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Congenital partial and complete absence of the pericardium
J A Van Son1, G K Danielson, H V Schaff
1Division of Thoracic and Cardiovascular Surgery, Mayo Clinic Rochester, Minnesota 55905.
Insights
Congenital pericardial defects, though rare, can cause serious heart issues like valve insufficiency. Most patients with pericardial absence, however, experience no complications after cardiovascular surgery.
Area of Science:
- Cardiology
- Thoracic Surgery
- Medical Genetics
Background:
- Congenital absence of the pericardium is a rare condition.
- It can be partial or complete, affecting the left or right side.
- This abnormality is often discovered incidentally during cardiac procedures.
Purpose of the Study:
- To investigate the clinical significance and outcomes of pericardial absence in patients undergoing cardiovascular surgery.
- To identify potential complications associated with partial or complete pericardial deficiency.
Main Methods:
- Retrospective review of 15 patients with pericardial absence identified between 1952 and 1991.
- Analysis of patient data, including surgical procedures, symptoms, and postoperative outcomes.
- Case study of a patient with complete left pericardial absence and tricuspid valve insufficiency.
Main Results:
- Fifteen patients with partial or complete pericardial absence were identified.
- One patient with complete left pericardial absence experienced tricuspid valve insufficiency, possibly due to cardiac displacement.
- Two patients with small pericardial defects underwent repair; three patients with complex congenital heart disease died postoperatively.
- The remaining 12 patients had no early or late postoperative complications.
Conclusions:
- Congenital pericardial deficiency is typically asymptomatic but can lead to severe complications like cardiac valvular insufficiency or herniation.
- Early diagnosis and management are crucial for patients with symptomatic pericardial absence.
- Surgical intervention for associated cardiac conditions can be successful, even in rare cases of pericardial abnormalities.
Abstract:
Between 1952 and 1991, 15 Mayo patients were found to have partial or complete absence of the pericardium at the time of a cardiovascular surgical procedure. One patient with complete absence of the left pericardium had symptoms possibly related to the pericardial abnormality. This 42-year-old man had severe insufficiency of the tricuspid valve attributable to chordal rupture of the anterior leaflet, possibly precipitated by complete displacement of the heart into the left pleural space. Excision of the ruptured chordae and plication of the anterior flail leaflet rendered a competent tricuspid valve. In two patients, a small defect in the pericardium was repaired. Three patients who underwent operation for complex congenital heart disease died: two early postoperatively and one late after a reoperation. In the other 12 patients, no early or late postoperative complications were encountered. Although rare and usually asymptomatic, complete and partial deficiency of the pericardium may lead to serious complications such as cardiac valvular insufficiency or incarceration of cardiac tissue.