Isolated congenital absence of the pericardium: clinical presentation, diagnosis, and management

Congenital total absence of the pericardium: A case report

“…Congenital absence of the pericardium is a rare anomaly [1]. Right-sided or bilateral (complete) deficiencies are even rarer than left-sided defects.…”

“…Described chest pain differ in severity, type, duration and frequency; however, pain can mimic coronary artery disease in some cases [1]. Probable causes of pain include impaction, ischemia or heart mobility due to the pericardial defect [1,7].…”

“…Most reported cases of pericardial absence have been incidental findings during thoracic surgery and imaging for unrelated medical conditions, or at postmortem examination [1,9].…”

“…In contrast to complete absence of the pericardium, which is benign, partial absence may lead to herniation or strangulation of the heart or its appendages through the defect [1]. Thus, it is important to distinguish the partial from complete type when considering whether prophylactic surgery should be performed.…”

“…Primary closure, pericardiectomy, left atrial appendectomy, division of adhesions, extension of the defect, or pericardioplasty are the surgical procedures employed for partial defects or symptomatic patients [1,6]. For our patient, we recommended a β-blocker to treat palpitations, with close follow-up to ensure symptom resolution.…”