Abstract 17194: Incremental Value of JTc Interval in Improving Risk Stratification for Life-Threatening Ventricular Arrhythmias in Patients Considered Low Risk by LVEF

Circulation ◽  
2018 ◽  
Vol 138 (Suppl_1) ◽  
Author(s):  
Mohsin Khan ◽  
Susan Olet ◽  
Mohammad E Mortada ◽  
Firas Zahwe ◽  
Jodi Zilinski ◽  
...  
Keyword(s):  
Cardiac Arrest ◽  
Risk Stratification ◽  
Ventricular Arrhythmias ◽  
Low Risk ◽  
Operating Characteristics ◽  
Icd Implantation ◽  
P Values ◽  
Net Reclassification Improvement ◽  
Life Threatening

Introduction: ICD implantation is recommended in patients with LVEF<35%, while those with LVEF between 35 to 40% are not considered at high risk for primary prevention ICD implantation. A subset of these patients develops life threatening ventricular arrhythmias (VA) and improvement in risk stratification may help identify and implement life-saving intervention. Hypothesis: Prolonged repolarization is a marker of electrical instability and JTc interval on ECG could provide prognostic information in patients with LVEF 35-40% incremental to that from LVEF. Methods: Patients ≥18 yr with no history of VA and an ECG and echocardiogram obtained at initial encounter between 11/2011 to 12/2016 with long-term follow-up were identified. The incremental predictive ability of JTc interval on improvement in risk stratification for VA was determined by receiver operating characteristics (ROC) curve, integrated discrimination improvement (IDA) and net reclassification improvement (NRI) analysis. All tests were performed at a 5% level of significance. Results: Out of 29,700 pts that met inclusion criteria, 1,102 (3.7%) had LVEF 35-40% (mean age 70.5±14.6 yrs, 49% males, CAD 67%) and 24,894 (84%) LVEF >40% (65.9±16.3 yrs , 61.8% M). Over the mean follow-up of 4.6±4.2 years, the incidence of VT/VF/cardiac arrest was 16.1% in patients with LVEF 35-40% compared to 4.1% with LVEF >40%. For every 50 ms increase in JTc interval above 300 ms, the risk for arrhythmic event in LVEF 35-40% increased two-fold (Odds Ratio=1.83 (95 % CI 1.72-1.94, P=0.013). Incorporation of JTc to LVEF improved the C statistics (95% Confidence Limit) in the model with only LVEF from 0.56 (0.54-0.57) to 0.72 (0.70-0.73) for the model combining LVEF and JTc. In addition, NRI was estimated at 0.57, which was statistically significant with p values <0.001 while IDI was estimated as 0.015 with p values <0.001 for the model incorporating JTc to LVEF. Conclusions: In patients with LVEF 35-40% considered low risk for life threatening VA by EF, incorporating JTc interval information improved risk stratification and identified those who subsequently developed VT/VF or cardiac arrest and thus identifies a subgroup that can benefit from prophylactic ICD implantation.

scholarly journals Frequency of ventricular tachyarrhythmias in patients with different risk scores for sudden cardiac death in hypertrophic cardiomyopathy and cardioverter defibrilators implanted in primary prevention

EP Europace ◽  
2021 ◽  
Vol 23 (Supplement_3) ◽  
Author(s):  
N Brankovic ◽  
NN Radovanovic ◽  
N Vujadinovic ◽  
B Kircanski ◽  
V Sajic ◽  
...  
Keyword(s):  
Primary Prevention ◽  
Cardiac Death ◽  
Ventricular Arrhythmias ◽  
Risk Scores ◽  
Icd Implantation ◽  
Life Threatening ◽  
Group A ◽  
Group B

