Sudden death as a presenting symptom of hypertrophic cardiomyopathy: treatment with an implantable cardioverter defibrillator

Aborted sudden death as the presenting manifestation of hypertrophic cardiomyopathy in a 14-year-old child is reported. Documented ventricular fibrillation was the cause of cardiac arrest. No ventricular arrhythmia was induced during programmed electrical stimulation. An implantable cardioverter-defibrillator was indicated. As the patient had a family history of myocardial disease, he had undergone a cardiovascular evaluation 4 years before the major event, and was found normal. It is suggested that normal physical examination, ECG, echocardiogram should not rule out the diagnosis of hypertrophic cardiomyopathy when a family history is present. Left ventricular hypertrophy may develop during childhood in patients with hypertrophic cardiomyopathy.     

Influence of the implantable cardioverter defibrillator on survival: retrospective studies and prospective trials

Frequent losses of heterozygosity observed at several chromosomal loci in primary lung cancers have indicated the existence of several tumor suppressor genes associated with this type of cancer. We have examined loss of heterozygosity on chromosomal arm 8p in 49 cases of non-small cell lung carcinoma, using 14 restriction fragment length polymorphism markers. Of 42 cases informative with at least one marker, 21 showed allelic loss, including 15 of 32 adenocarcinomas and 5 of 9 squamous cell carcinomas. The frequency of allelic loss on 8p was similar at all clinical stages. Deletion mapping defined a single common region of deletion in these tumors within an 8 cM interval at 8p21.3-p22 flanked by the loci defined by cMSR-32 and cC18-245.     

Bronchopericardial fistula after placement of an automatic implantable cardioverter defibrillator: radiographic and CT findings

OBJECTIVE: We describe our experience with bronchopericardial fistula as a complication of infection in patients who have undergone placement of automatic implantable cardioverter defibrillator systems. CONCLUSION: Bronchopericardial fistula should be suspected in patients who present with hemoptysis and who have undergone placement of an automatic implantable cardioverter defibrillator using pericardial or epicardial defibrillator patches. Air between a defibrillator patch and the heart on chest radiographs or CT is diagnostic.     

Compatibility of a nonthoracotomy lead system with a biphasic implantable cardioverter-defibrillator. Cadence-Endotak 60-Series IDE investigators

This prospective multicenter study was conducted under the Food and Drug Administration Investigational Device Exemption to evaluate the safety and efficacy of the combination of the Cadence implantable defibrillator (Ventritex, Inc.) and 60-series Endotak C leads (Cardiac Pacemakers, Inc.). Implantation was attempted in 148 patients with hemodynamically compromising ventricular tachycardia or fibrillation (VF), or with pace-terminable ventricular tachycardia. The system was successfully implanted in 97% of patients, with 96% of implants in a transvenous-lead-alone configuration. At implantation, the defibrillation threshold was 455 +/- 94 V (14 +/- 6 J) for lead-alone patients and 532 +/- 40 V (19 +/- 3 J) for those requiring a subcutaneous patch. VF conversion efficacy was reconfirmed in patients who underwent a 3-month chronic induction study. The system successfully detected all 763 induced arrhythmias and terminated 99.5% of them; after system modification, successful conversion was demonstrated in the 2 patients who initially had induced episodes requiring external defibrillation (1 lead revision; 1 reprogramming). All spontaneous episodes were terminated with an implantable-cardioverter defibrillator. Postshock VF redetection times were significantly shorter than initial detection times (4.5 +/- 1.8 seconds detection, 2.1 +/- 0.7 seconds redetection; p<0.0001). During an 8-month mean follow-up (range 1 to 31 months), 2 unwitnessed deaths were classified as sudden cardiac deaths, and 11 patients experienced a total of 12 complications, none of which was associated with the Cadence-Endotak combination.     

