Abstract 15165: Routine Ambulatory Heart Rhythm Monitoring in Patients With Transthyretin Cardiac Amyloidosis

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Zack Dale ◽  
Lana Rashdan ◽  
Pranav Chandrashekar ◽  
Miriam Elman ◽  
Stephen B Heitner ◽  
...  
Keyword(s):  
Cardiac Amyloidosis ◽  
Ambulatory Monitoring ◽  
Heart Rhythm ◽  
High Rate ◽  
Routine Practice ◽  
Cardiovascular Implantable Electronic Devices ◽  
Asymptomatic Patients ◽  
Atrial Fibrillation And Flutter

Introduction: Atrial fibrillation and flutter (AF/AFL) are common with transthyretin cardiac amyloidosis (ATTR-CM). These patients have higher risk for thromboembolism. Detection of AF/AFL may thus be especially important, but the role of routine ambulatory monitoring in asymptomatic patients is unclear. Methods: A single center observational study of patients seen at our Amyloidosis Center with wild-type or hereditary ATTR-CM diagnosed between 2005-2019. Records were reviewed to see if AF/AFL was present at baseline. In those without known AF/AFL, ambulatory monitor (duration 2-30 days) was done every 6 months when feasible as part of our routine practice. Patients with cardiovascular implantable electronic devices (CIED) instead had routine device interrogations. Results: Ninety-four patients with ATTR-CM (mean age 71.4±11.4 years, 88.3% male) had mean follow-up 2.2±1.9 years (200 patient-years). AF/AFL was seen in 62 patients (66.0%): 35 (56.5%) by symptoms, 18 (29.0%) incidentally by monitor or CIED, and 9 in whom method of AF/AFL diagnosis was unknown. Forty-one patients (66.1%) had AF/AFL before ATTR-CM diagnosis by of median 26.3 (IQR 4.3-40.9) months. New AF/AFL was diagnosed in 21/53 remaining patients with known ATTR-CM - 11 (52.4%) were incidentally diagnosed (Figure 1). Median time between ATTR-CM and AF/AFL diagnoses was 10.3 (IQR 7.0-25.8) months and 5.9 (IQR 2.2-22.3) months in symptomatic versus incidentally diagnosed patients (Mann-Whitney U=35, p=0.14). Anticoagulation (AC) was started in 9/11 (81.8%) due to incidental AF/AFL. One patient had AC for prior DVT; the other had no AC per patient preference. No strokes occurred in patients on AC. Conclusions: We found a high rate of incidentally diagnosed AF/AFL in patients with ATTR-CM. Detection of AF/AFL led to change in therapy. This finding affirms a need for routine ambulatory monitoring in asymptomatic patients with ATTR-CM. Optimal duration of monitoring needs further investigation.

scholarly journals The role of radiosurgery for hemangiopericytomas

2003 ◽  
Vol 14 (5) ◽  
pp. 1-5 ◽  
Author(s):  
Steven D. Chang ◽  
Gordon T. Sakamoto
Keyword(s):  
Stereotactic Radiosurgery ◽  
Distant Metastases ◽  
Residual Tumor ◽  
High Rate ◽  
Tumor Control ◽  
Small Subset ◽  
Patient Database ◽  
The Mean

Object Hemangiopericytomas represent a small subset of meningeal tumors. Despite their relatively uncommon nature, they are aggressive tumors known for recurrence. Resection is the standard treatment in most, although regrowth and metastases are common even after resection. The authors evaluate the role of stereotactic radiosurgery in the treatment of recurrent hemangiopericytomas. Methods In a review of the Stanford radiosurgery patient database between 1989 and 2002, the authors found eight patients with recurrent hemangiopericytoma who underwent stereotactic radiosurgery. The mean age of this population was 45.1 years (range 24–67 years). All patients had been previously treated with resection, and five patients (63%) had undergone conventional radiotherapy. The mean radiosurgery dose to the tumor margin was 20.5 Gy (range 16–24 Gy). The mean clinical and radiographic follow-up period was 44 months (range 8–77 months). Of the eight tumors treated with radiosurgery, six decreased in size and two ultimately progressed. There were no radiosurgery-related complications. Conclusions Stereotactic radiosurgery of hemangiopericytomas can result in increased tumor control and should be considered as a treatment option for patients in whom the diagnosis has been established and in whom residual tumor is demonstrated postoperatively. Close clinical and radiographic follow-up evaluation is necessary in this patient population because of the high rate of local recurrence and distant metastases.

