Kim Min Jeong, Kim Hyung-Kwan, Jung Ji-Hyun, Yoon Yeonyee E, Kim Hack-Lyoung, Park Jun-Bin, Lee Seung-Pyo, Kim Yong-Jin, Cho Goo-Young, Sohn Dae-Won, Oh Jae K
Section of Cardiovascular Imaging, Division of Cardiology, Cardiovascular Center, Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea.
Sejong General Hospital, Division of Cardiology, Department of Internal Medicine, Bucheon-si, Gyeonggi-do, Korea.
Heart. 2017 Aug;103(15):1203-1209. doi: 10.1136/heartjnl-2016-310870. Epub 2017 Mar 9.
Congenital absence of the pericardium (CAP) is often confused with other conditions presenting with right ventricular dilatation and usually warrants CT or cardiac MR (CMR) to confirm. It would be desirable to have more specific echocardiographic criteria to provide a conclusive diagnosis.
11 patients who were diagnosed with CAP (four patients with total CAP) based on CT/CMR were consecutively enrolled. Thirteen patients with atrial septal defect (ASD) and 16 normal subjects served as controls. To investigate spatial changes of heart in the thoracic cavity in CAP, following echocardiographic measurements were made in the left and right decubitus positions: the angle between the ultrasound beam and the left ventricular posterior wall (Angle-) in end-diastole at the parasternal long axis, and the distance between the chest wall and the most distal part of the left ventricular posterior wall (Distance-) at the parasternal mid-ventricular short axis.
Angle- in patients with CAP were significantly greater than in those with ASD (100.1±12.5° vs 74.5±8.6°, p<0.017) or in normal subjects (100.1±12.5° vs 69.9±7.6°, p<0.017) at the left decubitus, and the difference in Angle- according to posture (left vs right) was significantly greater in CAP compared with the other groups (CAP 20.7±12.7°, ASD 0.31±1.80°, normal 0.31±1.40°, all p<0.017). The differences in Distance- according to patient position (CAP 2.43±0.77°, ASD 0.42±0.45°, normal 0.26±0.55°) or cardiac cycle in each position (left: CAP 1.60±0.76°, ASD 0.41±0.27°, normal 0.17±0.12°; right: CAP 0.70±0.32°, ASD 0.22±0.19°, normal 0.22±0.13°) were significantly higher in the CAP group than in the other groups (all p<0.017).
Patients with CAP have dynamic alteration in cardiac position depending on posture, which is not observed in ASD or in normal controls. Hence, total or left-sided CAP can be reliably diagnosed with positional changes during routine echocardiography.
先天性心包缺如（CAP）常与其他导致右心室扩张的病症相混淆，通常需要进行CT或心脏磁共振成像（CMR）来确诊。若能有更具特异性的超声心动图标准以做出确定性诊断则更佳。
连续纳入11例经CT/CMR诊断为CAP的患者（4例为完全性CAP）。13例房间隔缺损（ASD）患者和16名正常受试者作为对照。为研究CAP患者胸腔内心脏的空间变化，在左侧卧位和右侧卧位进行以下超声心动图测量：胸骨旁长轴舒张末期超声束与左心室后壁的夹角（夹角-），以及胸骨旁心室短轴时胸壁与左心室后壁最远端的距离（距离-）。
左侧卧位时，CAP患者的夹角-显著大于ASD患者（100.1±12.5°对74.5±8.6°，p<0.017）或正常受试者（100.1±12.5°对69.9±7.6°，p<0.017），且与其他组相比，CAP患者根据体位（左侧与右侧）的夹角-差异显著更大（CAP为20.7±12.7°，ASD为0.31±1.80°，正常为0.31±1.40°，均p<0.017）。CAP组根据患者体位（CAP为2.43±0.77°，ASD为0.42±0.45°，正常为0.26±0.55°）或各体位心动周期（左侧：CAP为1.60±0.76°，ASD为0.41±0.27°，正常为0.17±0.12°；右侧：CAP为0.70±0.32°，ASD为0.22±0.19°，正常为0.22±0.13°）的距离-差异均显著高于其他组（均p<0.017）。
CAP患者心脏位置随体位有动态改变，而ASD患者或正常对照中未观察到这种情况。因此，在常规超声心动图检查中，根据位置变化可可靠诊断完全性或左侧CAP。