Use of atrial and ventricular electrograms from a dual chamber implantable cardioverter defibrillator to elucidate a complex dysrhythmia

Stored intracardiac electrograms provided by third-generation ICDs have proved their use in the analysis of the mechanism of tachydysrhythmic events. There are cases in which the analysis of ventricular electrograms is insufficient for the elucidation of certain dysrhythmias. The availability of atrial electrograms provided by dual chamber ICDs improves the diagnostic capability of electrogram analysis and could prove most useful especially in complex dysrhythmias.     

Problems in managing patients with long QT syndrome and implantable cardioverter defibrillators: a report of two cases

Implantable cardioverter-defibrillators (ICDs) have been a successful adjunct to the management of arrhythmias in patients with Long QT syndrome (LQTS). In two patients, interactions between LQTS and the ICD were diagnosed and corrected. Oversensing of T waves was confirmed in the first, while in the second, the arrhythmia disappeared when T wave abnormalities improved after cessation of H2 blocker therapy. In patients with LQTS and an ICD, T wave oversensing should be considered. Interventions that may have an adverse effect on repolarization should be avoided.     

Intracardiac QRS electrogram width--an arrhythmia detection feature for implantable cardioverter defibrillators: exercise induced variation as a base for device programming

Delivery of inappropriate therapy of implantable cardioverter defibrillators (ICD) due to inaccurate arrhythmia detection represents a major clinical problem. Different arrhythmia detection criteria such as the "stability" of the cycle length or the suddenness of "onset" of tachycardia have been implemented in ICD software to prevent inappropriate therapy. The new Medtronic model 7223Cx ICD offers an additional detection parameter (QRS width), which reflects changes in the duration of ventricular depolarization as a tool to distinguish supraventricular from ventricular tachycardias. Although this criterion can be programmed based on ECG parameters derived from resting ECGs, this may not be sufficient since QRS width is subject to considerable changes due to transient myocardial ischemia, changes in autonomic tone, or frequency dependent effects of antiarrhythmic drugs. The present study aimed to determine frequency dependent changes in QRS width in individual patients at rest and during symptom-limited exercise testing in 16 patients with documented ventricular tachycardia (N = 13) or ventricular fibrillation (N = 3). The optimal EGM slew threshold and the individual variation of QRS width were determined. Measurements obtained at the end of the implantation procedure were compared to those performed at hospital discharge. The majority of patients showed a wider variation in QRS duration as measured from 30 consecutive cycles during exercise as compared to rest. For example, the QRS range (i.e., the difference between the maximal and the minimal QRS width measured) averaged 7 +/- 3 ms at rest and increased to 11 +/- 3 ms during exercise (P = 0.004) with an increase of > or = 4 ms observed in 11 (69%) of 16 patients. In 13 (81%) of 16 patients a reprogramming of at least one QRS width parameter from its value at the time of implantation was necessary. Thus, the QRS width measured from the intracardiac EGM shows significant intraindividual variations in different physiological conditions. For optimal programming of the QRS width parameter, measurements obtained during exercise are important.     

Convulsions with meningiomas: incidence and significance

In a study of a consecutive series of 127 surgically treated meningiomas, it was found that 29% of the patients had reported with convulsions as their initial symptom. In this group, surgical excision of the meningioma stopped the convulsions in about half of the patients, but the others continued to have seizures after their operations. Among those patients with meningiomas who did not have preoperative convulsions, about one-sixth (15 patients) developed postoperative seizures. Patients in both groups required prolonged anticonvulsant medication. Factors predisposing to the occurrence of postoperative seizures were the site of the tumor, faulty surgical technique, and a preoperative history of seizures.     

Antitachycardia pacing in patients with implantable cardioverter-defibrillators: inverse circadian variation of therapy success and acceleration

We analyzed spontaneous ventricular tachycardias treated by antitachycardia pacing during long-term follow-up in 138 recipients of an implantable cardioverter-defibrillator. An inverse circadian variation of the antitachycardia pacing termination and acceleration rates with the worst antitachycardia pacing success during the time period with the highest episode frequency (morning hours) was demonstrated.     

