Brugada syndrome and syncope: a practical approach for diagnosis and treatment

Mascia, Giuseppe; Bona, Roberta Della; Ameri, Pietro; Canepa, Marco; Porto, Italo; Parati, Gianfranco; Crotti, Lia; Brignole, Michele

doi:10.1093/europace/euaa370

Cited by 34 publications

(30 citation statements)

References 53 publications

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“…In people with Brugada syndrome (BrS) there are multiple theories on the role of the sympathetic nervous system as a potential precipitant of sudden cardiac arrest. These people tend to have reduced cAMP and norepinephrine concentrations found in endomyocardial biopsies, ECG fluctuations with autonomic modulation and a higher incidence of cardiac arrest at night suggesting that cardiac vagal tone may have a significant contribution to arrhythmias in people with BrS ( 10 ). Whereas as individuals with Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) have polymorphic VT from adrenergic stimulation from physical or emotional stressors from an inherited dysfunction in the handling of calcium ions by the sarcoplasmic reticulum in myocardial cells ( 11 ).…”

Section: The Pathophysiology Of Arrhythmias and Sudden Death During Sexmentioning

confidence: 99%

Sex, Rhythm & Death: The effect of sexual activity on cardiac arrhythmias and sudden cardiac death

Dye

Engelstein

Swearingen

et al. 2022

Front. Cardiovasc. Med.

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Arrhythmias and sudden cardiac death with sexual activity are rare. However, the demographics are changing regarding the cardiovascular patients at risk for these events. Recent studies have highlighted that the individuals having cardiac events during sexual activity are becoming younger, with a higher proportion of female decedents than previously described. There needs to be an open dialog between the cardiovascular team and the cardiac patient to provide the education and reassurance necessary for cardiovascular patients to participate in sexual intercourse safely. This paper reviews how sexual activity can lead to an increase in cardiac arrhythmias and sudden cardiac arrest in patients that are not medically optimized or are unaware of their underlying cardiac condition. The most common cardiovascular diseases associated with sexually induced arrhythmias and arrest are discussed regarding their potential risk and the psychosocial impact of this risk on these patients. Finally, cardiovascular medications and implantable cardioverter-defibrillators (ICDs) are addressed by reviewing the literature on the safety profile of these cardiac interventions in this patient population. Overall, sexual activity is safe for most cardiac patients, and providing proper education to the patient and their partner can improve the safety profile for patients with higher risk cardiovascular conditions. To give the appropriate education and reassurance necessary, cardiovascular team members need an understanding of the pathophysiology of how sexual activity can provoke arrhythmias and sudden cardiac arrest. Healthcare providers also need to build comfort in speaking to all patients and ensure that sexual partners, female patients, and those in the LGBTQIA + community receive the same access to counseling but tailored to their individual needs.

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Section: The Pathophysiology Of Arrhythmias and Sudden Death During Sexmentioning

confidence: 99%

Sex, Rhythm & Death: The effect of sexual activity on cardiac arrhythmias and sudden cardiac death

Dye

Engelstein

Swearingen

et al. 2022

Front. Cardiovasc. Med.

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show abstract

“…Moreover, underlying cardiac conductivity defects, like prolonged QTc and Brugada syndrome, can also lead to cardiac arrhythmias [116][117][118][119][120] and may have the potential to cause detrimental cardiac outcome with aerobic activities. It is also important to note that there may be sex differences in arrhythmogenic potentials secondary to underlying conditions, such as catecholaminergic polymorphic ventricular tachycardia (CPVT) associated with cardiac ryanodine receptor 2 (RyR2) mutations and other cardiac pathologies [116][117][118][119][120][121][122][123][124][125].…”

