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Background: Cardioneuroablation (CNA) is a promising therapy for reflex asystolic syncope; however, convincing data on the mid-term safety and efficacy of this procedure are lacking., Objective: The purpose of this study was to assess the mid-term safety, efficacy, and patient acceptance of CNA., Methods: This prospective observational single-center study included 115 consecutive patients (mean age 39 ± 13 years; 58% female) treated between 2016 and 2022 who completed at least 1-year follow-up., Results: No significant procedure-related acute complications occurred. During median follow-up of 28 months (range 12-75), 95 (83%) remained free from syncope. Of the 20 patients (17%) with syncope recurrence, syncope burden decreased from a mean 17 (median 6.5) to 3.75 (median 2.5) episodes (P = .015). In 9 of 10 patients, pacing system removal was possible. Repeated CNA was needed in 3 patients (3%), whereas pacemaker implantation was performed in 5 (4%). The most frequent mid-term complication of CNA was sinus rhythm acceleration (from 60 ± 14 bpm to 90 ± 16 bpm; P <.0001), which was symptomatic in 31 patients (27%); 8 patients (7%) required chronic beta-blocker and/or ivabradine. Sinus node modification was necessary in 1 patient. Other complaints included dyspnea, chronic chest pain, and decreased exercise capacity, which were mild and reported by 16 patients (14%). Patient acceptance of CNA was very high: 96% stated that it was worth undergoing the procedure., Conclusions: Mid-term efficacy of CNA exceeds 80%, and acute complications are absent. The most frequent mid-term chronic complication is inappropriate sinus tachycardia, which in 7% required chronic treatment. The procedure is well accepted by patients., (Copyright © 2023 Heart Rhythm Society. Published by Elsevier Inc. All rights reserved.)

Introduction: Severe transitory episodes of bradycardia with subsequent syncope in children are common, and generally portend a benign prognosis. Rarely, patients may experience prolonged asystolic episodes secondary to significant sinus pauses (SP) or paroxysmal atrioventricular block (AVB). Cardioneuroablation (CNA) is a catheter-based intervention, used to identify and ablate the epicardial ganglionated plexi (GP), which results in disruption of the vagal-mediated parasympathetic input to the sinus and atrioventricular node., Objective: Describe the methodology and role of CNA for treatment of pediatric patients with functional AVB or SP., Methods: This is a single-center, case series study. Patients with SP or AVB, 21 years of age or younger, who underwent CNA between 2015 and 2021 were included. CNA was performed via anatomically guided and high-frequency stimulation methods., Results: Six patients were included. The median age was 18.9 years (range 12.3-20.9 years), 33% female. Two patients had prolonged SP, two had paroxysmal AVB, and two had both SP and AVB. Four patients had prior syncope. The median longest pause was 8.9 s (range 3.9-16.8) with 11 total documented pauses (range 2-231) during the 6 months pre-CNA. Post-CNA, the median longest pause was 1.3 s (range 0.8-2.2) with one documented SP after termination of atrial tachycardia at the 3-month follow-up. At 6 months, the median longest pause was 1.1 s (0.8-1.3) with 0 documented pauses. No patients had syncope post-CNA., Conclusion: CNA may be an effective alternative to pacemaker implantation in pediatric patients with syncope or significant symptoms secondary to functional SP or AVB., (© 2023 Wiley Periodicals LLC.)

Cardioneuroablation is an emerging alternative therapeutic modality for young patients with severe neurally-mediated syncope. We present two images of cardioneuroablation performed in young patients who suffered from recurrent neurally-mediated syncope with asystole and functional atrioventricular block. The patients remain syncope-free during follow-ups., Competing Interests: Conflict of interest There is no conflict of interest., (Copyright © 2023 Hellenic Society of Cardiology. Published by Elsevier B.V. All rights reserved.)

Background: Some patients suffering from intractable epileptic seizures, particularly drop attacks (DAs), are nonremediable by curative techniques. Palliative procedure carries a significant rate of surgical and neurological complications., Objective: To propose evaluation of safety and efficacy of Gamma Knife corpus callosotomy (GK-CC) as an alternative to microsurgical corpus callosotomy., Methods: This study included retrospective analysis of 19 patients who underwent GK-CC between 2005 and 2017., Results: Of the 19 patients, 13 (68%) had improvement in seizure control and 6 had no significant improvement. Of the 13/19 (68%) with improvement in seizures, 3 (16%) became completely seizure-free, 2 (11%) became free of DA and generalized tonic-clonic but with residual other seizures, 3 (16%) became free of DA only, and 5 (26%) had >50% reduction in frequency of all seizure types. In the 6 (31%) patients with no appreciable improvement, there were residual untreated commissural fibers and incomplete callosotomy rather than failure of Gamma Knife to disconnect. Seven patients showed a transient mild complication (37% of patients, 33% of the procedures). No permanent complication or neurological consequence was observed during the clinical and radiological workup with a mean of 89 (42-181) months, except 1 patient who had no improvement of epilepsy and then aggravation of the pre-existing cognitive and walking difficulties (Lennox-Gastaut). The median time of improvement after GK-CC was 3 (1-6) months., Conclusion: Gamma Knife callosotomy is safe and accurate with comparable efficacy to open callosotomy in this cohort of patients with intractable epilepsy suffering from severe drop attacks., (Copyright © Congress of Neurological Surgeons 2023. All rights reserved.)