Abstract Funding Acknowledgements Type of funding sources: None. Background The European Society of Cardiology’s (ESC) risk calculator for sudden cardiac death (SCD) in patients with hypertrophic cardiomyopathies (HCM) is currently a tool that facilitates the decision to implant a cardioverter defibrillator (ICD) in primary prevention, but doubts still exist in everyday clinical practice. Purpose We aimed to determine the incidence of life-threatening ventricular arrhythmias during long-term follow-up in patients with different ESC HCM risk scores for SCD calculated on ICD implantation in primary prevention. Methods This retrospective, observational study was conducted in a tertiary center among adult patients with HCM and ICD devices (ICD-VR, ICD-DR and CRT-ICD) implanted in primary prevention from January 2008 to December 2019. The patients followed up for less than one year were excluded from the analysis. Study group was divided into three subgroups, according to the ESC 5 years risk score of SCD and based on it the estimated need for ICD implantation: group A with risk &lt; 4%, where ICD is generally not indicated; group B with risk 4-6%, where ICD may be considered; group C with risk ≥6%, where ICD should be considered. Regularly, ICD was interrogated twice a year, while emergency controls were performed after delivering of ICD therapy. Follow-up data including information about sustained ventricular tachicardia (VT) and ventricular fibrillation (VF) episodes were collected from patients medical records. Results In the observed period, ICD devices were implanted in 60 adult patients with HCM. Ten of them had history of sustained ventricular arrhythmias and 3 were lost to follow-up. Therefore, we analysed 47 patients (55.3% male, mean age 51.0 ± 15.7 years), 13 from group A, 12 from group B and 22 from group C. Mean follow-up period was 80.6 ± 37.9 months. During the follow-up period ventricular tachyarrhythmias (VT and VF, either in monitoring or therapy zone) were recorded in one patient from group A (7.7%), in two patients from group B (16.7%) and in 7 patients from group C (31.8%). Using Kruskal-Wallis analysis of variance, we did not find a statistically significant difference in the occurrence of ventricular tachyarrhythmias among the observed groups (p = 0.225). Conclusions Patients with HCM and a 5-year risk of SCD ≥ 6% calculated on ICD implantation had more frequent life-threatening ventricular arrhythmias during long-term follow-up, but not statistically significant. There should be an individual approach when deciding on the need for device implantation in all patients with HCM.

scholarly journals Sex-specific differences in risk stratification of ventricular arrhythmias among implantable cardioverter defibrillator patients: do women differ from men?

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
A.C.J Van Der Lingen ◽  
D.A.M.J Theuns ◽  
M.A.J Becker ◽  
A.C Van Rossum ◽  
V.P Van Halm ◽  
...  
Keyword(s):  
Risk Factors ◽  
Risk Stratification ◽  
Secondary Prevention ◽  
Ventricular Arrhythmias ◽  
Study Cohort ◽  
Icd Implantation ◽  
Icd Therapy ◽  
Prevention Therapy ◽  
Male Patients

Abstract Background Implantable cardioverter defibrillator (ICD) guidelines and risk stratification models of sudden cardiac death (SCD) are applied without differentiation between men and women, based on the assumption that the incidence of ventricular arrhythmias and risk factors of SCD are similar in both sexes. Sex-specific risk factors of SCD may influence studies evaluating the benefit of ICD therapy, for both men and women. Purpose Aim of the study is to assess sex-specific differences in occurrence and predictors of appropriate device therapy (ADT) for ventricular arrhythmias. Methods A multicenter retrospective cohort of 2300 consecutive patients was evaluated, including patients referred for ICD implantation between the years 2009–2018 (age 62±13 years, LVEF 32±12%, 53% ischemic cardiomyopathy [CMP], 28% resynchronization therapy, 65% primary prevention). Exclusion criteria were: (1) patients with hypertrophic CMP, arrhythmogenic right ventricular CMP, systemic infiltrative cardiac disease or channelopathy; (2) lost to follow-up immediately after ICD implantation. Primary endpoint was ADT, defined as anti-tachycardia pacing or shock for ventricular tachyarrhythmia. Secondary endpoints were mortality and inappropriate ICD therapy. Univariable and multivariable Cox regression analyses, stratified by sex, were performed to assess predictors of ADT. Results The cohort primarily consisted of men (75%). After a mean follow-up of 4.8±3.0 years, men experienced more ADT compared to women (25% versus 16%, HR 1.71, p&lt;0.001) and men displayed a higher mortality compared to women (25% versus 19%, HR 1.37, p&lt;0.01). No difference in inappropriate ICD therapy was observed (9% versus 10%, HR 1.01, p=0.94). In the total study cohort, male sex (HR 1.55, p&lt;0.001), higher age (HR 1.15 per 10 years, p&lt;0.0019), left bundle branch block (LBBB, HR 0.74, p=0.01) and secondary prevention indication (HR 1.89, p&lt;0.001) were independently associated with ADT. In male patients, independent predictors of ADT were comparable with the total study cohort: higher age (HR 1.20 per 10 years, p&lt;0.001), LBBB (HR 0.72, p=0.01) and secondary prevention therapy (HR 1.80, p&lt;0.001). In contrast, age (p=0.54) or LBBB (p=0.29) were not associated with ADT in women. In women, only paroxysmal atrial fibrillation (HR 1.76, p=0.03) and secondary prevention therapy (HR 1.78, p&lt;0.01) were independently associated with ADT. Conclusion This study showed that men were at higher risk of ADT compared to women and that risk factors associated with SCD differ between both sexes. The results strongly suggests that SCD risk stratification models are primarily driven by male patients and sex-specific risk models of SCD are needed to identify those women at high risk of SCD. Figure 1 Funding Acknowledgement Type of funding source: None

