当前位置： SCI文献检索 > AMERICAN HEART JOURNAL期刊下所有文献 > New phenotype of familial dilated cardiomyopathy and conduction disorders.

New phenotype of familial dilated cardiomyopathy and conduction disorders.

Abstract：

BACKGROUND:Familial dilated cardiomyopathy (FDCM) is attributed to defects in cytoskeletal proteins, and different patterns of inheritance and phenotypic expressions according to assorted-protein modifications have been identified to date. We describe a clinical family study with 24 individuals in 3 generations affected by dilated cardiomyopathy (DCM) and cardiac conduction abnormalities. METHODS AND RESULTS:After a follow-up period of 25 +/- 14 months, DCM developed in 7 male adults, 6 with associated arterioventricular block (AVB); and 10 female and 7 male adults had several degrees of isolated AVB. This particular clinical expression, with a strong predominance of dilation of the heart developing in the male population and the vertical distribution of patients affected with AVB, is consistent with autosomal dominant inheritance involving both cardiac abnormalities. CONCLUSIONS:The presence of isolated AVB or that associated with DCM in a large number of individuals in the same family, in which members of the male sex seems to be predominantly affected by cardiac dilatation, differs from other FDCMs that have been described previously. This FDCM has an autosomal dominant pattern of inheritance with variable phenotypic expressivity, in which AVB may constitute in itself the only manifestation of this entity. To date, we have been unable to identify the mechanism of inheritance, and we advance some theoretical considerations about possible mechanisms.

journal_name

Am Heart J

journal_title

American heart journal

authors

Oropeza ES,Cadena CN

doi

10.1067/mhj.2003.141

subject

Has Abstract

pub_date

2003-02-01 00:00:00

pages

317-23

issue

2

eissn

0002-8703

issn

1097-6744

pii

S0002870302948313

journal_volume

145

pub_type

杂志文章

相关文献

AMERICAN HEART JOURNAL文献大全
  • Evaluation of a nine-lead Holter monitor for identifying and localizing ischemia and coronary artery disease detected by quantitative thallium-201 tomography.

    abstract：:We devised a nine-lead Holter monitor system with a lead-switching technique to record electrocardiograms from multiple sites in the anterior and the posterior or lateral chest. Leads CM1 to CM6, high lateral (HL), low lateral (LL), and low posterior chest (LB) were used. The sensitivity, specificity, and predictive a...

    journal_title：American heart journal

    pub_type： 临床试验,杂志文章

    doi：10.1016/0002-8703(94)90595-9

    authors： Tanabe T,Yoshioka K,Ide M,Kanemoto N,Suzuki Y

    更新日期：1994-11-01 00:00:00

  • Effect of direct stenting on clinical outcome in patients treated with percutaneous coronary intervention on saphenous vein graft.

    abstract：BACKGROUND:Percutaneous coronary intervention (PCI) of saphenous vein graft (SVG) is associated with frequent postprocedural enzyme elevation and late cardiac events. New strategies are proposed to minimize distal embolization and to improve the outcome of patients treated with stenting for SVG lesions. The objectives ...

    journal_title：American heart journal

    pub_type： 杂志文章

    doi：10.1016/S0002-8703(03)00309-0

    authors： Leborgne L,Cheneau E,Pichard A,Ajani A,Pakala R,Yazdi H,Satler L,Kent K,Suddath WO,Pinnow E,Canos D,Waksman R

    更新日期：2003-09-01 00:00:00

  • Improved Doppler echocardiographic assessment of the left atrial appendage by peripheral vein injection of sonicated albumin microbubbles.

    abstract：:We evaluated the usefulness of peripherally injected sonicated albumin microbubbles in transesophageal echo-Doppler cardiographic assessment of the left atrial appendage in 19 patients (age 61 +/- 19 [range 21 to 86] years; 12 [63%] women). Multiplane transesophageal echocardiography was performed before and after int...