Background: Corpus callosotomy (CC) is a surgical palliative procedure done for a selected group of patients with drug resistant epilepsy (DRE) to stop drop attacks and prevent falls., Methods: We performed a retrospective chart review of consecutive patients who underwent CC for DRE with drop attacks at our center between 2015 and 2019. Clinical, imaging details and surgical findings were noted. Clinical outcomes and functional status were evaluated., Results: During the study period, 17 patients underwent corpus callosotomy (Male: Female 14:3). The mean age at surgery was 10.3 years (standard deviation - 5.85, interquartile range [IQR] = 6.5). The mean age at onset of seizure was 2.23 years (standard deviation - 3.42, IQR = 1.5). Preoperative seizure frequency ranged from 2 to 60 attacks per day (median: 20, IQR= 36). All patients had atonic seizures/drop attacks. One patient underwent anterior CC and 16 underwent complete CC. Three patients had complications in the postoperative period. The median follow-up was 26 months. All patients had cessation of drop attacks immediately following surgery. One patient with anterior CC had a recurrence of drop attacks for which she underwent completion CC. Another patient had recurrent drop attacks 3 years later and was found to have a residual callosal connection. Three patients had complete seizure freedom and 4 patients had a <50% reduction in seizure frequency., Conclusions: Our study lends additional support to the efficacy of CC in patients with DRE, with the cessation of drop attacks. It also provided a reasonable reduction in seizure frequency. Complete CC led to better control of drop attacks., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background: The proclivity to atlantoaxial instability (AAI) has been widely reported for conditions such as rheumatoid arthritis and Down syndrome. Similarly, we have found a higher than expected incidence of AAI in hereditary connective tissue disorders. We demonstrate a strong association of AAI with manifestations of dysautonomia, in particular syncope and lightheadedness, and make preliminary observations as to the salutary effect of surgical stabilization of the atlantoaxial motion segment., Methods: In an institutional review board-approved retrospective study, 20 subjects (16 women, 4 men) with hereditary connective tissue disorders had AAI diagnosed by computed tomography. Subjects underwent realignment (reduction), stabilization, and fusion of the C1-C2 motion segment. All subjects completed preoperative and postoperative questionnaires in which they were asked about performance, function, and autonomic symptoms, including lightheadedness, presyncope, and syncope., Results: All patients with AAI reported lightheadedness, and 15 had refractory syncope or presyncope despite maximal medical management and physical therapy. Postoperatively, subjects reported a statistically significant improvement in lightheadedness (P = 0.003), presyncope (P = 0.006), and syncope (P = 0.03), and in the frequency (P < 0.05) of other symptoms related to autonomic function, such as nausea, exercise intolerance, palpitations, tremors, heat intolerance, gastroesophageal reflux, and sleep apnea., Conclusions: This study draws attention to the potential for AAI to present with syncope or presyncope that is refractory to medical management, and for surgical stabilization of AAI to lead to improvement of these and other autonomic symptoms., (Copyright © 2021 The Authors. Published by Elsevier Inc. All rights reserved.)

Deglutition syncope is a rare, neurally mediated reflex syncope associated with swallowing. It is typically associated with pharyngoesophageal disorders with secondary abnormal vagal reflex causing atrioventricular cardiac block and cerebral hypoperfusion. Diagnosis can be delayed and challenging. Various treatment options have been reported, including withholding cardiac blocker agents, diet modifications, and pacemaker placement. We present a case of persistent deglutition syncope secondary to esophagogastric junction outflow obstruction that failed medical therapy and was managed successfully with peroral endoscopic myotomy with excellent long-term outcome., (Copyright © 2020 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

