The Journal of Thoracic and Cardiovascular Surgery, Vol 81, 459-463, Copyright © 1981 by The American Association for Thoracic Surgery and The Western Thoracic Surgical Association
"Stone heart" in a neonate
JC Opie, G Taylor, PG Ashmore and D Kalousek
A neonate presented at this institution in September, 1978, with
interrupted aortic arch complex, including ventricular septal defect,
atrial septal defect, and patent ductus arteriosus. Additional anomalies
included bilateral cleft lip and palate, low set ears, and bilateral
colobomas. One-stage total correction of the interrupted aortic arch
complex was undertaken with standard hypothermic techniques. The repair was
completed within a 65 minute period of circulatory arrest at 18 degrees C,
perfusion was re-established, and the patient was returned to normothermia.
Tetanic contracture of the myocardium, stone heart, was evident at 27
degrees C. Cardiac action did not resume despite several therapeutic
maneuvers, and the infant died on the operating table. Extensive calcium
flooding was a notable feature on histological sections of the heart,
associated with myofibrillar degeneration (contraction bands). This
complication has never been reported before in a neonate with congenital
heart disease. Deep hypothermia did not prevent ischemic contracture of the
myocardium, and this complication must now be added to an already lengthy
list of complications of neonatal heart surgery.