scholarly journals Le suivi multidisciplinaire de patients adultes atteints de dystrophie myotonique de type 1 dans le Sud Aquitain

2021 ◽  
Vol 37 ◽  
pp. 32-35
Author(s):  
Olivier Flabeau ◽  
Thomas Bisson
Keyword(s):  
Multidisciplinary Care ◽  
Cognitive Status ◽  
High Rate ◽  
Cross Sectional Survey ◽  
Cross Sectional ◽  
Ctg Repeats ◽  
French Cohort

DM1 is characterized by a multisystemic involvement. Our objective was to determine the proportion of adequate follow-up for each affected organ in DM1 patients based on the recently published American and Spanish recommendations. To this end, we conducted a descriptive cross-sectional survey by phone in adult, genetically proven DM1 patients followed in the two French neuromuscular centers of Bayonne and Hendaye located in South Aquitaine, France. The questionnaire selected the most stringent criteria of the two international recommendations for each item of follow-up. Seventy-three patients were included, 55% of which were women (mean age of 48 years) with an average number of 467 CTG repeats. The proportion of patients receiving clinical follow-up in accordance with the recommendations was 90% in cardiology, 60% in neurology, 68% in ophthalmology, 53% in physiotherapy, 23% in pneumology, and 12% in rehabilitation. The high rate of neurological, cardiological, and ophthalmological monitoring might be explained by a locally dense medical demography whereas low rate of respiratory follow up and rehabilitation may reflect an incomplete knowledge of both the disease and the questionnaire. These results should be carefully interpretated as cognitive status may influence such a declarative study. Our study nevertheless disclosed important disparities according to the recommended multidisciplinary follow-up criteria in this French cohort of adult DM1 patients. These results highlight the major role of a multidisciplinary care and monitoring in DM1.

Fungal dysbiosis in cirrhosis

Gut ◽  
2017 ◽  
Vol 67 (6) ◽  
pp. 1146-1154 ◽  
Author(s):  
Jasmohan S Bajaj ◽  
Eric J Liu ◽  
Raffi Kheradman ◽  
Andrew Fagan ◽  
Douglas M Heuman ◽  
...  
Keyword(s):  
Standard Of Care ◽  
High Rate ◽  
Control Groups ◽  
Cross Sectional ◽  
And Control ◽  
Uninfected Culture ◽  
Culture Positive ◽  
Culture Negative

ObjectiveCirrhotics have a high rate of infections, which are increasingly fungal or culture-negative in nature. While infected cirrhotics have bacterial dysbiosis, the role of fungi is unclear. We aimed to evaluate gut bacterial and fungal dysbiosis in cross-sectional and longitudinal analyses of outpatient and inpatient cirrhotics and prediction of hospitalisations.MethodsCross-sectional: Age-matched controls, outpatients (with/without antibiotics) and hospitalised uninfected, culture-negative and culture-positive cirrhotics were included and followed for 90 days. Longitudinal: Three studies were conducted: (1) cirrhotics followed over 6 months, (2) outpatient cirrhotics administered antibiotics per standard of care for 5 days and (3) cirrhotics and controls administered omeprazole over 14 days. In all studies, stool bacterial/fungal profiles were analysed.ResultsCross-sectional: In 143 cirrhotics and 26 controls, bacterial and fungal diversities were significantly linked. Outpatients on antibiotics and patients with culture-positive infections had the lowest diversities. Bacterial and fungal correlations were complex in uninfected, outpatient and control groups but were markedly skewed in infected patients. 21% were admitted on 90-day follow-up. A lower Bacteroidetes/Ascomycota ratio was associated with lower hospitalisations. Longitudinal: Fungal and bacterial profiles were stable on follow-up (5 days and 6 months). After antibiotics, a significantly reduced bacterial and fungal diversity, higher Candida and lower autochthonous bacterial relative abundance were seen. After omeprazole, changes in bacterial diversity and composition were seen but fungal metrics remained stable.ConclusionThere is a significant fungal dysbiosis in cirrhosis, which changes differentially with antibiotics and proton pump inhibitor use, but is otherwise stable over time. A combined bacterial–fungal dysbiosis metric, Bacteroidetes/Ascomycota ratio, can independently predict 90-day hospitalisations in patients with cirrhosis.Clinical trial numberNCT01458990.