Syncope in patients with an implantable cardioverter-defibrillator: incidence, prediction and implications for driving restrictions

OBJECTIVES: This retrospective study was undertaken to provide information on occurrence, risk prediction and prevention of syncope in patients with an implantable cardioverter-defibrillator (ICD). BACKGROUND: ICDs effectively terminate ventricular tachycardia and fibrillation (VT/VF). Incapacitating symptoms, such as syncope, may still occur. METHODS: We performed a retrospective analysis of data from 421 patients (clinical history, outpatient chart reviews and episode data) with mean (+/-SD) follow-up of 26 +/- 18 months. RESULTS: Of 421 patients, 229 (54.4%) had recurrent VT/VF, and 62 (14.7%) had syncope. The actuarial survival rate free of VT/VF was 58%, 45% and 37% and that for survival free of syncope was 90%, 85% and 81% at 12, 24 and 36 months after implantation, respectively. Once VT/VF had occurred, 76%, 68% and 62% of patients remained free of syncope during the following 12, 24 and 36 months, and 68%, 64% and 56% remained free of second syncope 12, 24 and 36 months after first syncope, respectively. In cases of syncope, the mean cycle length (CL) of VT was 251 +/- 56 ms. A low baseline left ventricular ejection fraction (LVEF), induction of fast VT (CL <300 ms) during programmed ventricular stimulation and chronic atrial fibrillation (AF) were associated with an increased risk of syncope. If the LVEF was >40%, fast VT had not been induced, and patients had no chronic AF; 96%, 92% and 92% of patients remained free of syncope after 12, 24 and 36 months, respectively. Once patients had a VT recurrence, syncope during the first VT and a high VT rate were the strongest risk predictors of future syncope. CONCLUSIONS: Identification of patients with an ICD with a low and high risk of syncope seems to be feasible and might help as a guide to driving restrictions in such patients.     

Normal variations of the temporal bone on high-resolution CT: their incidence and clinical significance

We evaluated normal variations of the temporal bone on high-resolution computed tomograms (HR-CTs) and investigated their incidence. HR-CTs of the temporal bones of 325 patients were retrospectively reviewed. Six groups of variants, which were considered important for presurgical planning, were evaluated on HR-CTs. These included: (1) an incomplete bony covering of a high-positioned jugular bulb; (2) severe asymmetry of the jugular foramen; (3) an anteriorly located sigmoid sinus; (4) a deep sinus tympani; (5) a large internal auditory canal; and (6) a large cochlear aqueduct. The frequency of the variations were as follows: (1) 2.4%; (2) 4.0%; (3) 1.6%; (4) 5.9%; (5) 2.3%; and (6) 3.0%. Bilateral involvement with variation (4), (5) and (6) was frequently seen. Normal anatomical variations of the temporal bone are therefore not rare and awareness of the possible variants is necessary before surgery of the inner ear, middle ear and posterior fossa.     

Human papillomavirus detection in high-grade squamous intraepithelial lesions. Comparison of hybrid capture assay with a polymerase chain reaction system

The validity of human papillomavirus (HPV) detection using the hybrid capture assay (HCA) was compared with the polymerase chain reaction (PCR) in 38 patients with high-grade squamous intraepithelial lesions (HSILs). HCA and PCR showed 84% agreement for HPV detection. HCA missed a significant higher proportion of HSIL compared with PCR (21% vs. 5%; P = .04). Thus, the sensitivity of HCA should be increased before this test can be recommended for HSIL.     

