Abstract 2640: Clinical Characteristics and Risk Stratification in Symptomatic and Asymptomatic Patients with Brugada Syndrome - Multi-center Study in Japan-

Background Neither the clinical characteristics nor risk stratification of symptomatic and asymptomatic patients with Brugada syndrome have been clearly determined. We compared clinical and ECG characteristics of symptomatic and asymptomatic patients with Brugada syndrome to identify new markers for distinguishing high- from low-risk patients. Methods A total of 216 consecutive individuals with Brugada syndrome (mean age 52±14 years, 197 males) were enrolled in the Japan Idiopathic Ventricular Fibrillation Study (J-IVFS). Clinical and ECG characteristics were compared among 3 groups of patients: VF group; patients with aborted sudden death and documented VF (N=34), Syncope (Sy) group; patients with syncope without documented VF (N=70), and Asymptomatic (As) group; subjects without symptoms (N=112). Comparisons were made among the 3 groups as well as between the symptomatic (VF/Sy) and asymptomatic (As) groups. Short-term prognosis was also compared among the 3 groups, and between the VF/Sy and As groups. Results 1) Clinical characteristics: incidence of past history of AF was significantly higher in the VF and Sy groups than in the AS group (26, 26, and 12 %, respectively; [p=0.04]), though no other clinical parameters differed among the groups. 2) On resting 12-lead ECG, r-J interval (interval from QRS onset to J point) in lead V2 and QRS duration in lead V6 were highest in the VF group (104, 98, and 92 msec in V2 [p<0.001]; 106, 103, and 94 msec in V6 [p<0.0001], respectively, VF vs. Sy vs. As). 3) Positive late potential and inducibility of VF by EPS did not differ in incidence among the 3 groups. 4) Clinical follow-up: during a mean follow-up of 36±16 months, incidence of cardiac events (sudden death and/or VF) was higher in the VF/Sy groups than in the As group (29, 8, and 0 %, respectively [p<0.001]). Multivariate analysis showed that the frequencies of r-J interval ≥ 90 msec in lead V2 and QRS duration ≥ 90 msec in lead V6 were significantly higher in patients with cardiac events (p=0.02, 0.02, respectively). Conclusions In symptomatic patients, prolonged ventricular depolarization in precordial leads of the ECG was prominent in the VF group, and this sign can be used to distinguish high- from low-risk patients with Brugada syndrome.

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Cardiac Conduction Disorders as Markers of Cardiac Events in Myotonic Dystrophy Type 1

Journal of the American Heart Association ◽

10.1161/jaha.119.015709 ◽

2020 ◽

Vol 9 (17) ◽

Cited By ~ 1

Author(s):

Hideki Itoh ◽

Takashi Hisamatsu ◽

Takuhisa Tamura ◽

Kazuhiko Segawa ◽

Toshiaki Takahashi ◽

...

Keyword(s):

Sudden Death ◽

Myotonic Dystrophy ◽

Cardiac Conduction ◽

Myotonic Dystrophy Type 1 ◽

Myotonic Dystrophy Type ◽

Cardiac Events ◽

Conduction Disorders ◽

Qrs Duration

Background Myotonic dystrophy type 1 involves cardiac conduction disorders. Cardiac conduction disease can cause fatal arrhythmias or sudden death in patients with myotonic dystrophy type 1. Methods and Results This study enrolled 506 patients with myotonic dystrophy type 1 (aged ≥15 years; >50 cytosine‐thymine‐guanine repeats) and was treated in 9 Japanese hospitals for neuromuscular diseases from January 2006 to August 2016. We investigated genetic and clinical backgrounds including health care, activities of daily living, dietary intake, cardiac involvement, and respiratory involvement during follow‐up. The cause of death or the occurrence of composite cardiac events (ie, ventricular arrhythmias, advanced atrioventricular blocks, and device implantations) were evaluated as significant outcomes. During a median follow‐up period of 87 months (Q1–Q3, 37–138 months), 71 patients expired. In the univariate analysis, pacemaker implantations (hazard ratio [HR], 4.35; 95% CI, 1.22–15.50) were associated with sudden death. In contrast, PQ interval ≥240 ms, QRS duration ≥120 ms, nutrition, or respiratory failure were not associated with the incidence of sudden death. The multivariable analysis revealed that a PQ interval ≥240 ms (HR, 2.79; 95% CI, 1.9–7.19, P <0.05) or QRS duration ≥120 ms (HR, 9.41; 95% CI, 2.62–33.77, P < 0.01) were independent factors associated with a higher occurrence of cardiac events than those observed with a PQ interval <240 ms or QRS duration <120 ms; these cardiac conduction parameters were not related to sudden death. Conclusions Cardiac conduction disorders are independent markers associated with cardiac events. Further investigation on the prediction of occurrence of sudden death is warranted.

