대한흉부영상의학회 Weekly Chest Cases

Case No : 1192 Date 2020-08-24

Courtesy of Seung Jin Yoo, Yo Won Choi / Hanyang University Hospital, Hanyang University College of Medicine
Age/Sex 54 / M
Chief Complaintdyspnea on exertion

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Diagnosis With Brief Discussion

Diagnosis: Congenital absence of the right pericardium (pericardial Defect)
Radiologic Findings: Figure 1. Chest PA view shows rightward displacement of the enlarged heart and double contour of of the right heart border, suggesting mitral valvular heart disease with enlarged left atrium.
Figure 2. Chest lateral view shows abnormal radiolucency between the ascending aorta and the right pulmonary artery. The right pulmonary artery is unusually dense and the anterior margin is well demarcated.
Figure 3-4. Mediastinal and lung window setting of the Chest CT scan shows interposed lung between the right pulmonary artery and the ascending aorta through a partially absent right pericardium.
Brief Review: Congenital pericardial defect is a very rare malformation, with a reported frequency of 0.01%. Congenital pericardial defect can be characterized by location and whether there is partial or complete absence of the pericardium. Most pericardial defects are partial and occur on the left side. Right pericardial defect is extremely uncommon. Patients are usually asymptomatic, however herniation and strangulation of the heart or lungs through the pericardial defect can happen. Pericardial defects allows interposition of lung tissue between the aorta and the main segment of the ipsilateral pulmonary artery, and, occasionally bulging of the ipsilateral atrial appendage through the defect. Thirty percent of patients have associated defects: atrial septal defect, bicuspid aortic valve, patent ductus arteriosus, and tetralogy of Fallot are the most common associated cardiac defects and which are detectable on CT or MR images.
In cases of congenital absence of the right pericardium, interposed lung between the right pulmonary artery and the ascending aorta produces retroaortic radiolucency with prominent right pulmonary artery margin on chest radiograph. If inferior aspect of the right pericardium is absent the interposed lung may create radiolucency between the right inferior cardiac border and the right hemidiaphragm. In instances of cardiac tissue herniation through a right pericardial defect, prominent bulge of the right heart border may be seen. On CT and MR, absence of pericardium, interposed lung parenchyma through the defect and herniation of right heart structure can be demonstrated.
Please refer to: KSTR imaging conference 2018 Summer Case 12,
References: 1. Zhen J. Wang, Gautham P. Reddy, Michael B. Gotway, Benjamin M. Yeh, Steven W. Hetts, and Charles B. Higgins. CT and MR Imaging of Pericardial Disease. RadioGraphics 2003 23: 167S-180S.
2. Koo CW, Newburg A. Congenital absence of the right pericardium: embryology and imaging. J Clin Imaging Sci. 2015;5:12.
Keywords: heart, congenital, pericardial defect,

No. of Applicants : 60

▶ Correct Answer : 27/60, 45.0%

- Saitama-Sekishinkai Hosptal , Japan MIHOKO YAMAZAKI
- Pamukkale University, Faculty of Medicine, Dept. of Radiology , Turkey FURKAN UFUK
- ZIGONG TCM HOSPITAL OF CHINA , China Cao Cunyou
- Kinki University Faculty of Medicine, , Japan MITSURU MATSUKI
- Showa University Fujigaoka Hospital , Japan KYOKO NAGAI
- radiologist, aditya imaging centre , India VIVEK PATEL
- Mayo Clinic , United States AKITOSHI INOUE
- CH de la Cote Basque , France PAUL ARDILOUZE
- NIMS, HYDERABAD , India BHASKAR K
- Tiger Gate Hospital , Japan SHIN-ICHI CHO
- , Korea (South) JIN YOUNG LEE
- The University of Tokyo Hospital , Japan TOSHIHIRO FURUTA
- Chungbuk National University Hospital , Korea (South) JUNGHWAN KIM
- The University of Tokyo Hospital , Japan RYO KUROKAWA
- Other , Korea (South) MINSU KIM
- University of Tsukuba, Dept of Radiology , Japan MANABU MINAMI
- Chonbuk National University Hospital , Korea (South) KUM JU CHAE
- Other , Korea (South) KYU-CHONG LEE
- Fortis hospital , Mohali , India SHALEEN RANA
- Toranomon Hospital , Japan KAORU SUMIDA
- Ichinomiya Nishi Hospital , Japan Takao Kiguchi
- , Japan KAZUMA TERAUCHI
- Private sector , Greece VASILIOS TZILAS
- , Japan YUMI MAEHARA
- Chonnam National University Hwasun Hospital , Korea (South) WONGI JEONG
- Chonnam National University Hospital , Korea (South) JONG EUN LEE
- the first affiliatited hospital of nanjing medical univercity , China HAI XU

▶ Correct Answer as Differential Diagnosis : 2/60, 3.3%

- Gifu University Hospital , Japan Yo Kaneko
- TB centre kasaragod. , India rikhy krishnan

대한흉부영상의학회 Korean Society of Thoracic Radiology

Cases by Disease Category

Case No : 1192 Date 2020-08-24

Discussion

No. of Applicants : 60