Khayata Mohamed, Alkharabsheh Saqer, Shah Nishant P, Verma Beni Rai, Gentry James L, Summers Mathew, Xu Bo, Asher Craig, Klein Allan L
Internal Medicine, Cleveland Clinic, Cleveland, Ohio, USA.
Cardiology, Cleveland Clinic, Cleveland, Ohio, USA.
Open Heart. 2020 Jan 8;7(1):e001103. doi: 10.1136/openhrt-2019-001103. eCollection 2020.
Congenital pericardial defect (CPD) is a rare entity with an estimated frequency of 0.01%-0.04%. The recognition of this anomaly is important since it can be associated with serious complications. The aim of this study and review was to describe clinical and imaging features that help in establishing the diagnosis of this condition.
We retrospectively reviewed all adult patients at the Cleveland Clinic Health System with the diagnosis of CPD between the years 2000 and 2015. Baseline clinical characteristics, clinical manifestations, ECG, transthoracic echocardiography (TTE), cardiac CT and cardiac magnetic resonance (CMR) images were reviewed.
Eight patients were included in the study. Sixty-three percent of patients were males with mean age at diagnosis of 48 years, 63% had a partial pericardial defect on the left side and right ventricular (RV) dilation on TTE. Three patients had CMR. Levocardia was present in all CMRs. One patient had greater than 60° clockwise rotation and none of the CMRs showed ballooning of the left ventricular apex. One patient required surgical pericardioplasty. The remaining seven patients had a median follow-up of 17.3 months (5-144.9 months) and all remained asymptomatic.
CPDs are more likely to be partial on the left side and patients often have RV dilation on the TTE and levocardia on CMR. Most patients remain stable and do not require surgical intervention. TTE and CMR play an important role in making the diagnosis of this anomaly.
先天性心包缺损（CPD）是一种罕见疾病，估计发病率为0.01% - 0.04%。认识这种异常情况很重要，因为它可能与严重并发症相关。本研究及综述的目的是描述有助于确立该疾病诊断的临床和影像学特征。
我们回顾性分析了2000年至2015年间克利夫兰诊所医疗系统中所有诊断为CPD的成年患者。回顾了基线临床特征、临床表现、心电图、经胸超声心动图（TTE）、心脏CT和心脏磁共振（CMR）图像。
8例患者纳入研究。63%的患者为男性，诊断时平均年龄48岁，63%的患者左侧存在部分心包缺损，TTE显示右心室（RV）扩张。3例患者进行了CMR检查。所有CMR检查均显示心脏左位。1例患者顺时针旋转大于60°，所有CMR检查均未显示左心室心尖膨出。1例患者需要进行外科心包成形术。其余7例患者中位随访17.3个月（5 - 144.9个月），均无症状。
CPD更可能为左侧部分缺损，患者TTE常显示RV扩张，CMR显示心脏左位。大多数患者病情稳定，无需手术干预。TTE和CMR在该异常情况的诊断中起重要作用。