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564 Controversial role of intracardiac eletrophysiology study in Brugada syndrome: analysis of a single-centre retrospective cohort study

European Heart Journal Supplements ◽

10.1093/eurheartj/suab127.014 ◽

2021 ◽

Vol 23 (Supplement_G) ◽

Author(s):

Claudio Licciardello ◽

Jacopo Marazzato ◽

Michele Golino ◽

Francesca Seganfreddo ◽

Federica Matteo ◽

...

Keyword(s):

High Risk ◽

Risk Stratification ◽

Right Ventricular ◽

Brugada Syndrome ◽

Type I ◽

Drug Induced ◽

Programmed Ventricular Stimulation ◽

Asymptomatic Patients ◽

Ventricular Stimulation

Abstract Aims According to European guidelines, aborted sudden cardiac death (SCD) in Brugada syndrome (BrS) is regarded as a I class recommendation for secondary prevention implantable cardioverter defibrillator (ICD). However, the risk stratification of BrS patients for primary prevention ICD still represents a clinical conundrum. Although intracardiac electrophysiology (EP) study proved useful for the selection of high-risk patients in this setting. Therefore, aim of this study was to assess all clinical and EP variables associated with the induction of VA at EP study and the rate of appropriate/inappropriate ICD interventions and/or clinical SCD events in these patients occurring at follow-up. Methods and results From 2001 to 2021, all EP studies performed in symptomatic/asymptomatic patients (46 ± 14 years, M 88%) with/without family history of SCD spontaneous/drug-induced type I pattern (TIP) on ECG and no spontaneous ventricular arrhythmias were retrospectively considered at our study centre. Clinical variables, BrS pattern, EP study data (including right ventricular site and type of stimulation protocol), and ICD interventions (DC-shocks or Anti-Tachycardia Pacing events, ATP) and/or SCD events occurring at follow-up were all evaluated. EP study was deemed positive for any polymorphic VA induced during programmed ventricular stimulation; non-sustained episodes included. ICD was routinely implanted in all patients with a positive EP study. Follow-up data were detected by the collection of medical and home-monitoring recordings at study-site level. Follow-up data were available in 50 patients (9 ± 6 years on average). Patients were generally young with few cardiovascular comorbidities. SCD history was known in 21 (42%) with a significant number of asymptomatic patients (48%). Br patterns were equally distributed in the investigated population (spontaneous and drug-induced TIP in 52% and 48%, respectively) and AF history was fairly common (16%). In the study population, EP study tested positive in 30 patients (60%): spontaneous TIP (P = 0.0518), few extrastimuli during programmed ventricular stimulation (P = 0.0015), and right ventricular stimulation at the apical site (P ≤ 0.0001) were the only variables to be clearly associated with a positive EP study in the appraised patients. At follow-up, appropriate ICD shocks were documented in 4 out of 30 implanted patients (13%) at generally 5 ± 7 years from EP study evaluation. Although three ICD interventions (75%) occurred in patients with spontaneous TIP, one patient with drug-induced TIP pattern and positive EP study referred to Emergency Department for unrelenting VT storm after roughly 13 years from ICD implantation. Inappropriate ICD interventions for fast rate AF were detected in 10% of cases. Finally, no SCD events were documented at follow up in patients with a negative EP study. Conclusions In a retrospective analysis, EP study proved useful in the risk stratification of SCD in BrS patients. A few ventricular extrastimuli delivered at the right ventricular apex seem sufficient to prompt the induction of life-threatening VA in high-risk BrS patients during EP study. Moreover, in this setting, a negative EP study seems protective against the development of VA/SCD events at follow-up. However, not only is spontaneous TIP associated with an increased risk of arrhythmic death, but a drug-induced TIP, generally regarded as a low-risk condition, might also be associated with a long-term hazard of SCD in these patients.

