Known congenital pericardial agenesis on the left side, confirmed during surgery 8 years prior for an ascending aorta aneurysm (secondary to a bicuspid aortic valve.) A supracoronary ascending aorta replacement graft was performed at the time. Current imaging was performed to rule out a source of peripheral emboli.
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Heart in levoposition with the apex positioned posteriorly. Contrast can be seen entering the left atrium from the pulmonary veins, passing into the left ventricle, and leaving through the ascending aortic graft (sternotomy wires also present). Thrombus can be see in the ascending aorta.
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The patient was later placed on anticoagulation for treatment of the thrombus with follow-up scheduled in 3 months time to assess for resolution.
Pericardial agenesis is caused by defective development of the pleuropericardial membranes. Congenital pericardial agenesis is usually asymptomatic and discovered incidentally. Patients may present with a right bundle branch block on EKG, poor R wave progression, and prominent P waves. Complete pericardial agenesis has the best prognosis whereas partial agenesis may leave opportunity for herniation of tissues. Intervention should only be considered if complications of the defect are present.
This case was submitted with supervision and input from:
M. Attrach, M.D.
Erasmus Medical Center
Rotterdam, The Netherlands
- Kean-Wah Lau, Zee-Pin Ding. Partial Pericardial Defect. Circulation. 1998;97:1992, originally published May 19, 1998. doi:10.1161/01.CIR.97.19.1992
- Kim HJ, Cho YS, Cho GY, Choi SI. Congenital absence of the pericardium. Journal of cardiovascular ultrasound. 22 (1): 36-9. doi:10.4250/jcu.2014.22.1.36 - Pubmed