Isolated noncompaction cardiomyopathy is an exceedingly rare congenital cardiomyopathy. A case of isolated noncompaction cardiomyopathy is reported and the literature on the subject collected through a comprehensive literature search is reviewed. Fewer than 100 cases of this condition have been reported. Isolated noncompaction cardiomyopathy is caused by a defect in cardiac morphogenesis resulting in an arrest of compaction of loose interwoven meshwork of myocardial fibers during intrauterine life, which results in severe systolic dysfunction as well as undue hypertrophy of the involved walls of the ventricles. Although the most frequent sites involved are left ventricular apex and inferior wall, involvement of other left ventricular walls and right ventricle has also been reported. Etiology of the isolated noncompaction of myocardium is not clear. Familial cases have been reported and the mode of inheritance is heterogeneous. In X-linked form of the disease, a locus has been found on Xq28, and mutations have been reported in G4.5 gene. The age of onset of symptoms ranges from infancy to the geriatric age. Patients with isolated noncompaction cardiomyopathy have a high incidence of heart failure, arrhythmias, and thromboembolism. The most common presentation is congestive heart failure. Arrhythmias include atrial arrhythmias, ventricular tachycardia, and sudden cardiac death. The patient reported in this article presented with paroxysmal supraventricular tachycardia. Echocardiography is the procedure of choice to establish diagnosis. Due to the lack of associated cardiac anomalies, antenatal detection is difficult. The treatment is that for congestive heart failure, arrhythmias, and thromboembolism. The end-stage congestive heart failure is managed with heart transplantation and potential life-threatening ventricular tachyarrhythmias with an implantable cardioverter defibrillator. Prognosis is poor and the common causes of death are intractable heart failure and sudden cardiac death.
SummaryBackground: Use of adrenergic (inotropic and vasopressor) drugs is common after cardiac surgery.Hypothesis: The study was undertaken to evaluate the role of postoperative adrenergic drug use as a predictor of postoperative atrial fibrillation (AF) after cardiac surgery.Methods: The study population consisted of 199 patients post cardiac surgery. Postoperative adrenergic drug use and the baseline and clinical variables were analyzed as possible predictors of postoperative AF.Results: Of 199 patients, postoperative AF occurred in 59 patients (incidence 30%). The adrenergic drugs were used in 127 (64%) patients. Postoperative AF occurred in 49 of the 127 patients (39%) with and in 10 of the 72 patients (14%) without adrenergic drug use (p < 0.01). By univariate analyses, postoperative adrenergic drug use, age, left ventricular hypertrophy, left atrial size, valve surgery, aortic valve replacement, cross clamp time, bypass time, postoperative ventricular pacing, and hours in intensive care unit were predictors of development of postoperative AF. Atrial pacing was a predictor of freedom from developing AF. By multivariate logistic regression analysis, adrenergic drug use was an independent predictor of postoperative AF (odds ratio [OR] 3.35, 95% confidence interval [CI] 1.38-8.12, p = 0.016). Two other independent predictors were valve surgery (OR 2.88, 95% CI 1.31-6.35, p = 0.002) and age (OR 10.73, p =
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