The American journal of cardiology
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A total cavopulmonary connection (Fontan surgery) is rarely performed in a child with trisomy 21 (Down syndrome) for a univentricular heart, and the outcomes after surgery are not well defined, but the incidence of mortality has been reported to be higher. To determine the mortality rate and contributing factors after Fontan surgery in children with Down syndrome, mortality data after Fontan surgery from the Pediatric Cardiac Care Consortium Registry were evaluated. Among Fontan procedures (n = 2,853), all patients with Down syndrome (n = 17) were selected, of whom 13 had hemodynamic data available. ⋯ Almost all mortality was in the early postoperative period in children with Down syndrome. The relative risk ratio of mortality was 2.5 (95% confidence interval 0.63 to 10). In conclusion, Down syndrome was found to be an independent parameter associated with a significantly higher risk for mortality in the early postoperative period after Fontan surgery.
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Williams syndrome (WS) is a congenital disorder affecting the vascular, connective tissue, and central nervous systems of 1 in 8,000 live births. Previous reports have reported high frequencies of cardiovascular abnormalities (CVAs) in small numbers of patients with WS. A retrospective review was undertaken of patients with WS evaluated at our institution from January 1, 1980 through December 31, 2007. ⋯ In conclusion, CVAs are common in patients with WS, but supravalvar aortic stenosis and peripheral pulmonary stenosis occurred less frequently in this large cohort than previously reported. In patients with WS and CVAs, interventions are common and usually occur by 5 years of age. Most patients with WS do not require intervention during long-term follow-up, and the overall mortality has been low.
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Multicenter Study
The RecordAF study: design, baseline data, and profile of patients according to chosen treatment strategy for atrial fibrillation.
The REgistry on Cardiac rhythm disORDers assessing the control of Atrial Fibrillation (RecordAF) is the first worldwide, 1-year observational, longitudinal study of the management of paroxysmal/persistent atrial fibrillation (AF) in recently diagnosed patients. The study was conducted at 532 sites in 21 countries across Europe, America, and Asia; recruitment was completed in April 2008. The primary objectives were to prospectively assess the therapeutic success and clinical outcomes in rhythm- and rate-control strategies. ⋯ Rate-control patients more often had previous electrocardiographic evidence of AF and were not in sinus rhythm at inclusion (p <0.01 for both end points). Patients were followed at 6 and 12 months, and changes in therapeutic strategy and clinical outcomes were recorded. In conclusion, the RecordAF study results will provide a global perspective on current AF treatment strategies.
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Randomized Controlled Trial Multicenter Study
Relation of body mass index to sudden cardiac death and the benefit of implantable cardioverter-defibrillator in patients with left ventricular dysfunction after healing of myocardial infarction.
Obesity has been identified as a risk factor for cardiovascular disease and heart failure. However, data regarding the relation of body mass index (BMI) to outcome in patients with established heart failure are conflicting. We examined the risk of all-cause mortality and sudden cardiac death (SCD) in 1,231 patients after myocardial infarction with left ventricular dysfunction enrolled the Multicenter Automatic Defibrillator Implantation Trial-II (MADIT-II). ⋯ Consistently, patients with BMI <30 kg/m(2) exhibited 46% (p = 0.03) and 76% (p = 0.04) increases in risk of all-cause mortality and SCD, respectively, compared to patients who had higher BMI values. The benefit of the ICD was pronounced in higher-risk patients with BMI <30 kg/m(2) (hazard ratio 0.68, p = 0.017) and maintained in the lower-risk subgroup of patients with BMI > or =30 kg/m(2) (hazard ratio 0.73, p = 0.32; p = 0.86 for ICD-by-BMI interaction). In conclusion, our findings suggest an independent inverse association between BMI values and risk of all-cause mortality and SCD in patients after myocardial infarction with left ventricular dysfunction enrolled in the MADIT-II trial.
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N-terminal pro-brain natriuretic peptide (NT-pro-BNP) levels have been shown to be increased at baseline in patients undergoing surgical ventricular restoration (SVR) of the left ventricle. However, changes in the values of this marker in the early postoperative period and its prognostic significance remain less known in these patients. We evaluated 31 consecutive patients undergoing SVR who had NT-pro-BNP determined a day before SVR and from postoperative days 0 to 4. ⋯ Preoperative NT-pro-BNP had a high discrimination power on receiver operating characteristic analysis for major morbidity (area under the curve 0.84, sensitivity 68%, and specificity 88% for 1,304 pg/ml). Although NT-pro-BNP levels decreased after SVR in patients without major morbidity, their levels remained persistently increased in those with it. In conclusion, preoperative NT-pro-BNP determination may be of value in stratifying the risk for major morbidity after SVR.