Arrhythmias and myocardial fragility in Ehlers-Danlos syndrome: Complications after routine ICD placement

Garg, Vinisha; Bersohn, Malcolm; Han, Janet K.

doi:10.1016/j.hrcr.2018.03.008

Cited by 3 publications

(4 citation statements)

References 4 publications

(4 reference statements)

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“…Although the presence of dysautonomia or valvular disease in EDS is well proven, there is no clear evidence (basically based on a few case reports) in the literature between the occurrence of arrhythmias and its prevalence in EDS ( 13 – 15 ). As early as 1977, one of the first case reports of a patient with EDS and accompanying cardiovascular disorders, including frequent premature ventricular contractions, was published ( 16 ).…”

Section: Discussionmentioning

confidence: 99%

“…Case reports of metoprolol treatment of paroxysmal atrial fibrillation are also available ( 20 ). Chronic amiodarone therapy as an alternative to invasive arrhythmia treatment with RF ablation—due to the high risk of further perforations of cardiac structures—could be also used, as in the general population, with good therapeutic effect ( 13 ). To the best of our knowledge, the data which considers the usefulness of class IC of antiarrhythmic drugs in EDS is unavailable.…”

Section: Discussionmentioning

confidence: 99%

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Therapeutic difficulties in a patient with Ehlers-Danlos syndrome and numerous symptomatic premature ventricular contractions—case report and literature review

Sławiński

Wabich

Hawryszko

et al. 2023

Front. Cardiovasc. Med.

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A 28-year-old female patient diagnosed with Ehlers-Danlos syndrome type III (hypermobile EDS, hEDS) was admitted to the cardiology clinic due to a 3-year history of symptomatic ventricular arrhythmia in the form of multiple premature ventricular contractions (PVCs). Attempts at antiarrhythmic treatment with beta-blockers, propafenone, and verapamil were unsuccessful. Due to the diagnosis of hEDS and the high risk of vascular complications related to the ablation procedure, invasive treatment was abandoned, and it was decided to implement flecainide. After the flecainide treatment initiation, a spectacular improvement in the number of ventricular arrhythmias was observed, along with the disappearance of the complaints previously reported by the patient. To the best of our knowledge, this is the first described case of spectacular flecainide antiarrhythmic effect in a patient with numerous PVCs also diagnosed with EDS. Flecainide treatment in the EDS group could be a successful alternative to ablation, which can lead to serious vascular and even life-threatening complications, especially after the failure of propafenone and beta-blockers treatment.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Therapeutic difficulties in a patient with Ehlers-Danlos syndrome and numerous symptomatic premature ventricular contractions—case report and literature review

Sławiński

Wabich

Hawryszko

et al. 2023

Front. Cardiovasc. Med.

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show abstract

Section: Discussionmentioning

confidence: 99%

“…Other than case reports, there is minimal literature available on the safety and feasibility of cardiac (or indeed any) interventional procedures in EDS. The most relevant cardiac case report was of a 59-year-old EDS patient (of unknown subtype) who presented with syncope and recurrent VT. 6 A coronary angiogram was complicated by a femoral hematoma. An ICD was planned—the team avoided a subcutaneous device on the grounds of skin laxity and likelihood of device migration; however, the implanted transvenous ICD was complicated by delayed right ventricular apical lead perforation and pericardial effusion that required sternotomy (itself complicated by pneumothorax and subcutaneous emphysema) to explant the system.…”

Section: Discussionmentioning

confidence: 99%

Ventricular tachycardia ablation in a patient with Ehlers-Danlos syndrome

Calvert

Chu

Rao

et al. 2022

HeartRhythm Case Reports

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This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

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Prevalence of gastrointestinal, cardiovascular, autonomic and allergic manifestations in hospitalized patients with Ehlers-Danlos syndrome: a case-control study

2021

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Objective Previous observations suggest an association between Ehlers-Danlos syndrome (EDS) and gastrointestinal (GI), cardiovascular, immune, and autonomic nervous system dysfunction. We sought to determine whether a hospital diagnosis of EDS is associated with a higher prevalence of these manifestations vs hospitalized patients without EDS. We also evaluated hospital outcomes. Methods A total of 6,021 cases and matched controls were acquired from the 2016 National Inpatient Sample. In total, 2,007 EDS patients were identified via ICD-10 code. After bivariate analyses, multivariate logistic regression models were used to adjust for potential confounders. Results GI conditions were found in 44% of EDS patients vs 18% of controls [odds ratio (OR) = 3.57, 95% CI: 3.17, 4.02, P < 0.0001], with irritable bowel syndrome, gastroparesis and coeliac disease strongly associated with EDS. Autonomic dysfunction, including postural orthostatic tachycardia syndrome (POTS), neurocardiogenic syncope and orthostatic hypotension was found in 20% of EDS patients vs 6% of controls (OR = 4.45, 95% CI: 3.71, 5.32, P < 0.0001). EDS patients were more likely to have food allergy (OR = 3.88, 95% CI: 2.65, 5.66, P < 0.0001) and cardiovascular complications such as mitral valve disorders, aortic aneurysm and dysrhythmias (OR = 6.16, 95% CI: 4.60, 8.23, P < 0.0001). These conditions remained highly associated with EDS after considering confounders. EDS patients were 76% more likely to have longer than average hospitalizations (OR = 1.76, 95% CI: 1.54, 2.02, P < 0.0001). Conclusion GI, cardiovascular, autonomic and allergic manifestations are significantly more prevalent in EDS patients compared with hospitalized patients without EDS. Physicians should consider EDS in patients with unexplained GI, cardiovascular, autonomic and allergic conditions and exercise precautions when treating EDS patients in a hospital setting.

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Arrhythmias and myocardial fragility in Ehlers-Danlos syndrome: Complications after routine ICD placement

Cited by 3 publications

References 4 publications

Therapeutic difficulties in a patient with Ehlers-Danlos syndrome and numerous symptomatic premature ventricular contractions—case report and literature review

Therapeutic difficulties in a patient with Ehlers-Danlos syndrome and numerous symptomatic premature ventricular contractions—case report and literature review

Ventricular tachycardia ablation in a patient with Ehlers-Danlos syndrome

Prevalence of gastrointestinal, cardiovascular, autonomic and allergic manifestations in hospitalized patients with Ehlers-Danlos syndrome: a case-control study

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