Section: Detrimental Impacts Of Exercise On Cardiac Electrical Conductivitymentioning

confidence: 99%

Sex-Specific Impacts of Exercise on Cardiovascular Remodeling

Islam

Khalsa

Vyas

et al. 2021

JCM

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Cardiovascular diseases (CVD) remain the leading cause of death in men and women. Biological sex plays a major role in cardiovascular physiology and pathological cardiovascular remodeling. Traditionally, pathological remodeling of cardiovascular system refers to the molecular, cellular, and morphological changes that result from insults, such as myocardial infarction or hypertension. Regular exercise training is known to induce physiological cardiovascular remodeling and beneficial functional adaptation of the cardiovascular apparatus. However, impact of exercise-induced cardiovascular remodeling and functional adaptation varies between males and females. This review aims to compare and contrast sex-specific manifestations of exercise-induced cardiovascular remodeling and functional adaptation. Specifically, we review (1) sex disparities in cardiovascular function, (2) influence of biological sex on exercise-induced cardiovascular remodeling and functional adaptation, and (3) sex-specific impacts of various types, intensities, and durations of exercise training on cardiovascular apparatus. The review highlights both animal and human studies in order to give an all-encompassing view of the exercise-induced sex differences in cardiovascular system and addresses the gaps in knowledge in the field.

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“…Symptoms of BrS often occur during adulthood, at mean age of 41 years but patients may also suffer from symptoms in childhood. Of note, syncope in patients could be benign, related to neurally-mediated syncope, and distinguishing this syncope from the real, malignant syncope can be complicated [ 3 , 4 ]. Therefore, a critical diagnostic and treatment approach is required in these cases.…”

Section: Introductionmentioning

confidence: 99%

A Preclinical Study on Brugada Syndrome with a CACNB2 Variant Using Human Cardiomyocytes from Induced Pluripotent Stem Cells

Zhong

Schimanski

Zhang

et al. 2022

IJMS

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Aims: Some gene variants in the sodium channels, as well as calcium channels, have been associated with Brugada syndrome (BrS). However, the investigation of the human cellular phenotype and the use of drugs for BrS in presence of variant in the calcium channel subunit is still lacking. Objectives: The objective of this study was to establish a cellular model of BrS in the presence of a CACNB2 variant of uncertain significance (c.425C > T/p.S142F) using human-induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs) and test drug effects using this model. Methods and results: This study recruited cells from a patient with Brugada syndrome (BrS) and recurrent ventricular fibrillation carrying a missense variant in CACNB2 as well as from three healthy independent persons. These cells (hiPSC-CMs) generated from skin biopsies of healthy persons and the BrS patient (BrS-hiPSC-CMs) as well as CRISPR/Cas9 corrected cells (isogenic control, site-variant corrected) were used for this study. The hiPSC-CMs from the BrS patient showed a significantly reduced L-type calcium channel current (ICa-L) compared with the healthy control hiPSC-CMs. The inactivation curve was shifted to a more positive potential and the recovery from inactivation was accelerated. The protein expression of CACNB2 of the hiPSC-CMs from the BrS-patient was significantly decreased compared with healthy hiPSC-CMs. Moreover, the correction of the CACNB2 site-variant rescued the changes seen in the hiPSC-CMs of the BrS patient to the normal state. These data indicate that the CACNB2 gene variant led to loss-of-function of L-type calcium channels in hiPSC-CMs from the BrS patient. Strikingly, arrhythmia events were more frequently detected in BrS-hiPSC-CMs. Bisoprolol (beta-blockers) at low concentration and quinidine decreased arrhythmic events. Conclusions: The CACNB2 variant (c.425C > T/p.S142F) causes a loss-of-function of L-type calcium channels and is pathogenic for this type of BrS. Bisoprolol and quinidine may be effective for treating BrS with this variant.

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Brugada syndrome and syncope: a practical approach for diagnosis and treatment

Cited by 34 publications

References 53 publications

Sex, Rhythm & Death: The effect of sexual activity on cardiac arrhythmias and sudden cardiac death

Sex, Rhythm & Death: The effect of sexual activity on cardiac arrhythmias and sudden cardiac death

Sex-Specific Impacts of Exercise on Cardiovascular Remodeling

A Preclinical Study on Brugada Syndrome with a CACNB2 Variant Using Human Cardiomyocytes from Induced Pluripotent Stem Cells

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