0002 20 (135) 39 (276) .0001 Month 9 36 45 .0604 41 (136) 54 (267) .0203 Month 12 40 46 .2035 46 (133) 54 (269) .1386 Achievement of MMR according to average dose over the first 12 mo of treatment was greatest when the intended dose intensity (DI) was achieved (Table 2). Table 2. MMR at 12 mo according to DI (evaluable patients) Randomized Dose MMR DI (first 12 mo) (n) MMR rate, % [95% CI] 400 mg (n =133) 46% ≥ 400 mg (74) 50 [38−62] &lt; 400 mg (59) 41 [28−54] 800 mg (n =269) 54% 800 mg (52) 63 [49−76] 600 – 799 mg (134) 62 [53−70] 400 – 599 mg (69) 38 [26−50] &lt; 400 mg (14) 21 [5−51] CCyR occurred faster in the 800 mg/d arm, indicated by a higher response rate at 6 mo (57% vs. 45%, P = .0146). At 12 mo rates of MMR and CCyR (ITT population) were higher for the 800 mg/d arm but were no longer significantly different (MMR 46% vs. 40%, P= .2035; CCyR 70% vs. 66%, P= .3470). In pts with high Sokal risk scores, rates of MMR at 12 mo were 41% and 26% (P = .1565) for the 800 mg/d and 400 mg/d arms, respectively. Exploratory analysis of MR at 3 mo and its correlation with achievement of MMR at 12 mo follow. Of the pts in the 400 mg arm with BAC ratios &gt;0.1–≤ 1%, &gt;1–≤ 10% or &gt; 10% at 3 mo, 83%, 46%, and 11% later achieved an MMR at 12 mo. In the 800 mg arm 73%, 45% and 21% of the pts with respective BAC ratios achieved an MMR at 12 mo. Based on the BAC ratio at 6 mo, the observed MMR rate at 12 months was 52%, 11%, and 0% in the 400 mg/d arm compared to 46%, 14%, and 18% in the 800 mg/d arm. In the first year of follow-up, 6 pts had documented progression to AP/BC during treatment: 3 (1.9%) in the 400 mg/d arm and 3 (0.9%) in the 800 mg/d arm. At 12 mo, 85% of pts in the 400 mg/d arm were receiving the randomized dose compared to 62% of pts in the 800 mg/d arm. Median DI was 400 mg/d in the 400 mg arm and 750 mg/d in the 800 mg arm. Dose interruptions &gt; 5 days occurred more frequently in the 800 mg/d arm (67% vs 38%). Earlier achievement of MMR correlated with IM plasma trough level at 1 mo for the overall TOPS cohort; pts with IM concentrations &lt; 1165 ng/mL (lowest quartile of the aggregate group) achieved MMR slower than those with concentrations ≥ 1165 ng/mL (p=.0149). The most common grade 3/4 nonhematologic toxicities were rash, diarrhea and myalgia occurring slightly more frequently in the 800 mg/d arm. Grade 3/4 hematologic toxicity occurred more frequently in pts receiving 800 mg/d. Conclusions: TOPS confirms the efficacy and safety of IM in newly-diagnosed CML-CP. MMR occurred earlier in pts treated with 800 mg/d and in patients with plasma IM level above the lowest quartile, reinforcing the utility of IM blood level testing to optimize treatment. DI of IM 800 mg/d was maintained and tolerability was good. Additional follow-up is required to evaluate the effect of dose and MR on long-term clinical outcomes.


VASA ◽  
2013 ◽  
Vol 42 (4) ◽  
pp. 264-274
Author(s):  
Dagmar Krajíčková ◽  
Antonín Krajina ◽  
Miroslav Lojík ◽  
Martina Mulačová ◽  
Martin Vališ

Background: Intracranial atherosclerotic stenosis is a major cause of stroke and yet there are currently no proven effective treatments for it. The SAMMPRIS trial, comparing aggressive medical management alone with aggressive medical management combined with intracranial angioplasty and stenting, was prematurely halted when an unexpectedly high rate of periprocedural events was found in the endovascular arm. The goal of our study is to report the immediate and long-term outcomes of patients with ≥ 70 % symptomatic intracranial atherosclerotic stenosis treated with balloon angioplasty and stent placement in a single centre. Patients and methods: This is a retrospective review of 37 consecutive patients with 42 procedures of ballon angioplasty and stenting for intracranial atherosclerotic stenosis (≥ 70 % stenosis) treated between 1999 and 2012. Technical success (residual stenosis ≤ 50 %), periprocedural success (no vascular complications within 72 hours), and long-term outcomes are reported. Results: Technical and periprocedural success was achieved in 90.5 % of patients. The within 72 hours periprocedural stroke/death rate was 7.1 % (4.8 % intracranial haemorrhage), and the 30-day stroke/death rate was 9.5 %. Thirty patients (81 %) had clinical follow-up at ≥ 6 months. During follow-up, 5 patients developed 6 ischemic events; 5 of them (17 %) were ipsilateral. The restenosis rate was 27 %, and the retreatment rate was 12 %. Conclusions: Our outcomes of the balloon angioplasty/stent placement for intracranial atherosclerotic stenosis are better than those in the SAMMPRIS study and compare favourably with those in large registries and observational studies.


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