Outcomes of Implantable Loop Monitoring in Patients <21 Years of Age.

Rhythm-symptom correlation in pediatric patients with syncope/palpitations or at risk cohorts can be difficult, but important given potential associations with treatable or malignant arrhythmia. We sought to evaluate the use, efficacy and outcomes of implantable loop recorders (ILR) in pediatrics. We conducted a retrospective study of pediatric patients (<21 years) with implanted ILR. Patient/historical characteristics and ILR indication were obtained. Outcomes including symptom documentation, arrhythmia detection and ILR based changes in medical care were identified. Comparison of outcomes were performed based on implant indication. Additional sub-analyses were performed in syncope-indication patients comparing those with and without changes in clinical management. A total of 116 patients with ILR implant were identified (79 syncope/37 other). Symptoms were documented 58% of patients (syncope 68% vs nonsyncope 35%; p = 0.002). A total of 37% of patients had a documented clinically significant arrhythmia and 25% of patients had a resultant change in clinical management independent of implant indication. Arrhythmia type was dependent on implant indication with nonsyncope patients having more ventricular arrhythmias. Pacemaker/defibrillator implantation and mediation management were the majority of the clinical changes. In conclusion, IRL utilization in selected pediatric populations is associated with high efficacy and supports clinical management. ILR efficacy is similar regardless of indication although patients with nonsyncope indications had a higher frequency of ventricular arrhythmias as opposed to asystole and heart block in syncope indications. The majority of arrhythmic findings occurred in the first 12 months, and new technology that would allow for less invasive monitoring for 6 to 12 months may be of value.

Frequent occurrence of postbreakfast syncope due to carotid sinus syndrome after surgery for hypopharyngeal cancer: A case report.

RATIONALE: Syncope often occurs in patients with advanced head and neck cancers due to the stimulation of the autonomic nervous system by the tumor. Here, we describe a case of frequent syncopal episodes after laryngopharyngectomy for hypopharyngeal cancer. As all syncopal episodes were observed during the forenoon, we also evaluated the heart rate variability using ambulatory electrocardiography to determine why the syncopal episodes occurred during a specified period of the day. PATIENT CONCERNS: A 73-year-old Japanese man who underwent laryngopharyngectomy for recurrent hypopharyngeal cancer started experiencing frequent episodes of loss of consciousness that occurred during the same time period (10:00-12:00). He had never experienced syncopal episodes before the operation. From 23 to 41 days postoperatively, he experienced 9 syncopal episodes that occurred regardless of his posture. DIAGNOSES: Pharyngo-esophagoscopy revealed an anastomotic stricture between the free jejunum graft and the upper esophagus. Swallowing videofluoroscopy confirmed the dilatation of the jejunal autograft and a foreign body stuck on the oral side of the anastomosis. Contrast-enhanced computed tomography revealed that the carotid artery was slightly compressed by the edematous free jejunum. The patient was diagnosed with carotid sinus syndrome (CSS) as the free jejunum was dilated when consuming breakfast, which may have caused carotid sinus hypersensitivity and induced a medullary reflex. INTERVENTIONS: Administration of disopyramide was effective in preventing syncope. Heart rate variability analysis using ambulatory electrocardiography showed that parasympathetic dominancy shifted to sympathetic dominancy during 10:00 to 12:00. The significant time regularity of the syncopal episodes may have been affected by modified diurnal variation in autonomic tone activity. OUTCOMES: After the surgical release and re-anastomosis of the pharyngoesophageal stenosis via an open-neck approach, no recurrent episodes of syncope were reported. LESSONS: We reported a case of frequent syncopal episodes limited to the forenoon due to CSS after surgery for hypopharyngeal carcinoma. The patient was treated with anticholinergics followed by the release and re-anastomosis of the pharyngoesophageal stenosis. When syncope occurs after surgery for head and neck lesions, CSS due to postoperative structural changes should be considered as a differential diagnosis of syncope.

