Abstract:
BACKGROUND:Noncompaction of the ventricular myocardium is a rare congenital cardiomyopathy characterized by numerous excessively prominent trabeculations and deep intertrabecular recesses. Noncompaction of the ventricular myocardium is most often an isolated cardiac malformation presenting as a sporadic disease. Associated cardiac anomalies are present in some patients. We report a family with three adult males from consecutive generations having a biventricular form of noncompaction of the myocardium. Two of the patients have an associated Ebstein's malformation of the tricuspid valve. METHODS:Clinical evaluation and follow-up, electrocardiography, echocardiography, heart catheterization, coronary angiography, contrast cineventriculography, and magnetic resonance imaging. RESULTS AND CONCLUSIONS:The association of noncompaction of the ventricular myocardium and Ebstein's malformation has not been reported so far. We believe that both defects were caused by a developmental arrest of the right ventricular myocardium. Echocardiography is the diagnostic modality of choice in patients and in the male relatives, irrespective of their clinical status. Thromboembolic events, cardiac rhythm disorders and heart failure mandate treatment. Anticoagulation treatment and implantation of cardioverter-defibrillator pacemaker have to be strongly considered in these patients.
journal_name
Int J Cardioljournal_title
International journal of cardiologyauthors
Sinkovec M,Kozelj M,Podnar Tdoi
10.1016/j.ijcard.2004.05.033subject
Has Abstractpub_date
2005-07-10 00:00:00pages
297-302issue
2eissn
0167-5273issn
1874-1754pii
S0167-5273(04)00483-8journal_volume
102pub_type
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journal_title:International journal of cardiology
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journal_title:International journal of cardiology
pub_type: 评论,信件
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journal_title:International journal of cardiology
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pub_type: 杂志文章,评审
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journal_title:International journal of cardiology
pub_type: 杂志文章
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journal_title:International journal of cardiology
pub_type: 杂志文章,评审
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pub_type: 临床试验,杂志文章
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pub_type: 杂志文章,评审
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journal_title:International journal of cardiology
pub_type: 杂志文章
doi:10.1016/j.ijcard.2018.03.020
更新日期:2018-06-15 00:00:00
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journal_title:International journal of cardiology
pub_type: 杂志文章
doi:10.1016/0167-5273(94)02231-7
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pub_type: 杂志文章
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更新日期:2013-09-01 00:00:00
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pub_type: 杂志文章,随机对照试验
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pub_type: 杂志文章
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journal_title:International journal of cardiology
pub_type: 杂志文章
doi:10.1016/j.ijcard.2005.08.037
更新日期:2006-06-16 00:00:00
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pub_type: 杂志文章
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journal_title:International journal of cardiology
pub_type: 临床试验,杂志文章,随机对照试验
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更新日期:1993-12-31 00:00:00
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journal_title:International journal of cardiology
pub_type: 杂志文章,多中心研究
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更新日期:2013-10-09 00:00:00
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journal_title:International journal of cardiology
pub_type: 杂志文章
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journal_title:International journal of cardiology
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
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更新日期:2005-04-20 00:00:00
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journal_title:International journal of cardiology
pub_type: 杂志文章
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更新日期:2018-09-01 00:00:00
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journal_title:International journal of cardiology
pub_type: 信件
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