    journal_title：American heart journal

    pub_type： 杂志文章

    doi：10.1016/s0002-8703(97)70180-7

    authors： Yao SS,Ilercil A,Meisner JS,Strom JA,Shirani J

    更新日期：1997-04-01 00:00:00

  • Immediate unselected coronary angiography versus delayed triage in survivors of out-of-hospital cardiac arrest without ST-segment elevation: Design and rationale of the TOMAHAWK trial.

    abstract：:Patients experiencing out-of-hospital cardiac arrest (OHCA) without ST-segment elevation are a heterogenic group with a variety of underlying causes. Up to one-third of patients display a significant coronary lesion compatible with myocardial infarction as OHCA trigger. There are no randomized data on patient selectio...

    journal_title：American heart journal

    pub_type： 杂志文章,多中心研究,随机对照试验

    doi：10.1016/j.ahj.2018.12.005

    authors： Desch S,Freund A,Graf T,Fichtlscherer S,Haake H,Preusch M,Hammer F,Akin I,Christ M,Liebetrau C,Skurk C,Steiner S,Voigt I,Schmitz R,Mudra H,Ledwoch J,Menck N,Horstkotte J,Pels K,Lahmann AL,Otto S,Lenk K,Ohlow M

    更新日期：2019-03-01 00:00:00

  • Aggravated renal dysfunction during intensive therapy for advanced chronic heart failure.

    abstract：BACKGROUND:Chronic heart failure is associated with impaired renal function, which may worsen during therapy. The incidence, predictors, and consequences of aggravated renal dysfunction (ARD) in patients undergoing intensive therapy for advanced chronic heart failure are unknown. METHODS:We reviewed the experience of ...

    journal_title：American heart journal

    pub_type： 杂志文章

    doi：10.1016/s0002-8703(99)70113-4

    authors： Weinfeld MS,Chertow GM,Stevenson LW

    更新日期：1999-08-01 00:00:00

  • Value of color Doppler estimation of regurgitant volume in patients with chronic aortic insufficiency.

    abstract：:We studied 16 patients with chronic aortic insufficiency to compare a method for measuring regurgitant volume with color Doppler flow mapping to stroke count ratio determined by radionuclide ventriculography and to ventricular volumes assessed by two-dimensional echocardiography. A real-time color flow map of the left...

    journal_title：American heart journal

    pub_type： 杂志文章

    doi：10.1016/0002-8703(89)90868-5

    authors： Bouchard A,Yock P,Schiller NB,Blumlein S,Botvinick EH,Greenberg B,Cheitlin M,Massie BM

    更新日期：1989-05-01 00:00:00

  • Effects of substrate availability on myocardial C-11 palmitate kinetics by positron emission tomography in normal subjects and patients with ventricular dysfunction.

    abstract：:The possibility of demonstrating noninvasively with C-11 palmitate and positron emission tomography (PET) changes in myocardial substrate metabolism in normal and diseased human myocardium in response to altered substrate availability in blood and disease-related abnormalities was examined in five normal volunteers an...

    journal_title：American heart journal

    pub_type： 杂志文章

    doi：10.1016/0002-8703(86)90006-2

    authors： Schelbert HR,Henze E,Sochor H,Grossman RG,Huang SC,Barrio JR,Schwaiger M,Phelps ME

    更新日期：1986-06-01 00:00:00

  • Increasing the inotropic effect and toxic dose of digitalis by the administration of antikaliuretic drugs--further evidence for a cardiac effect of diuretic agents.

    abstract：:In prior studies, we have shown that the antikaliuretic drugs, triamterene and amiloride, through a direct cardiac effect reduce the loss of cardiac potassium induced by the administration of digitalis. Since loss of myocardial potassium is thought to underlie digitalis arrhythmias, this study was performed to determi...

