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.

scholarly journals A case of hypokalemia-induced bidirectional ventricular tachycardia

2020 ◽  
Vol 48 (11) ◽  
pp. 030006052097144
Author(s):  
Yanan Xie ◽  
Jingzhe Han ◽  
Jinming Liu ◽  
Jie Hao ◽  
Xiuguang Zu ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Potassium Level ◽  
Serum Potassium Level ◽  
Electrical Storm ◽  
Case Presentation ◽  
Cardioverter Defibrillator ◽  
Long Term Follow Up ◽  
History Of

Background Bidirectional ventricular tachycardia (BVT) is a rare, but serious, arrhythmia. Hypokalemia is commonly found in clinical practice, but hypokalemia-induced BVT has rarely been reported. Case presentation A 74-year-old male patient with the symptoms of chest distress and palpitations was admitted owing to frequent discharge of his implantable cardioverter defibrillator (ICD) for 4 days. Before admission, the patient experienced diarrhea after intake of crabs, and felt frequent discharge of his ICD with a total of approximately 17 discharges in 4 days. He had no history of digitalis use. The serum potassium level after admission was 3.1 mmol/L and an electrocardiogram was consistent with BVT. The diagnosis was ventricular tachycardia, electrical storm, and hypokalemia. His ventricular tachycardia was completely relieved after correction of hypokalemia. Conclusions After correction of hypokalemia in this patient, the episode of BVT was terminated and no recurrence of BVT was observed during long-term follow-up. Our findings suggest the diagnosis of hypokalemia-induced BVT.

Prognostic value of non-ischemic ring-like left ventricular scar pattern in patients with apparently idiopathic ventricular arrhythmias: a CMR imaging study

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
G Nucifora ◽  
D Muser ◽  
S Castro ◽  
R Casado Arroyo ◽  
D Benhayon ◽  
...  
Keyword(s):  
Ventricular Arrhythmias ◽  
Prognostic Significance ◽  
Imaging Study ◽  
Left Ventricular ◽  
High Rate ◽  
Life Threatening ◽  
History Of ◽  
Group A ◽  
Group B

Abstract Background The presence of left ventricular (LV) late gadolinium enhancement (LGE) at cardiac magnetic resonance (CMR) has been correlated to life-threatening arrhythmic events in patients with apparently idiopathic ventricular arrhythmias (VAs). Aim of the present study was to investigate the prognostic significance of a specific LV-LGE phenotype characterized by a subepicardial/midmyocardial “ring-like” pattern of fibrosis. Methods Out of a total of 518 consecutive patients with apparently idiopathic VAs who underwent CMR study, 79 (15%) had evidence of LV-LGE. Of these, 23 (4%) patients had LV LGE with ring-like pattern, defined as subepicardial or midmyocardial LGE involving at least 3 contiguous segments in the same slice (group A), while 56 (11%) patients had LV LGE with no ring-like pattern (group B). The remaining 439 patients had no LGE (group C). The end-point of the study was a composite SCD, resuscitated cardiac arrest and nonfatal episodes of ventricular fibrillation or documented sustained ventricular tachycardia. Results Group A patients were more frequently males compared to groups B and C (96% vs. 79% vs. 52%; p&lt;0.01) and had more frequently a family history of SCD and/or cardiomyopathy (30% vs. 11% vs. 5%; p&lt;0.01). All patients in Group A showed VAs with a predominant RBBB morphology vs. 38 (68%) patients in Group B and 65 (15%) in Group C (p&lt;0.01). During a follow-up of 63±39 months, the composite outcome occurred in 13 patients (57%) in Group A vs. 11 (20%) in Group B and 2 (1%) in Group C (p&lt;0.01). Conclusion In patients with apparently idiopathic VAs, a nonischemic LV-LGE with a ring-like pattern at CMR is associated with a high rate of malignant arrhythmic events during follow-up. Funding Acknowledgement Type of funding source: None

scholarly journals Outcome by Sex in Patients With Long QT Syndrome With an Implantable Cardioverter Defibrillator

2020 ◽  
Vol 9 (19) ◽  
Author(s):  
Arwa Younis ◽  
Mehmet K. Aktas ◽  
Spencer Rosero ◽  
Valentina Kutyifa ◽  
Bronislava Polonsky ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Ventricular Fibrillation ◽  
Implantable Cardioverter Defibrillator ◽  
Long Qt Syndrome ◽  
Syndrome Type ◽  
Long Qt ◽  
Cardioverter Defibrillator ◽  
Life Threatening ◽  
Qt Syndrome

