[A paradigmatic case of partial agenesis of the pericardium].
Complete or partial absence of the pericardium is an uncommon congenital abnormality. Since its first description, several cases have been reported. Although the clinical and instrumental features of this defect are well described, at present it is often overlooked. We report a case of a 19-year old boy referred to our Echocardiography laboratory because of a suspected interatrial septal defect. For this reason it has been recommended to undergo cardiac catheterization. The patient, completely asymptomatic, had a negative physical examination. The echocardiogram excluded the presence of an interatrial septal defect, but some abnormalities, regarding overall right-sided heart, were found (paradoxical interventricular septal movement, apparent right-sided heart enlargement, with unusual bulging of the apex of the right ventricle, an excessive basculant heart movement). In the absence of any other heart disease, we thought that these abnormalities reflected a congenital absence of the pericardium. Indeed, the chest X-ray showed some features, considered patognomonic for congenital absence of the pericardium (levoposition of the heart, lung interposition between the diaphragm and the base of the heart and between the aorta and pulmonary artery). Nuclear magnetic resonance study showed the absence of the pericardium in the posterior and posterolateral wall, confirming our suspicion. Furthermore, the posterior wall of the heart leaned to herniate through the pericardial defect. Since the partial absence of the pericardium can lead to severe complications, surgery was recommended. The correct diagnosis of this disease is very important because of its prognostic implications; this case represents a further contribution to the understanding of this defect.
Scapellato
F
F
Divisione di Cardiologia, Fondazione Salvatore Maugeri, IRCCS, Istituto Di Riabilitazione di Veruno.
Imparato
A
A
Brunetto
A
A
Temporelli
P L
PL
Giannuzzi
P
P
D000328:Adult; D003937:Diagnosis, Differential; D004452:Echocardiography; D006331:Heart Diseases / Q000150:complications / Q000473:pathology; D006801:Humans; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D010496:Pericardium / Q000000981:diagnostic imaging / Q000473:pathology*; D011859:Radiography