Return to Work Following Surgery for Incidental Diffuse Low-Grade Glioma: A Prospective Series With 74 Patients

Neurosurgery ◽  
2019 ◽  
Vol 87 (4) ◽  
pp. 720-729 ◽  
Author(s):  
Sam Ng ◽  
Guillaume Herbet ◽  
Sylvie Moritz-Gasser ◽  
Hugues Duffau
Keyword(s):  
Return To Work ◽  
Extent Of Resection ◽  
Low Grade Glioma ◽  
High Rate ◽  
Awake Surgery ◽  
Prophylactic Surgery ◽  
Low Grade ◽  
Oncological Treatment ◽  
Asymptomatic Patients

Abstract BACKGROUND Therapeutic strategy concerning incidental low-grade glioma (ILGG) is still debated. Early “prophylactic” surgery has been proposed in asymptomatic patients with favorable neurological and oncological outcomes. OBJECTIVE To assess postoperative ability to resume employment following awake surgery in asymptomatic ILGG patients. To assess extent of resection (EOR), timeline for adjuvant oncological treatment, and survival. METHODS A total of 74 patients with ILGG who underwent awake surgery with intraoperative mapping were prospectively included, with a minimum follow-up of 12 mo. All clinicoradiological data were collected, and statistical correlations with return to work (RTW) were performed. RESULTS A total of 66 patients (97.1%) among 68 patients with preoperative professional activities resumed their employment including 62 (91.2%) within 12 mo. Mean time before RTW was 6.8 mo (median: 6 mo, range: 1-36). Two patients experienced seizure-related legal issues impacting their RTW. Clinicoradiological features did not correlate with RTW apart from postoperative seizures (P = .02). Mean EOR was 95.7%. A total of 43 patients (58.1%) underwent supratotal/total resections. All patients recovered from transient deficits at 3 mo. No patients received consecutive adjuvant treatment. A total of 24 patients (32.4%) were reoperated, 24 patients received chemotherapy, and 7 patients (9.5%) received radiotherapy, on average 73.1 mo after surgery. Mean follow-up was 67 mo (range 12-240). Four patients (5.4%) died during the follow-up. CONCLUSION We observed a high rate of RTW (97.1%, including 91.2% within 12 mo) after awake surgery in ILGG patients. Delayed resumption of work was due to employer not clearing them for RTW, personal choice, and, in rare occasions, related to seizures.

The role of endoscopic third ventriculostomy in the treatment of hydrocephalus

2013 ◽  
Vol 12 (1) ◽  
pp. 54-61 ◽  
Author(s):  
Timothy W. Vogel ◽  
Biji Bahuleyan ◽  
Shenandoah Robinson ◽  
Alan R. Cohen
Keyword(s):  
Endoscopic Third Ventriculostomy ◽  
Third Ventricle ◽  
Public Health Problem ◽  
High Rate ◽  
Third Ventriculostomy ◽  
Major Public Health Problem ◽  
Shunt System

Object Hydrocephalus remains a major public health problem. Conventional treatment has relied on extracranial shunting of CSF to another systemic site, but this approach is associated with a high rate of complications. Endoscopic third ventriculostomy (ETV) is a novel treatment for select forms of hydrocephalus that can eliminate the need for implantation of a lifelong ventricular shunt system. However, the indications for ETV are contested and its long-term effectiveness is not well established. Methods The authors selected 100 consecutive patients who underwent ETV for hydrocephalus beginning in 1994. Patients were enrolled and treated at a single institution by a single surgeon. The primary outcome was success of ETV, with success defined as no need for subsequent surgery for hydrocephalus. Results Ninety-five patients satisfied the inclusion criteria. The mean follow-up period was 5.1 years (median 4.7 years) with follow-up data available for as long as 17 years. Patients commonly presented with headache (85%), ataxia (34%), emesis (29%), and changes in vision (27%). The success rate for ETV was 75%. Twenty-one patients (22%) in the series had malfunctioning shunts preoperatively and 13 (62%) were successfully treated with ETV. Preoperative inferior bowing of the third ventricle floor on MRI was significantly associated with ETV success (p < 0.05). Conclusions Endoscopic third ventriculostomy is an effective and durable treatment for select patients with hydrocephalus. When successful, the procedure eliminates the lifelong complications associated with implanted ventricular shunts.

