Apical hypertrophic cardiomyopathy is a rare form of hypertrophic cardiomyopathy that involves thickening of the distal portion of the left ventricular wall. Most commonly seen in the Japan, with a prevalence rate of about 15% of all HCM patient, its incidence in the USA is approximately 3% of HCM cases. We report a case of a 46-year-old woman with history of hypertension who presented to emergency department with worsening dyspnea and orthopnea with features of left ventricular hypertrophy (LVH) and diffuse large T-wave inversions in the lateral leads on a 12-lead ECG. Further work up revealed severe concentric LVH, with near obliteration of the LV cavity. Ventriculogram showed severe symmetric hypertrophy of the mid to lower septum, extending to the apex of left ventricle with significant pressure gradient of at least 160 mmHg across the apex to mid septal cavity, with no significant gradient across the left ventricular outflow tract. These findings were consistent with apical hypertrophic cardiomyopathy. She was treated with verapamil and metoprolol and has remained asymptomatic over last 2.5 years of follow-up. Although the clinical presentation of AHCM can be variable and nonspecific; however, hallmark findings on ECG and echo can be extremely important in its diagnosis.Abbreviations: AHCM: Apical hypertrophic cardiomyopathy; ECG: Electrocardiogram; LVH: Left ventricular hypertrophy; LVOT: Left ventricular outflow tract

Wellens’ syndrome is characterized by T-wave changes in electrocardiogram (EKG) during pain-free period in a patient with intermittent angina chest pain. It carries significant diagnostic and prognostic value because this syndrome represents a pre-infarction stage of coronary artery disease involving proximal left anterior descending (LAD) artery, which can subsequently lead to extensive anterior myocardial infarctions (MIs) and even death without coronary angioplasty. Therefore, it is crucial for every physician to recognize EKG features of Wellens’ syndrome in order to take appropriate immediate intervention to reduce mortality and morbidity for MI. Here, we report a case of an overweight man with 35 pack-year of smoking history who presented to Easton Hospital with intermittent pressing chest pain of 5/6 times within 10 day-period and was found to have type A Wellens’ sign, which was biphasic T-waves in precordial leads V2 and V3 during pain-free period with no cardiac enzymes elevation. He was given therapeutic lovenox and subsequently underwent coronary angioplasty and had 95–99% occlusion in proximal LAD artery. The unique feature of our case was that Wellens’ type B EKG changes were seen after reduction of stenosis with LAD artery stent, which was likely explained by the reperfusion of the ischemic myocardium. Therefore, it is important for physicians to recognize EKG features of Wellens’ syndrome in order to take appropriate therapy to reducing mortality and morbidity form impending MI.

Background: Clopidogrel (Plavix) is an antiplatelet medication that is routinely used in patients with cardiovascular disease. Cytochrome P2C19 enzymes play a major role in its metabolism, which determines its varied therapeutic level and its effectiveness.Objectives: To customize clopidogrel therapy and evaluate its efficacy by using CYP2C19 genotypic and phenotypic information to improve clinical outcomes in patients.Methods: A total of 465 patients with underlying cardiovascular disease were selected from our out-patient cardiology clinic. DNA sequences of CYP2C19 were analyzed in 465 patients.Results: Of 465 patients, 183 were wild-type homozygous (*1/*1) and 18.8% gain-of function and 19.8% loss-of-function alleles in our patient population The following changes were made: 1) Switching to prasugrel in patients whose genotype noted them to be “Slow metabolizers. This medication adjustment improved clinical outcomes in this patient group.2) Discontinuing or lowering clopidogrel doses in patients whose genotypes noted them to be “Fast or ultra-fast metabolizes” to decrease bleeding risk. For those who were not on clopidogrel but carried abnormal allele(s), “clopidogrel caution” was documented. These individuals were followed up for 3 years and there has not been any cardiac clinical symptoms, cardiac death or excessive bleeding reported.Conclusions: Given the varied effectiveness of clopidogrel due to its metabolism by CYP2C19 enzyme, and the relatively high frequency of both gain-of-function (18.8%) and loss-of-function (19.8%) alleles in our patient population, we believe that genotyping CYP2C19 is clinically important in order to improve patient outcomes and minimize patient risk.

BackgroundMyotonic dystrophy (DM) is an inherited progressive muscle disorder caused by defects in muscle proteins. As the incidence of this condition is low, not many are familiar with the multisystem involvement. At times, cardiac disease may even be the predominant manifestation in the form of arrhythmias, conduction defects, and cardiomyopathies. The progression of the disease can lead to sudden, unpredictable death. Thus, it is important to identify this subgroup and treat accordingly.ObjectiveTo identify patients with DM and assess their risk for sudden cardiac death.MethodsNine patients previously diagnosed with muscular dystrophy were evaluated by cardiologists for various reasons, from a general follow-up to cardiac arrest. All of them had electrocardiograms (EKG) and 2-D echocardiograms, and seven of them had further electrophysiological (EP) studies.ResultsOf the nine patients with DM, eight had EKG evidence of conduction abnormalities ranging from first-degree heart block to complete heart block. Of the seven who had EP studies, five had inducible ventricular tachycardia requiring immediate cardioversion and implantable cardioverter defibrillator (ICD) implant. Two of them underwent permanent pacemaker placement due to complete heart block and infra-Hissian block. The remaining two patients opted for a conservative approach with yearly EKG monitoring.ConclusionBecause one-third of the cardiac deaths in patients with DM are sudden, there is a strong need to identify these patients and intervene in those at high risk. Prophylactic pacemaker placement is recommended even in those with minimal conduction system abnormality. However, the common practice is to identify patients at high risk of conduction abnormalities by EP studies and then provide them with prophylactic invasive strategies.

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Patient: Female, 67Final Diagnosis: Infected pacemaker device secondary to Aspergillus fumigatusSymptoms: Swelling over the left pectoral regionMedication: VoriconazoleClinical Procedure: Pacemaker explantationSpecialty: CardiologyObjective:Unusual clinical courseBackground:With the increasing use of cardiac implantable electronic devices (CIED), there has been an associated increase in rate of complications. Infection accounts for about 1% of these, of which only a handful were reported secondary to Aspergillus fumigatus. All of these were seen in chronically-ill patients with several co-morbid conditions within a few years of implantation. None have been reported in an otherwise immunocompetent patient at 7 years after CIED implantation.Case Report:A 67-year-old woman with symptomatic sick sinus syndrome required a pacemaker 15 years ago with subsequent revision 7 years prior due to battery depletion. She now presented with a left pectoral non-tender mass that developed over several weeks. She denied history of recent fever, trauma, or infection. An elective pacemaker revision and pocket exploration led to the drainage of 150 cc of serosanguineous discharge from the pocket. She received peri-procedural prophylaxis with Vancomycin, but later, wound cultures grew Aspergillus fumigatus. She underwent complete removal of the pacemaker system along with a 6-week course of voriconazole and is doing well.Conclusions:Even though Staphylococcus aureus causes most CIED infections, there should be a suspicion for fungal organisms, especially in culture-negative infections, in immunocompromised states like diabetes mellitus or with minimal improvement on antibiotics. If not treated appropriately, aspergillosis may have catastrophic outcomes, including endocarditis, often leading to death. Appropriate treatment should include immediate initiation of antifungals and removal of the CIED. It is still unclear why an immunocompetent patient developed aspergillosis, but appropriate management helped avoid a grave outcome.

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