Unusual cause of syncope in young girl: arrhythmogenic right ventricular cardiomyopathy

Mutschelknauss, Marcus; Jörg, Lucas; Schmid, Florian; Rickli, Hans; Ammann, Peter

doi:10.1007/s00392-009-0110-5

Clin Res Cardiol

2010

DOI: 10.1007/s00392-009-0110-5

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Unusual cause of syncope in young girl: arrhythmogenic right ventricular cardiomyopathy

Marcus Mutschelknauss

Lucas Jörg

Florian Schmid

et al.

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Cited by 3 publications

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“…Frequently, it is not possible to achieve an obvious distinction between neurologic and cardiovascular causes, and the underlying mechanism for syncope often is not established. In cases of recurrent unexplained syncope, despite intensive testing, it may be reasonable to consider even rare differential diagnosis [1][2][3]. We report the case of a patient with paraganglioma of glomus caroticum, who had suffered from recurrent syncope as the major symptom.…”

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Glomus caroticum tumor as rare cause of recurrent syncope

Schmid¹,

Raithel²,

Hahn³

et al. 2012

Clin Res Cardiol

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Sirs:Syncope is a common and potentially dangerous symptom with interdisciplinary differential diagnosis. Frequently, it is not possible to achieve an obvious distinction between neurologic and cardiovascular causes, and the underlying mechanism for syncope often is not established. In cases of recurrent unexplained syncope, despite intensive testing, it may be reasonable to consider even rare differential diagnosis [1][2][3]. We report the case of a patient with paraganglioma of glomus caroticum, who had suffered from recurrent syncope as the major symptom.A 39-year-old woman without known cardiovascular risk factors and a normal state of health was referred to our cardiology department to get a second opinion medical examination of her repeated episodes of syncope.Approximately 4 months ago she had suffered from the first of five syncopal spells. A typical episode was characterized by sudden onset of dizziness, followed by a temporary loss of consciousness. The syncopal episode lasted a few seconds to minutes and did not include any seizure-like symptoms. It was not related to any particular movement of the head, to micturition, or to any specific activity. There was no relevant family history of disease. The patient was taking levothyroxine 100 lg, due to a subclinical hypothyroidism which is because of Hashimoto's thyroiditis.Prior to the admission to our department, several investigations already had been carried out. Electrocardiography, echocardiography, Holter monitoring, and electro-encephalography had demonstrated normal findings.Arrhythmias, orthostatic hypotension, or an abnormality of blood chemical levels had not been reported and all tested functions in the neurological examination were within normal ranges.A bilateral Doppler ultrasound showed normal perfusion of the common carotid artery, internal carotid artery, and external carotid artery. Interestingly, an enlarged lateral lymph gland in the region of the left carotid bifurcation was described, but referring to this finding no further diagnostic was carried out.Magnetic resonance imaging of the brain revealed a left-sided temporo-basal arachnoid cyst of 4 cm in diameter with dysplasia of the surrounding temporal lobe. During carotid sinus massage and head-turning, with continuous electrocardiographic and blood pressure monitoring, neither bradycardia nor asystole allegedly could be provoked. A positive result to tilt testing was classified as neurocardiogenic syncope (cardioinhibition without asystole). Due to the positive result to tilt testing and low blood pressure, a sympathomimetic medication with midodrine was recommended.Despite this medication, the symptoms persisted in the following weeks. Therefore, several additional out-patient investigations (orthopedic, psychosomatic) were performed without new consolidated findings.On examination after admission to our department, Doppler ultrasound revealed a 2.3 9 1.9 cm solid mass with a high degree of vascularization in the region of the left carotid artery bifurcation, causing carotid arter...

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mentioning

confidence: 95%

Glomus caroticum tumor as rare cause of recurrent syncope

Schmid¹,

Raithel²,

Hahn³

et al. 2012

Clin Res Cardiol

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Multiple old myocardial scars and new onset of myocarditis in two young patients presenting with ventricular tachycardias and dilated cardiomyopathy

et al. 2010

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Arrhythmogenic right ventricular cardiomyopathy/dysplasia: a review and update

et al. 2011

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Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a predominantly genetically determined and heritable form of cardiomyopathy that is characterized pathologically by the replacement of myocytes by adipose and fibrous tissue and leads to right ventricular failure, arrhythmias, and sudden cardiac death. The estimated prevalence of ARVC/D in the general population ranges from 1 in 2,000 to 1 in 5,000, men are more frequently affected than women, with an approximate ratio of 3:1. ARVC/D can be inherited as an autosomal dominant disease with reduced penetrance and variable expression, autosomal recessive inheritance is also described. There have been 12 genes identified which are linked to ARVC/D, encoding several components of the cardiac desmosome. Dysfunctional desmosomes resulting in defective cell adhesion proteins, such as plakoglobin (JUP), desmoplakin (DSP), plakophilin-2 (PKP-2), and desmoglein-2 (DSG-2) consequently cause loss of electrical coupling between cardiac myocytes, leading to myocyte cell death, fibrofatty replacement and arrhythmias. Diagnosis is based on the finding a combination of characteristic abnormalities in family history, electrocardiography, cardiac imaging as well as endomyocardial biopsy (original task force criteria). Therapeutic options remain limited because of the progressive nature of ARVC/D. Competitive athletics should be avoided. Patients with ARVC/D with a history of having been resuscitated from sudden cardiac death, patients with syncope, very young patients, and those who have marked right ventricular involvement are at the highest risk for arrhythmic death and also, the presence of left ventricular involvement is a risk factor. Several authors concluded that patients who meet the Task Force criteria for ARVC/D are at high risk for sudden cardiac death and should undergo ICD placement for primary and secondary prevention, regardless of electrophysiologic testing results. The role of electrophysiologic study and VT catheter ablation in ARVC/D remains poorly defined, and is frequently used as a palliative measure for patients with refractory VT. The progressive nature of ARVC/D suggests that catheter ablation would not be a long-term curative procedure. Sotalol proved to be highly effective in patients with ARVC/D and inducible as well as non-inducible ventricular tachycardia; if it is ineffective in inducible ventricular tachycardia response to other antiarrhythmic drugs is unlikely and therefore non-pharmacological therapy without further drug testing should be considered. Orthotopic heart transplantation is considered in patients with progressive heart failure and intractable recurrent ventricular arrhythmias.

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Unusual cause of syncope in young girl: arrhythmogenic right ventricular cardiomyopathy

Cited by 3 publications

References 15 publications

Glomus caroticum tumor as rare cause of recurrent syncope

Glomus caroticum tumor as rare cause of recurrent syncope

Multiple old myocardial scars and new onset of myocarditis in two young patients presenting with ventricular tachycardias and dilated cardiomyopathy

Arrhythmogenic right ventricular cardiomyopathy/dysplasia: a review and update

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