Cardiac and pulmonary function variability in Duchenne/Becker muscular dystrophy: an initial report.

The Duchenne and Becker forms of muscular dystrophy are associated with dilated cardiomyopathy and are diseases in which pulmonary function peaks and then progressively declines. In this report, the authors quantify cardiopulmonary function variability among brothers. Brothers in 3 of 7 eligible sibships had discordant pulmonary function, with significant differences between the brothers' peak forced vital capacities and their vital capacities at last comparable age. There was no relationship between pulmonary and cardiac function among the siblings. The authors concluded that despite identical genetic mutations, cardiac and pulmonary function variability was common among brothers in their clinic with Duchenne or Becker muscular dystrophy. If confirmed by larger studies, these results have negative implications for the use of genetic testing to predict cardiopulmonary course and response to therapies in Duchenne or Becker muscular dystrophy.

Cardiogenic shock in hypothyroidism.

A patient with minimal coronary artery disease presented in cardiogenic shock when her previously undiagnosed hypothyroid state was complicated by an episode of AV nodal re-entrant tachycardia. She did not respond to multiple pressors, and recovered dramatically after starting thyroid supplementation. Hypothyroidism caused her lack of responsiveness to pressors and perpetuated her hypotension and increased filling pressures long after she reverted to a sinus rhythm. Our case dramatically demonstrates the severe lack of physiologic reserve that can be associated with hypothyroidism.

QRS width and its impact on inducibility of ventricular arrhythmia at the time of electrophysiology study.

The inducibility of sustained ventricular arrhythmia by programmed electrical stimulation is a well-established marker of an increased risk of ventricular tachyarrhythmia. The purpose of this investigation was to determine whether there was a significant difference in the QRS duration or ejection fraction (EF) amongst patients who could be induced and those who could not be induced into ventricular arrhythmia at the time of standard electrophysiology study (EPS). All the patients who were studied for inducibility of ventricular arrhythmias between March 2003 and March 2004 (a total of 137 patients) were included in the study. An EF less than or equal to 30% was used to define decreased EF, and a QRS width greater than 120 ms was used to define widened QRS width. Patients were studied using standard EPS protocol after obtaining informed consent. In the univariate analysis, increasing age, male gender and low EF were predictors of inducibility, while widened QRS width did not have any predictive value. With multivariate analysis, there was a significant increase in the odds of inducibility with decreasing EF and widened QRS width. Interestingly, males had significantly more numbers of positive results.

Methadone-induced bradycardia.

Methadone is commonly used in opiate detoxification in high dose. It has a molecular structure similar to verapamil and has been reported to cause similar side effects with continuous high-dose infusion. We report the case of a patient with a history of addiction and abuse of alprazolam and hydrocodone who developed sinus bradycardia after being started on oral methadone for detoxification. The potential of methadone to cause side effects similar to verapamil must be considered in any patient taking methadone.

Ejection fraction and QRS width as predictors of event rates in patients with implantable cardioverter defibrillators.

OBJECTIVES: The Multicenter Automatic Defibrillator Implantation Trial II in 2002 recommended implantable cardioverter defibrillators (ICDs) prophylactically for all patients with a prior myocardial infarction and ejection fraction (EF) of 30% or less. In June of 2003, the Centers for Medicare and Medicaid Services approved reimbursement for ICD placement in patients with an EF of 30% or less who have a QRS interval greater than 120 ms. The purpose of this study was therefore to determine the value of QRS duration in predicting the occurrence of ventricular arrhythmias within the first year after ICD implantation. These ICDs were placed over the past 5 years for various indications. METHODS: EF cut points of 30% or less and 31% or greater and QRS duration of 120 ms or less and 121 ms or greater were used to assess the risk of events. RESULTS: There was a significant increase in events in subjects with EF of 30% or less, compared with patients with EF of 31% or greater (P < 0.05), and there was a trend toward increased likelihood of arrhythmias in patients with widened QRS width. CONCLUSIONS: This study confirms the conclusion of the Multicenter Automatic Defibrillator Implantation Trial II and implies that the Centers for Medicare and Medicaid Services criteria for reimbursement may not be scientifically valid.

Prion-associated dilated cardiomyopathy.

Creutzfeldt-Jakob disease is a spongiform encephalopathy affecting 1 individual per million population per year. We report on a previously healthy 43-year-old patient who presented with the simultaneous onset of a movement disorder, encephalopathy, cognitive decline, and dilated cardiomyopathy, and was found to have spongiform encephalopathy on brain biopsy. Although her neurological features could be explained by Creutzfeldt-Jakob disease, the etiology of the dilated cardiomyopathy could not be established. Finally, special staining of the endomyocardial biopsy specimen revealed the presence of abnormal prion, possibly infectious scrapie prion. As an exhaustive search for familial, ischemic, infectious, autoimmune, toxic, and metabolic causes of dilated cardiomyopathy was unrevealing, the presence of abnormal prion in the cardiac muscle suggested the possibility of prion-induced dilated cardiomyopathy in our patient.

Pancreatic abscess secondary to Alcaligenes faecalis.

We report a patient with pancreatic abscesses and necrosis secondary to Alcaligenes faecalis infection. He initially presented with alcohol-induced acute pancreatitis. Twenty days after the initial presentation, he re-presented with increasing pain and was found to have pancreatic necrosis and abscesses. Treatment was initiated with meropenem. Because of persistent fevers, computed tomography-guided drainage was performed. The fluid grew A faecalis resistant to meropenem and the patient continued to be febrile. He recovered only after adequate surgical intervention and appropriate antibiotic coverage. Although this is the first case of A faecalis reported to cause pancreatic abscess, we believe selection of this organism occurred because surgical drainage was delayed while the patient was on the recommended treatment with meropenem. This case emphasizes the need for early surgical drainage of pancreatic abscesses to avoid the selection of such resistant pathogens.

Focal origin of ventricular fibrillation in a patient with ischemic cardiomyopathy.

A 65-year-old patient with history of ischemic cardiomyopathy admitted to the hospital for chest pain and subsequently experienced incessant ventricular fibrillation (VF), requiring repeated defibrillation. Coronary angiogram was unchanged, compared to a study a year before, and acute ischemia was not considered to be the etiology of the VF. A particular premature ventricular contraction morphology was noted on telemetry prior to each episode of VF. The patient subsequently underwent successful radiofrequency ablation of a focus in the left ventricular free wall. Careful examination of initiating foci of VF or polymorphic ventricular tachycardia, with radiofrequency ablation in appropriate cases, could be potentially life-saving.

A presumptive case of toxocariasis associated with eosinophilic pleural effusion: case report and literature review.

Human toxocariasis is a helminthozoonosis caused by Toxocara sp. Larval migration of the organism through the tissues can result in eosinophila associated with a broad spectrum of clinical manifestations. We report a case of eosinophilic pleural effusion and CD8 cell deficiency associated with Toxocara sp. The symptoms of this patient responded promptly to a nonsteroidal anti-inflammatory medication (naproxen). This is only the fourth reported case of a pleural effusion associated with Toxocara.