Published in Circulation on August 22, 2000
Idiopathic restrictive cardiomyopathy is part of the clinical expression of cardiac troponin I mutations. J Clin Invest (2003) 2.04
Cardiomyopathy: an overview. Am Fam Physician (2009) 1.68
Cardiac-myocyte-specific excision of the vinculin gene disrupts cellular junctions, causing sudden death or dilated cardiomyopathy. Mol Cell Biol (2007) 1.63
Cardiomyopathy With Restrictive Physiology in Sickle Cell Disease. JACC Cardiovasc Imaging (2016) 1.42
Impaired relaxation is the main manifestation in transgenic mice expressing a restrictive cardiomyopathy mutation, R193H, in cardiac TnI. Am J Physiol Heart Circ Physiol (2008) 1.30
Correcting diastolic dysfunction by Ca2+ desensitizing troponin in a transgenic mouse model of restrictive cardiomyopathy. J Mol Cell Cardiol (2010) 1.17
Allele and species dependent contractile defects by restrictive and hypertrophic cardiomyopathy-linked troponin I mutants. J Mol Cell Cardiol (2008) 1.14
Inherited cardiomyopathies. Circ J (2014) 1.00
Inherited cardiomyopathies caused by troponin mutations. J Geriatr Cardiol (2013) 0.94
Cardiac troponin mutations and restrictive cardiomyopathy. J Biomed Biotechnol (2010) 0.93
Insights into restrictive cardiomyopathy from clinical and animal studies. J Geriatr Cardiol (2011) 0.93
Dose-dependent diastolic dysfunction and early death in a mouse model with cardiac troponin mutations. J Mol Cell Cardiol (2013) 0.92
Pediatric heart transplantation-indications and outcomes in the current era. J Thorac Dis (2014) 0.84
Utility of Doppler tissue imaging-derived indices in identifying subclinical systolic ventricular dysfunction in children with restrictive cardiomyopathy. Pediatr Cardiol (2011) 0.80
Idiopathic restrictive cardiomyopathy in children. Heart (2005) 0.80
Clinical practice: heart failure in children. Part I: clinical evaluation, diagnostic testing, and initial medical management. Eur J Pediatr (2009) 0.79
Pediatric cardiomyopathies: causes, epidemiology, clinical course, preventive strategies and therapies. Future Cardiol (2013) 0.79
Experimental models of inherited cardiomyopathy and its therapeutics. World J Cardiol (2014) 0.78
Applicability of published guidelines for assessment of left ventricular diastolic function in adults to children with restrictive cardiomyopathy: an observational study. Pediatr Cardiol (2014) 0.77
The impact of heart failure severity at time of listing for cardiac transplantation on survival in pediatric cardiomyopathy. J Heart Lung Transplant (2011) 0.77
Intraoperative management of atrial fibrillation in a patient with restrictive cardiomyopathy. Anesth Essays Res (2015) 0.75
Restrictive Cardiomyopathy Caused by Troponin Mutations: Application of Disease Animal Models in Translational Studies. Front Physiol (2016) 0.75
An unusual ECG pattern in restrictive cardimyopathy. Indian Heart J (2015) 0.75
Pathogenesis associated with a restrictive cardiomyopathy mutant in cardiac troponin T is due to reduced protein stability and greatly increased myofilament Ca2+ sensitivity. Biochim Biophys Acta (2014) 0.75
Clinical Characteristics and Treatment of Cardiomyopathies in Children. Curr Cardiol Rev (2016) 0.75
SCN5A mutations associated with an inherited cardiac arrhythmia, long QT syndrome. Cell (1995) 7.73
Spectrum of mutations in long-QT syndrome genes. KVLQT1, HERG, SCN5A, KCNE1, and KCNE2. Circulation (2000) 7.50
Genotype-phenotype correlation in the long-QT syndrome: gene-specific triggers for life-threatening arrhythmias. Circulation (2001) 7.47
Genetic basis and molecular mechanism for idiopathic ventricular fibrillation. Nature (1998) 7.26
Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome. Circulation (2000) 5.09
Ionic mechanisms responsible for the electrocardiographic phenotype of the Brugada syndrome are temperature dependent. Circ Res (1999) 4.57