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Delayed risk stratification, to include the response to initial treatment (surgery and radioiodine ablation), has better outcome predictivity in differentiated thyroid cancer patients

Acta Endocrinologica ◽

10.1530/eje-11-0466 ◽

2011 ◽

Vol 165 (3) ◽

pp. 441-446 ◽

Cited By ~ 152

Author(s):

Maria Grazia Castagna ◽

Fabio Maino ◽

Claudia Cipri ◽

Valentina Belardini ◽

Alexandra Theodoropoulou ◽

...

Keyword(s):

Thyroid Cancer ◽

High Risk ◽

Risk Stratification ◽

Initial Treatment ◽

Differentiated Thyroid Cancer ◽

Low Risk ◽

Risk Category ◽

Radioiodine Ablation ◽

Risk Patients

IntroductionAfter initial treatment, differentiated thyroid cancer (DTC) patients are stratified as low and high risk based on clinical/pathological features. Recently, a risk stratification based on additional clinical data accumulated during follow-up has been proposed.ObjectiveTo evaluate the predictive value of delayed risk stratification (DRS) obtained at the time of the first diagnostic control (8–12 months after initial treatment).MethodsWe reviewed 512 patients with DTC whose risk assessment was initially defined according to the American (ATA) and European Thyroid Association (ETA) guidelines. At the time of the first control, 8–12 months after initial treatment, patients were re-stratified according to their clinical status: DRS.ResultsUsing DRS, about 50% of ATA/ETA intermediate/high-risk patients moved to DRS low-risk category, while about 10% of ATA/ETA low-risk patients moved to DRS high-risk category. The ability of the DRS to predict the final outcome was superior to that of ATA and ETA. Positive and negative predictive values for both ATA (39.2 and 90.6% respectively) and ETA (38.4 and 91.3% respectively) were significantly lower than that observed with the DRS (72.8 and 96.3% respectively,P<0.05). The observed variance in predicting final outcome was 25.4% for ATA, 19.1% for ETA, and 62.1% for DRS.ConclusionsDelaying the risk stratification of DTC patients at a time when the response to surgery and radioiodine ablation is evident allows to better define individual risk and to better modulate the subsequent follow-up.

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Long-term monitoring of arrhythmias in cardiac sarcoidosis

European Heart Journal ◽

10.1093/ehjci/ehaa946.2138 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

A Bakker ◽

H Mathijssen ◽

J Balt ◽

V.F Van Dijk ◽

M Veltkamp ◽

...

Keyword(s):

Risk Assessment ◽

Risk Stratification ◽

Cardiac Death ◽

Cardiac Sarcoidosis ◽

Low Risk ◽

Risk Patients ◽

Long Term Monitoring ◽

Term Monitoring

Abstract Introduction Screening for cardiac sarcoidosis (CS) is recommended since it can manifest with ventricular arrhythmias (VA), atrioventricular conduction block (AVB) and sudden cardiac death (SCD). However, risk stratification for SCD is challenging, in particular in patients without overt cardiac symptoms. Purpose This study reports the practice-based risk stratification for SCD and the incidence of arrhythmias and mortality in CS patients by long-term monitoring of arrhythmias. Methods A retrospective, single center cohort study was performed in 537 patients with sarcoidosis screened for cardiac involvement with cardiac MRI and fluorodeoxyglucose PET in an hospital, a Dutch tertiary referral center. CS was diagnosed in 115 of 537 patients (21%), complete follow up was available in 108 patients (94%). After risk assessment for SCD (figure 1) an ICD was implanted in 16 high-risk patients. Within the92 low-risk patients, 80 had an internal loop recorder (ILR) implanted and 12 patients received no device. Chart review was performed to assess the occurrence of VA, AVB, death, ICD therapy and device related complications. Results During a mean follow-up of 31±15 months, 9 out of 80 ILR patients (11.3%) received an ICD of whom 7 (8.8%) based on recorded arrhythmias (VA in 5 and AVB in 2 patients). Five out of the total 25 ICD patients (20%) experienced sustained VA successfully treated with anti-tachycardia pacing in 2 (8%) and terminated spontaneously in all other patients. Two ICD patients experienced a mild pocket infection, treated with antibiotics. Two deaths occurred in the low-risk patients: 1 non-cardiac death and 1 SCD due to asystole. Conclusion The practice-based risk stratification supported an ICD implantation in up to 5% of sarcoidosis patients screened for CS. Sustained VA occurred in 20% of ICD patients.Early detection of important arrhythmias with an ILR can optimize risk assessment for SCD in CS. Practise-based risk stratification Funding Acknowledgement Type of funding source: None

