Cabrera A, Hernáez E, Clerigue N, Modesto C, Zubía A, Pastor E, Alcíbar J, Izquierdo M A, Galdeano J M
Servicio de Cardiología Pediátrica, Hospital Infantil Cruces, Vizcaya.
Rev Esp Cardiol. 1990 Apr;43(4):246-50.
Between 1971-1988 we have studied 46 children with dilated cardiomyopathy. Twenty were males and 18 females, with a median age of 17 months (range from 1 day to 11 years). The incidence of 3.4 cases a year during the first nine years dropped to 1.3 cases a year in the following years. Two patients were siblings. Seventeen patients were studied with Eco-Doppler, cardiac catheterization and angiocardiography. The most frequent presentation was heart failure, present in 45 cases (98%). By bidimensional echocardiography it was observed that all patients had a dilated left ventricle, with a left atrial/aorta ratio of 1.5 +/- 0.3; the ejection fraction was diminished in 12 patients (11-36%) and the length of the left ventricle in the long parasternal axis view 4.2 +/- 0.7. All the patients studied had an elevated telediastolic pressure in the left ventricle, four with pulmonary artery pressure of 96 +/- 11 and seven with right atrial pressure of 24 +/- 5. There was mitral insufficiency in 4 patients and tricuspid insufficiency in three. Clinical course was favorable in 18 patients with median age at present of 112 months (range: 11 months to 15 years). The last 6 patients, treated with vasodilators, are living. In conclusion, dilated cardiomyopathy is a moderately frequent disease, with trend to diminish in incidence prognosis improved after treatment with vasodilator was introduced, and easily assessed with non-invasive methods.
1971年至1988年间，我们研究了46例扩张型心肌病患儿。其中男性20例，女性18例，中位年龄为17个月（范围从1天至11岁）。最初9年的发病率为每年3.4例，随后几年降至每年1.3例。有2例患者为同胞兄妹。对17例患者进行了超声心动图、心导管检查和心血管造影研究。最常见的表现是心力衰竭，45例（98%）出现心力衰竭。通过二维超声心动图观察到，所有患者的左心室均扩张，左心房/主动脉比率为1.5±0.3；12例患者（11%-36%）的射血分数降低，在胸骨旁长轴视图中左心室长度为4.2±0.7。所有接受研究的患者左心室舒张末期压力均升高，4例患者肺动脉压力为96±11，7例患者右心房压力为24±5。4例患者存在二尖瓣关闭不全，3例患者存在三尖瓣关闭不全。18例患者临床病程良好，目前中位年龄为112个月（范围：11个月至15岁）。最后6例接受血管扩张剂治疗的患者仍存活。总之，扩张型心肌病是一种较为常见的疾病，发病率呈下降趋势，引入血管扩张剂治疗后预后改善，且可通过非侵入性方法轻松评估。