New insights in diagnostics and therapies in syncope: a novel approach to non-cardiac syncope.

This article aims to give advice on how to identify and manage patients with syncope who are at risk of severe outcomes, that is, at risk of trauma, potentially life-threatening episodes or frequent recurrences reducing quality of life. The first step of syncope diagnostic assessment is to identify patients with cardiac syncope, and once established, these patients must receive the adequate mechanism-specific treatment. If cardiac syncope is unlikely, reflex (neurally mediated) syncope and orthostatic hypotension are the most frequent causes of transient loss of consciousness. For these presentations, efficacy of therapy is largely determined by the mechanism of syncope rather than its aetiology or clinical features. The identified mechanism of syncope should be carefully assessed and assigned either to hypotensive or bradycardic phenotype, which will determine the choice of therapy (counteracting hypotension or counteracting bradycardia). The results of recent trials indicate that 'mechanism-specific therapy' is highly effective in preventing recurrences. Established mechanism-specific treatment strategies include withdrawal of hypotensive drugs, applying fludrocortisone and midodrine for the hypotensive phenotype and cardiac pacing in the bradycardic phenotype.

Pacemaker implantation in familial amyloid polyneuropathy: when and for whom?

BACKGROUND: Despite the important role of cardiac pacing in preventing syncope and sudden cardiac death in familial amyloid polyneuropathy (FAP), we lack clear guidelines as to the ideal timing and indications for permanent pacemaker implantation. PURPOSE: The purpose of this study was to evaluate the ideal timing for pacemaker implantation in FAP patients submitted to liver transplantation. METHODS: Retrospective study of 258 FAP patients submitted to liver transplantation between 1992 and 2012. Comparison of three groups: (A) patients without pacemaker (N = 122); (B) patients submitted to pacemaker implantation after liver transplantation, with documented conduction disorders (N = 73); and (C) patients submitted to "prophylactic" pacemaker implantation before transplantation, (N = 73). Patients were followed up for 12.2 ± 6.7 years. RESULTS: The majority of patients (57%) were referred for pacemaker implantation, which occurred before liver transplantation in 50% of cases. Patients who required pacemaker after transplantation presented significantly higher Machado-Joseph Score during pre-transplant evaluation than those who did not require pacemaker (24 ± 10 vs 20 ± 10, p = .025), and also exhibited higher levels of hepatic cytolysis enzymes and hyperbilirubinemia. The most common indication for permanent pacemaker was first degree atrioventricular block, with a mean time between transplantation and pacemaker implantation of 8.7 ± 4.2 years. During long-term follow-up, all-cause mortality was 27% and was lowest in the group submitted to pacemaker implantation only after liver transplantation (p = 0.002). CONCLUSION: The majority of FAP patients submitted to liver transplantation will need a pacemaker at some time of follow-up. However, it seems that there is no benefit in "prophylactic" cardiac pacing before liver transplantation.

Cochlear implant function in a patient with Jervell and Lange-Nielsen syndrome after defibrillation by countershock.

Jervell and Lange-Nielsen syndrome (JLNS), a rare autosomal recessive congenital QT prolongation syndrome, is characterized by cardiac arrhythmias, syncopal episodes, and profound deafness. A cochlear implant (CI) for patients with JLNS is expected to result in hearing improvement. Sometimes, defibrillation is required if a patient experiences lethal arrhythmia. In this paper, we report a pediatric patient with JLNS who received defibrillation after CI surgery in his right ear at the age of 2 years. With intensive care, the post-operative course was uneventful, and the patient acquired satisfactory speech and hearing abilities. Five years after the surgery, he underwent defibrillation because of the incidence of syncopal attack. Thereafter, arrhythmic syncope recurred three times, which necessitated defibrillation therapy. To prevent recurrence of cardiac arrhythmia, he underwent ICD (implantable cardioverter-defibrillator) implantation at the age of 11 years. At present, CI works well and provides good hearing, while syncopal attack is prevented by ICD. From the experience of this case, electronic circuit of CI is thought to tolerate emergency countershock if the speech processor is removed.

