Freedom in the chest

Abstract

Pericardial agenesis is a rarely seen congenital defect characterised by the partial or, more rarely complete, absence of the pericardium. Most often asymptomatic, it is usually incidentally discovered following the demonstration of heart's laevorotation on imaging, in the operating room or at autopsy. In this article, we report the case of an 80-year-old patient with asymptomatic complete pericardial agenesis fortuitous discovered. Pericardial agenesis observations are extremely uncommon reported in the literature, which substantiate its original epidemiological character. In addition, this observation brings some clinical, electrical as well as iconographic elements to better understand the pathology and raises clinical suspicions. Finally, this case report confirms the exceptionally symptomatic nature of the pathology, illustrating the irrelevance of treatment or specific follow-up.

Complete agenesis of pericardium in a young asymptomatic woman

“…It is possible to distinguish 6 types of congenital agenesis of pericardium: complete bilateral agenesis, left complete and partial agenesis, right complete and partial agenesis and finally a diaphragmatic form. More than 70% of cases are represented by complete left agenesis of pericardium [ 2 , 14 ]. About 30%-50% of cases are associated with other cardiac anomalies, like patent ductus arteriosus, mitral valve stenosis, bronchogenic cyst, ventricular septal defect as in the case of tetralogy of Fallot.…”

“…Surgical options include pericardioplasty or pericardiectomy with or without valve replacement if needed [ 12 ]. Left appendage strangulation is treated with excision and closure with patch [ 2 , 7 , 9 , 14 , 16 ]. In our case, due to the absence of symptoms there was no need of treatment.…”