von Bernuth G, Nissen H, Lang D, Hofstetter R
Z Kardiol. 1976 Nov;65(11):1022-32.
Three patients with congenital absence of the left pericardium and one patient with a partial left pericardial defect are described. Congenital absence of the left pericardium is characterized roentgenologically by levoposition of the heart, a prominent pulmonary artery segment and cardiac apex, interposition of lung between the left hemidiaphragm and the base of the heart, and unusual mobility of the heart. The electrocardiogram frequently shows right axis deviation, an incomplete right bundle branch block and leftward displacement of the transition zone in the precordial leeds. The anomaly has often been mistaken for other heart disease. As an isolated anomaly it is benign and does not require any treatment. Patients with a partial defect of the left pericardium can only be diagnosed lcinically if the left atrial appendage herniates through the defect. The herniation produces a protruding shadow along the left heart border and can be demonstrated angiographically and by fluoroscopy. In isolated cases death caused by herniation and strangulation of larger parts of the heart through a partial left pericardial defect has been described.
本文描述了3例先天性左心包缺如患者和1例部分性左心包缺损患者。先天性左心包缺如的X线特征为心脏左移、肺动脉段和心尖突出、左半膈肌与心底之间有肺组织插入以及心脏异常活动。心电图常显示电轴右偏、不完全性右束支传导阻滞和胸前导联过渡区左移。该异常常被误诊为其他心脏病。作为一种孤立的异常情况，它是良性的，无需任何治疗。部分性左心包缺损患者只有在左心耳通过缺损处疝出时才能在临床上被诊断出来。疝出会沿心脏左缘产生一个突出的阴影，血管造影和荧光透视检查可以显示该阴影。在个别病例中，曾有因部分性左心包缺损导致心脏较大部分疝出并绞窄而死亡的报道。