A 68-year-old woman presented with episodes of headache, palpitations, sweating and poorly controlled hypertension for the past 6 years. These symptoms were, at times, associated with micturition, and there were few episodes of micturition syncope as well. She had elevated 24-hour urinary normetanephrine and was found to have a paraganglioma arising from the urinary bladder infiltrating the sigmoid colon. She underwent laparotomy with excision of the bladder paraganglioma, following which her symptoms subsided. Paragangliomas are extra-adrenal catecholamine-producing tumours. Bladder paragangliomas need to be considered when evaluating hypertensive patients with headache, palpitations or syncope related to micturition., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Bladder Paraganglioma is a rare type of bladder tumor (0.06%). It is typically benign and the most common symptoms are hematuria, hypertension and headache. About 14% of these tumors are malign and consequently radio and chemoresistants. Therefore, surgery is the mainstay of treatment. As they are likely to recur and to metastize lifelong follow-up is required. The authors report a rare case of a 53 years old man with hematuria and a previous history of micturition syncope who was diagnosed with bladder lesion. During the transurethral ressection of bladder he became severely hypertensive. Plasma metanephrines, and urinary vanillylmandelic acid, were still high and the exams suggested residual tumor. The patient underwent radical cistoprostatectomy. After 4 years of follow-up the patient remains disease free., Competing Interests: The authors declare no competing interests., (Copyright: Gil Falcão et al.)

Rationale: Syncope caused by head and neck cancer (HNC) is rare. However, syncope caused by tongue cancer (TC) is even rarer. In TC, syncope is caused by tumor-mediated compression of the carotid sinus and stimulation of the glossopharyngeal nerve., Patient Concerns: In this study, we report the case of a 48-year-old male patient who was diagnosed with advanced TC and bilateral cervical lymph node metastasis. On the third day of admission, the patient experienced recurrent syncope with hypotension and bradycardia., Diagnoses: The patient was diagnosed with a well-differentiated squamous cell carcinoma of the tongue along with massive cervical lymph node metastasis and carotid sinus syndrome., Interventions: Initially, symptomatic treatment of syncope boosted the blood pressure and increased the heart rate. Thereafter, a temporary pacemaker was implanted. Finally, chemotherapy was used to control the tumor and relieve syncope., Outcomes: After chemotherapy, the tongue ulcers and cervical lymph node reduced in size; syncope did not recur., Lessons: This case shows that chemotherapy may be a valid treatment option in patients with cancer-related syncope; however, the choice of chemotherapeutic drugs is critical. Intensive care provides life support to patients and creates opportunities for further treatment.

An 84-year-old man presented with syncope. Prior to admission, ambulatory electrocardiogram had demonstrated non-sustained ventricular tachycardia. Echocardiography showed severe aortic stenosis. He was also diagnosed with hypertrophic cardiomyopathy (HCM) by cardiac magnetic resonance (CMR) showing remarkable inhomogeneous left ventricular hypertrophy and extensive late gadolinium enhancement (LGE) in the lesions at the upper border and right-ventricular side of the basal-mid septal wall. Finally, he showed complete atrioventricular (AV) block followed by a long pause and syncope several times after admission. In this case with several possible causes of syncope, the CMR findings suggested a clue concerning the etiology of his syncope: complete AV block in HCM.

Corpus callosotomy has been used as a form of surgical palliation for patients suffering from medically refractory generalized seizures, including drop attacks. Callosotomy has traditionally been described as involving a craniotomy with microdissection. MR-guided laser interstitial thermal therapy (MRg-LITT) has recently been used as a minimally invasive method for performing surgical ablation of epileptogenic foci and corpus callosotomy. The authors present 3 cases in which MRg-LITT was used to perform a corpus callosotomy as part of a staged surgical procedure for a patient with multiple seizure types and in instances when further ablation of residual corpus callosum is necessary after a prior open surgical procedure. To the authors' knowledge, this is the first case series of corpus callosotomy performed using the MRg-LITT system with a 3.3-year average follow-up. Although MRg-LITT is not expected to replace the traditional corpus callosotomy in all cases, it is a safe, effective, and durable alternative to the traditional open corpus callosotomy, particularly in the setting of a prior craniotomy.

