Abstract：
:Twenty-four patients with restrictive cardiomyopathy were identified at St. Thomas' Hospital during a 17-year period. All had endomyocardial biopsy, but in two patients the biopsy specimens were small and nondiagnostic. Seven patients had amyloidosis and five had other specific heart muscle diseases. The remaining 10 patients with primary restrictive cardiomyopathy had myocyte hypertrophy or interstitial fibrosis, or both. Patients with primary restrictive cardiomyopathy presented earlier but survived longer after presentation than did those with amyloidosis. In each group, survival after cardiac catheterization was related to cardiac index but not to filling pressures. Primary restrictive cardiomyopathy was associated with complete heart block in four patients, two of whom had skeletal myopathy. One had a family history of dominantly inherited skeletal myopathy. Primary restrictive cardiomyopathy was present in a mother and daughter. Two other patients had a family history of heart failure, sudden death or complete heart block, alone or in combination, at a young age. Restrictive hemodynamics and complete heart block were present in patients even in the absence of significant fibrosis. The data suggest that primary restrictive cardiomyopathy may be a distinct myopathy with dominant inheritance and incomplete penetrance that is expressed morphologically as myocyte hypertrophy and interstitial fibrosis. Skeletal myopathy may be associated with the cardiomyopathy.
journal_name
J Am Coll Cardioljournal_title
Journal of the American College of Cardiologyauthors
Katritsis D,Wilmshurst PT,Wendon JA,Davies MJ,Webb-Peploe MMdoi
10.1016/0735-1097(91)90540-psubject
Has Abstractpub_date
1991-11-01 00:00:00pages
1230-5issue
5eissn
0735-1097issn
1558-3597pii
0735-1097(91)90540-Pjournal_volume
18pub_type
杂志文章abstract：:The value of heart rate variability, ambulatory electrocardiographic (ECG) variables and the signal-averaged ECG in the prediction of arrhythmic events (sudden death or life-threatening ventricular arrhythmias) was assessed before hospital discharge in 416 consecutive survivors of acute myocardial infarction. During t...
journal_title：Journal of the American College of Cardiology
pub_type： 杂志文章
doi：10.1016/0735-1097(91)90791-7
更新日期：1991-09-01 00:00:00
abstract：OBJECTIVES:We sought to determine the prevalence of treatable left ventricular (LV) systolic dysfunction (LVSD) in patients who present with their first noncardiac vascular episode. BACKGROUND:Screening for LV dysfunction in patients who present with their first stroke (cerebrovascular accident), their first transient...
journal_title：Journal of the American College of Cardiology
pub_type： 杂志文章
doi：10.1016/s0735-1097(01)01725-9
更新日期：2002-01-16 00:00:00
abstract：OBJECTIVES:Percutaneous balloon valvotomy was studied retrospectively in newborns with critical pulmonary valve stenosis or atresia to assess its potential role as an alternative therapy to operation. BACKGROUND:Severe right ventricular outflow tract obstructions are life-threatening conditions requiring prostaglandin...
journal_title：Journal of the American College of Cardiology
pub_type： 杂志文章
doi：10.1016/0735-1097(95)00369-X
更新日期：1995-12-01 00:00:00
abstract：:In a retrospective analysis 139 patients with hypertrophic cardiomyopathy were followed up for 8.9 years (range 1 to 28 years). Patients were divided into two groups: Group 1 consisted of 60 patients with medical therapy and Group 2 of 79 patients with surgical therapy (septal myectomy). Groups 1 and 2 were subdivided...
journal_title：Journal of the American College of Cardiology
pub_type： 杂志文章,评审
doi：10.1016/s0735-1097(10)80176-7
更新日期：1991-03-01 00:00:00
abstract：BACKGROUND:His bundle pacing is a new method for delivering cardiac resynchronization therapy (CRT). OBJECTIVES:The authors performed a head-to-head, high-precision, acute crossover comparison between His bundle pacing and conventional biventricular CRT, measuring effects on ventricular activation and acute hemodynami...
