Electrocardiographic abnormalities and sudden death in myotonic dystrophy type 1.

BACKGROUND: Sudden death can occur as a consequence of cardiac-conduction abnormalities in the neuromuscular disease myotonic dystrophy type 1. The determinants of the risk of sudden death remain imprecise. METHODS: We assessed whether the electrocardiogram (ECG) was useful in predicting sudden death in 406 adult patients with genetically confirmed myotonic dystrophy type 1. A patient was characterized as having a severe abnormality if the ECG had at least one of the following features: rhythm other than sinus, PR interval of 240 msec or more, QRS duration of 120 msec or more, or second-degree or third-degree atrioventricular block. RESULTS: Patients with severe abnormalities according to the entry ECG were older than patients without severe abnormalities, had more severe skeletal-muscle impairment, and were more likely to have heart failure, left ventricular systolic dysfunction, or atrial tachyarrhythmia. Such patients were more likely to receive a pacemaker or an implantable cardioverter-defibrillator during the follow-up period. During a mean follow-up period of 5.7 years, 81 patients died; there were 27 sudden deaths, 32 deaths from progressive neuromuscular respiratory failure, 5 nonsudden deaths from cardiac causes, and 17 deaths from other causes. Among the 17 patients who died suddenly in whom postcollapse rhythm was evaluated, a ventricular tachyarrhythmia was observed in 9. A severe ECG abnormality (relative risk, 3.30; 95% confidence interval [CI], 1.24 to 8.78) and a clinical diagnosis of atrial tachyarrhythmia (relative risk, 5.18; 95% CI, 2.28 to 11.77) were independent risk factors for sudden death. CONCLUSIONS: Patients with adult myotonic dystrophy type 1 are at high risk for arrhythmias and sudden death. A severe abnormality on the ECG and a diagnosis of an atrial tachyarrhythmia predict sudden death. (ClinicalTrials.gov number, NCT00622453.)

Brugada syndrome in the presence of coronary artery disease and parasympathomimetic drug therapy.

Brugada pattern ECG changes have been described in various disease states and drug therapies, including electrolyte abnormalities, myocardial pathology, medications, and mechanical abnormalities. Therefore, the diagnosis of Brugada syndrome cannot be made in the presence of confounding variables according to prevailing guidelines. We present a case report which illustrates two important principles regarding Brugada syndrome, the dynamic ECG manifestations and the elimination of confounding variables (in this case significant coronary artery disease and cholinergic pharmacotherapy) prior to definitive diagnosis.

Prognostic value of transient and sustained increase in in-hospital creatinine on outcomes of patients admitted with acute coronary syndrome.

A history of renal insufficiency or increased creatinine level on admission is associated with poor outcomes in patients with acute coronary syndrome (ACS). This study sought to determine whether in-hospital worsening of renal function, either transient or sustained, is an independent risk factor for 6-month mortality in patients admitted with ACS. A total of 1,417 patients admitted with ACS from June 2000 to May 2003 were reviewed. Patients were classified into 3 groups. Group I included patients with an increase in creatinine during hospitalization of 0.5 mg/dl that resolved by discharge. Group III included patients with an increase in creatinine of >0.5 mg/dl that did not resolve. The primary end point was 6-month mortality from any cause. Patients in groups II and III had higher 6-month mortality rates (27% and 23%, respectively; both p<0.001) compared with patients in group I (7.4%). After adjustment for known risk factors, a transient increase in creatinine remained a significant independent predictor of 6-month mortality (odds ratio 2.07, 95% confidence interval 1.14 to 3.76), although a sustained increase in creatinine showed a trend (odds ratio 1.58, 95% confidence interval 0.68 to 3.70). In conclusion, independent of a history of renal insufficiency or increased admission creatinine, in-hospital worsening of renal function is an important risk factor for 6-month mortality in patients admitted with ACS. Furthermore, return to baseline function by discharge does not protect against this risk. These findings have implications for management of these high-risk patients.

Smoking cessation in patients with acute coronary syndrome.

BACKGROUND, In patients with acute coronary syndrome, smoking cessation rates, demographics, and management strategies havenot been well described. We hypothesized that hospitalized patients with acute coronary syndrome would have higher smoking cessation rates than other currently available therapies. In-hospital counseling and referral to cardiac rehabilitation may further improve cessation rates. METHODS, We reviewed 1098 consecutive admissions for acute coronary syndrome at the University of Michigan; 254 of thesepatients reported active smoking status on admission. Patients were divided into (i) those who continued smoking and (ii) those who quit smoking based on a 6-month telephonic interview. Clinical variables, management and therapies were com-pared for the two cohorts. RESULTS, The mean age of the 254 patients was 56 years and 65% were male. At six months, 49.2% of patients had quit smok-ing. Significant predictors of smoking cessation were coronary artery bypass grafting, pulmonary artery catheter placement, and need for mechanical ventilation. Patients who underwent cardiac rehabilitation post-discharge had a trendtoward higher cessation rates. Formal counseling during hospitalization did not seem to affect cessation rates. CONCLUSIONS, In this study, patients with acute coronary syndrome had a higher 6-month smoking cessation rate than previously published rates seen in ambulatory practice, and the more severely ill patients had higher cessation rates. Smoking cessation rates were not higher in those who received in-patient smoking counseling.

Adherence to medications by patients after acute coronary syndromes.

BACKGROUND: Nonadherence to medication may lead to poor medical outcomes. OBJECTIVE: To describe medication-taking behavior of patients with a history of acute coronary syndromes (ACS) for 4 classes of drugs and determine the relationship between self-reported adherence and patient characteristics. METHODS: Consenting patients with the diagnosis of ACS were interviewed by telephone approximately 10 months after discharge. The survey elicited data characterizing the patient, current medication regimens, beliefs about drug therapy, reasons for discontinuing medications, and adherence. The survey included the Beliefs About Medicine Questionnaire providing 4 scales: Specific Necessity, Specific Concerns, General Harm, and General Overuse, and the Medication Adherence Scale (MAS). Multivariate regression was used to determine the independent variables with the strongest association to the MAS. A p value < or = 0.05 was considered significant for all analyses. RESULTS: Two hundred eight patients were interviewed. Mean +/- SD age was 64.9 +/- 13.0 years, with 60.6% male, 95.7% white, 57.3% with a college education, 87.9% living with > or =1 other person, and 42% indicating excellent or very good health. The percentage of patients continuing on medication at the time of the survey category ranged from 87.4% (aspirin) to 66.0% (angiotensin-converting enzyme inhibitors). Reasons for stopping medication included physician discontinuation or adverse effects. Of patients still on drug therapy, the mean MAS was 1.3 +/- 0.4, with 53.8% indicating nonadherence (score >1). The final regression model showed R(2) = 0.132 and included heart-related health status and Specific Necessity as significant predictor variables. CONCLUSIONS: After ACS, not all patients continue their drugs or take them exactly as prescribed. Determining beliefs about illness and medication may be helpful in developing interventions aimed at improving adherence.