Abstract:
BACKGROUND:The syndrome with an electrocardiographic pattern of right bundle branch block, ST segment elevation in leads V(1)to V(3)and sudden death is genetically determined and caused by mutations in the cardiac sodium channel. The inheritance of the disease is autosomal dominant. Sudden death may, however, occur from a variety of causes in relatives and patients with this syndrome. PATIENTS AND METHODS:Twenty-five Flemish families with this syndrome with a total of 334 members were studied. Affected members were recognized by means of a typical electrocardiogram either occurring spontaneously or after the intravenous administration of antiarrhythmic drugs. Sudden deaths in these families were classified as related or not to the syndrome by analysis of the data at the time of the event, mode of inheritance of the disease, and data provided by survivors. Results Of the 25 families with the syndrome, 18 were symptomatic (at least one sudden death related to the syndrome) and seven were asymptomatic (no sudden deaths related to the syndrome). In total, there were 42 sudden cardiac deaths (12% incidence). Twenty-four sudden deaths were related to the syndrome and all occurred in symptomatic families. Eighteen sudden deaths (43% of total sudden deaths) were not related to the syndrome (nine cases) or were of unclear cause (nine cases). Three of them occurred in two asymptomatic families and the remaining 15 in five symptomatic families. Twenty-four of the 50 affected members (47%) suffered (aborted) sudden death and 18 of the 284 unaffected members (6%). This difference in the incidence of sudden death was statistically significant (P<0.0001). Patients with (aborted) sudden death caused by the syndrome were younger than patients with sudden death of other or unclear causes (38+/-4 years vs 59+/-3 years respectively, P=0.0003). CONCLUSIONS:In families at high risk of sudden death because of genetically determined diseases, the main cause of sudden death remains the disease. However, almost the half of sudden deaths are caused by unrelated diseases or are of unclear cause. Accurate classification of the causes of sudden death is mandatory for appropriate analysis of the causes of death when designing preventive treatments.
journal_name
Eur Heart Jjournal_title
European heart journalauthors
Brugada P,Brugada R,Brugada Jdoi
10.1053/euhj.1999.1751subject
Has Abstractpub_date
2000-02-01 00:00:00pages
321-6issue
4eissn
0195-668Xissn
1522-9645pii
S0195668X99917517journal_volume
21pub_type
杂志文章abstract::In untreated congestive heart failure, aldosterone plasma concentrations are elevated in proportion to the severity of the disease and are further increased by the use of diuretic treatment. Angiotensin II, plasma potassium concentration, and corticotropin are the major stimulators of aldosterone synthesis. During ang...
journal_title:European heart journal
pub_type: 杂志文章,评审
doi:10.1093/eurheartj/16.suppl_n.98
更新日期:1995-12-01 00:00:00
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journal_title:European heart journal
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pub_type: 临床试验,杂志文章,随机对照试验
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pub_type: 杂志文章,meta分析,评审
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pub_type: 临床试验,杂志文章
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pub_type: 临床试验,杂志文章
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abstract::Bisoprolol is a new cardioselective beta-blocker with a long half-life. The efficacy of once daily bisoprolol (10 mg) and atenolol (100 mg) was assessed in 20 patients with stable angina using a placebo controlled double-blind randomized crossover protocol. Efficacy was assessed by computer assisted treadmill exercise...
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pub_type: 临床试验,杂志文章,随机对照试验
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pub_type: 临床试验,杂志文章,随机对照试验
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更新日期:1992-03-01 00:00:00
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doi:10.1093/eurheartj/ehl360
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更新日期:2020-02-21 00:00:00
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journal_title:European heart journal
pub_type: 杂志文章
doi:10.1053/euhj.1998.1096
更新日期:1998-11-01 00:00:00
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更新日期:2002-09-01 00:00:00