Objective: To our knowledge, there have not been any reported cases of a meningioma of the craniocervical region presenting solely with syncope as its initial symptom. Only 1 case of meningioma presenting with syncope has been published, but it was associated with hydrocephalus. We report 2 cases of syncope caused by a craniocervical junction meningioma, with syncope being the sole presenting symptom and without hydrocephalus. We discuss the possible pathophysiology, as well as the clinical relevance of this type of presentation., Methods: We reviewed the charts, operative details, and imagery of 2 cases of meningioma in the region of the craniocervical junction, with syncope as their sole presenting feature. We also reviewed the literature., Results: In 1 case the syncope occurred spontaneously. In the other, it occurred during a Valsalva maneuver. Both meningiomas were surgically removed via a retromastoid approach. There was no recurrence of syncope following surgery. Following a literature review, we found 1 case of posterior fossa meningioma presenting with syncope, but hydrocephalus was also present., Conclusion: Syncope can be the sole manifestation of a meningioma of the craniocervical junction. Such syncopes are a consequence of transient dysfunction of the autonomous pathways in the medulla and/or of the medulla's output. In the absence of other causes of syncope, a meningioma in this region, even in the absence of hydrocephalus, should not be considered as fortuitous, but rather as the actual cause of syncope. Recognizing this possibility offers the potential for proper diagnosis and appropriate treatment of the syncope., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Intracardiac leiomyomatosis is a rare complication that occurs when a uterine leiomyoma (fibroid) undergoes vascular invasion and propagates within the inferior vena cava to reach the right atrium. This article describes a case of intracardiac leiomyomatosis in a middle-aged woman, exploring the presentation, diagnosis and surgical management of this condition. In this case the presenting complaints were syncope and atrial fibrillation, illustrating the importance of performing a transthoracic echocardiogram in patients presenting with their first episode of atrial fibrillation. Clinicians should consider intracardiac leiomyomatosis when evaluating women with right heart masses, especially those with a history of uterine leiomyomas., (Copyright © 2016 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). All rights reserved.)

The prognostic value of the seasonal variations of T-wave alternans (TWA) and heart rate variability (HRV), and the seasonal distribution of ventricular fibrillation (VF) in Brugada syndrome (Br-S) is unknown. We assessed the utility of seasonal variations in TWA and HRV for risk stratification in Br-S using a 24-h multichannel Holter electrocardiogram (24-M-ECG). We enrolled 81 patients with Br-S (grouped according to their history of VF, n = 12; syncope, n = 8; no symptoms, n = 61) who underwent 24-M-ECG in all four seasons. Precordial electrodes were attached to the third (3L-V2) and fourth (4L-V2, 4L-V5) intercostal spaces. We determined the maximum TWA (max-TWA) values and calculated HRV during night and morning time periods for all seasons. During a follow-up period of 5.8 ± 2.8 years, 11 patients experienced new VF episodes and there was a peak in new VF episodes in the summer. The VF group had the greatest 3L-V2 max-TWA value during morning time in the summer among the three groups and showed higher 3L-V2 max-TWA value than in the other seasons. The cutoff value for the 3L-V2 max-TWA during morning time in the summer was determined to be 42 µV using ROC analysis (82 % sensitivity, 74 % specificity; p = 0.0006). Multivariate analysis revealed that a 3L-V2 max-TWA value ≥42 µV during morning time in the summer and previous VF episodes were predictors of future VF episodes. The 3L-V2 max-TWA value during morning time in the summer may be a useful predictor of future VF episodes in Br-S.

Objective: To evaluate a novel approach to control epileptic drop attacks through a selective posterior callosotomy, sparing all prefrontal interconnectivity., Methods: Thirty-six patients with refractory drop attacks had selective posterior callosotomy and prospective follow-up for >4 years. Falls, episodes of aggressive behavior, and IQ were quantified. Autonomy in activities of daily living, axial tonus, and speech generated a functional score ranging from 0 to 13. Subjective effect on patient well-being and caregiver burden was also assessed., Results: Median monthly frequency of drop attacks decreased from 150 to 0.5. Thirty patients (83%) achieved either complete or >90% control of the falls. Need for constant supervision decreased from 90% to 36% of patients. All had estimated IQ below 85. Median functional score increased from 7 to 10 (p = 0.03). No patient had decrease in speech fluency or hemiparesis. Caregivers rated the effect of the procedure as excellent in 40% and as having greatly improved functioning in another 50%. Clinical, EEG, imaging, and cognitive variables did not correlate with outcome., Conclusions: This cohort study with objective outcome assessment suggests that selective posterior callosotomy is safe and effective to control drop attacks, with functional and behavioral gains in patients with intellectual disability. Results are comparable to historical series of total callosotomy and suggest that anterior callosal fibers may be spared., Classification of Evidence: This study provides Class III evidence that selective posterior callosotomy reduces falls in patients with epileptic drop attacks., (© 2016 American Academy of Neurology.)

