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Key Teaching Points • Late-onset asystole is a rare but significant complication in patients with a vagal nerve stimulator of which the clinician needs to be aware. A good clinical suspicion, thorough history, and physical examination are key to help discern cardiac vs neurogenic causes of syncope. • Autonomic neural input to the heart exhibits some “sidedness,” with the left vagal nerve having more influence on the atrioventricular node and right vagal nerve having more influence on the sinus node, which is why the stimulator is placed on the left side of the chest. This is thought to be secondary to the embryonic rotation of the body during development. However, it is important to know that there is some degree of overlap between the left and right vagal nerve innervation. • Very few cases of late-onset syncopal events associated with vagal nerve stimulation therapy have been reported that were treated with removal or deactivation of the device. Another approach could be to implant a permanent pacemaker in a patient who would not tolerate removal of the stimulator owing to intractable epilepsy.

Cardiac contractility modulation (CCM) is an investigational device-based therapy to enhance ventricular contractility in systolic heart failure patients who are not candidates for cardiac resynchronization therapy (CRT) owing to the absence of wide QRS complexes or who have failed to respond on CRT. The principal mechanism is based on the stimulation of cardiac muscles by nonexcitatory electrical signals to augment the influx of calcium ions into the cardiomyocytes. The majority of patients receiving CCM therapy have concurrent implantable cardioverter defibrillators, and the manufacturer declares both devices can be used in parallel without any interactions. Nevertheless, proper lead positioning of both devices are crucial, and it is mandatory to check device-device interactions during each and every cardiac electronic implantable device-related procedure to prevent adverse outcomes.

Infective endocarditis (IE) can lead to significant morbidity and mortality without appropriate treatment. Modified Duke Criteria are accepted by many professional societies to establish the diagnosis of IE, and cardiac imaging is one of the major diagnostic criteria. Transesophageal echocardiography is an algorithmic escalation to diagnose IE when transthoracic echo does not appreciate a positive finding. In patients with contraindications to transesophageal echocardiography, cardiac magnetic resonance imaging, cardiac computed tomography (CT), cardiac CT angiography, and fluorodeoxyglucose positron emission tomography with CT or CT angiography may be alternative diagnostic tools. However, these imaging modalities have their own limitations such as local unavailability, the presence of non–magnetic resonance imaging compatible implants, or impaired renal function. Intracardiac echocardiography could be a considerable alternative under those circumstances.

Bidirectional ventricular tachycardia (BVT) is a rare variety of tachycardia with morphologically distinct presentation: The QRS axis and/or morphology is alternating in the frontal plane leads. Since its original description in association with digitalis,1 numerous cases of this fascinating tachycardia with disparate etiologies and mechanisms have been postulated. We report a patient with BVT in association with non-ST elevation myocardial infarction and severe cardiomyopathy in the absence of digoxin toxicity.

Invasive ductal carcinoma (IDC) is the most common subtype of breast tumor. There were many cases reported about the treatment and adjuvant therapies. The simultaneously occurrence of breast carcinoma with cutaneous myiasis is, to our knowledge, a unique presentation. A 50-year-old female known case of breast cancer presented to the surgical department at Ziauddin Hospital Karachi with complaints of pain, redness, blackening, and a foul smelling, discharging wound on her left breast. The wound was debrided thoroughly with povidone-iodine and about 52 maggots were removed, which were identified asChrysomya bezziana. The patient was hospitalized and received amoxicillin and ivermectin according to protocol. This case report is pertinent to public health professionals and oncologists in the view of the social impact of myiasis.

