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Because of its powerful genetics, the adult zebrafish has been increasingly used for studying cardiovascular diseases. Considering its heart rate of ~100 beats per minute at ambient temperature, which is very close to human, we assessed the use of this vertebrate animal for modeling heart rhythm disorders such as sinus arrest (SA) and sick sinus syndrome (SSS). We firstly optimized a protocol to measure electrocardiogram in adult zebrafish. We determined the location of the probes, implemented an open-chest microsurgery procedure, measured the effects of temperature, and determined appropriate anesthesia dose and time. We then proposed an PP interval of more than 1.5 seconds as an arbitrary criterion to define an SA episode in an adult fish at ambient temperature, based on comparison between the current definition of an SA episode in humans and our studies of candidate SA episodes in aged wild-type fish and Tg(SCN5A-D1275N) fish (a fish model for inherited SSS). With this criterion, a subpopulation of about 5% wild-type fish can be considered to have SA episodes, and this percentage significantly increases to about 25% in 3-year-old fish. In response to atropine, this subpopulation has both common SSS phenotypic traits that are shared with the Tg(SCN5A-D1275N) model, such as bradycardia; and unique SSS phenotypic traits, such as increased QRS/P ratio and chronotropic incompetence. In summary, this study defined baseline SA and SSS in adult zebrafish and underscored use of the zebrafish as an alternative model to study aging-associated SSS., Competing Interests: The authors have declared that no competing interests exist.

We present a case of sinus arrest and junctional escape rhythm from sinus node artery (SNA) thrombus in a 55-year-old man after revascularisation of right coronary and proximal circumflex arteries for infero-posterior wall ST-segement elevation myocardial infarction (STEMI). Sinus arrest from occlusion of the SNA is uncommon. The ensuing bradycardia may have haemodynamic consequences requiring temporary pacing but is almost always self-limited., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Congenital Central Hypoventilation Syndrome (CCHS) is a rare neurocristopathy characterized by severe hypoventilation and autonomic dysregulation, with typical presentation in the neonatal period, and deficient cognitive skills in school-aged patients. We hypothesized that younger (preschool) children with CCHS would also show neurocognitive delay and that CCHS-related physiologic factors would impact neurocognitive test results., Methods: We studied developmental (Bayley) test results collected during routine clinical care in 31 children (mean age 25.0 ± 8.5 months; range, 6-40 months) with PHOX2B mutation-confirmed CCHS by comparing them with the normative reference mean from the Bayley standardization sample; we also examined associations between Bayley scores and CCHS disease-related factors., Results: Preschool patients with CCHS fell significantly below the normative mean of 100 on Bayley indices of mental (mean, 83.35 ± 24.75) and motor (mean, 73.33 ± 20.48) development (P < .001 for both). Significantly lower Bayley mental and motor scores were associated with severe breath-holding spells, prolonged sinus pauses, and need for 24 h/d artificial ventilation. Lower Bayley motor scores were also associated with seizures. Bayley scores differed among children with the three most common polyalanine repeat expansion mutation genotypes (mental, P = .001; motor, P = .006), being essentially normal in children with the 20/25 genotype but significantly lower in the other genotype groups (P < .05)., Conclusions: These results confirm neurodevelopmental impairment of CCHS preschoolers, with severity related to physiologic compromise and PHOX2B genotype. These findings suggest that adverse effects begin early in the disease process, supporting the need for neurodevelopmental monitoring and intervention from early infancy., (Copyright © 2016 American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.)

Purpose: Previous studies have shown that the underlying pathophysiologic mechanism in children with breath holding may be generalised autonomic dysregulation. Thus, we performed cardiac rhythm and heart rate variability analyses using 24-hour Holter monitoring to evaluate the cardiac effects of autonomic dysregulation in children with breath-holding spells., Methods: We performed cardiac rhythm and heart rate analyses using 24-hour Holter monitors to evaluate the cardiac effects of autonomic dysregulation in children during a breath-holding spell. Our study group consisted of 68 children with breath-holding spells - 56 cyanotic type and 12 pallid type - and 39 healthy controls., Results: Clinical and heart rate variability results were compared between each spell type - cyanotic or pallid - and the control group; significant differences (p<0.05) in standard deviation of all NN intervals, mean of the standard deviations of all NN intervals for all 5-minute segments, percentage of differences between adjacent RR intervals >50 ms, and square root of the mean of the sum of squares of the differences between adjacent NN intervals values were found between the pallid and cyanotic groups., Conclusions: Holter monitoring for 24 hours and heart rate variability parameters, particularly in children with pallid spells, are crucial for evaluation of cardiac rhythm changes.

