scholarly journals Aborted Sudden Cardiac Death and a Mother with Suspected Metabolic Myopathy

2014 ◽  
Vol 8 ◽  
pp. CMC.S16143
Author(s):  
Josef Finsterer ◽  
Claudia Stöllberger ◽  
Hans Keller

Aborted sudden cardiac death (SCD) has not been reported as initial manifestation of cardiac involvement in metabolic myopathy (MM). A 20-year-old female with a previous history of three syncopes, hyperhidrosis, and recurrent tick bites experienced aborted SCD. Her mother presented with MM, and a history of pituitary adenoma, nephroptosis, arterial hypertension, depression, migraine, goiter, pancreatitis, osteoporosis, hyperhidrosis, multiple muscle ruptures, and hyperlipidemia. After a few days of disorientation and amnesia, the young female recovered completely. Clinical neurological examination was noticeable for partial ophthalmoparesis and mild hyperprolactinemia. She received an implantable cardioverter defibrillator, which did not discharge so far. Recurrent syncopes and aborted SCD may be the initial manifestation of MM with multiple organ involvement. The family history is important in cases with aborted SCD to guide the diagnostic work-up. Phenotypic heterogeneity between the family members may be an indicator of MM.

EP Europace ◽  
2021 ◽  
Vol 23 (Supplement_3) ◽  
Author(s):  
R Alves Pinto ◽  
T Proenca ◽  
M Martins Carvalho ◽  
PD Grilo ◽  
CX Resende ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. Background  Sudden cardiac death (SCD) is an uncommon event in the absence of structural heart disease. However, ventricular fibrillation (VF) may occur in patients with unknown cardiac disease and a comprehensive work-up is needed to further improve diagnostic. Still, a significant and heterogenous group of patients remains labelled of Idiopathic VF and limited data is available regarding their natural history. Purpose The aim of this study was to evaluate the clinical outcomes of survivors of an aborted sudden cardiac death due to idiopathic VF or pulseless ventricular tachycardia (VT) and to assess possible predictors of recurrence. Methods  Patients who survived an idiopathic VF or pulseless VT between 2005 and 2019 referred to a cardiac defibrillator (ICD) implantation were included. Patients were followed for 1 to 15 years (median follow-up of 7 years). Clinical and device data were collected. Results A population of 29 patients, 59% male, with a median age of 50 years (age ranging from 18 to 76) at the time of the aborted SCD was studied. All patients implanted an ICD (69% single chamber, 24% dual chamber and 3% subcutaneous) at the index hospitalization. The initial rhythm was VF in 76% and pulseless VT in 24%. In relation to the context of the arrhythmic event, 48.3% occurred during daily life activities, 13.8% after an emotional stress, 6.9% during efforts and a similar percentage occurred either in rest or asleep. Of note, 12.5% of patients had previous history of syncope. Normal ECG was present in 83% of patients. Family history of SCD was present in 12% of the cases. As for the cardiovascular risk factors, 61.5% had hypertension, 19% dyslipidemia, 17% diabetes, 31% were smokers or previous smokers. Paroxysmal atrial fibrillation was present in 15% of patients. To exclude possible causes of VF, all patients were submitted to coronary angiogram and echocardiogram, 64% to genetic testing, 68% to cardiac magnetic resonance, 20% to electrophysiologic study, 12% to pharmacological provocative test and 4% were submitted to endomyocardial biopsy. At follow-up, an etiological diagnosis was established in 31% of patients: 3 events were attributed to coronary vasospasm, 3 to short coupled polymorphic VT, 1 patient had long QT syndrome, 1 had Brugada syndrome and in 1 patient an ANK2 mutation was identified. As for the clinical outcomes, 8% patients died (from non-arrhythmic causes), 31% patients received appropriate therapies and 19% had unappropriated shocks (of those 60% for sinus tachycardia and 40% for supraventricular tachycardia). Conclusion Etiologic diagnosis and prediction of recurrence of arrhythmic events in patients with idiopathic VF is challenging, even with a long-term follow-up and more sophisticated diagnostic evaluation.  Idiopathic VF is a rare but serious condition with recurrence in about one third of patients. Although not free of complications, ICD remains the gold standard of treatment.


2007 ◽  
Vol 262 (3) ◽  
pp. 360-367 ◽  
Author(s):  
P. Wennberg ◽  
M. Eliasson ◽  
G. Hallmans ◽  
L. Johansson ◽  
K. Boman ◽  
...  

