Congenital pericardium anomaly

Congenital pericardium anomaly, also known as congenital absence of the pericardium or congenital pericardial defect, refers to a rare developmental malformation in which the pericardium — the fibrous sac surrounding the heart — is partially or completely absent from birth. This condition results from premature atrophy of the left common cardinal vein (duct of Cuvier) during embryonic development, which disrupts the blood supply to the developing pleuropericardial membrane. The defect most commonly involves the left side of the pericardium, though right-sided and bilateral (complete) absence can also occur. Many individuals with congenital pericardial defects are asymptomatic, and the condition is often discovered incidentally during imaging or surgery performed for other reasons. However, some patients may experience chest pain, dyspnea, palpitations, or syncope. Partial left-sided defects carry a risk of cardiac herniation, in which part of the heart (particularly the left atrial appendage) can become trapped in the defect, potentially leading to strangulation, ischemia, or sudden death. Complete absence of the pericardium is generally better tolerated. The condition may be associated with other congenital anomalies, including bronchogenic cysts, atrial septal defects, patent ductus arteriosus, and mitral valve abnormalities. Diagnosis is typically made through echocardiography, CT scan, or MRI of the chest. Asymptomatic patients with complete pericardial absence generally require no treatment beyond periodic monitoring. Symptomatic patients or those with partial defects at risk for cardiac herniation may require surgical intervention, which can include pericardioplasty (reconstruction of the pericardium using synthetic material or autologous tissue) or surgical widening of the defect to prevent herniation.

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