doi: 10.1161/hc2501.092237
(Circulation. 2001;103:3154.)
© 2001 American Heart Association, Inc.
Images in Cardiovascular Medicine |
Congenital Complete Absence of the Pericardium
From the Department of Radiological Sciences (O.M.) and the Ahmanson/UCLA Adult Congenital Heart Disease Center (J.K.P.), University of California, Los Angeles, and the Department of Surgery (W.G.W.), the Toronto Hospital, Toronto, Ontario, Canada.
Correspondence to Joseph K. Perloff, MD, Division of Cardiology, Room 47-123, UCLA Center for the Health Sciences, 10833 LeConte Avenue, Los Angeles, CA 90095-1679. E-mail josephperloff{at}earthlink.net
A 38-year-old woman
with ill-defined chest pain had an abnormal x-ray 8 years before her
only pregnancy. She had been unable to bear down during delivery, but
gestation was otherwise uncomplicated, yielding a normal female
offspring. She subsequently experienced left-sided chest pain that
awakened her from sleep when turning into either a right or left
lateral decubitus position. The pain was pulsating, stabbing, heavy,
moderate in severity, and sometimes lasted for hours. Thoracic MRI in
the supine and left lateral recumbent positions diagnosed congenital
complete absence of the pericardium
(Figure 1
, left). The pain was ascribed to torsion at the
thoracic inlet because of striking positional changes (mobility) of the
heart
(Figure 1, left). Congenital complete absence of the
pericardium was confirmed during an operation designed to stabilize the
cardiac position
(Figure 1, right, and
Figure 2). The heart was encapsulated in a Gore-Tex sack.
The postoperative magnetic resonance images are shown in
Figures 1 and 2.
Figure 3 shows hypoplasia of the left lung and left
pulmonary artery, which sometimes coexist with congenital
complete absence of the
pericardium.
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