Pericardial agenesis

Case contributed by Sean Huggins , 28 Aug 2017
Diagnosis certain
Changed by Sean Huggins, 7 Sep 2017
Hidden edits. Some edits not affecting the appearance of this case have been suppressed.

Updates to Study Attributes

Modality was set to CT.
Findings was added:

Heart in levoposition with the apex positioned posteriorly. Contrast can be see 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. 

Updates to Study Attributes

Modality was set to CT.
Findings was added:

Heart in levoposition with the apex positioned posteriorly. Contrast can be see 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. 

Updates to Study Attributes

Findings was changed:

Heart in levoposition with the apex positioned posteriorly. Contrast can be see 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. Hypoplastic left lower lobe.

Images Changes:

Image CT (C+ arterial phase) ( update )

Perspective was set to Coronal.
Specifics was set to C+ arterial phase.

Image CT (C+ arterial phase) ( update )

Perspective was set to Sagittal.
Specifics was set to C+ arterial phase.

Image 2 CT (C+ arterial phase) ( create )

Image 3 CT (C+ arterial phase) ( create )

Updates to Case Attributes

Presentation was changed:
Known congenital pericardial agenesis on the left side, confirmed during surgery 8 years prior for an ascending aorta graft performed 8 years prior (hadaneurysm (secondary to a bicuspid aortic valve [tricuspid with a fused raphe].) receivedA supracoronary ascending aorta replacement graft was performed at the time. Current imaging was performed to look forrule out a source of peripheral emboli.
Body was changed:

The patient was later placed on anticoagulation for treatment of the thrombus with follow-up scheduled in a month's3 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.Cardiovascular RadiologistErasmus Medical CenterRotterdam, The Netherlands

  • -<p>The patient was later placed on anticoagulation for treatment of the thrombus with follow-up scheduled in a month's time to assess for resolution.</p><p>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.</p><p>This case was submitted with supervision and input from:</p><p>M. Attrach, M.D.<br>Cardiovascular Radiologist<br>Erasmus Medical Center<br>Rotterdam, Netherlands</p>
  • +<p>The patient was later placed on anticoagulation for treatment of the thrombus with follow-up scheduled in 3 months time to assess for resolution.</p><p>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.</p><p>This case was submitted with supervision and input from:</p><p>M. Attrach, M.D.<br>Cardiovascular Radiologist<br>Erasmus Medical Center<br>Rotterdam, The Netherlands</p>

Updates to Quizquestion Attributes

Answer was changed:
Partial agenesis is more likely to have complications, herniation with incarceration of tissues in particular.

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