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A Case Report of Congenitally Absent Pericardium Masquerading as Recurrent Pericarditis
Tomoki Sempokuya1, Corey J Lum1, Mahdi Veillet-Chowdhury1
1Division of Cardiology, Department of Medicine, John A. Burns School of Medicine, University of Hawai'i, Honolulu, HI.
Summary
A rare congenital absence of the pericardium was diagnosed in a patient with recurrent chest pain, initially misdiagnosed as pericarditis. Characteristic imaging findings, including the "Snoopy Sign," aided diagnosis.
Area of Science:
- Cardiology
- Radiology
- Medical Imaging
Background:
- Congenital absence of the pericardium is a rare condition.
- It is often undiagnosed or misdiagnosed, leading to delayed treatment.
- Swyer-James-MacLeod syndrome can present with similar symptoms.
Observation:
- A 24-year-old female with Swyer-James-MacLeod syndrome presented with pleuritic chest pain.
- Initial diagnosis was acute pericarditis, treated successfully with anti-inflammatory medication.
- Recurrent symptoms prompted a re-evaluation of cardiac imaging.
Findings:
- Re-review of cardiac imaging revealed signs of congenitally absent pericardium.
- Characteristic findings included the "Snoopy Sign" on chest X-ray.
- Cardiac CT confirmed the diagnosis of absent pericardium.
Implications:
- This case highlights the importance of recognizing rare conditions.
- The "Snoopy Sign" is a key diagnostic indicator on chest X-ray.
- Increased awareness among healthcare providers can improve diagnosis and management of absent pericardium.
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