Background Sex differences in outcome have been reported in patients with congenital long QT syndrome. We aimed to report on the incidence of time‐dependent life‐threatening events in male and female patients with long QT syndrome with an implantable cardioverter defibrillator (ICD). Methods and Results A total of 60 patients with long QT syndrome received an ICD for primary or secondary prevention indications. Life‐threatening events were evaluated from the date of ICD implant and included ICD shocks for ventricular tachycardia, ventricular fibrillation, or death. ICDs were implanted in 219 women (mean age 38±13 years), 46 girls (12±5 years), 55 men (43±17 years), and 40 boys (11±4 years). Mean follow‐up post‐ICD implantation was 14±6 years for females and 12±6 years for males. At 15 years of follow‐up, the cumulative probability of life‐threatening events was 27% in females and 34% in males (log‐rank P =0.26 for the overall difference). In the multivariable Cox model, sex was not associated with significant differences in risk first appropriate ICD shock (hazard ratio, 0.83 female versus male; 95% CI, 0.52–1.34; P =0.47). Results were similar when stratified by age and by genotype: long QT syndrome type 1 (LQT1), long QT syndrome type 2 (LQT2), and long QT syndrome type 3 (LQT3). Incidence of inappropriate ICD shocks was higher in males versus females (4.2 versus 2.7 episodes per 100 patient‐years; P =0.018), predominantly attributed to atrial fibrillation. The first shock did not terminate ventricular tachycardia/ventricular fibrillation in 48% of females and 62% of males ( P =0.25). Conclusions In patients with long QT syndrome with an ICD, the risk and rate of life‐threatening events did not significantly differ between males and females regardless of ICD indications or genotype. In a substantial proportion of patients with long QT syndrome, first shock did not terminate ventricular tachycardia/ventricular fibrillation.

scholarly journals RECURRENT SYNCOPE AS A SYMPTOM OF ELECTRICAL STORM – CASE PRESENTATION

2021 ◽  
Vol 8 (4) ◽  
pp. 271-273
Author(s):  
Przemysław Skoczyński ◽  
Paweł Pochciał
Keyword(s):  
Ventricular Arrhythmias ◽  
Electrical Storm ◽  
Monitoring Equipment ◽  
Case Presentation ◽  
Diffi Cult ◽  
Patient Reported ◽  
Cardiology Clinic ◽  
Life Threatening ◽  
History Of

Dangerous ventricular arrhythmias leading to sudden cardiac death (SCD) are some of the most diffi-cult diagnostic challenges. They are often mildly symptomatic. Their often self-limiting nature means that they are difficult to capture on ECG. A 75-year old woman with chronic heart failure due to nonis-chemic cardiomyopathy reported to the cardiology clinic for a scheduled routine follow-up of the ICD, implanted three years prior as primary prevention of SCD. The patient reported recent episodes of sud-den weakness and described the episodes as hypotension. The patient associated it with too aggressive treatment of arterial hypertension. During the visit the patient experienced one of these episodes that she had previously described. The monitoring equipment in the clinic revealed ventricular tachycardia (VT). The history of the implanted ICD revealed many similar previous episodes including 5 episodes in the last 24 hours which led to a diagnosis of electrical storm. Dangerous ventricular arrhythmias may be mildly symptomatic and they are often underestimated by the patient. Fainting, especially in situa-tions unusual for the vasovagal reflex or orthostatic hypotension, should always arouse vigilance to-wards life-threatening ventricular arrhythmia.

scholarly journals Traumatic Tension Pneumothorax as a Cause of ICD Failure: A Case Report and Review of the Literature

2014 ◽  
Vol 2014 ◽  
pp. 1-4
Author(s):  
Ehtesham Ul Haq ◽  
Bassam Omar
Keyword(s):  
Ventricular Tachycardia ◽  
Ventricular Arrhythmias ◽  
Tension Pneumothorax ◽  
African American Female ◽  
Nonischemic Cardiomyopathy ◽  
Review Of The Literature ◽  
Cardioverter Defibrillator ◽  
The Third ◽  
History Of ◽  
High Index Of Suspicion