P4772Role of anticoagulation in patients with subclinical AF and its association with cardiovascular events: a SILENT sub study

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M Martinelli Filho ◽  
S F Siqueira ◽  
G A T Athayde ◽  
K M Dias ◽  
A O Pinheiro ◽  
...  
Keyword(s):  
Previous History ◽  
Intervention Group ◽  
High Rate ◽  
Control Group ◽  
Thromboembolic Events ◽  
Short Term ◽  
Cardiac Devices ◽  
Increased Risk

Abstract Background Atrial fibrillation (AF) is a well-established thromboembolic event risk factor. Episodes of subclinical AF (SCAF) recorded in implantable electronic cardiac devices (IECD) have been related to clinical AF and increased risk of stroke. However, there is no scientific evidence regarding the role of anticoagulation in this population. Objective: Our objective is to assess the association of SCAF with clinical AF and rate of systemic thromboembolic events, in a short-term follow-up. Methods This is a sub-study of SILENT, a prospective, randomized, unicentric study which included patients with sinus rhythm, IECD, with CHA2DS2-VASc ≥2, without previous history of AF. Patients were randomized to the Intervention Group and to the Control Group in the 1: 1 ratio. Patients of the Intervention Group with SCAF episodes (>6 min) received anticoagulation, as well as those with clinical AF in both groups. The primary end point was systemic thromboembolic phenomena and the secondary endpoints were SCAF rate, total and cardiovascular mortality, cardiovascular hospitalization and bleeding. Results A total of 758 patients were evaluated, with a mean age of 72.81 years (± 9.73), of which 461 (60.8%) were female. The mean follow-up was 19.59±4.24 months. Baseline characteristics were similar in both groups. Only 3 patients presented the primary outcome (two of them from Intervention Group). There were 16 deaths (2,1%) and 44 cardiovascular hospitalizations (5,8%), with no difference between groups. Atrial high rate episodes (AHRE) and clinical AF were more prevalent in Control Group, leading to an equal rate of anticoagulation between groups. Clinical AF was statistically associated to previous atrial high rate episodes of any duration (p=0.001) and correlated with SCAF (p<0.01 and R: 0,60) previously recorded in the device. Conclusion This sub study showed that, in a short term follow-up, SCAF has a good correlation with clinical AF occurrence with low rate of thromboembolic events. The Silent study will evaluate in an extended population the role of anticoagulation, in the long term. Acknowledgement/Funding None

Prognostic role of global work index in asymptomatic patients with aortic stenosis

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
F Ilardi ◽  
A Postolache ◽  
R Dulgheru ◽  
S Marchetta ◽  
M Cicenia ◽  
...  
Keyword(s):  
Aortic Stenosis ◽  
Control Group ◽  
Funding Source ◽  
Cardiac Damage ◽  
Asymptomatic Patients ◽  
Work Index ◽  
Advanced Stages ◽  
Global Work

Abstract Background In asymptomatic patients with aortic stenosis (AS), the optimal timing for intervention is still challenging. Previous studies demonstrated that advanced stages of cardiac damage are associated with excess mortality. The role of myocardial work to identify cardiac dysfunction in AS and to predict prognosis has not been investigated. Purpose We aimed to evaluate the modification of myocardial work indices related to AS stages and their prognostic value. Methods This study analysed clinical, echocardiographic and outcome data of 170 patients with asymptomatic AS (aortic valve area ≤1.5 cm2) and preserved ejection fraction (LVEF ≥50%). Exclusion criteria were: significant associated cardiac valve lesion, left bundle branch block and suboptimal quality of speckle-tracking image analysis. The control group included 50 patients matched for age and sex. Global work index (GWI), global constructive work (GCW), global wasted work (GWW) and global work efficiency (GWE) were estimated by LV pressure-strain loops. In AS group, LV pressure was evaluated by adding trans-aortic mean gradient to systolic blood pressure. The following staging classification was used: no cardiac damage associated with the valve stenosis (Stage 0), left ventricular damage (Stage 1), left atrial or mitral valve damage (Stage 2), pulmonary hypertension or tricuspid valve damage (Stage 3), or right ventricular damage or subclinical heart failure (Stage 4). Results While global longitudinal strain was significantly lower in AS than in control group (18.7±2.8 vs 20.7±2.1%, p&lt;0.001), increased values of GCW and GWI (respectively 2948±598 vs 2360±353 mmHg%, and 2528±521 vs 2005±302 mmHg%, p&lt;0.001) were observed in patients with AS. Besides, GWW was significantly increased in AS vs controls (139±90 vs 90±49 mmHg%, p=0.001), with no changes in terms of GWE (95±4 vs 96±2%, p=0.110). When patients were stratified according the stages of cardiac damage, MW indices didn't different significantly, except for the GWI, which was significantly lower in Stage 3 to 4 compared to Stage 0 and Stage 2 (2268±469 vs 2623±503 vs 2610±503 mmHg% respectively, p=0.025). During a mean follow up of 27 months (IQ range 12–48 mo), 18 patients had a CV death. The best GWI value associated with outcome was 1866 mmHg% (sensitivity 45%, specificity 96%, AUC= 0.701, p=0.01). The presence of a GWI at baseline lower than 1866 mmHg% was associated with a higher rate of CV events at 4-year follow-up (57% vs 7%, log-rank p&lt;0.001). On multivariable Cox-regression analysis, BNP values (P=0.014) and GWI &lt;1866 mmHg% (P=0.033) emerged as independently associated with CV death. Conclusion In asymptomatic patients with AS, advanced stages of cardiac damage are characterized by reduced values of GWI, that are associated with increased mortality. Thus, the evaluation of MW indices may allow a better phenotyping of asymptomatic patients at higher risk of developing cardiovascular events during follow-up. Funding Acknowledgement Type of funding source: Private grant(s) and/or Sponsorship. Main funding source(s): Cardiopath PhD program