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Low-Risk Polycythemia Vera Treated with Phlebotomies: Clinical Characteristics, Hematologic Control and Complications in 358 Patients from the Spanish Registry of Polycythemia Vera

Blood ◽

10.1182/blood-2021-145012 ◽

2021 ◽

Vol 138 (Supplement 1) ◽

pp. 3621-3621

Author(s):

Ana Triguero ◽

Alexandra Pedraza ◽

Manuel Pérez ◽

María Isabel Mata ◽

Beatriz Bellosillo ◽

...

Keyword(s):

Polycythemia Vera ◽

Research Funding ◽

Clinical Characteristics ◽

Treatment Strategies ◽

Myeloproliferative Disorders ◽

Primary Prophylaxis ◽

Low Risk ◽

Major Hemorrhage ◽

Risk Patients

Abstract Introduction: Current recommendations for patients with low-risk polycythemia vera (PV) include hematocrit (Htc) control with phlebotomies and primary prophylaxis of thrombosis with low-dose aspirin. There is scarce information regarding the hematological control, the incidence of complications and the need for cytoreduction in PV patients treated with phlebotomies only. Methods: A total of 358 patients with low-risk PV (<60 years old and without history of thrombosis) from the Spanish Registry of Polycythemia Vera were included in the present study. PV-related symptoms and blood counts were collected at 6, 12, 18, 24, 36, 48 and 60 months from diagnosis while the patients were treated with phlebotomies only. The duration of the treatment with phlebotomies, the indication of starting cytoreduction and the incidence of thromboembolic and hemorrhagic events during the cytoreduction-free period was also analyzed. Results: Baseline characteristics at the time of diagnosis are described in Table 1. Table 2 summarizes the main hematological and clinical characteristics under treatment with phlebotomies. Inadequate control of the Htc (> 45%) was reported in 61-70% of the patients, leukocytosis >15x10 9/l in 10% and thrombocytosis >1000x10 9/l in 5%. In addition, about 20% of the patients had pruritus and 10% had microvascular symptoms. Of the 358 patients included, 275 (77%) required cytoreduction, 261 (73%) with hydroxyurea and 14 (4%) with IFN. The main indication of cytoreduction was thrombocytosis (20%), followed by age >60 years old (15%) and microvascular symptoms (13%). Median duration of cytoreduction abstention was 4.7 (0.1-30.4) years being significantly longer in patients younger than 50 years (6 and 2 years for patients younger and older than 50 years, respectively, p<0.0001). With a follow-up of 1659 person-years under phlebotomy only treatment, 14 thrombosis were observed (arterial n=9, venous n= 5), 12 hemorrhages (major n=4, minor n=8) and 4 solid tumors (1 melanoma and 3 non-cutaneous carcinomas). The incidence of complications during the cytoreduction-free period by person-years was: 0.8% for thrombosis, 0.2% for major hemorrhage and 0.2% for second neoplasia. The median follow-up until last visit including the time after starting cytoreductive therapy was 8.4 (0.2-39) years. Of 14 deaths observed, none occurred during the phlebotomy period. Half of the patients died from PV related reasons but the other 50% were not related. The median survival estimation by K-M was 36.5 years. Disease progression was documented in 27 (7.5%) patients, 26 of them to myelofibrosis, 1 to myelodysplastic syndrome and none to acute leukemia. Progression to myelofibrosis occurred during the cytoreduction-free period in 5 patients (1.4%) after a median of 5.8 years (Range: 4.9-8.9). Conclusions The incidence of thrombotic and hemorrhagic complications was very low in this series of low-risk patients treated with phlebotomies, even though only 30-40% of patients maintained the Htc <45%. The data from the present study show that low-risk patients have different therapeutic needs than other PV patients and support the development of new treatment strategies. Representing the Spanish Group of Myeloproliferative Disorders. GEMFIN Figure 1 Figure 1. Disclosures Bellosillo: Qiagen: Consultancy, Speakers Bureau; Roche: Consultancy, Research Funding; Thermofisher Scientific: Consultancy, Speakers Bureau. Ferrer Marin: Cty: Research Funding; Incyte: Consultancy, Research Funding; Novartis: Speakers Bureau. Garcia Gutierrez: Novartis: Consultancy, Honoraria, Research Funding; BMS: Consultancy, Honoraria, Research Funding; Pfizer: Consultancy, Honoraria, Research Funding; Incyte: Consultancy, Honoraria, Research Funding.

