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Apical hypertrophic cardiomyopathy (HC) has been considered a "benign" form of HC, with limited data on long-term outcome. We compared apical HC patients with a non-HC, age- and gender-matched Minnesota white population to identify outcomes and prognostic factors. Between 1976 and 2006, 193 patients (62% men) with apical HC were seen at our clinic. Their(More)
OBJECTIVE To examine the relationship among age, septal morphological subtype, and presence of hypertrophic cardiomyopathy (HCM)-associated myofilament mutations. PATIENTS AND METHODS Comprehensive mutation analysis of the 8 HCM susceptibility genes that encode the myofilaments of the cardiac sarcomere was performed previously in 382 unrelated patients(More)
Hypertrophic cardiomyopathy (HCM) can be classified into at least four major anatomic subsets based upon the septal contour, and the location and extent of hypertrophy: reverse curvature-, sigmoidal-, apical-, and neutral contour-HCM. Here, we sought to identify genetic determinants for sigmoidal-HCM and hypothesized that Z-disc-HCM may be associated(More)
Four-and-a-half LIM domain protein 1 isoform A (FHL1A) is predominantly expressed in skeletal and cardiac muscle. Mutations in the FHL1 gene are causative for several types of hereditary myopathies including X-linked myopathy with postural muscle atrophy (XMPMA). We here studied myoblasts from XMPMA patients. We found that functional FHL1A protein is(More)
BACKGROUND Alcohol septal ablation has emerged as a therapy for patients with obstructive hypertrophic cardiomyopathy (HCM). However, there are limited data on the predictors of success with the procedure. METHODS We examined patient characteristics and cardiac morphology as well as procedural data on 166 HCM patients (mean age, 63 years; 43% men), who(More)
Hypertrophic cardiomyopathy (HC) is associated frequently with heart failure symptoms and diastolic dysfunction. Although the influence of brain natriuretic peptide (BNP) levels in the management of patients with systolic dysfunction is evolving, there are few data on the role of BNP in the management of patients with HC. BNP was compared with clinical and(More)
BACKGROUND Although dual-chamber pacing therapy for obstructive hypertrophic cardiomyopathy has been relegated to a limited role, there remains a subset of patients who are not good candidates for more definitive therapies. OBJECTIVE The goal of this investigation was to determine whether preprocedural variables could help identify patients most likely to(More)
BACKGROUND Apical outpouching, including wall motion abnormalities and aneurysms, has been described in apical hypertrophic cardiomyopathy (ApHCM). METHODS Between 1976 and 2006, 193 patients with ApHCM (120 men; overall mean age, 61 ± 17 years) were evaluated. RESULTS Apical outpouching was found in 29 patients (15%) and in 22 of the 78 patients (28%)(More)
BACKGROUND We investigated whether the aortic augmentation index (AIx), a measure of arterial wave reflection and stiffness, is associated with cardiorespiratory fitness in men without known coronary heart disease (CHD). METHODS Asymptomatic men (n = 201, mean age 51 +/- 9.2 years) referred for a screening exercise electrocardiogram (ECG) underwent(More)
BACKGROUND X-linked myopathy with postural muscle atrophy is a novel X-linked myopathy caused by mutations in the four-and-a-half LIM domain 1 gene (FHL1). Cardiac involvement was suspected in initial publications. We now systematically analyzed the association of the FHL1 genotype with the cardiac phenotype to establish a potential cardiac involvement in(More)