Influence of polarity reversal on defibrillation success with biphasic shocks and a transvenous/subcutaneous defibrillator system in a porcine animal model

Clinical studies show that polarity reversal affects defibrillation success in transvenous monophasic defibrillators. Current devices use biphasic shocks for defibrillation. We investigated in a porcine animal model whether polarity reversal influences defibrillation success with biphasic shocks. In nine anesthetized, ventilated pigs, the defibrillation efficacy of biphasic shocks (14.3 ms and 10.8 ms pulse duration) with "initial polarity" (IP, distal electrode = cathode) and "reversed polarity" (RP, distal electrode = anode) delivered via a transvenous/subcutaneous lead system was compared. Voltage and current of each defibrillating pulse were recorded on an oscilloscope and impedance calculated as voltage divided by current. Cumulative defibrillation success was significantly higher for RP than for IP for both pulse durations (55% vs 44%, P = 0.019) for 14.3 ms (57% vs 45%, P < 0.05) and insignificantly higher for 10.8 ms (52% vs 42%, P = ns). Impedance was significantly lower with RP at the trailing edge of pulse 1 (IP: 44 +/- 8.4 vs RP: 37 +/- 9.3 with 14.3 ms, P < 0.001 and IP: 44 +/- 6.2 vs RP: 41 +/- 7.6 omega with 10.8 ms, P < 0.001) and the leading edge of pulse 2 (IP: 37 +/- 5 vs RP: 35 +/- 4.2 omega with 14.3 ms, P = 0.05 and IP: 37.5 +/- 3.7 vs RP: 36 +/- 5 omega with 10.8 ms, P = 0.02). In conclusion, in this animal model, internal defibrillation using the distal coil as anode results in higher defibrillation efficacy than using the distal coil as cathode. Calculated impedances show different courses throughout the shock pulses suggesting differences in current flow during the shock.     

Indwelling catheter and risk of urinary infection: a clinical investigation with a new closed-drainage system

In 108 cases a postmortal pancreatography followed by histological examination was performed. The size of the pancreas could be measured on X-ray films after complete acinar filling. In normal cases organ size was proportional to the diameter of the duct, the diameter ranging between 2 and 4 mm (average 3,7 mm). In subjects with chronic pancreatitis, the diameter varied from 4 to 8 mm (average 5.7 mm). From these results it could be concluded, that the S/D-quotient (pancreatic surface seen on X-ray in cm2 divided by the greatest diameter of the main duct in mm) might be an aid in establishing the diagnosis of chronic pancreatitis in doubtful cases. In chronic pancreatitis the width of the duct generally increases while the size of the organ decreases due to scar formation, thereby clearly reducing the S/D-quotient. The average quotient of our normal cases was 24,2 and that for cases of chronic pancreatitis was 12,0.     

Hymenoptera sting anaphylaxis: detection and clinical significance of individual bee and wasp venoms specific IgE and IgG4 antibodies

In Japan, an average of 37 fatalities per year related to bee or wasp stings were reported during the years 1979-1988. To confirm fatal anaphylaxis serologically, we measured bee or wasp venom specific IgE (sIgE) and IgG4 (sIgG4) antibodies in the sera of 22 patients who visited hospitals with either allergic or anaphylactic reactions after bee or wasp stings by using Enzyme-Linked Immunosorbent Assay. Specific IgE or IgG4 antibodies against Polistes apachus (Paper wasp) and/or a mixture of Polistes annularis, P. exceramans, P. fuscatus, and P. metricus venoms of Polistes genus were detected in 11 patients and the detection frequencies were the highest among positive antibodies against bee or wasp venoms. The severity of allergic reactions was graded from 0-4 according to Mueller. The detection frequencies of sIgE in Mueller grade 0-3 patients were in the range of 33% to 67%, and in grade 4 were 100%. Whereas, the detection frequency of sIgG4 was high (67-100%) in grade 2-4 patients. Especially high levels of sIgE and sIgG4 were detected in patients experiencing the most severe clinical reactions. The detection of venom specific IgE and IgG4 antibodies appears to be useful when determining bee or wasp stings as the cause of fatalities.     

Psychiatric disorder in a birth cohort of young adults: Prevalence, comorbidity, clinical significance, and new case incidence from ages 11–21.

