Centre de Cardiologie Clinique et Interventionnelle, Hôpital du Bocage, Centre Hospitalo-Universitaire de Dijon, Dijon, France.
Clin Cardiol. 2010 Feb;33(2):E52-7. doi: 10.1002/clc.20607.
Congenital defects of the pericardium are rare and poorly known cardiac malformations. Most of them are left-sided and asymptomatic and are usually incidentally diagnosed.
To describe the clinical, electrocardiographic, and imaging features of total absence of the left pericardium.
We report the cases of 3 patients who have been seen in our institution with a diagnosis of total congenital absence of the pericardium. All of them complained of precordial pain; one of them experienced disabling symptoms that justified surgical intervention. All of them had previously been suspected to have an atrial septal defect because of the echocardiographic appearance of right ventricular volume overload. Electrocardiogram, chest x-ray, echocardiography, and magnetic resonance imaging of the heart consistently showed remarkably similar features including leftward displacement, increased mobility, and interposition of lung tissue between the heart and other intrathoracic structures.
Congenital absence of the left pericardium should be known by clinicians as a possible differential diagnosis of chest pain or pseudo-right heart overload.
先天性心包缺损是罕见且知之甚少的心脏畸形。它们大多为左侧且无症状，通常为偶然诊断。
描述完全性左心包缺失的临床、心电图和影像学特征。
我们报告了 3 例在我院就诊的患者，他们被诊断为完全性先天性心包缺失。他们都主诉胸痛；其中 1 例因超声心动图显示右心室容量超负荷而出现致残性症状，需要手术干预。所有患者之前均因超声心动图表现怀疑房间隔缺损。心电图、胸部 X 线、超声心动图和心脏磁共振成像均显示出非常相似的特征，包括心脏向左移位、活动度增加以及肺组织介于心脏和其他胸腔内结构之间。
临床医生应将先天性左心包缺失视为胸痛或假性右心负荷过重的可能鉴别诊断。