    journal_title：American heart journal

    pub_type： 杂志文章

    doi：10.1016/0002-8703(75)90257-4

    authors： Seller RH,Greco J,Banach S,Seth R

    更新日期：1975-07-01 00:00:00

  • The association between socioeconomic status of high-risk patients with coronary heart disease and the treatment rates of evidence-based medicine for coronary heart disease secondary prevention in China: Results from the Bridging the Gap on CHD Secondary

    abstract：BACKGROUND:In China, low socioeconomic status (SES) may be a barrier for patients with coronary heart disease (CHD) to receive adequate treatment because of their inadequate access to health resources. This study aims to evaluate whether and to what extent SES is associated with the treatment of CHD patients. METHODS:...

    journal_title：American heart journal

    pub_type： 杂志文章

    doi：10.1016/j.ahj.2008.12.009

    authors： Niu S,Zhao D,Zhu J,Liu J,Liu Q,Liu J,Wang W,Smith SC Jr,BRIG project.

    更新日期：2009-04-01 00:00:00

  • Population screening for plasma cholesterol: community-based results from Connecticut.

    abstract：:Plasma cholesterol levels were measured in 15,892 participants of a cholesterol screening in Hartford, Connecticut. The screenees were typically health conscious as evidenced by the low prevalence of current cigarette smoking (12%). However, more than 25% of the men and 35% of the women over the age of 50 years had ch...

    journal_title：American heart journal

    pub_type： 杂志文章

    doi：10.1016/0002-8703(89)90741-2

    authors： Wynder EL,Harris RE,Haley NJ

    更新日期：1989-03-01 00:00:00

  • Treatment of anxiety in patients with coronary heart disease: Rationale and design of the UNderstanding the benefits of exercise and escitalopram in anxious patients WIth coroNary heart Disease (UNWIND) randomized clinical trial.

    abstract：BACKGROUND:Anxiety is highly prevalent among patients with coronary heart disease (CHD), and there is growing evidence that high levels of anxiety are associated with worse prognosis. However, few studies have evaluated the efficacy of treating anxiety in CHD patients for reducing symptoms and improving clinical outcom...

    journal_title：American heart journal

    pub_type： 杂志文章,随机对照试验

    doi：10.1016/j.ahj.2016.03.003

    authors： Blumenthal JA,Feger BJ,Smith PJ,Watkins LL,Jiang W,Davidson J,Hoffman BM,Ashworth M,Mabe SK,Babyak MA,Kraus WE,Hinderliter A,Sherwood A

    更新日期：2016-06-01 00:00:00

  • The ω-3 Fatty Acids for Prevention of Post-Operative Atrial Fibrillation trial--rationale and design.

    abstract：:Postoperative atrial fibrillation/flutter (PoAF) commonly complicates cardiac surgery, occurring in 25% to 60% of patients. Postoperative atrial fibrillation/flutter is associated with significant morbidity, higher long-term mortality, and increased health care costs. Novel preventive therapies are clearly needed. In ...

    journal_title：American heart journal

    pub_type： 杂志文章,多中心研究,随机对照试验

    doi：10.1016/j.ahj.2011.03.035

    authors： Mozaffarian D,Marchioli R,Gardner T,Ferrazzi P,O'Gara P,Latini R,Libby P,Lombardi F,Macchia A,Page R,Santini M,Tavazzi L,Tognoni G

    更新日期：2011-07-01 00:00:00

  • Effect of viscosity and iodine concentration of nonionic radiographic contrast media on coronary arteriography in patients.

    abstract：:To assess the influence of viscosity and iodine concentration, three matched and standardized left coronary arteriograms were obtained in 20 patients using iopamidol (Isovue-370), ioversol (Optiray-320), and iohexol (Omnipaque-350). The order of contrast media was randomized and the administration of contrast was doub...

    journal_title：American heart journal

    pub_type： 临床试验,杂志文章,随机对照试验

    doi：10.1016/0002-8703(92)90760-s

    authors： Kern MJ,Roth RA,Aguirre FV,Beauman G,Vogel R

    更新日期：1992-01-01 00:00:00

  • The effect of Aliskiren on exercise capacity in older patients with heart failure and preserved ejection fraction: A randomized, placebo-controlled, double-blind trial.