We discuss the case of a 38-year-old black man who presented at our hospital with his first episode of syncope, recently developed atrial arrhythmias refractory to pharmacologic therapy, and a left atrial thrombus. He was diagnosed with primary cardiac sarcoidosis characterized by predominant involvement of the epicardium that caused atrial fibrillation and atrial flutter. Histologic analysis of his epicardial lesions yielded a diagnosis of sarcoidosis. This patient's atrial arrhythmia was successfully treated with a hybrid operation that involved resection of his atrial appendage, an Epicor maze procedure, and radiofrequency ablation during a catheter-based electrophysiologic study. The cardiac sarcoidosis was successfully managed with corticosteroid therapy. Our case report shows that sarcoidosis can initially manifest itself as syncope with new-onset atrial arrhythmia. Sarcoidosis is important in the differential diagnosis because of its progressive nature and its potential for treatment with pharmacologic, surgical, and catheter-based interventions.

Two young Labrador retriever dogs with bradycardia-induced syncope resulting from atrial myopathy underwent permanent transvenous pacemaker implantation. Both dogs developed heart failure 3-5 years after pacemaker implantation. Both were managed medically for approximately 7 years after pacemaker implantation and, ultimately, were humanely euthanized due to refractory heart failure signs and quality of life concerns. Long-term management of dogs with atrial myopathy and secondary atrial standstill with pacemaker implantation and medical therapy for heart failure is feasible and prognosis may be better than previously reported or speculated., (Copyright © 2016 Elsevier B.V. All rights reserved.)

Background: Cardio neuroablation (CNA) is a lesser-known technique for management of patients with excessive vagal activation on the basis of radiofrequency catheter ablation (RFCA) of the areas related to the three main autonomic ganglia around the heart. We investigated the effectiveness of selective and/or stepwise RFCA of these areas via right atrium (RA) and/or left atrium (LA) in the patients with recurrent syncope due to excessive vagal activity., Methods: Twenty-two patients presenting symptomatic functional bradyarrhythmias, neurally mediated reflex syncope (NMS), symptomatic atrioventricular (AV) block, and symptomatic sinus node dysfunction (SND; number = 8, 7, 7, respectively) were enrolled. The three main paracardiac ganglia were targeted via RA and LA in the patients with NMS and SND. The procedure was performed via RA in the patients with AV block, followed by RFCA of all ganglia via LA, if AV conduction disorder persists. The sites showing fragmented potentials were identified by electrical mapping and verified by high-frequency stimulation and ablated until atrial electrical potential was completely eliminated (<0.1 mV)., Results: The patients with NMS and SND were free from new syncopal episode at a mean 12.3 ± 3.4 months and 9.5 ± 3.1 months follow-up, respectively. Ablation from RA was successful in six of seven patients with AV block. Despite the increased heart rate, the resolution of AV block after the RFCA could not be achieved in one patient who had partial resolution with atropine infusion on admission., Conclusion: CNA may be an alternative and safe strategy to reduce NMS episodes, and to treat functional AV block and symptomatic SND, especially in young patients., (© 2015 Wiley Periodicals, Inc.)

Some cases of Coffin-Lowry syndrome recognized episodic drops and it tended to be intractable for medical treatment. We reported here a patient with the Coffin-Lowry syndrome associated with obstructive sleep apnea syndrome (OSAS). The patient had epileptic seizures and drop attacks only during night-time and it was not recognized during the daytime. His sleep-induced electroencephalogram was normal. At 12-years old of his age, his OSAS was worse, so we performed a tracheotomy. Notably after the operation, his epileptic episodes were disappeared.

Objective: Gradient magnetic field topography (GMFT) with magnetoencephalography (MEG) has been developed to demonstrate magnetic-field gradients of epileptic spikes on a volume-rendered brain surface. We evaluated GMFT in patients with anterior 2/3 corpus callosotomy (ACC) for drop-attacks., Methods: Eight patients (age; 11-37 years) underwent ACC. GMFT evaluated the predominant distributions (anterior/posterior) and the spreading patterns (unilateral/bilateral) of pre- and postoperative interictal MEG spikes corresponding to generalized spikes on EEG. We compared the occurrence of four types of spikes; anterior unilateral spike (AUS), posterior unilateral spike (PUS), anterior bilateral spike (ABS), posterior bilateral spike (PBS) between 5 patients (group G) with good control of drop attacks and 3 patients (group P) with residual drop attacks., Results: Preoperatively, GMFT showed the proportion of ABS in group G (mean ± SD, 57.4 ± 9.7%) was significantly (p=0.024) higher than that in group P (31.6 ± 15.2%). The number and proportion of postoperative ABS and PBS in group G were significantly decreased (p<0.05 in all)., Conclusion: GMFT is valuable to evaluate pre- and post-operative predominant spikes in patients with drop attacks., Significance: GMFT revealed a higher proportion of preoperative ABS and postoperative decline of both ABS and PBS in patients with good control of drop attacks after ACC., (Copyright © 2015. Published by Elsevier Ireland Ltd.)

