"Stone heart" in a neonate.

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.