Mental health data were gathered at ages 11, 13, 15, 18, and 21 in an epidemiological sample using standardized diagnostic assessments. Prevalence of Diagnostic and Statistical Manual of Mental Disorders-III-Revised (DSM-III-R) mental disorders increased longitudinally from late childhood (18%) through mid- (22%) to late-adolescence (41%) and young adulthood (40%). Nearly half of age-21 cases had comorbid diagnoses; and comorbidity was associated with severity of impairment. The incidence of cases with adult onset was only 10.6%: 73.8% of adults diagnosed at age 21 had a developmental history of mental disorder. Relative to new cases, those with developmental histories were more severely impaired and more likely to have comorbid diagnoses. The high prevalence rate and significant impairment associated with a diagnosis of mental disorder suggests that treatment resources need to target the young adult sector of the population. The low new-case incidence in young adulthood, however, suggests that primary prevention and etiological research efforts need to target children and adolescents. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

系统性红斑狼疮患者血清增殖诱导配体水平的检测及其临床意义

目的:检测系统性红斑狼疮(SLE)和类风湿性关节炎(RA)患者血清中增殖诱导配体(APRIL)的水平,探讨APRIL水平与SLE患者临床指标的相关性及APRIL在SLE发病中的作用.方法:采用ELISA法检测SLE患者组(48例)、RA患者组(16例)和正常对照组(16例)血清APRIL水平,并与SLE活动指数(SLEDAI)及实验室指标进行对比分析.结果:SLE组和RA组血清APRIL水平高于正常对照组(P<0.01),SLE组高于RA组(P<0.01).SLE组中抗Sm抗体阳性患者APRIL水平高于阴性患者( P<0.05).抗U1-RNP抗体阳性患者APRIL水平高于阴性患者( P<0.05),抗SSA /SSB抗体、抗ACL抗体、抗ds-DNA抗体阳性和阴性患者APRIL水平差异无统计学意义.SLE患者血清APRIL水平与其补体C3、C4呈负相关关系(r1=-0.819,P<0.01;r2=-0.549,P<0.01),与SLEDAI评分、免疫球蛋白、抗核抗体谱等免疫学指标无相关性.结论:APRIL在SLE患者中特异性升高,可能在狼疮发病中起重要作用;血清APRIL水平可能与SLE患者疾病活动程度有关联,但尚不能确定是否可作为疾病活动性指标.     

Interactive MR imaging-guided biopsy and aspiration with a modified clinical C-arm system

OBJECTIVE: This investigation was performed to test the hypotheses that interactive guidance of MR image acquisition during needle-directed procedures using a clinical 0.2-T C-arm open MR imaging system integrated with a frameless optically linked stereotaxy system is feasible, and that procedure times can be sufficiently short to be well tolerated by the patient. SUBJECTS AND METHODS: One hundred six MR-guided procedures were performed in 86 patients (ranging in age from 5 months to 88 years) using a clinical C-arm imaging system supplemented with an in-room RF-shielded liquid crystal display monitor, a frameless stereotaxy system, rapid gradient-echo sequences for needle guidance, and MR-compatible monitoring and surgical lighting equipment. We performed 50 biopsies and aspirations of the head and neck in 37 patients, 23 biopsies of musculoskeletal lesions in 22 patients, 16 biopsies of abdominal sites in 10 patients, six biopsies of the thoracolumbar spine or sacrum in six patients, and 11 shoulder joint injections for MR arthrography in 11 patients, in addition to 38 MR arthrographic injections on the same imaging system described in a previous report. Tissue sampling included fine-needle aspiration (n = 90) and cutting needle core biopsy (n = 41). Thirty-five patients underwent both procedures. Procedures were evaluated for success of needle placement, procedure time, and complications. RESULTS: Needle placement was successful in all cases, and no complications occurred. Tissue was sufficient for pathologic diagnosis for all but eight patients. Passes per patient averaged 2.1. For fine-needle aspiration, instrument time averaged 7 min 42 sec per pass, cutting needle core biopsy averaged 6 min 24 sec, and shoulder injection averaged 8 min. CONCLUSION: MR imaging guidance for needle procedures on a clinical 0.2-T C-arm system with supplemental interventional accessories is feasible, with relatively rapid needle placement.