Background. Tension pneumothorax can infrequently cause ventricular arrhythmias and increase the threshold of defibrillation. It should be suspected whenever there is difficulty in defibrillation for a ventricular arrhythmia.Purpose. To report a case of traumatic tension pneumothorax leading to ventricular tachycardia and causing defibrillator failure.Case. A 65-year-old African-American female was brought in to our emergency department complaining of dyspnea after being forced down by cops. She had history of mitral valve replacement for severe mitral regurgitation and biventricular implantable cardioverter defibrillator inserted for nonischemic cardiomyopathy. Shortly after arrival, she developed sustained ventricular tachycardia, causing repetitive unsuccessful ICD shocks. She was intubated and ventricular tachycardia resolved with amiodarone. Chest radiograph revealed large left sided tension pneumothorax which was promptly drained. The patient was treated for congestive heart failure; she was extubated on the third day of admission, and the chest tube was removed.Conclusion. Prompt recognition of tension pneumothorax is essential, by maintaining a high index of suspicion in patients with an increased defibrillation threshold causing ineffective defibrillations.

scholarly journals Ventricular fibrillation ablation in cardiomyopathies and arrhythmic storm

2021 ◽  
Vol 23 (Supplement_E) ◽  
pp. E112-E117
Author(s):  
Carlo Pappone ◽  
Gabriele Negro ◽  
Giuseppe Ciconte
Keyword(s):  
Ventricular Tachycardia ◽  
Cardiac Death ◽  
Ventricular Arrhythmias ◽  
Healthy Individuals ◽  
Therapeutic Tool ◽  
Cardioverter Defibrillator ◽  
Structural Abnormalities ◽  
Life Threatening ◽  
Underlying Mechanisms ◽  
Screening And Prevention

Abstract Sudden cardiac death (SCD) is a relevant contributor to cardiovascular mortality, often occurring as a dramatic event. It can be the consequence of a ventricular tachycardia/fibrillation (VT/VF), a common and life-threatening arrhythmia. The underlying mechanisms of this catastrophic arrhythmia are poorly known. In fact, it can occur in the presence of a structural heart condition which itself generates the suitable substrate for this arrhythmia. Nevertheless, a VF may cause SCD also in young and otherwise healthy individuals, without overt structural abnormalities, generating difficulties in the screening and prevention of these patients. The implantable cardioverter-defibrillator represents the only therapy to contrast SCD by treating a VT/VF; however, it cannot prevent the occurrence of such arrhythmias. Catheter ablation is emerging as an essential therapeutic tool in the management of patients experiencing ventricular arrhythmias.

scholarly journals Catheter Ablation of Life-Threatening Ventricular Arrhythmias in Athletes

Medicina ◽  
2021 ◽  
Vol 57 (3) ◽  
pp. 205
Author(s):  
Nicola Tarantino ◽  
Domenico G. Della Rocca ◽  
Nicole S. De Leon De La Cruz ◽  
Eric D. Manheimer ◽  
Michele Magnocavallo ◽  
...  
Keyword(s):  
Catheter Ablation ◽  
Ventricular Arrhythmias ◽  
Individual Factors ◽  
Substantial Part ◽  
Arrhythmogenic Substrate ◽  
Life Threatening ◽  
History Of ◽  
Anatomic Distribution ◽  
Artery Disease ◽  
Surveillance Analysis

A recent surveillance analysis indicates that cardiac arrest/death occurs in ≈1:50,000 professional or semi-professional athletes, and the most common cause is attributable to life-threatening ventricular arrhythmias (VAs). It is critically important to diagnose any inherited/acquired cardiac disease, including coronary artery disease, since it frequently represents the arrhythmogenic substrate in a substantial part of the athletes presenting with major VAs. New insights indicate that athletes develop a specific electro-anatomical remodeling, with peculiar anatomic distribution and VAs patterns. However, because of the scarcity of clinical data concerning the natural history of VAs in sports performers, there are no dedicated recommendations for VA ablation. The treatment remains at the mercy of several individual factors, including the type of VA, the athlete’s age, and the operator’s expertise. With the present review, we aimed to illustrate the prevalence, electrocardiographic (ECG) features, and imaging correlations of the most common VAs in athletes, focusing on etiology, outcomes, and sports eligibility after catheter ablation.

scholarly journals Cardioverter-Defibrillator Implantation to Treat Cardiac Fibroma-Induced Ventricular Tachycardia in a 70-Year-Old Woman

2014 ◽  
Vol 41 (3) ◽  
pp. 329-331 ◽  
Author(s):  
Fayaz Ahmad Hakim ◽  
Anil Pandit ◽  
Farouk Mookadam ◽  
Sylvia Mamby
Keyword(s):  
Ventricular Tachycardia ◽  
Implantable Cardioverter Defibrillator ◽  
Clinical Symptoms ◽  
Surgical Excision ◽  
Orthotopic Heart Transplantation ◽  
Implantable Cardioverter Defibrillator Therapy ◽  
Cardiac Fibroma ◽  
Outflow Obstruction ◽  
Cardioverter Defibrillator