    abstract：:In older patients (70 ± 7 years) with chronic well-compensated heart failure with preserved ejection and controlled blood pressure, 6 months treatment with aliskiren (direct renin inhibitor) showed non-significant trends for modest improvements in peak exercise oxygen consumption (14.9 ± 0.2 mL kg-1 min-1 versus 14.4 ...

    journal_title：American heart journal

    pub_type： 信件

    doi：10.1016/j.ahj.2018.03.019

    authors： Upadhya B,Brubaker PH,Morgan TM,Eggebeen JD,Jao GT,Stewart KP,Kitzman DW

    更新日期：2018-07-01 00:00:00

  • Incidence, presentation, diagnosis, and management of malfunctioning implantable cardioverter-defibrillator rate-sensing leads.

    abstract：:Recognition of tachyarrhythmia by an implantable cardioverter-defibrillator (ICD) requires an intact rate-sensing lead. We retrospectively examined 266 consecutive patients requiring an ICD to characterize the incidence, clinical presentation, diagnosis, and management of a defective rate-sensing lead. To identify cli...

    journal_title：American heart journal

    pub_type： 杂志文章

    doi：10.1016/0002-8703(94)90585-1

    authors： Daoud EG,Kirsh MM,Bolling SF,Strickberger SA,Morady F,Kou WH

    更新日期：1994-11-01 00:00:00

  • Vitamin D deficiency and coronary artery disease: a review of the evidence.

    abstract：:Coronary artery disease remains the leading cause of death in developed countries despite significant progress in primary prevention and treatment strategies. Older patients are at particularly high risk of poor outcomes following acute coronary syndrome and impaired nutrition, including low vitamin D levels, may play...

    journal_title：American heart journal

    pub_type： 杂志文章,评审

    doi：10.1016/j.ahj.2013.11.012

    authors： Kunadian V,Ford GA,Bawamia B,Qiu W,Manson JE

    更新日期：2014-03-01 00:00:00

  • Prospective evaluation of shoulder-related problems in patients with pectoral cardioverter-defibrillator implantation.

    abstract：BACKGROUND:The pectoral approach to implantation of cardioverter defibrillators (ICDs) has become a standard in defibrillator therapy because of reduced generator size, weight, and volume. But the size of these devices is still comparable to the size of the early conventional antibradycardia pacemakers that were associ...

    journal_title：American heart journal

    pub_type： 杂志文章

    doi：10.1016/s0002-8703(98)70270-4

    authors： Korte T,Jung W,Schlippert U,Wolpert C,Esmailzadeh B,Fimmers R,Schmitt O,Lüderitz B

    更新日期：1998-04-01 00:00:00

  • Response of B-type natriuretic peptide to exercise in hypertensive patients with suspected diastolic heart failure: correlation with cardiac function, hemodynamics, and workload.

    abstract：BACKGROUND:Diastolic heart failure (DHF) is characterized by dyspnea due to increased left ventricular (LV) filling pressures during stress. We sought the relationship of exercise-induced increases in B-type natriuretic peptide (BNP) to LV filling pressures and parameters of cardiovascular performance in suspected DHF....

    journal_title：American heart journal

    pub_type： 杂志文章

    doi：10.1016/j.ahj.2004.02.012

    authors： Mottram PM,Haluska BA,Marwick TH

    更新日期：2004-08-01 00:00:00

  • Brain natriuretic peptide predicts right heart failure in patients with acute pulmonary embolism.

    abstract：BACKGROUND:Plasma levels of brain natriuretic peptide (BNP) are increased in patients with left heart failure. In patients with severe pulmonary embolism (PE), primary right ventricular (RV) dysfunction is frequent. Little is known about BNP secretion in acute RV failure. METHODS:We prospectively studied 50 consecutiv...

