A 1-year-old boy with long pauses caused by paroxysmal atrioventricular block and sinus arrest: Vagal reflex and effect of pacing.

Paroxysmal atrioventricular block (PAVB) is rare in children. A 1-year-old boy presented with PAVB and sinus arrest that resulted in refractory life-threatening symptomatic long pauses. Continuous heart rate variability analysis with high time resolution (wavelet analysis) revealed an abrupt increase in parasympathetic activity just before a long pause, which indicated a vagal reflex. Although a pacemaker is not always effective because of a concomitant vasodepressive response in such cases, the complete stabilization after pacemaker implantation in this case supports the necessity and usefulness of pacemaker implantation in patients with reflex-induced highly symptomatic bradycardia.

Vagoglossopharyngeal Neuralgia Occurred Concomitantly with Ipsilateral Hemifacial Spasm and Versive Seizure-Like Movement: A First Case Report.

Vagoglossopharyngeal neuralgia (VGPN) is a very rare condition. VGPN with convulsive like attack is even rarer All of the cases had their head turned to the opposite side of facial pain. Hemifacial spasm occurring concurrently with VGPN has never been reported. Herein, we present the first case of VGPN that had ipsilateral hemifacial spasm and versive seizure-like movement to the same side of facial pain. We reported a 71-year-old man presenting with multiple episodes of intermittent sharp shooting pain arising on the right middle neck, followed by hemifacial spasm on right face. Then the patient became syncope while his head and gaze turned to the same side of the painful neck. Electrocardiography showed sinus arrest. Interictal Electroencephalography was normal. This patient initially responded to pregabalin for two weeks, then the symptoms became worse. Microvascular decompression and carbamazepine resulted in the complete remission of all symptoms after six months of follow-up. We could not explain the pathophysiology of unilateral versive seizure like movement.

Assessment of heart rate variability in breath holding children by 24 hour Holter monitoring.

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.

[Dizziness and syncope : clinical case examples]. / Schwindel und Synkope : Klinische Fallbeispiele.

Syncope is defined as a transient, self-limiting loss of consciousness and postural tone due to transient global cerebral hypoperfusion. After syncope the following questions have to be answered: was it a syncopal episode, has the etiological diagnosis been determined, are there data suggestive of a high risk of cardiovascular events or death and what are the therapeutic options? Therefore, a standardized diagnostic work-up is necessary. This diagnostic work-up with differential diagnostic considerations is given for three clinical cases: a 52-year-old man experienced syncope while driving a car and on the morning of the same day syncope had previously occurred while in a standing position. The initial cardiological and neurological evaluation revealed no pathological findings but after implantation of a loop recorder a further syncope with a sinus arrest of 17 s occurred 1 year later. The patient received a single chamber pacemaker. The second case is a 79-year-old female with Parkinson's disease for many years and a primary autonomic dysfunction leading to dizziness and syncope due to pronounced blood pressure fluctuations with hypertensive and hypotensive phases. The last patient is a 22-year-old female with postural orthostatic tachycardia syndrome and recurrent syncope. The diagnostic evaluation and treatment proved to be difficult.

An unexpected Holter monitor result: multiple sinus arrests in a patient with lateral medullary syndrome.

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.

Ethics in electrophysiology: who speaks for this man?

In this case, a patient who is incapable of participating in health-care decisions requires a pacemaker generator replacement. Because the pacemaker may no longer be necessary, the issues of surrogacy and surrogate decision-making are considered. Where there are no involved family members, each state has procedures for finding an appropriate ombudsman for the patient who can assist in healthcare choices.

A pause for thought: exercise-induced sinus arrest causing syncope in a young male.

A 32-year-old Spanish man presented to hospital after a second episode of syncope immediately following exercise. On admission, his vitals signs were stable and he had a regular heart rate of 60 bpm. ECG and transthoracic echocardiogram were normal. He completed 15 min of a BRUCE protocol exercise test. One minute and ten seconds into recovery, he lost consciousness. His ECG demonstrated sinus arrest with pauses of up to 5 s and subsequently junctional ectopy. After 38 s, his heart returned to sinus rhythm at a rate of 140 bpm and he regained consciousness. Vasovagal syncope following exercise in the absence of structural heart disease is uncommonly reported. When cases of exercise-related syncope in patients with structurally normal hearts have been reported, the typical patient is a young male who engages in physical training. Treatment strategies in patients suffering with vasovagal asystole are necessarily empirical, and careful judgement based on the specific features of the individual cases needs to be employed.

Implantation of a permanent pacemaker in a pregnant woman under the guidance of electrophysiologic signals and transthoracic echocardiography.

We describe the case of a 30-year-old pregnant woman who underwent pacemaker implantation for recurrent syncope caused by sinus arrest. In order to minimize radiation exposure, which may potentially have teratogenic effects, we decided to perform pacemaker implantation by means of a hybrid technique involving the evaluation of electrophysiologic signals and transthoracic echocardiographic imaging to guide lead positioning within the right ventricle. After the procedure, the patient was always asymptomatic and had no recurrences of syncope. Six months later, she experienced uncomplicated natural labor, and the newborn was absolutely healthy. On a 10-month follow-up examination, the lead position was still optimal, and ventricular sensing and pacing thresholds were good. Our case demonstrates that pacemaker implantation under the guidance of electrophysiologic signals and transthoracic echocardiographic imaging, with only a short view by pulsed fluoroscopy in order to verify the correct lead position, is an effective and well-tolerated procedure in pregnant women.