journal_title：Journal of the American College of Cardiology
pub_type： 杂志文章
doi：10.1016/j.jacc.2018.09.073
更新日期：2018-12-18 00:00:00
abstract：BACKGROUND:Peripartum cardiomyopathy (PPCM) remains a major cause of maternal morbidity and mortality. OBJECTIVES:This study sought to prospectively evaluate recovery of the left ventricular ejection fraction (LVEF) and clinical outcomes in the multicenter IPAC (Investigations of Pregnancy Associated Cardiomyopathy) s...
journal_title：Journal of the American College of Cardiology
pub_type： 杂志文章,多中心研究
doi：10.1016/j.jacc.2015.06.1309
更新日期：2015-08-25 00:00:00
abstract：OBJECTIVES:This study was designed to prospectively determine, in patients with an acute coronary syndrome, whether the inflammatory marker, C-reactive protein (CRP), measured at hospital admission, discharge, and 1 month later has incremental value to predict outcomes at 1 year. BACKGROUND:The clinical utility of CRP...
journal_title：Journal of the American College of Cardiology
pub_type： 杂志文章,多中心研究
doi：10.1016/j.jacc.2008.03.019
更新日期：2008-06-17 00:00:00
abstract：:Mechanisms of death among patients who died within 18 h of enrollment in the Thrombolysis in Myocardial Infarction Phase II (TIMI II) study were analyzed. Of 3,339 patients enrolled, 32 died within the 1st 4 h and 31 died within the subsequent 14 h. Thirteen of the 63 patients had shock at enrollment; 22 had advanced ...
journal_title：Journal of the American College of Cardiology
pub_type： 临床试验,杂志文章,多中心研究,随机对照试验
doi：10.1016/0735-1097(92)90313-c
更新日期：1992-05-01 00:00:00
abstract：OBJECTIVES:The aim of this study was to investigate the effects of bariatric surgery on small artery function and the mechanisms underlying this. BACKGROUND:In lean healthy humans, perivascular adipose tissue (PVAT) exerts an anticontractile effect on adjacent small arteries, but this is lost in obesity-associated con...
journal_title：Journal of the American College of Cardiology
pub_type： 杂志文章
doi：10.1016/j.jacc.2013.04.027
更新日期：2013-07-09 00:00:00
abstract：:The symptoms of chronic heart failure (CHF) are predominantly shortness of breath and fatigue during exercise and reduced exercise capacity. Disturbances of central hemodynamic function are no longer considered to be the major determinants of exercise capacity. The two symptoms of fatigue and breathlessness are often ...
journal_title：Journal of the American College of Cardiology
pub_type： 杂志文章,评审
doi：10.1016/S0735-1097(96)00323-3
更新日期：1996-11-01 00:00:00
abstract：OBJECTIVES:This study was undertaken to elucidate the prevalence of aortic valve abnormalities in the elderly. BACKGROUND:The age of persons treated actively for valve disorders is increasing. More information is needed about the prevalence of aortic valve disease in old age. METHODS:Randomly selected men and women i...
journal_title：Journal of the American College of Cardiology
pub_type： 杂志文章
doi：10.1016/0735-1097(93)90249-z
更新日期：1993-04-01 00:00:00
abstract：BACKGROUND:Familial restrictive cardiomyopathy (FRCM) has a poor prognosis due to diastolic dysfunction and restrictive physiology (RP). Myocardial stiffness, with or without fibrosis, underlie RP, but the mechanism(s) of restrictive remodeling is unclear. Myopalladin (MYPN) is a messenger molecule that links structura...
journal_title：Journal of the American College of Cardiology
pub_type： 杂志文章
doi：10.1016/j.jacc.2014.09.071
更新日期：2014-12-30 00:00:00
abstract：OBJECTIVES:To elucidate signal transduction pathways regulating expression of myocardial gap junction channel proteins (connexins) and to determine whether mediators of cardiac hypertrophy might promote remodeling of gap junctions, we characterized the effects of angiotensin II on expression of the major cardiac gap ju...