Abstract 18297: Clinical and Prognostic Utility of Cardiac Magnetic Resonance Imaging in Multiple Myeloma Patients With Suspected Amyloidosis

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Sabha Bhatti ◽  
Evan Watts ◽  
Fahd Syed ◽  
Abdul Hakeem
Keyword(s):  
Magnetic Resonance Imaging ◽  
Multiple Myeloma ◽  
Cardiac Mri ◽  
Cardiac Amyloidosis ◽  
Cardiac Involvement ◽  
Al Amyloidosis ◽  
Resonance Imaging ◽  
Prognostic Utility

Background: Upto 30% of patients with multiple myeloma have AL amyloidosis and cardiac involvement is associated with worse outcomes in these patients. Traditional screening modalities including EKG, echocardiography and biomarkers have limited value. The aim of this study was to evaluate the role of “screening” cardiac MRI in a large multiple myeloma population at a large specialized referral center. Methods & Results: 164 consecutive patients with multiple myeloma who underwent cardiac MRI between 6/2005 and 10/2011 were enrolled in this study. Primary endpoint was all cause death. Clinical, EKG, echocardiographic, biomarker and MRI predictors for death were analyzed. Mean age of population was 63+10 years, 40% females and 16% African Americans. 30% of the population had MRI evidence of cardiac involvement. There were 26 patients who had biopsy proven systemic amyloidosis, of whom 62% showed cardiac involvement on MRI. 81% patients with confirmed cardiac amyloidosis on endomyocardial biopsy had typical MR pattern of cardiac involvement. During a median follow up period of 702 days (mean 1019 (950) days), there were 59 deaths (36%). Amyloid pattern on cardiac MRI (OR 2.19), elevated BNP and increased LV wall thickness on MRI were significant predictors of mortality . (All p<0.05) Conclusions: This is the largest study to date evaluating the role of cardiac MRI in multiple myeloma patients with suspected cardiac amyloidosis. Cardiac MRI is a clinically robust tool for risk stratification of this subset of patients.

scholarly journals Incidental demyelinating inflammatory lesions in asymptomatic patients: a Brazilian cohort with radiologically isolated syndrome and a critical review of current literature

2012 ◽  
Vol 70 (1) ◽  
pp. 5-11 ◽  
Author(s):  
Antonio Carlos Martins Maia Jr. ◽  
Antonio José da Rocha ◽  
Bernardo Rodi Barros ◽  
Charles Peter Tilbery
Keyword(s):  
Cognitive Abilities ◽  
Resonance Imaging ◽  
Gadolinium Enhancement ◽  
Inflammatory Lesions ◽  
Radiologically Isolated Syndrome ◽  
Asymptomatic Patients ◽  
Definition Of ◽  
Brazilian Cohort

Despite the definition of specific diagnostic criteria to identify radiologically isolated syndrome (RIS) suggestive of multiple sclerosis, its natural history remains incompletely understood. We retrospectively analyzed a Brazilian cohort of 12 patients to clarify their features and to emphasize the role of imaging predictors in clinical conversion. We demonstrated that, although some individuals did not exhibit progression over a lengthy follow-up period (16.7%), most patients will progress clinically or radiologically in the initial years of the follow-up (83.3%). Infratentorial and spinal cord involvement, as well as the total number of lesions, were more relevant predictors of progression than gadolinium enhancement. Further studies remain necessary to define the risk of conversion in males and to clarify the cognitive abilities of RIS patients. This study may provide an improved understanding of the natural course and evolution of incidental magnetic resonance imaging lesions, and further assists with the management of RIS in clinical practice.

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