MYOCARDITIS IN BETA-THALASSEMIA MAJOR - A CAUSE OF HEART-FAILURE
MYOCARDITIS IN BETA-THALASSEMIA MAJOR - A CAUSE OF HEART-FAILURE
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DOI：
10.1161/01.cir.91.1.66
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发表时间：
1995-01-01
期刊：
影响因子：
37.8
通讯作者：
TOUTOUZAS, PK
中科院分区：
文献类型：
--
作者：
KREMASTINOS, DT;TINIAKOS, G;TOUTOUZAS, PK
背景虽然急性心包炎是一种常见的并发症β-地中海贫血重大，心肌炎在这种疾病的患病率和后果还没有investigated.Methods和结果一个前瞻性的5年随访研究进行了所有患者与β-地中海贫血重大急性感染性心肌炎的诊断可以建立在1977年和1986年之间。同样数量的年龄和性别匹配的β地中海贫血和左心室功能正常且无心肌炎证据的对照受试者也被随访5年。在1048例重型β地中海贫血患者中，47例心前区胸痛患者（年龄15 ± 2.5岁）被诊断为急性感染性心肌炎。心肌活检诊断性26例，临界性14例，非诊断性7例。11例（23.4%）心肌炎患者发生急性心力衰竭伴左室功能不全（左室射血分数25 ± 11%），其中8例在确诊后1个月~ 1年内死亡。13例心肌炎患者（27.6%）在3 ± 1.3年内发生慢性心力衰竭（左心室射血分数，26 ± 13%），其中10例在8 ± 3个月内死亡。对照组受试者的左心室收缩和舒张功能在5年期间没有显著变化（左心室射血分数，63+/-11% vs 65+/-7%; P=NS）。然而，3例平均血清铁蛋白水平极高的患者出现左心室限制性异常（早心室/晚心室，>2.2;减速时间，内径30 mm）和右侧心力衰竭。血清铁蛋白和输血前血红蛋白的平均水平与那些没有心肌炎。结论在β地中海贫血患者，心肌炎似乎参与左心室收缩功能障碍的发病机制，是主要的死亡原因。铁超载似乎引起左心室限制性异常，并伴有右心室扩大和功能障碍。
Background Although acute pericarditis is a common complication of beta-thalassemia major, the prevalence and consequences of myocarditis in this disease have not been investigated.Methods and Results A prospective 5-year follow-up study was carried out in all patients with beta-thalassemia major in whom the diagnosis of acute infectious myocarditis could be established between 1977 and 1986. A similar number of age- and sex-matched control subjects with beta-thalassemia and normal left ventricular function and no evidence of myocarditis were also followed for 5 years. Of 1048 patients with beta-thalassemia major, 47 patients (age, 15 +/- 2.5 years) with precordial chest pain were diagnosed as having acute infectious myocarditis. Myocardial biopsy was diagnostic in 26 patients, borderline in 14 patients, and nondiagnostic in 7 patients. Acute heart failure with left ventricular dysfunction (left ventricular ejection fraction, 25+/-11%) developed in 11 patients (23.4%) with myocarditis, and 8 of them died within 1 month to 1 year after diagnosis. Thirteen patients with myocarditis (27.6%) developed chronic heart failure (left ventricular ejection fraction, 26+/-13%) within 3+/-1.3 years, and 10 of them died within 8+/-3 months. Left ventricular systolic and diastolic functions of the control subjects did not change significantly during the 5-year period (left ventricular ejection fraction, 63+/-11% versus 65+/-7%; P=NS). However, left ventricular restrictive abnormalities (early diastole/late diastole, >2.2; deceleration time, 30 mm internal diameter) and right-sided heart failure developed in 3 patients with extremely high mean serum ferritin levels. No significant difference was found in mean levels of serum ferritin and pretransfusion hemoglobin between patients with and those without myocarditis.Conclusions In patients with beta-thalassemia, myocarditis appears to be involved in the pathogenesis of left ventricular systolic dysfunction, being the main cause of death. Iron overload appears to provoke left ventricular restrictive abnormalities combined with right ventricular enlargement and dysfunction.