2397Lesion-specific risk for sudden cardiac death or life-threatening ventricular arrhythmias in adult congenital heart disease

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
P Gallego Garcia De Vinuesa ◽  
A E Gonzalez Garcia ◽  
P Avila ◽  
A Alonso ◽  
D Garcia Hamilton ◽  
...  
Keyword(s):  
Risk Stratification ◽  
Incidence Rate ◽  
Cardiac Death ◽  
Ventricular Arrhythmias ◽  
Adult Congenital Heart Disease ◽  
Low Risk ◽  
Discriminative Ability ◽  
Specific Risk ◽  
Adult Congenital ◽  
Life Threatening

Abstract Background Risk models for primary prevention strategies in adult congenital heart disease (ACHD) must incorporate the heterogeneous risk for sudden cardiac death (SCD) and life-threatening ventricular arrhythmias (LTVA) as stratified by underlying lesion. Objectives To determine lesion-specific risk for SCD and LTVA in ACHD. Methods We analyzed 3311 ACHD patients (50% males) prospectively followed-up for 37510 person/years. SCD cases were confirmed by means of the Spanish National Death Registry. In addition, we identified all cases of resuscitated cardiac arrest or ventricular tachycardia requiring cardioversion. According to the incidence rate of the composite end-point of SCD and LTVA, lesions were stratified into four groups of risk. Cumulative freedom from SCD or LTVA in patients at high, moderate, low and very low risk were compared by using Cox regression model with left truncation. The c-index of this lesion-specific risk stratification was calculated by using the β-coefficients. The discriminative ability of this lesion-specific risk stratification was also tested in an external cohort of 203 SCD-LTVA cases and 2287 controls from 20 different centers. Results 71 patients experienced an event (53 SCD, 18 LTVA). Patients at highest risk (incidence rate >1%) were those with Rastelli procedure, severe coronary abnormalities, complex Fallot and cyanotic patients, either Eisenmenger or non-Eisenmenger; at moderate risk (incidence rate 0.25–1.0%) non-complex Fallot, Mustard/Senning repair, Fontan procedures and congenitally corrected transposition; at low risk (incidence rate 0.1–0.25%) Ebstein anomaly and left heart lesions; and at very low risk (incidence rate <0.1%) left-to-right shunts and right ventricular outflow lesions. The discriminative ability in a multicenter external cohort was excellent (c-index ranged from 0.748 to 0.819 by center). Lesion-specific risk and C-index Conclusions A lesion-specific risk stratification based on the incidence rate of SCD and LTVA was performed and validated. This approach could result in a more individualized risk assessment. Acknowledgement/Funding Instituto de Salud Carlos III, Ministerio de Economía y Competividad, Spain (Exp PI14/02099 and PI17/01327) and co-financed by FEDER

scholarly journals Mortality and ventricular arrhythmia after acute myocarditis: a nationwide registry-based follow-up study