Swallow-induced syncope and carotid sinus hypersensitivity: Coincident or associated conditions?

Swallow induced syncope is a rare clinical condition which is thought to result from an abnormal vagal reflex leading to bradycardia and cerebral hypoperfusion. It mostly occurs in patients with organic or functional disorders of the esophagus, and often requires permanent pacemaker implantation, along with treatment of the underlying esophageal pathology. In the following case, we report of a 71-year-old male with achalasia post per oral endoscopic myectomy, who presented with syncope and documented AV-block while eating solid food. In addition, long sinus pauses were noted during carotid sinus massage, suggesting that the two distinct entities may be associated, and further supporting the mechanism of neurally mediated syncope in the pathophysiology of swallow-induced syncope.

Two Cases of LQT Syndrome with Malignant Syncope after Switch from Propranolol to Bisoprolol.

Propranolol in slow-release form has been the first-line treatment in long QT (LQT) until it was withdrawn from the market. We describe two cases where a switch to bisoprolol resulted in worsening of arrhythmia control: A man with LQT2, asymptomatic on propranolol, experienced syncope after switching to bisoprolol 5 mg daily. He switched back to propranolol and has remained asymptomatic during subsequent 12 months. A man with classical Jervell Lange-Nielsen syndrome, previous gangliectomy, and ICD implantation, switched to bisoprolol 5 mg daily. Four months later he experienced a tachycardia storm. He switched back to propranolol and has remained free from arrhythmias during subsequent 12 months.

Twelve tips for medical students to maximise learning in theatre.

BACKGROUND: Introduction into the clinical environment can be a daunting experience for medical students, especially in the operating theatre. Prior knowledge of how to prepare for theatre and cope with surgical placements is advantageous, as learning opportunities can be maximised from the start. AIM: This article provides medical students with 12 tips devised to help make the most out of their initial theatre placements. METHODS: Tips were formulated based on the experiences of three senior medical students and a review of the literature. RESULTS: The 12 tips are (1) Know the patient and procedure, (2) Be familiar with your surgical department, (3) Familiarise yourself with different surgical attire, (4) Revise your clinical skills, (5) Be time-efficient, (6) Learn how to work in a sterile environment, (7) Avoiding syncope, (8) Impress the operating surgeon, (9) Be aware of the professional, ethical, and legal issues in surgery, (10) Use mentors to enhance your learning, (11) Embrace extra-curricular activities to enhance your insight into surgery and (12) Be acquainted with relevant support systems. CONCLUSIONS: These 12 tips provide guidance and opportunities to maximise learning for new clinical-phase medical students being introduced to the operating theatre for the first time.

[Acute jaw-thoracic pain and syncope in a 41-year-old man]. / Akuter Kiefer-Thorax-Schmerz und Synkope bei einem 41-jährigen Patienten.

A 41-year-old physically active man with no significant past medical history presented with sudden thoracic pain. The patient was referred to the next tertiary care hospital. A CT scan showed an ectasia of the ascending aorta with irregularities of the aortic wall without dissection. Despite initial refusal, the patient was referred to a university hospital with experience in aortic surgery. A triphase ECG-synchronized cardiothoracic flash protocol performed on a 256 line CT scanner confirmed an aortic intramural hematoma and a covered aortic perforation. Shortly afterwards the patient collapsed and had to be resuscitated.

G-suited for prevention of syncope in patients with vasovagal syncope: a pilot study.