A 2-stage corpus callosotomy is accepted as a palliative procedure for patients older than 16 years with, in particular, medically intractable generalized epilepsy and drop attack seizures and is preferable for a lower risk of disconnection syndrome. Although the methods by which a previously performed craniotomy can be reopened for posterior callosotomy have already been reported, posterior corpus callosotomy using a parietooccipital interhemispheric approach with the patient in a semi-prone park-bench position has not been described in the literature. Here, the authors present a surgical technique for posterior callosotomy using a parietooccipital interhemispheric approach with a semi-prone park-bench position as a second surgery. Although this procedure requires an additional skin incision in the parietooccipital region, it makes the 2-stage callosotomy safer and easier to perform because of reduced intracranial adhesion, less bleeding, and an easier approach to the splenium of the corpus callosum.

Objectives: Primary tumors of the inferior vena cava are rare, with leiomyosarcoma representing the vast majority., Method: A 60-year-old man was admitted in emergency for fainting and mild anemia. A whole-body computed tomography revealed a retroperitoneal mass of approximately 8 cm in diameter, invading the lumen of the inferior vena cava, extending to the renal vein confluence. An en bloc resection of the solid mass was performed. Macroscopically the tumor did not seem to insist on the resection margin., Results: Histopathological examination confirmed the diagnosis of leiomyosarcoma of the inferior vena cava. Postoperative recovery was uneventful and the patient was discharged after eight days, starting adjuvant chemotherapy. During the follow-up, the patient did not show other fainting episode, and at 24 months he is disease free., Conclusions: Unusually, fainting could even be the isolated sign of a large leiomyosarcoma of the inferior vena cava, also when it affects its middle portion., (© The Author(s) 2014.)

Background: A dilemma occurs in the treatment of second-sided Ménière's disease in the only hearing ear, particularly in patients with severe symptoms such as 'drop attacks'. This paper describes a patient treated with contralateral cochlear implantation prior to vestibular nerve section of the symptomatic ear., Case Report: A 53-year-old man, with second-sided Ménière's disease and drop attacks in the only serviceable right ear, underwent successful left cochlear implantation 30 years after hearing loss, followed by right vestibular nerve section. The patient achieved control of Ménière's attacks and improved hearing. Although the patient experienced oscillopsia post-operatively, he was satisfied with his improved everyday functioning., Conclusion: Patients with severe second-sided Ménière's disease in the only hearing ear are a small but difficult treatment group. In those that are suitable for cochlear implantation in the non-serviceable ear, it is suggested that this be employed prior to surgical treatment of the Ménière's symptoms, even if the implanted ear has had no auditory stimulation for many years.

Arrhythmias during pregnancy pose a dilemma for the treating physician. Most antiarrhythmic drugs are classified as category C in the FDA labeling system during pregnancy. We describe the case of a pregnant woman who presented syncope due to drug-refractory supraventricular tachycardia who underwent catheter ablation without the use of fluoroscopy., (Copyright © 2014 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.)

Isolated pulmonary regurgitation (PR) is a rare occurrence with only a handful of documented cases worldwide. Though usually well tolerated in childhood, chronic PR can eventually lead to RV dysfunction and ventricular arrhythmias. In this cardiac vignette, we describe a case of isolated PR in a young female presenting with syncope and explore the natural history, different investigative modalities as well as issues in clinical management of this rare condition., (Crown Copyright © 2014. Published by Elsevier B.V. All rights reserved.)

We report on the efficacy and safety of extended, one-stage anterior callosal section that spares the splenium, which is performed in a large series of pediatric patients with drop attacks. Twenty-nine pediatric patients with drop attacks were studied (19 males and 10 females; mean age: 9.9 years). As presurgical factors, the age at surgery, age at seizure onset, age at drop attack onset, sex, hemiparesis, severe mental retardation, electroencephalograph abnormalities, magnetic resonance imaging abnormalities, and (18)fluorodeoxyglucose positron emission tomography abnormalities were analyzed. All patients had multiple seizure types, including drop attacks, atypical absence seizures, complex partial seizures, tonic seizures, and generalized tonic-clonic seizures. All patients were developmentally impaired and had electroencephalograph results showing marked secondary bilateral synchrony. All patients received an extended, one-stage callosal section, leaving only the splenium intact. The mean follow-up time was 5.2 years. Seizure outcome (cessation of seizures or ≥ 90% seizure reduction) was achieved in 79.3% of patients with drop attacks. The families assessed the overall daily function as improved in 62.1% of the patients, unchanged in 24.1%, and worse in 13.8%. Family satisfaction with callosotomy was achieved in 82.8% of the patients. The majority of the patients had some degree of a transient acute postoperative disconnection syndrome that disappeared within 3 weeks. Postoperatively, patients showed a consistent increase in attention levels. We conclude that extended callosal sectioning that leaves the splenium intact should be considered a good palliative surgical option for pediatric patients with drop attacks and that diminishment of epileptic discharge synchrony is a good prognostic sign following callosotomy. We also found that the postoperative increase in attention levels was as useful as seizure control in improving the quality of life of these patients., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Hydatid disease is a parasitic infestation that is caused by the larvae of the tapeworm Echinococcus granulosus. Clinical manifestations are extremely variable and related to the location and the size of the cysts. Syncope as the first clinical presentation of hydatid cyst is a very rare condition. Herein, we report two cases of patients with cardiac and pleural hydatid cysts who had syncope as the initial symptom.