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Indication and prognostic significance of programmed ventricular stimulation in asymptomatic patients with Brugada syndrome

EP Europace ◽

10.1093/europace/euaa003 ◽

2020 ◽

Vol 22 (6) ◽

pp. 972-979 ◽

Cited By ~ 1

Author(s):

Saori Asada ◽

Hiroshi Morita ◽

Atsuyuki Watanabe ◽

Koji Nakagawa ◽

Satoshi Nagase ◽

...

Keyword(s):

High Risk ◽

Ventricular Fibrillation ◽

Brugada Syndrome ◽

Event Rate ◽

Prognostic Significance ◽

Fragmented Qrs ◽

Asymptomatic Patients ◽

Ventricular Stimulation ◽

Risk Patients

Abstract Aims To establish the indication for programmed ventricular stimulation (PVS) for asymptomatic patients with Brugada syndrome (BrS), we evaluated the prognostic significance of PVS based on abnormal electrocardiogram (ECG) markers. Methods and results One hundred and twenty-five asymptomatic patients with BrS were included. We performed PVS at two sites of the right ventricle with up to three extrastimuli [two pacing cycle lengths and minimum coupling interval (MCI) of 180 ms]. We followed the patients for 133 months and evaluated ventricular fibrillation (VF) events. Fragmented QRS (fQRS) and Tpeak-Tend (Tpe) interval were evaluated as ECG markers for identifying high-risk patients. Fragmented QRS and long Tpe interval (≥100 ms) were observed in 66 and 37 patients, respectively. Ventricular fibrillation was induced by PVS in 60 patients. During follow-up, 10 patients experienced VF events. Fragmented QRS, long Tpe interval, and PVS-induced VF with an MCI of 180 ms or up to two extrastimuli were associated with future VF events (fQRS: P = 0.015, Tpe ≥ 100 ms: P = 0.038, VF induction: P < 0.001). However, PVS-induced VF with an MCI of 200 ms was less specific (P = 0.049). The frequencies of ventricular tachyarrhythmia events during follow-up were 0%/year with no ECG markers and 0.1%/year with no VF induction. The existence of two ECG factors with induced VF was strongly associated with future VF events (event rate: 4.4%/year, P < 0.001), and the existence of one ECG factor with induced VF was also associated (event rate: 1.3%/year, P = 0.011). Conclusion We propose PVS with a strict protocol for asymptomatic patients with fQRS and/or long Tpe interval to identify high-risk patients.

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Long-term prognosis in patients with non-type 1 Brugada ECG: results from a large Japanese cohort of Brugada syndrome

European Heart Journal ◽

10.1093/ehjci/ehaa946.0736 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

M Takagi ◽

T Shinohara ◽

T Kamakura ◽

Y Sekiguchi ◽

Y Yokoyama ◽

...

Keyword(s):