Patient: Male, 41 Final Diagnosis: Sick Sinus Syndrome and absent left main coronary artery • separate ostia of left anterior descending and circumflex arteries in Holt-Oram Syndrome Symptoms: Conduction disturbance • seizure-like activity • upper extremity malformations Medication: — Clinical Procedure: Electro physiology study • coronary catheterization • pacemaker Specialty: Cardiology Objective: Congenital defects/diseases Background: Holt-Oram syndrome (HOS) is a rare but significant syndrome consisting of structural heart defects, conduction abnormalities, and upper extremity anomalies. It was first described in the British Heart Journal in 1960 by Mary Holt and Samuel Oram as a report of atrial septal defect, conduction disturbances, and hand malformations occurring in family members. Patients can present with heart blocks or symptoms of underlying congenital heart defects. Case Report: A 41-year-old man with Holt-Oram syndrome presented with seizure-like activity and was found to have an underlying conduction disturbance. Physical exam showed bilateral atrophic upper extremities with anatomic disfiguration, and weakness of the intrinsic hand muscles. Cardiovascular exam revealed a slow heart rate with irregular rhythm. EKG showed sinus arrest with junctional escape rhythm. Cardiac catheterization revealed coronary anomalies, including absent left main coronary artery and separate ostia of the left anterior ascending and left circumflex coronary artery. Coronary arteries were patent. Following electrophysiology study, sick sinus syndrome and AV block were diagnosed, and the patient received implantation of a permanent pacemaker. Conclusions: This patient presented with a seizure-like episode attributed to hypoxia during asystole from an underlying cardiac conduction defect associated with Holt-Oram syndrome. Arrhythmias and heart blocks are seen in these patients, and conduction defects are highly associated with congenital heart defects. Holt-Oram syndrome rarely presents with coronary artery anomalies. There is no reported case of separate coronary ostia and absent left main coronary artery. Prompt diagnosis is important since anomalies in coronary and upper extremity vasculature might be challenging for invasive procedures.

We report a case of 78-year-old man admitted to the hospital due to palpitations and lightheadedness. On EKG advanced atrioventricular block with ventricular rate of 37 beats per minute was noted. On electrophysiology study a common type of atrioventricular nodal reentrant tachycardia was inducible with maintenance of advanced AV block. Radiofrequency ablation of slow pathway followed by placement of a permanent pacemaker resulted in elimination of tachycardia and resolution of symptoms.

Bilateral testicular tumours are rare and 80% occur metachronously. Synchronous bilateral testicular tumours are rare, and bilateral synchronous testicular tumours in bilateral cryptorchidism are extremely rare with only few cases reported previously. A 36 year old male patient presented to surgical OP with complaints of pain in scrotal region of both sides since 6-8 months. He was asymptomatic previously except for complaints of empty scrotal sac. Magnetic resonance imaging performed 6 months back revealed bilateral large oval to mildly irregular inguinal swellings with iso to hyperintense areas measuring 36×23×20 mm on right side and 34×26×20 mm on left side and patient was diagnosed with bilateral undescended testis. Bilateral orchidectomy was performed and the resected specimen was subjected to histopathologic examination. Gross visual examination of the bilateral orchidectomy specimen revealed the larger specimen to measure 7×4×1 cm with grey coloured external surface. Cut section revealed testis measuring 3.5×2 cm and surrounded with fibro adipose tissue. The testicle shows grey brown and grey white areas surrounding a central yellow nodule measuring 1×0.8 cm. The other mass had a yellowish nodular external surface and measured 5.5×4×1 cm. On cut section, normal testicular tissue could not be identified and the entire testis appeared to be replaced by a solid yellowish white homogenous tumour. Sections from the yellowish smaller mass revealed tumour composed of sheets and trabeculae of large polygonal cells with large vesicular nuclei with prominent nucleoli and abundant clear to vacuolated cytoplasm. Focal collections of histiocytes and occasional multinucleated giant cells were also seen. Sections from the other larger mass showed a thick fibrotic tunica albuginea with sclerosed and hypoplastic seminiferous tubules in areas of dense hyaline fibrosis. In the centre, a well circumscribed tumour was seen showing similar histologic features as in the contralateral mass. Epididymis did not show tumour infiltration.

Implantable cardioverter defibrillators have an established role in the management of life-threatening tachyarrhythmias. These devices use sophisticated sensing circuitry to detect and promptly treat a vast majority of these arrhythmias. However, they are not foolproof. We report one case where the device failed to sense every other QRS complex during induced ventricular fibrillation due to marked electrical alterans. Thus, undersensing can be a potentially fatal problem despite the use of auto adjustable gain.

