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

Abstract

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.

Congenital Partial Absence of Pericardium in an Elderly Patient

“…It is usually detected incidentally during imaging or by autopsy [ 1 ]. It is often associated with other congenital cardiac defects such as the tetralogy of Fallot or atrial septal defects [ 4 ]. Most patients remain asymptomatic and are diagnosed incidentally, and few patients present with palpitations and chest pain [ 7 ].…”

“…Patients with a complete absence of pericardium require no intervention as they are mostly asymptomatic, and the risk of cavity herniation and coronary artery damage is low. Due to the risk of herniation and other fatal complications, patients with partial absence of pericardium tend to present with symptoms and may benefit from treatment [ 4 ]. If the herniation is causing major defects, the pericardium can be removed in order to transform the partial defect into a complete defect resulting in a reduction of symptoms [ 4 ].…”

“…Echocardiography, CT scan, and magnetic resonance imaging are used for the diagnosis [ 1 ]. Patients with a complete absence of pericardium require no treatment, while patients with a partial absence of pericardium are at risk for herniation, and treatment with pericardial reconstruction is reasonable for this group [ 4 ].…”

Complete Pericardial Agenesis in a Pregnant Patient: A Clinical Dilemma

“…4,6 Complete bilateral absence of the pericardium is the rarest presentation, accounting for 9% of the cases, whereas complete absence of the left pericardium is the most reported one. 7,8 This classification has prognostic implications, with incomplete defects being more associated with herniation of cardiac structures. There are reports of three patients with partial absence of left pericardium with documented sudden death.…”

It’s Never Too Late: Spotting Congenital Absence of the Pericardium in an Older Adult

“… 2 , 3 The teardrop shaped heart (elongated atria and bulbous ventricles 2 ) within a displaced apical window flagged the suspicion for CAP. Additional echocardiographic features include paradoxical septal motion (present but confounded by paced rhythm), cardiac hypermobility, and apparent right ventricular enlargement (due to leftward shifting of the cardiac chambers 5 ) with tricuspid regurgitation. 2 The latter can be confused for right ventricular cardiomyopathy or trigger searches for intracardiac shunts, leading to potential unnecessary tests or treatment.…”