A 41 year-old African-American male presented with syncope preceded by shortness of breath at outside facility and transferred to us for management of extensive pulmonary embolism with unstable vital signs. Electrocardiogram showed sinus tachycardia with S1Q3T3 pattern. A transthoracic echocardiogram revealed a freely mobile strand like mass in the left atrium. A transoesophageal echocardiogram showed a very large freely mobile thrombus extending from a patent foramen ovale in to the left atrium. He underwent emergent surgery for the extraction of clot followed by thromboembolectomy from both pulmonary arteries. He made a remarkable recovery and was discharged after seven days of hospital stay., (Copyright © 2013 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.)

Background: To investigate the long term efficacy of carotid denervation by adventitial stripping of the internal carotid artery (ICA) for carotid sinus syndrome (CSS). Secondly, the long term safety of this technique is investigated with emphasis on the effects on blood pressure (BP), heart rate (HR) and carotid artery diameter., Methods: Characteristics of patients that were operated for CSS in a single institute between 1980-2007 were studied by a retrospective chart review. Alive and fit patients additionally received a standardized interview investigating symptoms of residual CSS or baroreflex failure. They underwent a test panel consisting of office BP measurement, carotid sinus massage (CSM), table tilt testing, 24-hour ECG and ambulatory BP measurement (ABPM) and carotid duplex. Unoperated, age- and sex- matched individuals without CSS served as controls., Results: After a total follow up of 91±34 months, 22 of 26 patients (85%) were asymptomatic and 20 of them (77%) without a pacemaker. Of the 7 surviving and fit patients, six were free of CSS symptoms (follow up 114 ± 81 months). Recurrence of CSS after an initial successful carotid denervation was not observed. BP level, BP variability and carotid diameters were not different compared to controls., Conclusion: Carotid denervation by adventitial stripping of the ICA for CSS seems effective and safe on the long term. A randomized controlled trial comparing the efficacy and safety of carotid denervation, pacing and medical treatment is needed for optimal future treatment of patients suffering from CSS.

A 65-year-old woman was admitted to the hospital because of a syncopal episode with documented transient complete atrioventricular block. A DDD pacemaker was implanted. Post implantation, the patient was diagnosed with bidirectional ventricular tachycardia. Analysis of the arrhythmia and differential diagnosis is performed., (©2013 Wiley Periodicals, Inc.)

Some forms of focal epilepsy, including temporal lobe epilepsy, are rarely associated with ictal bradycardia and sinus node arrest. We report a case of a previously healthy man presenting with syncope in whom telemetry revealed sinus arrest. Initial treatment was with permanent pacemaker implantation and it was only following a subsequent grand mal seizure that other symptoms suggestive of temporal lobe epilepsy were documented. Anti-epileptic medication was subsequently commenced with resolution of all symptoms. There are few previously reported cases of syncope and documented sinus node arrest as the presenting feature of temporal lobe epilepsy.

Background: Medically intractable epilepsy involving drop attacks can be difficult to manage and negatively affect quality of life. Most studies investigating the effect of corpus callosotomy (CC) on seizures have been limited, focusing on the pediatric population or drop seizures alone, with little attention to other factors influencing seizure outcome., Objective: To assess seizure outcomes after CC in adults and children., Methods: Retrospective analysis was performed on all patients who underwent CC (anterior two thirds, 1- or 2-stage complete) at our institution between 1990 and 2011. Change in frequency after CC was assessed for drop seizures and other seizure types. Multiple factors were evaluated for impact on seizure outcome., Results: Fifty patients met inclusion criteria. The median age was 1.5 years at seizure onset and 17 years at time of surgery. Anterior two-thirds CC was performed in 28 patients, 1-stage complete in 17, and 2-stage complete in 5. All 3 groups experienced a significant decrease in drop seizures (P < .001, P < .001, and P = .020, respectively), with 40% experiencing complete resolution, and 64% dropping at least 1 frequency category. Other seizure types significantly decreased in anterior two-thirds CC and 1-stage complete (P = .0035, P = .001, respectively). Younger age at surgery correlated with better seizure outcomes (P = .043)., Conclusion: CC for medically refractory generalizing epilepsy is effective for both drop seizures and other seizure types. CC should be considered soon after a patient has been deemed medically refractory because earlier age at surgery results in lower risk and better outcome.

