Chan Sherwin S, Whitehead Kevin K, Kim Timothy S, Fu Gregory L, Keller Marc S, Fogel Mark A, Harris Matthew A
Department of Radiology, Children's Mercy Hospital and Clinics, 2401 Gillham Road, Kansas City, MO, 64108, USA,
Pediatr Radiol. 2015 Sep;45(10):1465-71. doi: 10.1007/s00247-015-3358-0. Epub 2015 May 3.
There is an established association between tetralogy of Fallot and partial anomalous pulmonary venous connections. This association is important because surgically repaired tetralogy patients have increased risk of right heart failure. We hypothesize that partial anomalous venous connections increase right ventricular volumes and worsen right ventricular failure.
We reviewed cardiac MRI exams performed at a tertiary pediatric hospital from January 2005 to January 2014. We identified patients with repaired tetralogy and unrepaired partial anomalous pulmonary venous connection. We used age- and gender-matched repaired tetralogy patients without partial anomalous pulmonary venous connection as controls. We analyzed the MRI results and surgical course and performed comparative statistics to identify group differences.
There were eight patients with repaired tetralogy and unrepaired partial anomalous pulmonary venous connection and 16 controls. In all cases, the partial anomalous pulmonary venous connection was not detected on preoperative echocardiography. There were no significant differences in surgical course and body surface area between the two groups. Repaired tetralogy patients with unrepaired partial anomalous pulmonary venous connection showed significantly higher indexed right ventricular end diastolic volume (149 ± 33 mL/m(2) vs. 118 ± 30 mL/m(2)), right ventricle to left ventricle size ratios (3.1 ± 1.3 vs. 1.9 ± 0.5) and a higher incidence of reduced right ventricular ejection fraction compared to controls (3/8 vs. 0/16).
Repaired tetralogy of Fallot with unrepaired partial anomalous pulmonary venous connection is associated with reduced right ventricular ejection fraction and more significant right ventricular dilation.
法洛四联症与部分性肺静脉异位连接之间存在既定关联。这种关联很重要，因为接受手术修复的法洛四联症患者发生右心衰竭的风险增加。我们假设部分性肺静脉异位连接会增加右心室容积并加重右心室衰竭。
我们回顾了2005年1月至2014年1月在一家三级儿科医院进行的心脏MRI检查。我们确定了接受修复的法洛四联症且存在未修复的部分性肺静脉异位连接的患者。我们将年龄和性别匹配的无部分性肺静脉异位连接的接受修复的法洛四联症患者作为对照。我们分析了MRI结果和手术过程，并进行比较统计以确定组间差异。
有8例接受修复的法洛四联症且存在未修复的部分性肺静脉异位连接的患者和16例对照。在所有病例中，术前超声心动图均未检测到部分性肺静脉异位连接。两组在手术过程和体表面积方面无显著差异。与对照组相比，存在未修复的部分性肺静脉异位连接的接受修复的法洛四联症患者显示出显著更高的右心室舒张末期指数容积（149±33 mL/m² 对 118±30 mL/m²）、右心室与左心室大小比值（3.1±1.3 对 1.9±0.5）以及更低的右心室射血分数发生率（3/8 对 0/16）。
存在未修复的部分性肺静脉异位连接的接受修复的法洛四联症与右心室射血分数降低和更显著的右心室扩张相关。