Long QT syndrome patients with mutations of the SCN5A and HERG genes have differential responses to Na+ channel blockade and to increases in heart rate. Implications for gene-specific therapy. Circulation (1995) 4.48
Sodium channel blockers identify risk for sudden death in patients with ST-segment elevation and right bundle branch block but structurally normal hearts. Circulation (2000) 3.99
ECG T-wave patterns in genetically distinct forms of the hereditary long QT syndrome. Circulation (1995) 3.79
Specific mutations in methyl-CpG-binding protein 2 confer different severity in Rett syndrome. Neurology (2008) 3.74
Influence of genotype on the clinical course of the long-QT syndrome. International Long-QT Syndrome Registry Research Group. N Engl J Med (1998) 3.41
Computerized three-dimensional segmented human anatomy. Med Phys (1994) 3.26
Water source as risk factor for Helicobacter pylori infection in Peruvian children. Gastrointestinal Physiology Working Group. Lancet (1991) 3.09
Proposed diagnostic criteria for the Brugada syndrome. Eur Heart J (2002) 3.01
Spectrum of ST-T-wave patterns and repolarization parameters in congenital long-QT syndrome: ECG findings identify genotypes. Circulation (2000) 2.95
Postmortem molecular analysis of SCN5A defects in sudden infant death syndrome. JAMA (2001) 2.89
Implantation of balloon-expandable intravascular grafts by catheterization in pulmonary arteries and systemic veins. Circulation (1988) 2.65
The Brugada syndrome: clinical, electrophysiologic and genetic aspects. J Am Coll Cardiol (1999) 2.56
Age- and sex-related differences in clinical manifestations in patients with congenital long-QT syndrome: findings from the International LQTS Registry. Circulation (1998) 2.53
Soluble TNF-alpha receptor 1 and IL-6 plasma levels in humans subjected to the sleep deprivation model of spaceflight. J Allergy Clin Immunol (2001) 2.46
Novel gene mutations in patients with left ventricular noncompaction or Barth syndrome. Circulation (2001) 2.24
Feeding strategies for premature infants: randomized trial of gastrointestinal priming and tube-feeding method. Pediatrics (1999) 2.18
Gene-specific response of dynamic ventricular repolarization to sympathetic stimulation in LQT1, LQT2 and LQT3 forms of congenital long QT syndrome. Eur Heart J (2002) 2.17
Human herpes virus 6 in archival cardiac tissues from children with idiopathic dilated cardiomyopathy or congenital heart disease. J Clin Pathol (2008) 2.07
Influence of mutation type and X chromosome inactivation on Rett syndrome phenotypes. Ann Neurol (2000) 2.03
Mutations in the human delta-sarcoglycan gene in familial and sporadic dilated cardiomyopathy. J Clin Invest (2000) 1.91
KCNE1 mutations cause jervell and Lange-Nielsen syndrome. Nat Genet (1997) 1.90
Evaluation of postmortem endomyocardial biopsy specimens from 38 patients with lymphocytic myocarditis: implications for role of sampling error. Mayo Clin Proc (1989) 1.82
Association of viral genome with graft loss in children after cardiac transplantation. N Engl J Med (2001) 1.78
Evidence for a dystrophin missense mutation as a cause of X-linked dilated cardiomyopathy. Circulation (1997) 1.76
Detection of microorganisms in the tracheal aspirates of preterm infants by polymerase chain reaction: association of adenovirus infection with bronchopulmonary dysplasia. Pediatr Res (2000) 1.72
Decreased single-photon emission computed tomographic [123I]beta-CIT striatal uptake correlates with symptom severity in Parkinson's disease. Ann Neurol (1995) 1.70
Characterization of the developmental stages of sucking in preterm infants during bottle feeding. Acta Paediatr (2000) 1.69
Subacute postictal aggression. Neurology (1998) 1.68
Failure to detect connexin43 mutations in 38 cases of sporadic and familial heterotaxy. Circulation (1996) 1.65
