MECHANISMS OF SUDDEN-DEATH AND AUTOPSY FINDINGS IN PATIENTS WITH ARNOLD-CHIARI MALFORMATION AND VENTRICULOATRIAL CATHETERS

Neurological and vascular complications of Arnold-Chiari malformation treated with ventriculoatrial shunting may result in sudden or unexpec ted death. Two patients with Arnold-Chiari malformation and ventriculo atrial shunting had variable clinical manifestations and diagnostic di fficulties. A 3-year-old girl with a 1-day history of right-sided hear t failure died unexpectedly soon after cardiac catheterization. At aut opsy examination an adherent thrombus around the ventriculoatrial cath eter tip, pulmonary infarction, and embolic pulmonary arterial hyperte nsive changes were found. In the second case, a 21-year-old man died s uddenly after a brief episode of dyspnea. He had a I-year history of ' 'asthma'' before death. Autopsy examination confirmed pulmonary infarc tion and embolic pulmonary arterial hypertensive changes. There was no histological evidence of asthma. Deaths in both cases were due to pul monary infarction stemming from thromboemboli derived from ventriculoa trial catheterization. Both patients had evidence of long-standing cli nically unsuspected vascular disease, which may have contributed to de ath. Cardiac catheterization may also have precipitated death in the f irst patient. Other possible problems leading or contributing to sudde n death in such patients include pulmonary hypertension with chronic c or pulmonale, airway obstruction from recurrent laryngeal nerve paraly sis, and shunt blockage with acute hydrocephalus. Lethal brainstem com pression may also accompany relatively minor trauma associated with ch ronic cerebellar tonsillar herniation in these patients.