    journal_title：American heart journal

    pub_type： 杂志文章

    doi：10.1016/s0002-8703(03)00528-3

    authors： Krüger S,Graf J,Merx MW,Koch KC,Kunz D,Hanrath P,Janssens U

    更新日期：2004-01-01 00:00:00

  • Relationship of age and exercise performance in patients with heart failure: the HF-ACTION study.

    abstract：BACKGROUND:More than three fourths of patients with heart failure (HF) are 65 years and older, and older age is associated with worse symptoms and prognoses than is younger age. Reduced exercise capacity is a chief HF complaint and indicates poorer prognosis, especially among elderly persons, but the mechanisms underly...

    journal_title：American heart journal

    pub_type： 杂志文章

    doi：10.1016/j.ahj.2009.07.018

    authors： Forman DE,Clare R,Kitzman DW,Ellis SJ,Fleg JL,Chiara T,Fletcher G,Kraus WE,HF-ACTION Investigators.

    更新日期：2009-10-01 00:00:00

  • Mixed venous oxygen saturation for differentiating stable from unstable tachycardias.

    abstract：:Current antitachycardia systems are incapable of adequately distinguishing stable from unstable tachycardias. Previously, integration of a pressure sensor or an impedance sensor, together with electrogram analysis, has been investigated as an improved method of identifying unstable arrhythmias. A mixed venous oxygen s...

    journal_title：American heart journal

    pub_type： 杂志文章

    doi：10.1016/0002-8703(91)90519-n

    authors： Cohen TJ,Liem LB

    更新日期：1991-09-01 00:00:00

  • Effects of heart rate on phasic coronary blood flow pattern and flow reserve in patients with normal coronary arteries: a study with an intravascular Doppler catheter and spectral analysis.

    abstract：:To assess the effects of pacing-induced tachycardia on phasic coronary blood flow pattern and flow reserve of left anterior descending artery, we examined 16 patients with chest pain and angiographically normal coronary arteries by using an intravascular Doppler catheter with spectral analysis of the velocity signal. ...

    journal_title：American heart journal

    pub_type： 杂志文章

    doi：10.1016/0002-8703(94)90661-0

    authors： Hongo M,Nakatsuka T,Watanabe N,Takenaka H,Tanaka M,Kinoshita O,Okubo S,Sekiguchi M

    更新日期：1994-03-01 00:00:00

  • Effects of intra-aortic balloon counterpulsation on coronary pressure in patients with stenotic coronary arteries.

    abstract：BACKGROUND:The benefits of intra-aortic balloon pumping (IABP) usage reportedly occur through systolic unloading of the left ventricle and the augmentation of diastolic coronary flow. The aim of this study was to assess the change in intracoronary pressure distal to the coronary stenosis after the IABP by using an intr...

    journal_title：American heart journal

    pub_type： 杂志文章,随机对照试验

    doi：10.1016/j.ahj.2007.05.019

    authors： Yoshitani H,Akasaka T,Kaji S,Kawamoto T,Kume T,Neishi Y,Koyama Y,Yoshida K

    更新日期：2007-10-01 00:00:00

  • Value and limitations of Doppler ultrasound in the evaluation of aortic stenosis: a statistical analysis of 70 consecutive patients.

    abstract：:Continuous-mode Doppler ultrasound and cardiac catheterization were performed within 1.4 +/- 2.0 days in 70 patients, aged 26 to 84 (mean 67 +/- 11) years, with suspected aortic stenosis. Optimal Doppler spectral display signal was recorded from the apical window in 43% of the patients, the second or third right paras...

    journal_title：American heart journal

    pub_type： 杂志文章

    doi：10.1016/0002-8703(86)90694-0

    authors： Panidis IP,Mintz GS,Ross J

    更新日期：1986-07-01 00:00:00

  • A neural link to explain the "muscle hypothesis" of exercise intolerance in chronic heart failure.