Design and implementation of the North American Pediatric Cardiomyopathy Registry. Am Heart J (2000) 1.65
Delineation of the Marfan phenotype associated with mutations in exons 23-32 of the FBN1 gene. Am J Med Genet (1996) 1.65
Body composition during the first 2 years of life: an updated reference. Pediatr Res (2000) 1.64
The epidemiology of Helicobacter pylori in Peruvian children between 6 and 30 months of age. Am J Gastroenterol (1994) 1.62
Growth failure in children with inflammatory bowel disease: a prospective study. Gastroenterology (1993) 1.62
Coordination of suck-swallow and swallow respiration in preterm infants. Acta Paediatr (2003) 1.60
Sizing of atrial septal defects to predict successful closure with transcatheter cardioSEAL device. Tex Heart Inst J (2001) 1.57
Two long QT syndrome loci map to chromosomes 3 and 7 with evidence for further heterogeneity. Nat Genet (1994) 1.55
New mutations in the KVLQT1 potassium channel that cause long-QT syndrome. Circulation (1998) 1.53
Duchenne muscular dystrophy: deficiency of dystrophin-associated proteins in the sarcolemma. Neurology (1993) 1.52
Practice variations by population: training significance. Pediatrics (1996) 1.52
Whole-body biodistribution, radiation absorbed dose and brain SPECT imaging with iodine-123-beta-CIT in healthy human subjects. J Nucl Med (1994) 1.49
Adjustments in energy expenditure and substrate utilization during late pregnancy and lactation. Am J Clin Nutr (1999) 1.48
Genetic and molecular basis of cardiac arrhythmias: impact on clinical management parts I and II. Circulation (1999) 1.44
Rett syndrome: analysis of deaths in the British survey. Eur Child Adolesc Psychiatry (1997) 1.43
The "final common pathway" hypothesis and inherited cardiovascular disease. The role of cytoskeletal proteins in dilated cardiomyopathy. Herz (2000) 1.42
Quantitative echocardiographic analysis of the aortic arch predicts outcome of balloon angioplasty of native coarctation of the aorta. Circulation (1996) 1.40
Closed head injury resulting in paradoxical improvement of a seizure disorder. Seizure (2000) 1.39
Energy expenditure and deposition of breast-fed and formula-fed infants during early infancy. Pediatr Res (1990) 1.39
Infant feeding mode affects early growth and body composition. Pediatrics (2000) 1.38
Survival after aberrant right subclavian artery-esophageal fistula: case report and literature review. J Vasc Surg (1996) 1.38
Early feeding, antenatal glucocorticoids, and human milk decrease intestinal permeability in preterm infants. Pediatr Res (1998) 1.38
A coordinating center in a clinical trial: the Hypertension Detection and Followup Program. Control Clin Trials (1983) 1.36
Coronary arteries in transposition of the great arteries. Am J Cardiol (1994) 1.29
Genetic and molecular basis of cardiac arrhythmias; impact on clinical management. Study group on molecular basis of arrhythmias of the working group on arrhythmias of the european society of cardiology. Eur Heart J (1999) 1.29
Familial hypertrophic cardiomyopathy in maine coon cats: an animal model of human disease. Circulation (1999) 1.28
Left-right axis malformations associated with mutations in ACVR2B, the gene for human activin receptor type IIB. Am J Med Genet (1999) 1.27
Outcome of home mechanical ventilation in children. J Pediatr (1985) 1.27
Comparison of clinical and genetic variables of cardiac events associated with loud noise versus swimming among subjects with the long QT syndrome. Am J Cardiol (1999) 1.25
Difference images calculated from ictal and interictal technetium-99m-HMPAO SPECT scans of epilepsy. J Nucl Med (1995) 1.23
Clinic attendance in the hypertension detection and follow-up program. Hypertension (1982) 1.22
Effect of maternal diet and body composition on lactational performance. Am J Clin Nutr (1984) 1.21