Open Heart ◽  
2021 ◽  
Vol 8 (2) ◽  
pp. e001806
Author(s):  
Kristian Hay Kragholm ◽  
Filip Lyng Lindgren ◽  
Tomas Zaremba ◽  
Phillip Freeman ◽  
Niels Holmark Andersen ◽  
...  
Keyword(s):  
Heart Failure ◽  
Cardiac Arrest ◽  
Ventricular Arrhythmias ◽  
Acute Myocarditis ◽  
Icd Implantation ◽  
Follow Up Study ◽  
Background Population ◽  
All Cause Mortality ◽  
Population Controls

ObjectiveIncidence and severity of acute myocarditis vary significantly in previous reports and there is a lack of epidemiological studies on the short-term risks of mortality, heart failure and ventricular arrhythmias in patients with acute myocarditis. Therefore, study aims were to examine 90-day risks of mortality, heart failure (HF) and ventricular arrhythmias in patients with acute myocarditis in comparison to age-matched and sex-matched background population controls.MethodsIn this nationwide register-based follow-up study of patients hospitalised with myocarditis between 2002 and 2018 in Denmark, 90-day risks of all-cause mortality, HF, ventricular arrhythmias (ventricular tachycardia, ventricular fibrillation (VF)), cardiac arrest and implantable cardioverter-defibrillator (ICD) implantation were compared with age-matched and sex-matched controls from the background population (1:5 matching). Absolute risks standardised to the age, sex and comorbidity distribution of the entire study population were derived from multivariable Cox regression.ResultsA total of 2523 patients hospitalised with myocarditis were included. Median age was 48 years (Q1–Q3: 30–69) and 67.7% were men. Comorbidity burden was more pronounced among patients with myocarditis relative to controls. Standardised 90-day all-cause mortality risk was 4.9% for patients with acute myocarditis versus 0.3% for controls (p<0.001). Ninety-day standardised risks for other endpoints were 7.5% versus 0.1% for HF, 1.9% versus <0.1% for VF/VF/arrest risk and 1.6% versus <0.1% for ICD implantation (all p<0.001).ConclusionsIn this large nationwide register-based follow-up study, patients hospitalised with myocarditis had significantly higher 90-day risks of all-cause mortality, HF, ventricular arrhythmias, cardiac arrest and ICD implantation compared with background population controls.

Abstract 2851: Temporal QT Variability from Bipolar Ventricular Tip-to-Ring Electrograms Predicts Sustained Ventricular Tachyarrhythmia.

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Larisa G Tereshchenko ◽  
Barry J Fetics ◽  
Peter P Domitrovich ◽  
Ronald D Berger
Keyword(s):  
Risk Stratification ◽  
Right Ventricular ◽  
Effective Means ◽  
Ventricular Tachyarrhythmia ◽  
Icd Implantation ◽  
Area Index ◽  
Icd Therapy ◽  
Increased Risk ◽  
Qt Variability

We assessed the hypothesis that ventricular tachycardia / ventricular fibrillation (VT/VF) risk stratification based on the repolarization assessment of intracardiac electrograms (EGMs) from implantable devices is feasible. Methods: Bipolar right ventricular tip-to-ring EGMs were recorded at rest (mean heart rate 66 ± 16 bpm) for 5.5 ± 2.6 minutes in 75 patients (58 ± 14 years, 72% men) with ischemic (60%) and non-ischemic (40%) cardiomyopathy who underwent single-chamber Medtronic ICD implantation for primary (77%) or secondary (23%) prevention of SCD. QT variability index (QTVI), variability of Tpeak-Tend area index, and T-wave alternans (TWA) were calculated as previously described elsewhere. Only 41 out of 75 recordings (55%) were eligible for analysis as determined by data quality requirements of the custom software (less than 10% non-analyzable beats or 5% ectopic beats). The endpoint was appropriate ICD therapy for VT/VF during follow-up > 6 months. Results: During mean follow-up of 12 months (range 6–19 months), 12 patients had appropriate ICD therapy. The survival analysis showed that the top quartile of QTVI (> - 0.5) predicts an event-free survival rate from appropriate ICD therapies (p = 0.027). Neither increased Tpeak-Tend area variability nor TWA was associated with a significant increased risk for VT/VF. Conclusions: In this prospective study, temporal QT variability measured from right ventricular tip-to-ring EGMs is associated with increased risk of sustained VT/VF events. Repolarization lability may be present throughout the ventricular myocardium, such that single-site EGMs may provide an effective means for VT/VF risk stratification.