Aims/objectives: Patients with implantable cardioverter defibrillators (ICD) often receive an adjunctive anti-arrhythmic therapy. We propose that an addition of spironolactone will reduce the number of clinically significant ventricular arrhythmias and ICD-related therapies. Methods and results: In a multicentre retrospective study, 64 patients with ischaemic and non-ischaemic dilated cardiomyopathy whose left ventricular ejection fraction (LVEF) was

A 70 year old lady presented with symptoms of changed bowel movements and rectal bleeding. She was operated and the resected specimen of intestine was brought to Department of pathology for histopathological examination. The specimen was fixed in 10% formal saline. Tissue bits were taken from the representative areas in the mass for further fixation and processing. Sections were stained with hematoxylin and eosin and thoroughly examined under microscope for establishing the diagnosis of Spindle variant of melanotic melanoma. Anorectal melanomas are rare malignancies and an infrequent occurrence in the seventh decade of life. The aim was to evaluate a pigmented anorectal mass and establish its diagnosis in a 70 year old woman by thorough histopathological examination.

Background: Chronic kidney disease (CKD) has been linked to serious arrhythmias. We studied the impact of CKD upon implantable cardioverter-defibrillator (ICD) recipients. Methods and Results: Baseline estimated glomerular filtration rate (eGFR) was calculated from variables at the time of ICD implantation in 95 patients. Patients with eGFR below 60 ml/min and those with end-stage renal disease (ESRD) were considered to have significant CKD. Among 95 patients who underwent ICD implantation for VT/VF, the mean age was 66.5±12.2 years, 27 (29.0%) were women and 20 (21.5%) were African American. The CKD groups (eGFR < 60 ml/min and ESRD) and control group (eGFR≥60 ml/min) were similar with respect to background histories and medications. A significant difference in all-cause mortality in those with eGFR≥60 ml/min, 3 patients (8.6%), compared to either those with eGFR < 60 ml/min, 28 (60.9%), or ESRD 7 patients (58.3%), p < 0.0001, was noted. Proportionately more patients died from arrhythmic deaths in those with eGFR < 60 ml/min, 8 patients (17.39%) and ESRD 3 patients (25%), than those with eGFR ≥60 ml/min, no patient. P ≤ 0.0001. There was progressive increase in DFT's with worsening renal failure. The Cox proportional hazards model for time until death, found independent predictors to be: age, OR = 1.04 (per year), 95% CI 1.00–1.08, p = 0.04; CKD group, OR 2.59, 95% CI 1.27–5.30, p = 0.009; and use of beta-blockers, OR 0.25, 95% CI 0.10–0.61, p = 0.002. Conclusions: Significant CKD was related to overall poor survival, arrhythmic death and high DFTs.

Internal component failure of the Implantable Cardioverter Defibrillators (ICD) is a rare complication, which can have a potentially catastrophic outcome. We describe a patient with random component failure, which occurred due to electrical overstress damage to the high voltage hybrid as a result of inappropriate arcing, as detected by the manufacturer. This in turn damaged other components in the device. This secondary damage caused the device to reset, emitting a constant tone. A high current state was created, thereby depleting the battery prematurely. The problem was promptly detected and rectified by ICD generator replacement.

