Shingu Manami, Matsumoto Kensuke, Yamazaki Tetsu, Kawasaki Satoru, Nishisaki Hogara
Division of Cardiovascular Medicine, Department of Internal Medicine, Hyogo Prefectural Tamba Medical Center, 2002-7, Isou, Hikami-cho, Tamba-shi, Hyogo, Tamba 669-3495, Japan.
Eur Heart J Case Rep. 2025 Apr 21;9(5):ytaf200. doi: 10.1093/ehjcr/ytaf200. eCollection 2025 May.
The principal roles of the pericardium include protection from microorganisms, prevention of cardiac friction, and restriction of unlimited dilation of the heart. In the case of a congenital pericardial defect in which structural, electrophysiological, and haemodynamic abnormalities manifested during postural changes, we propose another indispensable pericardial function of cardiac central anchorage.
A 29-year-old man with atypical chest pain was referred to our hospital. Electrocardiography revealed fluctuations in the QRS transitional zone, electrical axis, and atrial polarity with body posture. Echocardiography revealed a far dorsally displaced heart, paradoxical motion of the interventricular septum (IVS), and hyperdynamic motion of the posterior wall in the left lateral decubitus position, which normalized to the right lateral decubitus position, along with significant haemodynamic alterations. Multidetector-row computed tomography revealed a complete absence of the left pericardium.
When pericardial fixation is impaired, the heart falls dorsally owing to gravity in the left lateral decubitus position, resulting in right ventricular overstretch and enlargement, which, in turn, results in compression of the left ventricle via a leftward shift of the IVS and a consequent reduction in cardiac output. Moreover, the energy generated by the myocardium, which should normally be concentrated only on blood ejection, would be distributed between the energy used for ejection and that used for the leap-up movement of the heart, reducing its energy efficiency. Through detailed observation of the congenital pericardial defect, haemodynamic insights into the important functions of the pericardium, which were not visible through static observation, were obtained.
心包的主要作用包括抵御微生物、防止心脏摩擦以及限制心脏无限制扩张。对于先天性心包缺损患者，在体位改变时会出现结构、电生理和血流动力学异常，我们提出心包还具有心脏中央固定这一不可或缺的功能。
一名29岁非典型胸痛男性被转诊至我院。心电图显示QRS波过渡区、电轴和心房极性随体位波动。超声心动图显示在左侧卧位时心脏明显向后移位、室间隔矛盾运动以及后壁运动增强，而在右侧卧位时恢复正常，同时伴有显著的血流动力学改变。多排螺旋计算机断层扫描显示左心包完全缺失。
当心包固定受损时，在左侧卧位心脏因重力作用向后下垂，导致右心室过度伸展和扩大，进而通过室间隔向左移位压迫左心室，从而使心输出量减少。此外，心肌产生的能量，正常情况下应仅集中用于射血，此时会在射血所用能量和心脏上移运动所用能量之间分配，降低其能量效率。通过对先天性心包缺损的详细观察，获得了关于心包重要功能的血流动力学见解，这些功能通过静态观察无法看到。