BACKGROUND: Vasovagal syncope (VVS) represents by far the most common cause of syncope as it is diagnosed in around 50% of all patients that come to an emergency department. Although VVS is not fatal, it can cause an injury. Even serious injuries are not common, but there are reports of serious injuries of up to 5%. There are no current studies that demonstrate the effectiveness of any treatment. Past studies found that an Anti-Gravity suit (G-suit) can increase blood pressure and has been reported to prevent orthostatic hypotension effectively in patients with diabetes. It is possible that the G-suit can prevent VVS. OBJECTIVE: In the present study, the authors assessed the efficacy of G-suit for vasovagal syncope prevention. MATERIAL AND METHOD: In this open-label, randomized controlled study, we used the Italian tilt protocol, namely 60 degree passive tilting followed by 0.4 mg nitroglycerin challenge when the passive phase fails to induce syncope. If test was positive, then patient was enrolled. Tilt table test was repeated to compare G-suited and no G-suited to assess efficacy of G-suit for vasovagal syncope prevention. RESULTS: 10 patients were enrolled. There is no difference between the control group and an experimental group. In this study there is no cardio-inhibition vasovagal syncope. Positive tilt table test occurred in 50% of the patients receiving G-suited and 100% in control group (p 0.133). CONCLUSION: G-suit is unable to prevent syncope in patients with positive tilt table test but the result is not statistically significant. However, the number of patients may be too small.

Presumed mitochondrial disease manifesting with recurrent syncopes.

OBJECTIVES: Loss of consciousness may be due to neurological or cardiac involvement in mitochondrial disease, and is often difficult to attribute to either cause, as in the following case. CASE REPORT: A 67-year-old man with hypertension, diabetes, elevated serum creatine kinase, glaucoma, optic atrophy, and vertigo had experienced recurrent losses of consciousness since 63 years of age. Diagnostic work-up revealed paroxysmal supraventricular arrhythmias, hyperlipidemia, steatosis hepatis, renal insufficiency, polyneuropathy, first-degree atrio-ventricular block, orthostasis, and cataract. From the age of 66 years, he developed tonic-clonic seizures. Electrocardiography loop recording showed some losses of consciousness as associated with supraventricular tachycardias and others with epileptic activity or arterial hypotension. Neurological investigations and muscle biopsy were indicative of mitochondrial disease with multisystem involvement. Losses of consciousness disappeared after catheter ablation and treatment with levetiracetam. CONCLUSION: Recurrent loss of consciousness in mitochondrial disease may not only be due to arrhythmias but also seizure activity, or autonomic neuropathy. Arrhythmias, seizures, and polyneuropathy may have a common underlying cause affecting various tissues.

Diagnosis and management of inherited cardiomyopathies.

Inherited heart conditions are the most common cause of sudden cardiac death in those under the age of 35 and the leading cause of non-traumatic death in young athletes. Hypertrophic cardiomyopathy (HCM) is the most common inherited heart disease affecting 1 in 500 of the population. Some patients may exhibit severe left ventricular hypertrophy, others may show nothing more than an abnormal ECG. Left ventricular hypertrophy most commonly manifests in the second decade of life. Sudden death is rare and usually affects patients in the first three decades whereas older patients present with heart failure, atrial fibrillation and stroke. Arrhythmogenic right ventricular cardiomyopathy is a rare, autosomal dominant heart muscle disorder which affects between 1 in 1,000 and 1 in 5,000 of the population. Dilated cardiomyopathy (DCM) is characterised by a dilated left ventricle with impaired function that cannot be explained by ischaemic heart disease, hypertension or valvular heart disease. At least 25% of cases of DCM are familial. DCM may be associated with multisystem conditions such as muscular dystrophy. Chemotherapy and certain other drugs, alcohol abuse and myocarditis may also lead to a dilated and poorly contracting left ventricle. In many cases the first manifestation of an inherited cardiomyopathy can be a sudden cardiac arrest. Other presentations include chest pain or breathlessness during exertion, palpitations and syncope. In many of the cardiomyopathies, the diagnosis can be made with a standard ECG and echocardiogram. However if the diagnosis is not certain or the cardiologist wishes to look at the heart structure in greater detail, a cardiac MRI may be performed.

[72-year-old man with syncope and left hemiparesis]. / 72-jähriger Patient mit Synkope und linksseitiger Hemisymptomatik.

