Akagi T, Benson L N, Lightfoot N E, Chin K, Wilson G, Freedom R M
Department of Pediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada.
Am Heart J. 1991 May;121(5):1502-6. doi: 10.1016/0002-8703(91)90158-e.
To assess the natural history and potential risk factors in childhood dilated cardiomyopathy, we investigated 25 patients (ages 9.6 +/- 4.4 years) who presented after they were 2 years old. All patients had symptoms of congestive heart failure and reduced contractility with a dilated left ventricle at presentation. Two factors at presentation were significantly different between patients who died less than 1 year after the presentation (n = 14) and those who survived for more than 1 year (n = 9); cardiothoracic ratio (65.1% +/- 6.8% vs 57.1% +/- 6.1%, p less than 0.01) and left ventricular ejection fraction (31.3% +/- 7.0% vs 40.0% +/- 6.2%, p less than 0.05). Irrespective of intensive medical therapy, dilated cardiomyopathy in children had a poor prognosis; the actuarial survival rate was 41% at 1 year and 20% at 3 years. Other forms of therapy should be considered in the early stages of dilated cardiomyopathy in this high-risk group.
为评估儿童扩张型心肌病的自然病史和潜在危险因素，我们对25例2岁以后发病的患者（年龄9.6±4.4岁）进行了调查。所有患者在就诊时均有充血性心力衰竭症状，且左心室扩张，收缩力降低。在就诊后1年内死亡的患者（n = 14）和存活超过1年的患者（n = 9）之间，有两个就诊时的因素存在显著差异；心胸比率（65.1%±6.8%对57.1%±6.1%，p＜0.01）和左心室射血分数（31.3%±7.0%对40.0%±6.2%，p＜0.05）。无论采取强化药物治疗，儿童扩张型心肌病的预后都很差；1年时的精算生存率为41%，3年时为20%。对于这个高危组的扩张型心肌病早期患者，应考虑其他治疗方式。