2017 ◽  
Vol 45 ◽  
pp. 25-29 ◽  
Author(s):  
Alban-Elouen Baruteau ◽  
Elijah R. Behr

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Kari S Kaikkonen ◽  
Marja-Leena Kortelainen ◽  
Heikki V Huikuri

Introduction. There is little information on the specific risk factors leading to sudden cardiac death (SCD) during an acute coronary event, because the risk variables may overlap with those of non-fatal coronary event. This study was designed to compare the risk profiles of SCD victims and survivors of an acute coronary event. Methods and Results. A case-control study included consecutive victims of SCD (n=425, mean age 64±11 years) verified to be due to an acute coronary event at medicolegal autopsy and consecutive patients surviving an acute myocardial infarction (AMI, n=644, mean age 62±10 years). Common cardiovascular risk factors, cardiac hypertrophy, and severity of coronary artery disease (CAD) were assessed in both groups. Family history of SCD (odds ratio 1.5, 95% CI 1.0 to 2.2, p=0.03), male gender (odds ratio 1.8, 95% CI 1.3 to 2.4, p<0.001), current smoking (odds ratio 2.0, 95% CI 1.5 to 2.6, p<0.001), cardiac hypertrophy (odds ratio 3.0, 95% CI 2.3 to 3.9, p<0.001) and 3-vessel CAD (odds ratio 5.4, 95% CI 3.6 to 8.2, p<0.001) were more common among the victims of SCD as compared to survivors of AMI. On the contrary, history of hypercholesterolemia (p<0.001) was less common among the SCD victims. There was a cumulative increase of risk of being a SCD victim vs. AMI survivor when more than one risk factor was present, the odds ratio being 44.3 (95% CI 8.0 to 246.7) in a current male smoker with a family history of SCD and cardiac hypertrophy. When 3-vessel CAD was added to the combined risk score, all subjects (7% of the SCD victims) were in the group of SCD giving a 100% sensitivity and specificity, respectively, in differentiating between the SCD victims and AMI survivors. Conclusions. There are specific features that differentiate the victims of SCD from survivors of an acute coronary event. Clustering of several variables, such as family history of SCD, smoking, cardiac hypertrophy, and 3-vessel CAD indicate a very high risk of SCD.


Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Anne G Rosenfeld ◽  
Mohamud Daya ◽  
Vivian Christensen ◽  
Rebecca Rawson

Sudden cardiac death (SCD) is accompanied by preceding symptoms in a significant proportion of victims, with a median duration of up to 2 hours in some cases. The purpose of this study was to describe the characteristics of SCD victims with heralding symptoms who refused medical care. We conducted a secondary data analysis of interview data from witnesses of 99 cases of out-of-hospital presumed myocardial infarction death with known symptoms. Qualitative description methods were used to analyze qualitative data. Logistic regression was used to test the influence of type of symptoms (chest pain vs. non-chest pain), history of heart disease, and age on refusal of medical care. Categorization as refusal of medical care required conversation with someone where refusal was expressed verbally by the victim. There were 19 cases (19%) that refused medical care; their mean age was 72. The majority were male (16/19, 84%). Fifteen cases involved persistent refusal, defined as refusing care until collapse (range of <15 minutes to 60 hours). Four victims initially refused care and then permitted access to medical care. The suggestion for seeking medical care came from someone else in all but one case, and usually included multiple attempts. The care options offered but refused included calling 911 or a doctor, as well as going to the hospital, emergency department or a doctor’s office. Reasons for refusal of medical care (more than one reason in some cases) included stating the symptoms were due to something not urgent (n=10), other obligations (n=3), expressed dislike of hospitals or doctors (n=4), or recent medical reassurance of health status (n=7). Controlling for age, victims with chest pain vs. non-chest pain symptoms were more likely (OR = 3.56, p = .036, 95% CI = 1.09 –11.66) to refuse medical care and those with a history of heart disease were less likely (OR = .16, p = .004, 95% CI = .05–.55) to refuse medical care. Patients with chest pain and no history of heart disease are more likely to refuse advice to seek medical care. Public health messages about how to respond to cardiac symptoms should include strategies to overcome the reasons people refuse medical care. This research has received full or partial funding support from the American Heart Association, AHA Pacific/Mountain Affiliate (Alaska, Arizona, Colorado, Hawaii, Idaho, Montana, Oregon, Washington & Wyoming).


EP Europace ◽  
2020 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
R Zhang ◽  
I T Fazmin ◽  
A Porto ◽  
K Divulwewa ◽  
A Reddy ◽  
...  

