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Summary
Rare pericardial malformations, including absence of the pericardium, are uncommon. While complete absence often requires no surgery, partial defects need intervention to prevent fatal outcomes.
Area of Science:
- Cardiology
- Thoracic Surgery
- Developmental Biology
Background:
- Pericardial malformations and celomic pericardial cysts (CPC) are rare congenital anomalies.
- Literature review indicates a significant number of reported cases, with authors contributing to the case count.
Purpose of the Study:
- To review and analyze the incidence and characteristics of rare pericardial malformations and CPC.
- To delineate management strategies for different types of pericardial defects.
Main Methods:
- Systematic literature review of reported cases of pericardial malformations and CPC up to the year 2000.
- Analysis of defect types, patient demographics, and clinical presentations.
Main Results:
- 187 rare pericardial malformations and 1047 CPC cases identified in the literature.
- Complete absence of the pericardium occurred in 9.6% of cases, with left-sided absence being more common (40.6%) and prevalent in males.
- Partial pericardial defects were observed in 30.48% of malformation cases, with left-sided defects being significantly more frequent than right-sided ones.
Conclusions:
- Absence of the pericardium or its hemifield typically does not necessitate surgical intervention.
- Partial pericardial defects require surgical repair (suturing or plastic closure) to mitigate risks of strangulation and mortality.
- Celomic pericardial cysts are often asymptomatic, but symptomatic cases may present with dyspnea or palpitations; cystectomy is an effective treatment.