A 72-year-old man was admitted with left hemiparesis after a painless syncope with suspected ischemic stroke. So far he was a healthy man without any cardiovascular risk factors. A computed tomography scan of the head did not show any abnormal findings. In the clinical evaluation we revealed pulse deficits in the left-sided extremities and the blood pressure of the left arm was not measurable. The final diagnosis was an acute aortic dissection, beginning in the proximal portion of the ascending aorta, leading to the iliacal arteries. The patient was transferred and surgery was performed immediately with success. Painless acute aortic dissection presenting only with neurologic symptoms made the correct diagnosis extremely difficult. Correct diagnosis, however, is essential, otherwise thrombolytic therapy - indicated for acute-stage cerebral infarction - would have been performed, probably with a fatal outcome.

Long-term follow-up of seizure outcomes after corpus callosotomy.

PURPOSE: Corpus callosotomy can be an effective surgical treatment for medically intractable generalized seizures, particularly for drop attacks. We studied long-term seizure outcomes after callosotomy, mainly focusing on drop attacks as the seizure type. METHODS: This study reviews 78 patients who underwent callosotomy and were followed up for more than 3 years after surgery. Seizure outcome of callosotomy was analyzed for seizure type, including drop attacks and other types of seizures. RESULTS: The followed-up time ranged from 3 to 13 years (mean; 7.0+/-2.9 years, median; 8 years). When callosotomy was total section, drop attack seizure-free rate was 90%. However, partial section yielded a drop seizure-free rate of only 54%. Thirty-five of the 46 (76%) patients who were free of drop attacks 6 years after callosotomy had no relapse thereafter. Relapse of drop attacks was also significantly different depending on the range of callosotomy. With total section, only 7% showed relapse of drop attacks. On the contrary, patients with partial section had a 31% relapse rate. In 21% patients, postural seizures newly developed after callosotomy. CONCLUSIONS: These findings confirmed that callosotomy is the treatment of choice for disabling generalized seizures, especially for drop attacks. Total section is far more effective than partial section in terms of control of drop attacks and prevention of seizure relapse. However, new types of seizure could occur after callosotomy. When newly developed postural seizures were very severe, patients may fall due to sudden torsion of body, but the entire process of falling was not as sudden as that observed during previous drop attacks.

Prevention of human papillomavirus infection: provisional recommendations for immunization of girls and women with quadrivalent human papillomavirus vaccine.

This policy statement contains provisional recommendations for use of the quadrivalent human papillomavirus vaccine in girls and women. A full policy statement from the American Academy of Pediatrics is forthcoming.

Efficacy and safety of radiosurgical callosotomy: a retrospective analysis.

PURPOSE: Anterior callosotomy is a surgical option for the treatment of generalized tonic or atonic seizures associated with drop attacks. Besides open surgery, a radiosurgical callosal disconnection using the gamma knife (GK) also can be performed, but reliable data about tolerability and efficacy are sparse. METHODS: Eight patients (three female, five male age range, 5 to 69 years) with severe generalized epilepsy associated with disabling drop attacks underwent GK callosotomy between 1993 and 2004. In six patients, the anterior third of the corpus callosum was radiosurgically disconnected. In one patient a second procedure with GK treatment of the middle third of the corpus callosum was added 17 months later. In two patients posterior GK callosotomy had followed partial hemispherotomy. RESULTS: Drop attacks (DAs) were completely abolished in three patients, and two patients had a marked DA seizure reduction of 60%. Two of four patients with additional generalized tonic-clonic seizures showed a reduction of 100%, and the remaining, a 50% and 60% decrease, respectively. Other seizure types responded less well to the radiosurgical treatment. In both patients with posterior GK callosotomy after hemispherotomy, partial seizures decreased. Beside transient headache in two patients, no immediate or long-term postradiosurgical side effects were observed. CONCLUSIONS: Palliative radiosurgical callosotomy is an efficient and safe noninvasive alternative to the open procedure with comparable results. No signs of postradiosurgical side effects were noted within an up to 12-year posttreatment period.