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Congenital absence of the left pericardium is a rare phenomenon occurring as a result
of abnormal embryologic development of the left cardinal vein which normally develops
into the left pleuropericardial membrane. Congenital pericardial defects can occur
alone or in association with other congenital anomalies. Although diagnosis with conventional
imaging techniques is difficult to make, this condition can be identified through
chest X-ray by: (1) levo-rotation of the heart with displacement of the apex to the
left, (2) linear shape of the left cardiac silhouette with erasure of the aortic knob,
and (3) a flattened right cardiac silhouette in the absence of longstanding tricuspid
valve regurgitation. Fig. 1 displays the above mentioned signs in an 18-year-old lady who underwent surgical
closure of an ostium secondum atrial septal defect, in whom diagnosis of congenital
partial absence of the left pericardium was suspected preoperatively.
Fig. 1(A) Preoperative chest X-ray showing: (1) the leftward displacement of the heart,
(2) a linear shape of the left cardiac silhouette (arrow), and (3) a flattened right
cardiac silhouette by erasure of its normally lower convexity (solid arrow). (B) Operative
surgeon's view after median pericardiotomy showing congenital partial absence of the
left pericardium; note the inferior left lower pulmonary lobe (asterisk).
