Cecchi F, Olivotto I, Montereggi A, Santoro G, Dolara A, Maron B J
Cardiologia di San Luca, Ospedale di Careggi, Florence, Italy.
J Am Coll Cardiol. 1995 Nov 15;26(6):1529-36. doi: 10.1016/0735-1097(95)00353-3.
Our aim was to study a population of patients with hypertrophic cardiomyopathy from the well defined geographic region of Tuscany in central Italy, a group virtually free of selective referral bias and therefore probably closely representative of the true patient population with this disease.
Most available information on clinical course, natural history and prognosis of hypertrophic cardiomyopathy is based on data generated from tertiary referral centers and therefore constitutes a potentially biased perspective of the disease process in this complex and diverse condition.
The study group comprised 202 patients aged 1 to 74 years (mean +/- SD 41 +/- 17) at initial diagnosis and followed up for 1 to 30 years (mean 10 +/- 5).
Largely with the use of single or multiple drug therapy, the vast majority of patients (n = 154 [76%]) were asymptomatic or mildly symptomatic and in stable or improved condition over the period of follow-up, whereas the remaining patients (n = 48 [24%]) experienced deterioration, had substantial functional impairment or died. Of the 13 patients (6%) who died of cardiovascular causes related to hypertrophic cardiomyopathy, 11 had progressive congestive heart failure (including 6 in the end-stage phase) and only 2 died suddenly. The annual mortality rate for cardiovascular disease was 0.6% and that due to sudden cardiac death was only 0.1%; the cumulative survival rate was 97%, 95% and 92%, respectively, at 5, 10 and 15 years of follow-up. Atrial fibrillation proved to be a relatively common (n = 57 [28%]) and particularly unfavorable clinical feature, with premature death occurring in 9 of the 57 patients. The cumulative survival rate after 15 years was 76% for patients with atrial fibrillation versus 97% for patients with sinus rhythm. Syncope occurred in 33 patients (16%) but did not appear to be of prognostic significance.
In an unselected regional population, hypertrophic cardiomyopathy had a relatively benign prognosis inconsistent with its prior characterization as a generally progressive disorder, based primarily on the experience of selected referral institutions. Sudden unexpected cardiac death was distinctly uncommon, although a sizable proportion of patients (particularly the subset prone to atrial fibrillation), did experience clinical deterioration.
我们的目的是研究来自意大利中部托斯卡纳地区这个明确地理区域的肥厚型心肌病患者群体，该群体几乎不存在选择性转诊偏倚，因此可能非常接近这种疾病真实患者群体的代表。
关于肥厚型心肌病临床病程、自然病史和预后的大多数现有信息基于三级转诊中心产生的数据，因此在这种复杂多样的病症中，构成了对疾病进程的潜在偏倚观点。
研究组包括202例初诊时年龄为1至74岁（平均±标准差41±17）且随访1至30年（平均10±5）的患者。
在很大程度上通过使用单一或多种药物治疗，绝大多数患者（n = 154 [76%]）无症状或症状轻微，在随访期间病情稳定或改善，而其余患者（n = 48 [24%]）病情恶化、有严重功能障碍或死亡。在13例（6%）死于与肥厚型心肌病相关的心血管原因的患者中，11例有进行性充血性心力衰竭（包括6例处于终末期阶段），仅2例猝死。心血管疾病的年死亡率为0.6%，心源性猝死的年死亡率仅为0.1%；在随访5年、10年和15年时，累积生存率分别为97%、95%和92%。心房颤动被证明是一种相对常见（n = 57 [28%]）且特别不利的临床特征，57例患者中有9例过早死亡。心房颤动患者15年后的累积生存率为76%，而窦性心律患者为97%。33例患者（16%）发生晕厥，但似乎没有预后意义。
在一个未经选择的区域人群中，肥厚型心肌病的预后相对良好，这与其先前主要基于选定转诊机构的经验而被描述为一种通常进行性疾病的特征不一致。意外心源性猝死明显不常见，尽管相当一部分患者（特别是易于发生心房颤动的亚组）确实经历了临床恶化。
