대한흉부영상의학회 Weekly Chest Cases

Case No : 454 Date 2006-07-10

Courtesy of Young Jin Kim, MD, Ji Eun Nam, MD, Kyu Ok Choe, MD. / Severance Hospital, Yonsei University College of Medicine, Seoul, Korea
Age/Sex 48 / F
Chief ComplaintNon-exertonal periodic chest pain

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Diagnosis With Brief Discussion

Diagnosis: Congenital Absence of the Pericardium
Radiologic Findings: Chest radiograph shows levoposition of the cardiac silhouette, loss of the right heart border, prominent main pulmonary artery, and a “tongue” of lung tissue interposing between the main pulmonary artery and aorta.

MRI demonstrates marked displacement of the heart into the left hemithorax with the cardiac apex pointing posteriorly, the main pulmonary artery extending far beyond the mediastinal margins into the left lung, and the pathognomic presence of lung parenchyma in the usually lung-free aortopulmonary space. No pericardium is present over most of the heart. Coronal image shows the lung extending below the heart, interposing between its inferior surface and the left diaphragm.
Brief Review: Congenital defects (or absence) of the pericardium are a relatively rare anomaly that was first described in 1559 by Columbus. Pericardial defects have generally been classified as being complete or partial. In the majority of cases, pericardial defects affect the left side of pericardium rather than the right.

Congenital absence of the pericardium has a common presentation pattern with periodic stabbing chest pain that may mimic coronary artery disease. Chest radiography and MRI are required for definitive diagnosis. Characteristic imaging findings are described above.
References: 1. Gatzoulis MA, Munk MD, Merchant N, et al. Isolated congenital absence of the pericardium: clinical presentation, diagnosis, and management. Ann Thorac Surg. 2000;69:1209-1215

2. Raman SV, Daniels CJ, Katz SE, et al. Congenital absence of the pericardium. Circulation. 2001;104:1447-1448.

3. Faridah Y, Julsrud PR. Congenital absence of pericardium revisited. Int J Cardiovasc Imaging. 2002;18:67-73.
Keywords: Pericardium, Congenital,

No. of Applicants : 32

▶ Correct Answer : 19/32, 59.4%

- Hopital Calmette, Chru, Lille, France Toledano Manuel
- IRCCS S.Luca Hospital, Milano,Italy Filippo Casolo
- Annecy Hospital, France Gilles Genin
- Max Hospital , Saket, New Delhi. India Anurag Jain
- Maimonides Medical Center, New York, USA Naomi Twersky
- Asan Medical Center, Korea Eun Jin Chae
- University of Colorado Health Sciences Center, Denver, USA Jeff Grossman
- Annemasse, Polyclinique de Savoie, Gay-Dpassier Philippe
- Gwangmyoung Sungae Hospital, Korea Jiyong Rhee
- Mubarak Al Kabeer Hospital, Kuwait Varghese Sajeev P.J.
- Sam Anyang Hospital, Korea Jae Seung Seo
- Doctors Hospital, Nassau, Bahamas N.B.S.Mani
- Seoul National University Hospital, Korea Chang Min Park
- University of Colorado, USA Seth Kligerman
- Radiologie Guiton, La Rochelle, France Denis Chabassiere
- China Medical University Hospital,Taiwan Jun-Jun Yeh
- Shinchon Severance, Yonsei University, Korea Hye-Jeong Lee
- Shinchon Severance, Yonsei University, Korea Yong Eun Chung
- Hangang Sacred Heart Hospital, Korea Eil Seong Lee

▶ Correct Answer as Differential Diagnosis : 2/32, 6.3%

- Zulekha Hospital, Dubai Saurabh Khandelwal
- Diskapi Yildirim Beyazit Hospital, Ankara, Turkey Meric Tuzun

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 454 Date 2006-07-10

Discussion

No. of Applicants : 32