journal_title：Journal of the American College of Cardiology
pub_type： 杂志文章
doi：10.1016/s0735-1097(98)00317-9
更新日期：1998-09-01 00:00:00
abstract：OBJECTIVES:We hypothesized that orthotopic heart transplantation with bicaval and pulmonary venous anastomoses preserves atrial contractility. BACKGROUND:The standard biatrial anastomotic technique of orthotopic heart transplantation causes impaired function and enlargement of the atria. Cine magnetic resonance imagin...
journal_title：Journal of the American College of Cardiology
pub_type： 杂志文章
doi：10.1016/0735-1097(94)00465-3
更新日期：1995-03-15 00:00:00
abstract：OBJECTIVES:This study was undertaken to test the hypothesis that lipoprotein(a) [Lp(a)] impairs endothelial function. BACKGROUND:Elevated Lp(a) plasma levels have been demonstrated to be associated with an increased risk of coronary heart disease. In atherosclerosis, endothelial dysfunction is known to be an early ind...
journal_title：Journal of the American College of Cardiology
pub_type： 杂志文章
doi：10.1016/s0735-1097(97)00497-x
更新日期：1998-02-01 00:00:00
abstract：:In symptomatic severe aortic regurgitation, left ventricular diastolic pressure increases rapidly, often exceeding left atrial pressure in late diastole. This characteristic hemodynamic change should be reflected in the Doppler mitral inflow velocity, which is the direct result of the diastolic pressure difference bet...
journal_title：Journal of the American College of Cardiology
pub_type： 杂志文章
doi：10.1016/0735-1097(89)90021-1
更新日期：1989-12-01 00:00:00
abstract：OBJECTIVES:The aim of this study was to characterize fully in vivo aortic compliance over a wide range of passive distending pressures, and to study pharmacologically induced alterations in compliance using an intravascular ultrasound-based technique in the canine model of heart failure. BACKGROUND:Altered aortic comp...
journal_title：Journal of the American College of Cardiology
pub_type： 杂志文章
doi：10.1016/s0735-1097(98)00530-0
更新日期：1999-01-01 00:00:00
abstract：:Baseline coronary artery calcification (CAC) accurately identifies coronary atherosclerosis and might improve prediction of future cardiac events. Serial assessment of CAC scores has been proposed for monitoring atherosclerosis progression and for assessing the effectiveness of medical therapies aimed at reducing card...
journal_title：Journal of the American College of Cardiology
pub_type： 杂志文章,评审
doi：10.1016/j.jacc.2010.06.038
更新日期：2010-11-09 00:00:00
abstract：OBJECTIVES:The aim of this randomized, single-blind, controlled trial was to assess N-acetylcysteine effects on contrast-induced nephropathy and reperfusion injury in ST-segment elevation myocardial infarction patients undergoing primary angioplasty with moderate contrast volumes. BACKGROUND:High-dose N-acetylcysteine...
journal_title：Journal of the American College of Cardiology
pub_type： 杂志文章,随机对照试验
doi：10.1016/j.jacc.2009.08.091
更新日期：2010-05-18 00:00:00
abstract：OBJECTIVES:This study sought to determine if nesiritide increases diuresis in congestive heart failure patients. BACKGROUND:In the ASCEND-HF (Acute Study of Clinical Effectiveness of Nesiritide and Decompensated Heart Failure), 7,141 patients hospitalized with acute decompensated heart failure (ADHF) were randomized t...
journal_title：Journal of the American College of Cardiology
pub_type： 杂志文章
doi：10.1016/j.jacc.2013.04.073
更新日期：2013-09-24 00:00:00
abstract：OBJECTIVES:This study sought to develop a regression-based method for characterization of QT interval behavior during exercise and to define the normal range of the resulting "dynamic" measures of repolarization during submaximal treadmill testing in men and women. BACKGROUND:The Bazett-corrected QT (QTc) interval dur...
journal_title：Journal of the American College of Cardiology
pub_type： 杂志文章
doi：10.1016/s0735-1097(96)00351-8
更新日期：1996-11-15 00:00:00
abstract：:Both activation of platelets and elevation of plasminogen activator inhibitor type 1 (PAI-1) activity in plasma have been associated with acute myocardial infarction. Growth factors from platelet alpha-granules have been shown to increase PAI-1 synthesis in liver and endothelial cells in culture. The present study was...