A 67-year-old man who had a history of syncope was admitted because of effort angina. The sinus node (SN) was the single blood supply from the right coronary artery (RCA). After we implanted 2 everolimus-eluting stents for RCA, slow-flow occurred and the SN artery was occluded, and junctional escape rhythm was sustained. After the wiring to the occluded SN artery, junctional escape rhythm immediately recovered to sinus rhythm, and the patient achieved continuous sinus rhythm and stable hemodynamics. Given that acute SN ischemia is a possible cause of sinus dysfunction, careful choice of a percutaneous coronary intervention strategy should be taken into consideration if the SN artery is the single blood supply from the RCA and if syncopal history is present.

We describe asymptomatic sinus arrest and post-hiccup cough syncope in a patient with medullary infarction. A 78-year-old woman developed arrhythmia, hiccup, and cough syncope attacks. Neurological examination was not remarkable. Cough syncope occurs after hiccup attacks. Bradycardia and decreased blood pressure were also present after the beginning cough. Holter 24-hour electrocardiography monitor exhibited 65 episodes of asymptomatic sinus arrest more than 3 seconds. Magnetic resonance imaging disclosed acute infarction in the bilateral medial regions and the right tegmentum of the upper and middle medulla oblongata. Cerebral angiography showed severe atherosclerotic changes in the vertebral arteries. These clinicoradiological findings suggested that a distinct topography of medullary lesions could cause a series of cardiovascular and respiratory dysfunction. Thus, physicians should pay more attention to the medullary lesion in patients with arrhythmia and syncope., (Copyright © 2014 National Stroke Association. Published by Elsevier Inc. All rights reserved.)

Objectives: Concomitant surgical atrial fibrillation (AF) ablation is a safe and feasible procedure, recommended in guidelines. Pacemaker dependency is a known complication of AF ablation. We sought to determine independent predictors for pacemaker implantation after surgical AF ablation., Methods: Between January 2003 and November 2012, 594 patients underwent concomitant surgical AF ablation. Various energy sources, including cryoablation (n = 139), unipolar radiofrequency (n = 278), and bipolar radiofrequency (n = 177), were used. Left atrial (n = 463, 77.9%) and biatrial (n = 131, 22.1%) ablation was performed. Univariate and multivariate logistic regression analysis was used to identify independent predictors for pacemaker implantation within 30 days after surgical AF ablation., Results: The mean patient's age was 68.6 ± 9.4 years, and 66.8% were male. No major ablation-related complications occurred. A total of 41 (6.9%) of patients received pacemaker implantation during the 30-day follow-up period. Indications for pacemaker implantation were atrioventricular block in 25 (60.9%) of patients, sinus bradycardia or sinus arrest in 9 (22.0%) of patients, and bradyarrhythmia in 7 (17.1%) of patients. Demographic data, type of surgical procedure, and type of energy source did not have a significant impact on pacemaker implantation rate. However, biatrial ablation led to a significant pacemaker implantation rate compared with isolated left-sided ablation (6.3% vs 13.6%; P = .028)., Conclusions: Concomitant surgical AF ablation showed a pacemaker implantation rate of 6.9% after 30-day follow-up. Univariate and multivariate analysis showed biatrial lesion set as the only statistically significant predictor for pacemaker implantation after surgical AF ablation., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Aims: Although patients with paroxysmal atrial fibrillation (AF) and prolonged sinus pauses [tachycardia-bradycardia syndrome (TBS)] are generally treated by permanent pacemaker, catheter ablation has been reported to be a curative therapy for TBS without pacemaker implantation. The purpose of this study was to define the potential role of successful ablation in patients with TBS., Methods and Results: Of 280 paroxysmal AF patients undergoing ablation, 37 TBS patients with both AF and symptomatic sinus pauses (age: 62 ± 8 years; mean maximum pauses: 6 ± 2 s) were analysed. During the 5.8 ± 1.2 years (range: 5-8.7 years) follow-up, both tachyarrhythmia and bradycardia were eliminated by a single procedure in 19 of 37 (51%) patients. Repeat procedures were performed in 14 of 18 patients with tachyarrhythmia recurrence (second: 12 and third: 2 patients). During the repeat procedure, 79% (45 of 57) of previously isolated pulmonary veins (PVs) were reconnected to the left atrium. Pulmonary vein tachycardia initiating the AF was found in 46% (17 of 37) and 43% (6 of 14) of patients during the initial and second procedure, respectively. Finally, 32 (86%) patients remained free from AF after the last procedure. Three patients (8%) required pacemaker implantation, one for the gradual progression of sinus dysfunction during a period of 6.5 years and the others for recurrence of TBS 3.5 and 5.5 years after ablation, respectively., Conclusion: Catheter ablation can eliminate both AF and prolonged sinus pauses in the majority of TBS patients. Nevertheless, such patients should be continuously followed-up, because gradual progression of sinus node dysfunction can occur after a long period of time.

