Response to Anesthetic management of a rare case of Shprintzen-Goldberg craniosynostosis syndrome

Abstract

pulmonary hypertension secondary to left atrial hypertension to have developed. Frequently, such pulmonary hypertension gradually settles following decompression of the left heart by the LVAD, but right ventricular function may be impaired, and sudden changes in pulmonary vascular resistance are extremely poorly tolerated. An apparently stable patient receiving LVAD support alone may acutely decompensate because of inadvertent elevation in pulmonary vascular resistance. Effective analgesia and normocapnoea minimize the risk of increases in pulmonary vascular resistance. Inhaled nitric oxide and vasoactive therapy (usually epinephrine and milrinone) must be to hand. Right ventricular failure is the likeliest cause of decompensation in the LVAD patient during the course of anesthesia. It is not safe to assume that passive pulmonary blood flow will always suffice.Third, Pratap and Wilmhurst counsel against external cardiac compressions. We carried out external cardiac compressions for a prolonged period in a child with hypertrophic cardiomyopathy associated with a malignant dysrhythmia; the left ventricular apical cannula had become obstructed as the left ventricle decompressed following VAD insertion with subsequent loss of output. This child had effective cardiac compression until cardiopulmonary bypass was instituted and the cannula repositioned following resection of left ventricular muscle. The cannulae and anastomoses remained intact during the period of cardiac compressions.