EP Characteristics in Familial WPW. Introduction: A familial form of Wolff-Parkinson-White syndrome (WPW) occurs in association with hypertrophic cardiomyopathy and intraventricular conduction ahnormalities. This syndrome, demonstrating autosomal dominant inheritance and segregating with a high degree of penetrance but variable expressivity, has been genetically linked to chromosome 7q3. The purpose of this study is to detail the electrophysiologic characteristics of accessory atrioventricular connections (AC) in four members of a kindred with this syndrome. Methods and Results: We clinically evaluated 32 members of a single kindred and identified 20 individuals with ventricular preexcitation, abnormal intraventricular conduction including complete AV block and/or ventricular hypertrophy, Genetic linkage analysis mapped the disease gene in this kindred to the chromosome 7q3 locus (maximum logarithm of the odds score = 6.88, θ= 0); recombination events in affected individuals reduced the genetic interval from 7 centimorgans (cM) to 5 cM. Electrophysiologic study of four individuals with preexcitation, identified seven AC (1 right sided, 3 septal, and 3 left sided). All four individuals had inducible orthodromic tachycardia; while three had multiple AC. Bidirectional conduction was demonstrated in 6 of 7 AC. Successful ablation was accomplished in 5 of 7 AC. Conclusion: The electrophysiologic characteristics and location of AC in family members having this complex cardiac phenotype are similar to those seen in individuals with isolated WPW. Identification of WPW in more than one family member should prompt clinical evaluation of relatives for additional findings of ventricular hypertrophy or conduction abnormalities.(J Cardiovasc Electrophysiol. Vol. 10, pp. 629-635, May 1999)

Vasodepressor syncope is believed to be preceded by hypercontractility which leads to mechanoreceptor stimulation. Therefore, a preserved left ventricular function is supposedly required to trigger the reflex. We analyzed our experience in patients with positive head-up tilt test. Out of 500 patients 32 had previous history of structural heart disease. Of them 8 patients had severe left ventricular dysfunction (EF l 30%). The mean age was 52 ± 8 years, 5 were male and 3 female. Mean EF was 22 ± 5%. Six patients had dilated cardiomyopathy and 2 had myocardial infarction. Electrophysiologic study was negative in 6 patients while the remaining two had inducible sustained monomorphic ventricular tachycardia requiring defibrillator implant. Syncope was witnessed by physicians in 6 out of 8 patients and was associated with bradycardia and hypotension. In all 8 patients head-up tilt reproduced symptoms. Upright tilt was positive at baseline in 7 patients and during isoproterenol infusion in 1 patient. Five patients were treated with theophylline, 1 with ephedrine, and 2 with disopyramide. After a mean follow-up of 2.2 ± 1.3 years all 8 patients were free of vasodepressor-related syncope. Conclusion We conclude that vasodepressor syncope is possible even in the presence of severely impaired ventricular function and should, therefore, be entertained in such cases. Alternative mechanisms may mediate the circulatory response in this population.

Objectives. This study analyzed the advantage of combining a biphasic device with a transvenous system and compared the results with those obtained with a standard monophasic device. Background. Available lead system use monophasic pulses and may require lengthy intraoprative testing to achieve adequate defibrillation threshold in a conspicuous number of patients. The option of biphasis waveform may provide further benefits. However, clinical experience with a permanent implant is lacking. Methods. Fifty-five patients underwent testing and received a permanent implant using the Endotak lead system associated with a CPI monophasic device. The remaining 36 patients received a permanent implant with the Endotak lead system connected to a biphasic device. In both groups a subcutaneous patch was combined when needed to obtain acceptable defibrillation thresholds. Results. Biphasic pulses resulted in lower mean (±SD) defibrillation threshold (monophasic 15 ± 4.7 J vs. biphasic 12 ± 5 J, p = 0.03) and a better implantation rate (100% biphasic vs. 89% monophasic, p = 0.07). Biphasic pulses allowed implantation with less ventricular fibrillation induction (7.4 ± 3.2 vs. 3.5 ± 1.8, p < 0.01) and a mean shorter procedure time (168 ± 39 vs. 111 ± 30 min, p < 0.01). With the biphasic waveform a grater proportion of patients met the implantation criteria with the lead system alone (83% vs. 45%, p < 0.01). When needed, the left prepectoral location of the patch electrode was always sufficient in patients receiving the biphasic device, whereas placement in the left subscapular position was required in 15 patients in the monophaisic group. Implantation of the biphasic device was associated with a shorter mean hospital stay (3.8 ± 0.8 vs. 5.4 ± 2.2 days, p < 0.01). Conclusions. Incorporation of a biphasic device in a transvenous implantable cardioverter-defibrillator uniformly incresses the efficacy of the system and the case of implantation.