1175Structural progression increases the risk of ventricular arrhythmias in patients with arrhythmogenic cardiomyopathy

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M Chivulescu ◽  
Ø.H Lie ◽  
H Skulstad ◽  
B A Popescu ◽  
R O Jurcut ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Ventricular Arrhythmias ◽  
Arrhythmogenic Cardiomyopathy ◽  
Cardioverter Defibrillator ◽  
Structural Progression ◽  
Life Threatening ◽  
Arrhythmic Event ◽  
History Of ◽  
Structural Disease

Abstract Background Arrhythmogenic cardiomyopathy (AC) is an inheritable cardiomyopathy with incomplete penetrance, variable phenotype severity and poorly described disease progression. It is characterized by high risk of life-threatening ventricular arrhythmias and sudden cardiac death in young individuals. Risk stratification and selection of patients presenting without history of life-threatening arrhythmic events for cardioverter-defibrillator implantation in primary prevention remains challenging. Purpose We aimed to assess the impact of disease progression on arrhythmic outcomes in AC patients. Methods We included consecutive AC probands and mutation-positive family members with at least one complete follow-up evaluation. Echocardiographic and electrical parameters were defined according to the 2010 Revised Task Force criteria at inclusion and at last follow-up. Structural progression was defined as development of new echocardiographic diagnostic criteria. Electrical progression was defined as the development of new diagnostic depolarization, repolarization and/or premature ventricular complex count criteria during follow-up. Non-sustained ventricular tachycardia or ventricular tachycardia occurring during follow-up defined incident ventricular arrhythmic events. Results We included a total of 144 patients (48% female, 47% probands, 40±16 years old). At inclusion, 54 patients (37%) had a history of arrhythmic events, 30 patients (21%) had overt structural disease and 114 (79%) had no or minor structural disease. During 7.0 (IQR: 4.5 to 9.4) years of follow-up, 49 patients (43%) with no or minor structural disease at inclusion developed new structural criteria being defined as progressors. Among 80 participants with no or minor structural disease and no arrhythmic history at inclusion, a first arrhythmic event occurred in 14 (17%). The incidence of arrhythmic events was higher in progressors (11/27, 41%) than in non-progressors (3/53, 6%) (p<0.001) (Figure). Structural progression was associated with higher risk of first arrhythmic events during follow-up when adjusted for sex, age at inclusion and follow-up duration, independent of electrical progression (7.6, 95% CI [1.5, 37.2], P=0.01). Incident arrhythmic events distribution Conclusion Almost half of patients without overt structural cardiac disease at genetic diagnosis develop new structural criteria during 7 years follow-up and 17% experienced their first ventricular arrhythmic event. Structural progression was independently associated with ventricular arrhythmic events during follow-up. These findings highlight the increased risk of arrhythmias when structural abnormalities are detected. Their finding may initiate the evaluation for primary prevention cardioverter-defibrillator implantation.

Cost Effectiveness in Low Risk MGUS Patients

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 2360-2360
Author(s):  
Tiffany Pompa ◽  
Mark Maddox ◽  
Adonas Woodard ◽  
Jeurkar Chet ◽  
Maelys Amat ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Risk Stratification ◽  
Medical Costs ◽  
Low Risk ◽  
Bone Lesions ◽  
Office Visits ◽  
Excess Cost ◽  
Total Cost ◽  
The Cost