journal_title：Journal of the American College of Cardiology
pub_type： 杂志文章
doi：10.1016/0735-1097(91)90689-7
更新日期：1991-11-15 00:00:00
abstract：OBJECTIVES:The aim of this study was to characterize the relationship between pericardial fat and atrial fibrillation (AF). BACKGROUND:Obesity is an important risk factor for AF. Pericardial fat has been hypothesized to exert local pathogenic effects on nearby cardiac structures above and beyond that of systemic adipo...
journal_title：Journal of the American College of Cardiology
pub_type： 杂志文章
doi：10.1016/j.jacc.2010.11.045
更新日期：2011-04-26 00:00:00
abstract：OBJECTIVES:The objective of this study was to examine gender differences in left ventricular (LV) function and expression of cardiac genes in response to LV pressure overload due to ascending aortic stenosis in rats. BACKGROUND:Clinical studies have documented gender differences in the pattern of adaptive LV hypertrop...
journal_title：Journal of the American College of Cardiology
pub_type： 杂志文章
doi：10.1016/s0735-1097(99)00165-5
更新日期：1999-07-01 00:00:00
abstract：BACKGROUND:Renin-angiotensin system activation is a feature of many cardiovascular conditions. Activity of myocardial reduced nicotinamide adenine dinucleotide phosphate oxidase 2 (NADPH oxidase 2 or Nox2) is enhanced by angiotensin II (Ang II) and contributes to increased hypertrophy, fibrosis, and adverse remodeling....
journal_title：Journal of the American College of Cardiology
pub_type： 杂志文章
doi：10.1016/j.jacc.2015.05.020
更新日期：2015-07-21 00:00:00
abstract：BACKGROUND:Bacterial growth in cultures of resected heart valves of patients with infective endocarditis (IE) is influenced by pre-operative antibiotic treatment (preop-AT). OBJECTIVES:This study sought to evaluate the time dependency of valve culture results (positive valve culture [PVC] vs. negative valve culture) o...
journal_title：Journal of the American College of Cardiology
pub_type： 杂志文章
doi：10.1016/j.jacc.2020.04.075
更新日期：2020-07-07 00:00:00
abstract：:The management of anticoagulant therapy in patients with atrial fibrillation undergoing electrical cardioversion remains controversial, largely because of inadequate studies demonstrating risk or benefit, a relatively inconvenient anticoagulation management strategy and the increasing use of transesophageal echocardio...
journal_title：Journal of the American College of Cardiology
pub_type： 杂志文章,评审
doi：10.1016/0735-1097(94)90443-x
更新日期：1994-02-01 00:00:00
abstract：:This study was performed 1) to determine the ability of dobutamine stress echocardiography to detect stenoses in individual coronary arteries by utilizing a new model of coronary artery distribution; 2) to evaluate its ability to detect coronary artery stenosis with a minimal lumen diameter less than 1 mm; and 3) to c...
journal_title：Journal of the American College of Cardiology
pub_type： 杂志文章
doi：10.1016/0735-1097(92)90324-g
更新日期：1992-05-01 00:00:00
abstract：OBJECTIVES:We sought to determine the predictive ability of total white blood cell (WBC) count and its subtypes for risk of death or myocardial infarction (MI). BACKGROUND:An elevated WBC count has been associated with cardiovascular risk, but which leukocyte subtypes carry this risk is uncertain. METHODS:Consecutive...
journal_title：Journal of the American College of Cardiology
pub_type： 杂志文章
doi：10.1016/j.jacc.2005.02.054
更新日期：2005-05-17 00:00:00
abstract：OBJECTIVES:The aim of this study was to determine the incidence and significance of second- or third-degree heart block among patients with inferior myocardial infarction treated with thrombolytic therapy. BACKGROUND:Data from the prethrombolytic era suggest that heart block occurs in approximately 20% of patients wit...
journal_title：Journal of the American College of Cardiology
pub_type： 临床试验,杂志文章,多中心研究,随机对照试验
doi：10.1016/0735-1097(92)90004-7
更新日期：1992-09-01 00:00:00
