Panidis I P, Ren J F, Holsclaw D S, Kotler M N, Mintz G S, Ross J
J Am Coll Cardiol. 1985 Sep;6(3):701-6. doi: 10.1016/s0735-1097(85)80134-0.
Two-dimensional and Doppler echocardiography were performed in 17 consecutive hospitalized patients with cystic fibrosis aged 6 to 38 years (mean 21 +/- 9) and in 10 normal subjects aged 24 +/- 7 years. Left ventricular and right ventricular ejection fraction were measured by a computerized light pen system and Simpson's rule from two-dimensional echocardiographic apical four and two chamber views. Right ventricular wall thickness, inferior vena cava size and the presence of tricuspid regurgitation by Doppler recording were also assessed. National Institutes of Health (NIH) score of clinical severity ranged from 22 to 72 (mean 51 +/- 15) (100 = excellent, 0 = poor). Four patients, all with an NIH score of 40 or less, died of respiratory failure within 1 year of the echocardiographic study. There was no significant difference between patients with cystic fibrosis and normal subjects with regard to right ventricular ejection fraction (59 +/- 11 versus 61 +/- 10%), left ventricular ejection fraction (67 +/- 8 versus 70 +/- 8%) and right ventricular systolic (5 +/- 1 versus 5 +/- 0.5 mm) and diastolic (2.4 +/- 0.5 versus 2.5 +/- 0.5 mm) wall thicknesses. A dilated inferior vena cava and mild tricuspid regurgitation by Doppler recording were detected in only one patient. A poor correlation was found between right ventricular ejection fraction and NIH clinical score (r = 0.26), chest X-ray score (r = 0.29) and pulmonary function tests. It is concluded that right and left ventricular systolic function is preserved in patients with moderately severe cystic fibrosis; clinical status in these patients is probably determined by the pulmonary rather than cardiac involvement.
对17例年龄在6至38岁（平均21±9岁）的住院囊性纤维化患者及10例年龄在24±7岁的正常受试者进行了二维和多普勒超声心动图检查。通过计算机光笔系统和Simpson法则，从二维超声心动图心尖四腔和两腔视图测量左心室和右心室射血分数。还评估了右心室壁厚度、下腔静脉大小以及通过多普勒记录检测到的三尖瓣反流情况。美国国立卫生研究院（NIH）临床严重程度评分范围为22至72（平均51±15）（100分表示极佳，0分表示极差）。4例患者，均NIH评分在40分及以下，在超声心动图检查后1年内死于呼吸衰竭。囊性纤维化患者与正常受试者在右心室射血分数（59±11%对61±10%）、左心室射血分数（67±8%对70±8%）以及右心室收缩期（5±1对5±0.5mm）和舒张期（2.4±0.5对2.5±0.5mm）壁厚度方面无显著差异。仅1例患者检测到下腔静脉扩张和多普勒记录显示的轻度三尖瓣反流。右心室射血分数与NIH临床评分（r = 0.26）、胸部X线评分（r = 0.29）和肺功能测试之间相关性较差。结论是中度严重囊性纤维化患者的左右心室收缩功能得以保留；这些患者的临床状况可能由肺部而非心脏受累决定。