Prolongation of the QT interval and the sudden infant death syndrome. N Engl J Med (1998) 1.20
Fetal and postnatal growth of children born to narcotic-dependent women. J Pediatr (1983) 1.18
Viral infection of the myocardium in endocardial fibroelastosis. Molecular evidence for the role of mumps virus as an etiologic agent. Circulation (1997) 1.17
Comparison of Indium-111 platelet scintigraphy and two-dimensional echocardiography in the diagnosis of left ventricular thrombi. N Engl J Med (1982) 1.16
Pubertal African-American girls expend less energy at rest and during physical activity than Caucasian girls. J Clin Endocrinol Metab (1999) 1.12
Sympathetic stimulation produces a greater increase in both transmural and spatial dispersion of repolarization in LQT1 than LQT2 forms of congenital long QT syndrome. J Am Coll Cardiol (2001) 1.12
Pathologic features of myocardial hamartomas causing childhood tachyarrhythmias. Circulation (1987) 1.12
Detection of adenoviral genome in the myocardium of adult patients with idiopathic left ventricular dysfunction. Circulation (1999) 1.11
Genetic and molecular basis of cardiac arrhythmias: impact on clinical management part III. Circulation (1999) 1.11
Benign extra-axial collections of infancy. Pediatr Neurosci (1987) 1.11
Association of parvovirus B19 genome in children with myocarditis and cardiac allograft rejection: diagnosis using the polymerase chain reaction. Circulation (1997) 1.10
Amphetamine-stimulated dopamine release competes in vivo for [123I]IBZM binding to the D2 receptor in nonhuman primates. Synapse (1992) 1.10
Energy metabolism, nitrogen balance, and substrate utilization in critically ill children. Am J Clin Nutr (2001) 1.09
Improvement of repolarization abnormalities by a K+ channel opener in the LQT1 form of congenital long-QT syndrome. Circulation (1998) 1.08
Factors affecting head growth and intellectual function in children of drug addicts. Pediatrics (1985) 1.08
A novel approach to assess oral feeding skills of preterm infants. Neonatology (2011) 1.08
[13C]bicarbonate kinetics in humans: intra- vs. interindividual variations. Am J Physiol (1983) 1.07
Measurement of breast volume by ultrasound during normal menstrual cycles and with oral contraceptive use. Obstet Gynecol (1985) 1.07
A comparison of dietary methods in nutritional studies. Am J Clin Nutr (1983) 1.07
Gene mapping of familial autosomal dominant dilated cardiomyopathy to chromosome 10q21-23. J Clin Invest (1996) 1.06
20p12.3 microdeletion predisposes to Wolff-Parkinson-White syndrome with variable neurocognitive deficits. J Med Genet (2008) 1.04
Surgery without catheterization for congenital heart defects: management of 100 patients. J Am Coll Cardiol (1987) 1.03
Possible association of the human KCNE1 (minK) gene and QT interval in healthy subjects: evidence from association and linkage analyses in Israeli families. Ann Hum Genet (2005) 1.02
Etiologies of cardiomyopathy and heart failure. Nat Med (1999) 1.02
Age-gender influence on the rate-corrected QT interval and the QT-heart rate relation in families with genotypically characterized long QT syndrome. J Am Coll Cardiol (1997) 1.01
Outcome of cardiac transplantation in children. Survival in a contemporary multi-institutional experience. Pediatric Heart Transplant Study. Circulation (1996) 1.01
13C-breath tests for sucrose digestion in congenital sucrase isomaltase-deficient and sacrosidase-supplemented patients. J Pediatr Gastroenterol Nutr (2009) 1.01
Familial ventricular arrhythmias in boxers. J Vet Intern Med (1999) 1.01
Cerebrospinal fluid biogenic amines and biopterin in Rett syndrome. Ann Neurol (1989) 1.01
Echocardiographic predictors of adverse clinical events in children with dilated cardiomyopathy: a prospective clinical study. Heart (2004) 1.00