Russell A. Morgan Department of Radiology and Radiological Sciences, Johns Hopkins University School of Medicine, 601 N Caroline St, JHOC 3142, Box 0818, Baltimore, MD 21287, USA.
J Cardiovasc Comput Tomogr. 2013 Jan-Feb;7(1):11-7. doi: 10.1016/j.jcct.2013.01.003. Epub 2013 Jan 19.
Congenital absence of the pericardium is a rare entity, with less than 400 cases reported in the literature. Pericardial absence is typically left sided, which results in herniation of the great vessels or portions of the heart. Patients may be asymptomatic, typical for complete defects, or can present with various degrees of chest pain in the setting of partial absence and strangulation. The finding may be isolated or associated with complex heart disease. We present a number of cases of pericardial absence that show isolated right- and left-sided defects, as well as entities in the differential diagnosis. Early recognition may decrease morbidity and mortality, resulting from delay in proper treatment.
先天性心包缺失较为罕见，文献中报道的病例不足 400 例。心包缺失通常为左侧，导致大血管或部分心脏疝出。患者可能无症状，这是完全性缺损的典型表现，也可能表现为部分缺失和绞窄时不同程度的胸痛。心包缺失可孤立存在，也可与复杂心脏病相关。我们展示了一些心包缺失病例，既有单纯的左侧和右侧缺陷，也有鉴别诊断中的其他情况。早期识别可能会降低因治疗不及时而导致的发病率和死亡率。