A 52-year-old man presented with vomiting, dysphagia, left-sided ataxia and dissociated sensory loss. Diffusion-weighted MRI showed evidence of acute infarct in the left lateral medulla and left medial cerebellar hemisphere, probably secondary to thromboembolism from left vertebral artery dissection. While making an uneventful recovery as an inpatient, a routine 24 h ECG was performed 2 weeks after the stroke to investigate possible paroxysmal atrial fibrillation. The recording instead revealed 56 asymptomatic episodes of sinus arrest, necessitating implantation of a permanent pacemaker to prevent sudden cardiac death. The medulla contains key structures involved in autonomic regulation, including the dorsal vagal nucleus and the nucleus tractus solitarius. Acute infarction may disrupt cardiac autonomic regulation pathways, resulting in altered parasympathetic and sympathetic outflow to the sinoatrial and atrioventricular nodes, with potentially life-threatening effects.

Cardiac arrhythmias during sleep are relatively common and include a diverse etiology, from benign sinus bradycardia to potentially fatal ventricular arrhythmias. Predisposing factors include obstructive sleep apnea and cardiac disease. Rapid eye movement (REM)-related bradyarrhythmia syndrome (including sinus arrest and complete atrioventricular block with ventricular asystole) in the absence of an underlying cardiac or physiologic sleep disorder was first described in the early 1980s. Although uncertain, the underlying pathophysiology likely reflects abnormal autonomic neural-cardiac inputs during REM sleep. The autonomic nervous system (ANS) is a known key modulator of heart rate fluctuations and rhythm during sleep and nocturnal heart rate reflects a balance between the sympathetic-parasympathetic systems. Whether the primary trigger for REM-related bradyarrhythmias reflects abnormal centrally mediated control of the ANS during REM sleep or anomalous baroreflex parasympathetic influences is unknown. This review focuses on the salient features of the REM-related bradyarrhythmia syndrome and explores potential mechanisms with a particular assessment of the relationship between the ANS and nocturnal heart rate fluctuations., (Published by Elsevier Ltd.)

A 5-month-old female infant presented with new-onset acute lymphoblastic leukemia and hyperleukocytosis with white blood cell count of 352 × 10(9) cells/L. She developed sinus pauses and hypoxemic respiratory failure. A manual single volume exchange blood transfusion was done with complete resolution of sinus pauses and hypoxemia.

Emotional reactions involve changes in both cognitive and bodily processes. Therefore, effective emotion regulation may also involve modulation of responses in both of these systems. The present study investigated the relationship between regulation of cognition and regulation of the heart in children and adolescents, using a go/nogo task in combination with the induction of negative emotions. Behavioral, temperamental and event-related brain potential (ERP) indicators of inhibitory cognitive control were collected, as was a measure of parasympathetic control of the heart (respiratory sinus arrhythmia, RSA). Independently of age, RSA was correlated with nogo N2 magnitudes during the emotion-induction procedure. RSA during the task was also correlated with N2 latencies and with behavioral accuracy before, during and after the emotion induction. Resting RSA was correlated with individual differences in the capacity for effortful cognitive control, as measured by questionnaire. These results suggest that emotional responses in seemingly distinct neurophysiological systems may be regulated in an integrated fashion throughout the developmental span tested., (Copyright 2010 Elsevier B.V. All rights reserved.)

Pyrethroids are common household insecticides. Even though they are less toxic to humans, reports of accidental and suicidal poisoning are not uncommon. Cardiotoxicity due to pyrethroid poisoning is rare. We report a case of cardiac conduction disturbance due to a pyrethroid, prallethrin. A 28-year-old female presented after a suicidal consumption of prallethrin. Her clinical and laboratory parameters were normal during the first 24 h of hospital stay. On the second hospital day, she developed metabolic acidosis and sinus arrest with escape junctional rhythm. Despite correction of metabolic acidosis, the sinus arrest persisted for 3 days. She reverted back to sinus rhythm with bradycardia after this period and was discharged on the seventh hospital day. Her follow-up was uneventful. Pyrethroid poisoning can affect the gastrointestinal, respiratory, and nervous system. Most serious effects of the toxin in humans are seizures and coma. Mechanism of pyrethroid neurotoxicity is believed to be due to its ability to modify sodium, chloride, and calcium channels of the neurons. Our case raises the possibility that cardiac arrhythmia due to pyrethroid poisoning can occur due to its effect on sodium channels in the heart.

