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Congenital defects of the pericardium: a review
Ankit B Shah1, Itzhak Kronzon2
1Lenox Hill Hospital, 100 East 77th Street, 2nd Floor Non-invasive Cardiology, New York, NY 10075, USA.
Insights
Congenital pericardial defects are rare but can be diagnosed using cardiac magnetic resonance. While often asymptomatic, severe cases pose life-threatening risks requiring tailored treatment.
Area of Science:
- Cardiology
- Medical Imaging
- Congenital Disorders
Background:
- Pericardial defects, a rare condition, present as either acquired or congenital.
- Congenital defects vary in location (left/right-sided) and extent (partial/complete absence of the pericardium).
Purpose of the Study:
- To review the diagnostic modalities and clinical implications of congenital pericardial defects.
- To highlight the gold standard for diagnosing congenital absence of the pericardium.
Main Methods:
- Review of diagnostic imaging techniques including chest radiographs, echocardiography, and cardiac magnetic resonance.
- Discussion of clinical presentation, diagnostic challenges, and treatment strategies.
Main Results:
- Physical examination and electrocardiogram are not diagnostic for pericardial defects.
- Chest radiographs and echocardiography can suggest the diagnosis.
- Cardiac magnetic resonance is the gold standard for diagnosing congenital absence of the pericardium.
- Most patients have a normal life expectancy, but complications like cardiac herniation can be fatal.
Conclusions:
- Congenital pericardial defects require accurate diagnosis, with cardiac MRI being the preferred method.
- While often benign, potential life-threatening complications necessitate individualized treatment plans based on defect characteristics and patient presentation.
Abstract:
Pericardial defects are a rare disorder that can be characterized as acquired or congenital. Congenital defects can be further characterized by location and size of the defect, e.g. left- or right-sided and partial or complete absence of the pericardium. While physical examination and electrocardiogram are not diagnostic, chest radiographs and echocardiography have findings that should alert the clinician to the absence of the pericardium as a possible diagnosis. Despite its limitations with visualizing the normal pericardium in areas of minimal adipose, cardiac magnetic resonance is currently the gold standard for diagnosing the congenital absence of the pericardium. Patients have a similar life expectancy to those without pericardial defects; however in certain cases, herniation and strangulation of cardiac chambers can be life threatening and lead to sudden cardiac death. Treatment is tailored to the patient's symptoms, presentation, and the size and location of the defect.
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