Abstract Monoclonal gammopathy of undetermined significance (MGUS) is a premalignant disorder characterized by the asymptomatic presence of a monoclonal protein. It is defined by an M protein < 3 gm/dl, less than 10% clonal plasma cells in the bone marrow, and the absence of anemia, hypercalcemia, renal insufficiency and bone lesions. In 2010 the International Myeloma Working Group (IMWG) advocated for MGUS patients to be stratified into low risk disease, which carries a 5% risk of progression to multiple myeloma at 20 years, and high risk disease, which represents a 20% risk at 20 years. This stratification model categorizes patients as low risk if they have an IgG paraprotein with an M-component < 1.5 g/dl and a normal free light chain (FLC) ratio. As such, it is suggested that the initial workup be comprised of a serum protein electrophoresis (SPEP), an immunofixation (IFE), and a FLC ratio. A bone marrow biopsy (BM) and bone survey should only be performed if anemia, hypercalcemia or an elevated creatinine of unclear etiology is noted. If these studies place a patient into the low risk, it is suggested the patient follow up at 6-months with only an SPEP. If the SPEP is stable, the next follow-up is recommended to occur at 2 to 3 year intervals unless symptoms arise suggestive of a plasma cell dyscrasia. The risk stratification of MGUS patients was validated in 2013 by Turesson et al. in a Swedish cohort (Blood, 2014; 123:338-345). Nevertheless, the risk model is not universally accepted and unnecessary office visits along with laboratory studies are performed on low risk patients. The purpose of this study was to perform an internal retrospective review of our patients diagnosed with low risk MGUS, evaluating excess medical costs incurred when patients were not risk stratified by the IMWG recommendations. Methods: MGUS patients seen in the Hematology Oncology Division of Drexel University between 2014 and 2016 were retrospectively categorized into high and low risk based on the IMWG criteria. Those determined to be low risk were evaluated over two years for extra costs incurred outside the IMWG recommendations. Extra cost was tallied based on initial workup and surveillance studies performed up to two years from diagnosis. Costs per test and follow up visits were based on our office appointment pricing and BM biopsy charges. Laboratory costs were obtained based on pricing from ACCU reference lab. Cost per test (varies by lab/provider) SPEP $67 UPEP $130 Serum IFE $200 Urine IFE $72 IgA $27 IgG $27 IgM $27 K/L ratio $120 B2 microglobulin $42 Office Visit $40 - $100 Bone Survey $500 - $1200 BM biopsy $500- $1000 Results: Sixty patients seen between 2014 and 2016 met the criteria for MGUS. Twenty-eight patients were determined to have low risk disease. Of the 28 patients, five were diagnosed prior to 2010 and were excluded. In the remaining 23 patients, four followed up at exactly six months from diagnosis and only one had an SPEP. The most common test ordered was quantitative immunoglobulins (QI) aside from a CBC and CMP. The total number of excess office visits was 49. Three patients had unnecessary BM biopsies (total cost $1,000 - $2,000), and 11 had unnecessary bone surveys (Total $5,500 - $13,200). The total cost of unnecessary lab tests within 2 years was $6,024 and the total cost of unnecessary office visits within 2 years was $1960 - $4900. Thus, the average excess spent per patient was $630 - $1135, for a total excess cost for the 23 patients of $14,484 - $26,124. Conclusion: This internal review highlights the excess medical costs incurred when patients are not risk stratified by the IMWG recommendations. Ideally, no further health care dollars should be spent for low risk MGUS patients who have a stable SPEP at the 6-month visit until the 2 or 3 year follow up visit. The actual excess amount spent in our office in 2 years for these patients was $14,484 - $26,124 beyond the cost of the standard of care recommended by the IMWG guidelines. Additionally, these values did not include excess basic labs such as a CBC or CMP and it did not include extension of our investigation out to three years which would result in further unnecessary costs. One patient was noted to accumulate excess cost due to his co-morbid condition of prostate cancer, which led to increased surveillance for his low risk MGUS. The risk stratification model allows physicians to offer patients a better understanding of their disease, decrease the patient's burden and reduce the cost on healthcare. Disclosures No relevant conflicts of interest to declare.

scholarly journals Loperamide Induced Life Threatening Ventricular Arrhythmia

2016 ◽  
Vol 2016 ◽  
pp. 1-3 ◽  
Author(s):  
Ankit Upadhyay ◽  
Vijaykumar Bodar ◽  
Mohammad Malekzadegan ◽  
Sharanjit Singh ◽  
William Frumkin ◽  
...  
Keyword(s):  
Cardiac Arrest ◽  
Ventricular Arrhythmias ◽  
Cardiac Toxicity ◽  
Drug Abusers ◽  
Qtc Interval ◽  
Over The Counter ◽  
Life Threatening ◽  
Irritable Bowel ◽  
Antidiarrheal Agent ◽  
Congenital Long Qt Syndrome

