Congenital Complete Absence of the Pericardium
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.
- Copyright © 2001 by American Heart Association