Brugada Syndrome ◽

Clinical Risk Factor ◽

Cardiac Events ◽

Drug Induced ◽

Japanese Cohort ◽

Qrs Duration ◽

Long Term Prognosis

Abstract Background Most recent consensus conference report recommends Brugada syndrome (BrS) is diagnosed in patients with ST segment elevation with spontaneous, drug-induced or fever-induced type 1 morphology. Prognosis in patients with type 2 or 3 ECG without drug-induced or fever-induced type 1 ECG is still unknown. Purpose To evaluate a long-term prognosis in patients with non-type 1 Brugada ECG in a large Japanese cohort of BrS (The Japan Idiopathic Ventricular Fibrillation Study [J-IVFS]). Methods From 528 patients in J-IVFS, a total of 28 consecutive non-type 1 patients (54±14 years, all male, previous sustained ventricular tachyarrhythmias (VTs) 1, syncope 11, asymptomatic 16) were enrolled. Cardiac events (CI: sudden cardiac death or VTs) during the follow-up period were evaluated, and risk factors for the cardiac events were assessed. Results During a mean follow-up period of 111±91 months (median 134 months), 4 patients experienced cardiac events (1.5%/yr), who all had received implantable cardioverter defibrillator implantation. There was no statistically significant clinical risk factor for cardiac events. However, the incidences of cardiac events tended to be higher in symptomatic patients (CI: 25.0, non-CI: 6.3%, p=0.17), those with wide QRS duration >90 msec in lead V2 (CI: 30.0, non-CI: 6.3%, p=0.11), and those with inducible VTs (CI: 21.1, non-CI: 0%, p=0.20), as determined by the Kaplan-Meier method. The annual incidences of cardiac events in patients with symptom, wide QRS duration >90msec in lead V2, or inducible VTs were 2.8, 3.5, and 2.0%/yr, respectively. The incidences of cardiac events were significantly higher in patients with all these 3 factors (9.9%/yr) than those without (p=0.01). Conclusions Our large-scaled multicentre study revealed long-term prognosis in patients with non-type 1 Brugada ECG. The combination of symptom, wide QRS duration in lead V2, and inducible VTs may be useful to evaluate risk for cardiac events. The patients with all these parameters showed high risk for cardiac events and need to be carefully followed. Funding Acknowledgement Type of funding source: None

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Abstract 6102: Integration of Non-Contrast Cardiac Computed Tomography and Stress Myocardial Perfusion Imaging in the Emergency Department: Identifying Which Patients with Chest Pain Can be Safely Discharged Home

Circulation ◽

10.1161/circ.118.suppl_18.s_1061-c ◽

2008 ◽

Vol 118 (suppl_18) ◽

Author(s):

Faisal Nabi ◽

Su Min Chang ◽

Lemuel A Moye ◽

Robert G Hust ◽

Craig M Pratt ◽

...

Keyword(s):

Computed Tomography ◽

Emergency Department ◽

Chest Pain ◽

Cardiac Computed Tomography ◽

Low Risk ◽

Stress Myocardial Perfusion Imaging ◽

Cardiac Events ◽

Stress Myocardial Perfusion ◽

Risk Patients

Over 5 million emergency department (ED) visits occur annually for evaluation of chest pain. A rapid simple imaging algorithm is needed to identify patients with noncardiac chest pain so as to avoid unnecessary hospital admission. We conducted a prospective trial in 1031 low risk patients (60% women; mean age 54±13 years) admitted through the ED to our chest pain unit who had no prior cardiac history, a nondiagnostic ECG for ischemia, and a normal initial troponin. All patients had stress myocardial perfusion imaging (SPECT) with a coronary artery calcium score (CACS) by noncontrast cardiac computed tomography (CT) within 24 hours. Mean patient follow-up was 7.4±3.3 months. SPECT and CT studies were interpreted independently and the CACS quantified as an Agatston score. The mean TIMI risk score was 1.5±0.7. Cardiac events occurred in 29 patients (2.8%): acute myocardial infarction (N=4) or an acute coronary syndrome (ACS, N=21) during admission; or ACS following hospital discharge (N=4). Abnormal SPECT and cardiac events significantly increased with CACS (p<.001), with over a 40-fold increase in event rates for patients with a CACS>400 vs 0(Table ). Only 5 (0.8%) patients with CACS=0 had an abnormal SPECT and none had significant coronary artery disease by angiography. The 2 patients who had a CACS=0 and a cardiac event during their hospitalization both had a normal gated SPECT and no subsequent event in follow-up. The sensitivity of an abnormal CT was significantly higher than an abnormal SPECT for identifying patients with events (93% vs 65%, p<.01, respectively). A sizeable percentage (61%) of our low risk patient cohort had CACS=0 by CT which predicted both a normal SPECT and an excellent short-term outcome. Our data support that low risk patients with chest pain and a CACS=0 can be safely discharged home from the ED, with SPECT reserved for those with an abnormal CT result.

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