Aims: The efficacy of catheter-based pulmonary vein isolation (PVI) for paroxysmal atrial fibrillation (AF) and prolonged sinus pauses [bradycardia-tachycardia syndrome (BTS)] has been already described. However, the effects of PVI on autonomic modulation in BTS patients remain to be determined. We, therefore, examined the alteration in the autonomic modulation through the PVI procedure by using a heart rate variability (HRV) analysis of 24 h ambulatory monitoring., Methods and Results: This study consisted of 26 symptomatic paroxysmal AF patients either with prolonged sinus pauses on termination of AF (>3.0 s, BTS group, n = 11) or without any evidence of sinus node dysfunction (control group, matched for sex and age, n = 15) who underwent PVI. All 11 BTS patients became free from both AF and prolonged sinus pauses without pacemaker implantation (23 +/- 14 months of observation). The mean heart rate significantly increased in the control group (P < 0.05), but not in the BTS group after the PVI procedure, although the HRV parameters of root-mean-square successive differences in the adjacent NN intervals, standard deviation of the NN intervals, and high frequency did significantly decrease in both groups (P < 0.05)., Conclusion: Although the parasympathetic modulation was significantly attenuated after the PVI procedure, the mean heart rate did not increase in the BTS patients, probably due to the pre-existing sinus node dysfunction.

Familial hypokalemic periodic paralysis is an autosomal dominant muscle disorder characterized by episodic attacks of muscle weakness, accompanied by a decrease in blood potassium levels. It is based on genetic mutations in the genes CACNA1S (most frequent, encoding the skeletal muscle calcium channel) and SCN4A (10% of cases, encoding the sodium channel). Few cases have been reported with cardiac dysrhythmia. We report a rare case of a patient with a novel SCN4A mutation who presented, on ECG, extreme bradycardia and syncopal sinus arrest that required a temporary pacemaker implant

The aim of the work was to evaluate the prevalence of cardiac rhythm and conductance disturbances in patients with early manifestations of metabolic syndrome (MS). 24-hour ECG monitoring was undertaken in 105 patients meeting AHA/NHLBI (2005) MS criteria and in 79 healthy subjects. Exclusion criteria were the presence of diabetes mellitus, CHD, and obesity (body mass index > 40 kg/m2). MS was associated with an increased number of supraventicular extrasystoles (628.9 +/- 49.5 vs 415.9 +/- 57.9, p < 0.05) and ventricular extrasystoles (34.4 +/- 9.9 vs 11.8 +/- 6.5 for paired ones and 9.5 +/- 3.7 vs 2.2 +/- 4.0 for group ones, p < 0.05), higher frequency of tachyarrhythmia (supraventicular tachycardia: 18.1 vs 7.6%, p < 0.05; atrial fibrillation: 9.5 vs 2.5, p < 0.05; sinus node arrest: 6.7 vs 0%, p < 0.05). Regression analysis revealed significant correlation between arrhythmias and the number of components of the disease. It is concluded that cause-and-effect relationship between MS and cardiac rhythm disturbances is apparent at the early stage of the disease.

Radiofrequency ablation has been associated with changes in autonomic function. In this case, a 52-year-old woman was referred for electrophysiologic study of recurrent supraventricular tachycardia. Typical slow/fast atrioventricular node re-entry tachycardia was induced and confirmed during the study. Radiofrequency ablation of the slow pathway of the atrioventricular node led to sinus arrest, which resolved once ablation was stopped. Given the distance of the ablation site from the sinus node, we inferred that mechanisms other than direct injury to the sinus node were involved. To our knowledge, this is only the 2nd reported finding of sinus arrest during slow-pathway ablation of the atrioventricular node. This case highlights the complex interaction between the autonomic nervous system and the cardiac conduction system. Possible mechanisms include a Bezold-Jarisch reflex and modification of the intrinsic autonomic nervous system.

We report a patient with herpes simplex virus (HSV) encephalitis presenting as recurrent syncope due to sinus node arrest. Although the patient's initial presentation suggested a primary cardiac cause, an eventual diagnosis of HSV encephalitis was supported by computed tomography scan and magnetic resonance imaging, and confirmed by HSV polymerase chain reaction. The mechanism of cardiac arrhythmias in HSV encephalitis remains unclear; however, cardiac monitoring should be considered in all patients in whom the diagnosis is suspected. With diagnosis and appropriate management, a permanent pacemaker is generally not indicated. This case report highlights the importance of considering potentially reversible causes of collapse secondary to sinus node dysfunction beyond primary cardiac causes.