DIAGNOSIS OF CONGENITAL PERICARDIAL DEFECTS, INCLUDING A PATHOGNOMIC SIGN FOR DANGEROUS APICAL VENTRICULAR HERNIATION, ON MAGNETIC-RESONANCE-IMAGING

Objective-To establish criteria for the accurate diagnosis of differen t forms of left sided pericardial defects on magnetic resonance imagin g. Early detection of a partial apical defect is essential as it is po tentially fatal. Design-Examination of four children with congenital p ericardial defects by magnetic resonance imaging, the results being co mpared with the features on conventional chest radiography and echocar diography and with published data. Results-Magnetic resonance imaging improved the ability to diagnose and distinguish between complete and partial left sided pericardial defects. A deep myocardial crease was v isualised in a patient with apical pericardial defect, indicating the risk of a life threatening ventricular strangulation. A prominent left atrial appendage was, in contrast to many reports, not a reliable sig n for partial left sided pericardial defect. Conclusions-The various f orms of congenital left sided pericardial defects cannot reliably be d iagnosed in plain chest radiographs or on echocardiography. Their diag nosis and the distinction between partial and complete defects, howeve r, is of clinical importance and can be accomplished more confidently by magnetic resonance imaging.