Purpose: Syncope is associated with poor prognosis in patients with pulmonary hypertension. Atrial septostomy improves cardiac index and functional class in appropriately selected patients with pulmonary hypertension, and has been shown to improve syncope. One of the major challenges to its effectiveness is maintaining septostomy patency. We report the case of percutaneous deployment of a modified peripheral stent to create an atrial septostomy in a man with severe pulmonary hypertension and syncope, initially intolerant of medical therapy., Procedures: Percutaneous butterfly stent deployment across the interatrial septum using intracardiac echocardiography and fluoroscopy., Findings: The patient improved in all clinical parameters (BNP, six-minute walk test, dyspnoea score), and was subsequently able to tolerate targeted pulmonary hypertension therapies., Principal Conclusions: Atrial septostomy using butterfly stents to maintain patency may play a role in the treatment of patients with advanced pulmonary hypertension who do not respond to targeted therapy., (Copyright © 2013 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.)

The presence of syncope despite medical therapy in patients with hypertrophic cardiomyopathy (HC) is considered an indication for surgical myectomy; however, no study has examined the long-term effects on recurrent syncope and survival after surgery in these patients. We examined 239 patients with HC and a history of syncope who had undergone surgical myectomy (mean age 48 ± 17 years; 56% men). The patients were age- and gender-matched to patients with HC and syncope who were treated medically without myectomy (mean age 51 ± 16 years; 59% men). The median follow-up period was 4.7 years (0.8, 11.3). The recurrence rate of syncope was 11% in the myectomy patients and 40% in the medical group (p <0.0001). Multiple episodes of syncope, left ventricular outflow tract obstruction, and recent syncope were identified as baseline predictors of recurrent syncope. Survival free of all-cause mortality was greater for patients who had undergone surgical myectomy than for the medically treated patients (10-year estimate 82 ± 4% vs 69 ± 4%; p = 0.01). In conclusion, surgical myectomy in patients with HC and a history of syncope was associated with a reduction in recurrent syncope and increased survival., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Cor triatriatum sinister, a congenital cardiac anomaly involving a fibromuscular membrane that partitions the left atrium into 2 chambers, has been reported in only 0.1% to 0.4% of patients with congenital heart disease. The posterosuperior chamber receives blood from the pulmonary veins, and the anteroinferior chamber contains the left atrial appendage and mitral valve orifice. Most patients are diagnosed with the condition in infancy or childhood; adult cases are rare. We describe a case of cor triatriatum sinister in a 43-year-old man whose only presenting symptom was recurrent syncope. He underwent corrective resection of the membrane and was asymptomatic thereafter. In addition to discussing the patient's case, we review the relevant medical literature.

Lower cranial nerve schwannomas are benign tumors of the neurolemmocytes of the cranial nerves. Among children, cranial nerve schwannomas are extremely rare and are predominantly associated with neurofibromatosis (NF) type 2. The purpose of the current case report is to describe a unique giant extra- and intracranial foramen jugular schwannoma in a young boy with lower cranial nerve deficits and glossopharyngeal neuralgia syncope syndrome and to review the pertinent literature. In the current case report, we illustrate the course of disease in a 14-year-old boy with a 4-month history of recurrent syncope and a big bulge on the left side of his neck. Audiometry showed deafness of the left ear. Magnetic resonance imaging (MRI) demonstrated a giant unilateral dumbbell-shaped intra- and extracranial foramen jugular schwannoma with a volume of 156 cm3 causing severe brain stem compression and obstructive hydrocephalus. The tumor was removed completely in a two-step surgery. The tumor was confirmed during surgery to originate from the glossopharyngeal nerve. The histological examination revealed the characteristic features of a schwannoma. The MRI 3 months after the second surgery confirmed complete tumor removal. The genetic examination for NF was negative. Review of literature showed that dumbbell-shaped lower cranial nerve schwannomas in the childhood population are rare., (Georg Thieme Verlag KG Stuttgart · New York.)