Benign cardiac fibroma is rarely reported in adults. Its clinical symptoms are related to outflow obstruction or dysrhythmias. We present the case of a 70-year-old woman who had a syncopal episode from ventricular tachycardia caused by cardiac fibroma. Because of unfavorable tumor anatomy, the patient was not a candidate for surgical excision, and she declined orthotopic heart transplantation. To prevent sudden cardiac death, we placed an implantable cardioverter-defibrillator, and the patient remained well throughout the 2-year follow-up period. To our knowledge, this is the first report of implantable cardioverter-defibrillator therapy to treat an adult patient's unresectable cardiac fibroma.

Coeliac crisis mimicking nephrotic syndrome in a post-partum patient

2019 ◽  
Vol 64 (3) ◽  
pp. 116-118
Author(s):  
Özant Helvacı ◽  
Seyma Yıldız ◽  
Berfu Korucu ◽  
Ulver Derici ◽  
Turgay Arinsoy
Keyword(s):  
Nephrotic Syndrome ◽  
Coeliac Disease ◽  
Post Partum ◽  
Lower Extremity Weakness ◽  
Case Presentation ◽  
Multisystem Involvement ◽  
Life Threatening ◽  
History Of ◽  
Rare Occasion

Background Coeliac crisis is a life-threatening presentation of coeliac disease. Severe diarrhoea, weight loss, electrolyte imbalances and malnutrition are prominent features. Although mainly a disease of childhood, it can on the rare occasion be diagnosed in adults. Case presentation A 25-year-old female with severe generalised oedema, lower extremity weakness, hypokalemia and profound hypoalbuminemia was referred with an initial diagnosis of nephrotic syndrome. Three months previously she had given birth to a healthy child following an uneventful pregnancy. She did not have proteinuria. She had a history of diarrhoea with gluten-containing food since childhood but lacked a formal diagnosis of coeliac disease. A duodenal biopsy confirmed the suspected diagnosis. Coeliac crisis was diagnosed with life-threatening multisystem involvement. Introduction of a gluten-free diet abolished all disease symptoms and ameliorated laboratory parameters at six months’ follow-up. Conclusion Coeliac crisis is a rare, yet dangerous presentation of coeliac disease in adults. As this case suggests, it can present with generalised oedema and hypoalbuminemia mimicking nephrotic syndrome. Rapid diagnosis is the key to successful treatment.

scholarly journals Ventricular tachycardia storm management in a COVID-19 patient: a case report

2020 ◽  
Vol 4 (FI1) ◽  
pp. 1-6 ◽  
Author(s):  
Gianfranco Mitacchione ◽  
Marco Schiavone ◽  
Alessio Gasperetti ◽  
Giovanni B Forleo
Keyword(s):  
Ventricular Tachycardia ◽  
Ventricular Arrhythmias ◽  
Cardiac Involvement ◽  
Previous History ◽  
Left Ventricular ◽  
Electrical Storm ◽  
Increased Risk ◽  
History Of ◽  
Myocardial Involvement ◽  
Artery Disease

Abstract Background Coronavirus disease 2019 (COVID-19) has been associated with myocardial involvement. Among cardiovascular manifestations, cardiac arrhythmias seem to be fairly common, although no specifics are reported in the literature. An increased risk of malignant ventricular arrhythmias and electrical storm (ES) has to be considered. Case summary We describe a 68-year-old patient with a previous history of coronary artery disease and severe left ventricular systolic disfunction, who presented to our emergency department describing cough, dizziness, fever, and shortness of breath. She was diagnosed with COVID-19 pneumonia, confirmed after three nasopharyngeal swabs. Ventricular tachycardia (VT) storm with multiple implantable cardioverter defibrillator (ICD) shocks was the presenting manifestation of cardiac involvement during the COVID-19 clinical course. A substrate-based VT catheter ablation procedure was successfully accomplished using a remote navigation system. The patient recovered from COVID-19 and did not experience further ICD interventions. Discussion To date, COVID-19 pneumonia associated with a VT storm as the main manifestation of cardiac involvement has never been reported. This case highlights the role of COVID-19 in precipitating ventricular arrhythmias in patients with ischaemic cardiomyopathy who were previously stable.

Sign in / Sign up

Export Citation Format

Share Document