Succinylcholine in Rubinstein-Taybi syndrome

Rubinstein-Taybi syndrome is a congenital anomaly characterized by broad thumbs and first toes facial abnormalities, and mental lietardation. Cardiac anomalies are frequently preset. I predicted that "Succinylcholine ... might cause arrhythmia in patients with Rubinstein-Taybi syndrome". Recently, I once again anesthetized the patient with Rubinstein-Taybi syndrome whom I had earlier described in some detail. The patient, now 14 years old and weighing 22 kg, was admitted to the hospital on the morning of scheduled restorative dentistry. The lead II electrocardiogram with the patient awake was similar to that obtained by Holter monitor 2 years earlier, and showed occasional instances (1-3 beats/ min) of retrograde conduction. Heart rate was 105 beats/min. A. rapid sequence induction of anesthesia with nasal intubation was planned. Premedication consisted of 0.1 mg glycopyrrolate, iv, and heart rate and rhythm remained unchanged. Thiopental, 100 mg, iv, was followed by succinylcholine 20 mg, iv, and cricoid pressure was applied. Beginning 15 s after succinylcholine, a variety of abnormal cardiac rhythms occurred for the next 5 min. Short runs of supraventricular tachycardia were interspersed with multifocal premature ventricular contractions (5-10 min) and occasional premature atrial contractions. Blood pressure remained stable at 100/60 mmHg. Intubation was performed and the case proceeded uneventfully. The frequency of cardiac abnormalities in Rubinstein-Taybi syndrome (approximately 33% of patients in each of two series had known conduction or structural defects) would seem to predispose such patients to abnormal responses to cardioactive drugs. Indeed, 2 years earlier, the patient described above displayed a variety of arrhythmias following administration of neostigmine and atropine for antagonism of neuromuscular block. However, at that time, succinylcholin use did not cause arrhythmias. In this instance, it did. "Special attention to ... the cardiac effects of neromuscular blocking agents ...is advisable in order to minimize the risks of ... cardiac arrhythmias in patients with Rubinstein-Taybi syndrome." Anesthesiology 11 1982, Vol.57, 429.