Abstract Introduction Little is known regarding the aetiology or outcome of atrial fibrillation (AF) occurring in young adults. This retrospective analysis was performed to explore the demographics and efficacy of AF ablation in this population. Methods Patients were included who had undergone ≥1 AF ablation under the age of 40 between 2006-2018. Recurrence was defined as return of either documented AF or previous symptoms for &gt;30s following a 3-month blanking period. Initial exploratory co-variates were included in a univariate analysis and those terms with P-value of &lt;0.1 were then used to generate a Cox proportional-hazards multivariate model. Results 124 patients (33.6 ± 4.7 yrs, 77% men), initially presenting with paroxysmal AF (pAF; n = 97) or persistent AF (n = 27), underwent 175 AF ablation procedures. 22.6% (n = 28) also had atrial flutter. Time from symptom onset to first ablation was 50.7 ± 46.2 months. Relevant cardiovascular-related demographics were analysed: hypertension in 8.9% (n = 11); diabetes in 1.6% (n = 2); positive family history of AF in 12.9% (n = 16); and family history of sudden cardiac death in 2.4% (n = 3). Mean CHA2DS2-VASc score was 0.35. Of those patients with documented echocardiogram imaging (n = 91), 26.4% (n = 24) had LA dilatation and 6.6% (n = 6) had LV dysfunction. Patients with LA dilatation underwent more ablations (2.3 ± 0.3) compared to controls (1.5 ± 0.1; p &lt; 0.001). Ablation strategy was pulmonary vein isolation (PVI) only in 67.2% (n = 119), with additional ablation in the remaining: roof line in 18.9% (n = 33); cavotricuspid isthmus line in 13.1% (n = 23); mitral isthmus line in 2.3% (n = 4); superior vena cava isolation in 2.3% (n = 4); complex fractionated atrial electrograms in 14.9% (n = 26). Mean procedure time was 155 ± 41 min, mean ablation time was 1657 ± 991 s and mean fluoroscopy time was 32.6 ± 23.4 min. General anaesthesia was used in 43.4% (n = 76). Complications included femoral haematoma (n = 2), tamponade (n = 1) and pulmonary vein stenosis (n = 2). 90 days of follow-up was available for 137 procedures performed for pAF (n = 105) and persistent AF (n = 32). For pAF, overall recurrence was 61.9% for first ablations and 62.9% overall. Recurrence was 56.3% for persistent AF. Factors significantly associated with increased AF recurrence in univariate analysis were male gender (hazard ratio (HR) 2.3, 95% confidence interval (CI): 1.2-4.4, p = 0.011), hypertension (HR 0.5, CI: 0.2-1.1, p = 0.067), family history of sudden cardiac death (HR 6.8, CI: 1.6-29.0 , p = 0.010) and enlarged LA size (HR 2.2, CI: 1.3-3.6, p = 0.003). In multivariate analysis, the only significant predictor of poor outcome was enlarged LA size (HR 2.0, 95% CI: 1.2-3.5, p = 0.011). Conclusions Young patients with AF may have structurally abnormal hearts, and therefore do not only present with lone AF. LA size may be used as a predictor for success. Surveillance imaging may be useful to detect future structural change, which will be the subject of future prospective studies. Abstract Figure. AF ablation recurrence in young adults


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
C Cotrim ◽  
F Costa ◽  
D Severino ◽  
L Baquero ◽  
J Guardado

Abstract Background Some publications, on exercise induced intraventricular gradients, admit the possibility they can be related to some cases of unexplained sudden cardiac death (SCD). Clinical case We present the case of a young male athlete (16 years) that after winning a triathlon competition has sudden cardiac death. No cardiovascular risk factors. No family history of SCD A previous episode of dizziness, accompanied by nausea and vomiting related to intense training happens 6 months before. In September 2018 about 30 minutes after winning a triathlon competition has SCD episode having been resuscitated on site by the competition physician having been defibrillated and transported to intensive care unit. After discharge, cardiac MRI, Coronary AngioTC, complete genetic study for heart diseases, flecainid test, transthoracic echocardiogram, stress echocardiogram with hyperventilation and ergometrine. All have normal results (Figure) During 24 hours Holter ECG isolated premature ventricular complexes were detected and during exercise stress echocardiography a significant intraventricular gradient without systolic anterior movement of mitral valve was detected (Figure). The athlete was disqualified for sports practice, refuses CDI implantation and started bisoprolol 2,5 mg daily. To the best of our knowledge this is the first case of association between SCD and exercise induced intraventricular gradient. This possible association should be studied in the future. Abstract P859 Figure. Intraventricular gradient in SCD athlete


2019 ◽  
Vol 5 (3) ◽  
pp. 169-172 ◽  
Author(s):  
Johnni Rudbeck-Resdal ◽  
Jens Cosedis Nielsen ◽  
Henning Bundgaard ◽  
Henrik K. Jensen

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