    abstract：BACKGROUND:In chronic heart failure the cause of exercise limitation is still unclear: ergoreceptors, muscle afferents sensitive to exercise metabolites, are proposed as a neural link between muscular abnormalities and the limited exercise responses in this syndrome. METHODS:In 92 stable patients with heart failure (3...

    journal_title：American heart journal

    pub_type： 杂志文章

    doi：10.1016/s0002-8703(99)70361-3

    authors： Piepoli M,Ponikowski P,Clark AL,Banasiak W,Capucci A,Coats AJ

    更新日期：1999-06-01 00:00:00

  • Radionuclide angiographic evaluation of left ventricular performance at rest and during exercise in patients with aortic regurgitation.

    abstract：:Radionuclide angiographic evaluation of LV performance at rest and during exercise in patients with AR have shown that an abnormal EF response to exercise may be observed in asymptomatic patients with normal resting LV function. The EF response to exercise has been correlated with a number of clinical and exercise mea...

    journal_title：American heart journal

    pub_type： 杂志文章

    doi：10.1016/0002-8703(86)90019-0

    authors： Iskandrian AS,Heo J

    更新日期：1986-06-01 00:00:00

  • The effect of drug-eluting stents on intermediate angiographic and clinical outcomes in diabetic patients: insights from randomized clinical trials.

    abstract：OBJECTIVE:Implantation of drug-eluting stents has emerged as the predominant percutaneous revascularization strategy in diabetic patients, despite limited outcomes data. Accordingly, our aim was to conduct a meta-analysis to assess the benefit and safety profile of drug-eluting stents in diabetic patients. METHODS:We ...

    journal_title：American heart journal

    pub_type： 杂志文章,meta分析,评审

    doi：10.1016/j.ahj.2007.11.017

    authors： Kumbhani DJ,Bavry AA,Kamdar AR,Helton TJ,Bhatt DL

    更新日期：2008-04-01 00:00:00

  • Thrombus Aspiration during Percutaneous coronary intervention in Acute myocardial infarction Study (TAPAS)--study design.

    abstract：BACKGROUND AND OBJECTIVE:Embolization of atherothrombotic material is common during percutaneous coronary intervention (PCI) in acute myocardial infarction (MI). This may lead to distal vessel occlusion resulting in impaired myocardial perfusion, which is associated with larger infarct size and increased mortality. Adj...

    journal_title：American heart journal

    pub_type： 杂志文章

    doi：10.1016/j.ahj.2005.11.010

    authors： Svilaas T,van der Horst IC,Zijlstra F

    更新日期：2006-03-01 00:00:00

  • Computer analysis of exercise-induced changes in QRS duration in patients with angina pectoris and in normal subjects.

    abstract：:Exercise-induced changes in QRS duration were assessed in 25 normal subjects and in 17 patients with stable ischemic heart disease. None had bundle branch block or were taking medications, and all patients had angina pectoris induced during the test. QRS duration and ST60 amplitude were measured by computer during res...

    journal_title：American heart journal

    pub_type： 杂志文章

    doi：10.1016/0002-8703(86)90640-x

    authors： Ahnve S,Sullivan M,Myers J,Froelicher V

    更新日期：1986-05-01 00:00:00

  • Direct pharmacologic and osmolal effects of contrast media on the mechanics of heart muscle isolated from cats.

    abstract：:We studied 56 right ventricular papillary muscles isolated from cats to determine whether the direct mechanical effects of contrast media are due to their pharmacologic action or to the hyperosmolality caused by them. A 10% solution of Renografin-76 abruptly decreased force 22% below control, then slowly increased it ...

    journal_title：American heart journal

    pub_type： 杂志文章

    doi：10.1016/0002-8703(84)90550-7

    authors： Chuck LH,Refsum H,Rouleau JL,Mathey D,Sievers RE,Parmley WW

    更新日期：1984-07-01 00:00:00