Loperamide is over-the-counter antidiarrheal agent acting on peripherally locatedμopioid receptors. It is gaining popularity among drug abusers as opioid substitute. We report a case of a 46-year-old male that was presented after cardiac arrest. After ruling out ischemia, cardiomyopathy, pulmonary embolism, central nervous system pathology, sepsis, and other drug toxicity, we found out that patient was using around 100 mg of Loperamide to control his chronic diarrhea presumably because of irritable bowel syndrome for last five years and consumed up to 200 mg of Loperamide daily for last two days before the cardiac arrest. We hypothesize that the patient’s QTc prolongation and subsequent cardiac arrest are due to Loperamide toxicity. Patient experienced gradual resolution of tachyarrhythmia and gradual decrease in QTc interval during hospitalization which supports the evidence of causal relationship between Loperamide overdose and potentially fatal arrhythmias. It also provided the clue that patient may have congenital long QT syndrome which was unmasked by Loperamide causing ventricular arrhythmias. This case adds one more pearl in the literature to support that Loperamide overdose related cardiac toxicity does exist and it raises concerns over Loperamide abuse in the community.

scholarly journals Cardiomyopathy phenotyping and risk-stratification in danon disease

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
M Rigolli ◽  
A Khan ◽  
M Brambatti ◽  
F Contijoch ◽  
E Adler
Keyword(s):  
Heart Failure ◽  
Risk Stratification ◽  
Heart Transplant ◽  
Left Ventricular ◽  
Clinical Severity ◽  
Funding Source ◽  
Icd Implantation ◽  
Danon Disease ◽  
Imaging Characteristics

Abstract Background Danon Disease (DD) is a rare, X-linked vacuolar myopathy due to mutations in Lysosomal Associated Membrane Protein 2 (LAMP-2). Though it is strongly associated with severe cardiomyopathy, heart failure and sudden death, there is no data on typical cardiac magnetic resonance (CMR) imaging characteristics in DD and their association with clinical severity and outcome. Purpose To phenotype and risk-stratify DD patients. Methods CMR scans of confirmed DD patients recruited in a global registry were prospectively analyzed for biventricular volumes, ejection fraction (EF), left ventricular (LV) strain, mass (LVM) and late gadolinium enhancement (LGE) in a core-lab blinded fashion. A major adverse cardiac event (MACE) was a composite of death, heart transplant and implantable cardioverter defibrillator (ICD) for secondary prevention. Results 12 DD patients (5 males [42%], median age 13 yrs [interquartile range (IQR) 5]) were included. LV hypertrophy (LVH) was present in 10/12 (83%), associated with LV dilation in 2 females. LVH was typically asymmetric in females (5/7) and concentric in males (3/5); right ventricular (RV) hypertrophy frequent in females (4/7). LV strain was reduced (global circumferential strain [GCS] −12±4%) and LGE common (73%), often extensive and always sparing the basal-mid septum. LGE was strongly associated with heart failure (BNP r=0.9, p=0.0021). Patients with MACE (6 [50%], median follow-up 2.9 yrs) had elevated LVM (241±63 g, p=0.032), impaired LV strain (GCS: −9.8±3.9, p=0.02) and higher LGE mass (median 56 g [IQR 35], p=0.021) compared to those without events during follow-up (LVM 155±56 g, GCS −14.9±1.6, LGE mass 0 g [IQR 8]). CMR characteristics were predictors of MACE (LV strain: hazard ratio [HR] 1.4, p=0.021; LGE mass: HR 1.1, p=0.03). Conclusions LGE sparing the basal-mid septum was pathognomonic in DD. LVH with reduced LV strain was the most common DD phenotype but the spectrum included LV dilation and RV hypertrophy in females. CMR characteristics (LV strain and LGE) were associated with heart failure and predicted worse outcome (heart transplant and fatal arrhythmias). CMR phenotyping and risk-stratification of this severe and underrecognized cardiomyopathy may aid diagnosis and clinical management in DD patients who need selection for early heart transplant, ICD implantation and targeted gene therapy. Danon Disease Phenotypes and Outcome Funding Acknowledgement Type of funding source: Other. Main funding source(s): National Institute of Health and Rocket Pharmaceuticals

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