Diagnosis and management of congenital absence of pericardium: a case report

BACKGROUND: Absence of the pericardium is a rare congenital defect with an approximate incidence of <1/10 000. We review a case of complete pericardial agenesis in a symptomatic patient with gross cardiac mobility, for which pericardial reconstruction was undertaken successfully. CASE SUMMARY: A 24-year-old otherwise fit and well patient, with debilitating exertional chest pain was found to have complete pericardial agenesis on the left side and on the diaphragmatic surface. There was gross cardiac mobility demonstrated on cardiac magnetic resonance imaging. His pericardium was reconstructed surgically using Gore-tex(®) patches. There were no complications, and the patient was discharged 8 days later. Three months later at follow-up, the patient required no analgesia and has had complete resolution of his chest pains. DISCUSSION: Congenital hemi-pericardial agenesis is a very rare condition which often remains undetected due to its asymptomatic nature. It is important to consider this as a differential diagnosis of exertional chest pains. Cardiac magnetic resonance imaging remains the investigation of gold standard. There is no consensus on whether surgical intervention in symptomatic or asymptomatic patients has any prognostic value. However, we have demonstrated that by reconstructing the pericardium in a highly symptomatic patient, there has been a resolution in size of a previously dilated right ventricle and most importantly an improvement in quality of life.