2019
DOI: 10.1259/bjrcr.20180117
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Congenital absence of pericardium: two cases and a comprehensive review of the literature

Abstract: Congenital absence of pericardium (CAP) is a rare condition, generally asymptomatic or paucisymptomatic, nevertheless sporadic cases complicated by sudden death are described. CAP can be diagnosed by CT and MRI. It is classified as total or partial, and partial defects are divided into left defects and right defects. Interestingly, several articles highlight the correlation between CAP and some anatomical lung abnormalities, such as presence of lung parenchyma between the main pulmonary artery and ascending … Show more

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Cited by 7 publications
(9 citation statements)
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“…Congenital absence of pericardium is often difficult to diagnose, and it has no specific symptoms and signs, so it is often ignored. CAP has certain imaging diagnostic features ( 6 , 7 ): (1) pulmonary parenchyma between the main pulmonary artery and the ascending aorta; (2) pulmonary parenchyma between the base of the heart and the left diaphragmatic muscle; (3) pulmonary parenchyma between the ascending main aorta and the right pulmonary artery; (4) “SNOOPY” sign on chest radiograph. Even so, it is still very difficult to clinically diagnose CAP.…”
Section: Discussionmentioning
confidence: 99%
“…Congenital absence of pericardium is often difficult to diagnose, and it has no specific symptoms and signs, so it is often ignored. CAP has certain imaging diagnostic features ( 6 , 7 ): (1) pulmonary parenchyma between the main pulmonary artery and the ascending aorta; (2) pulmonary parenchyma between the base of the heart and the left diaphragmatic muscle; (3) pulmonary parenchyma between the ascending main aorta and the right pulmonary artery; (4) “SNOOPY” sign on chest radiograph. Even so, it is still very difficult to clinically diagnose CAP.…”
Section: Discussionmentioning
confidence: 99%
“…The most frequent defect is the left side absence of the pericardium while right side defects and complete agenesis are quite rare (Abbas et al 2005;Southworth and Stevenson 1938). Partial defects are rarer but of clinical importance as they can cause myocardial strangulation and death; however, they are usually asymptomatic or paucisymptomatic (Rehkämper et al 2017;Centola et al 2009;Sergio et al 2019).…”
Section: Discussionmentioning
confidence: 99%
“…In addition, there are congenital abnormalities outside the heart, such as bronchogenic cyst reported in this case, as well as pulmonary isolation, and diaphragmatic hernia. Congenital pericardial defect is also associated with abnormal pulmonary anatomy, such as the presence of pulmonary parenchyma between the pulmonary artery and aorta[ 21 ]. Imperatori et al [ 19 ] has reported 18 cases of bronchogenic cyst with congenital pericardial defect.…”
Section: Discussionmentioning
confidence: 99%