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Abstract

Introduction: Williams syndrome, consists of a constellation of signs and symptoms including mental retardation, distinctive emotional and behavioral traits, cranio-facial anomalies and association with difficult airway, growth deficiency, genitourinary manifestations, and supravalvular aortic stenosis.1,2 Literature is rampant with reports of sudden cardiac death under general anesthesia in these patients. 3-5 We describe a patient with William's syndrome, who presented for revision cardiac surgery of a stenosed prosthetic aortic valve and possible aortic root repair. The perioperative challenges required teamwork from the ICU and surgical teams in combination. Case report: A 44 y/o female with a known history of William's syndrome with congenital supravalvular Aortic Stenosis complicated by dissection of subvalvular stenosis who had previously undergone a resection of supraventricular ridge, patch repair of ascending aorta, re-implant of right coronary artery, and aortic valve replacement (St. Jude #17), presented with prosthetic valve re-stenosis. She had the typical 'elfin facies" as described with William's syndrome and was short stature (Ht 142.2 cm (4' 8")). The baseline pre-procedure trans-thoracic echo (TTE) ( Figure 1 a and b) showed a left ventricle with mildly decreased systolic function. EF = 48 ± 5%. Moderate (2+) mitral valve regurgitation (MR) and moderate (2+) aortic valve regurgitation (AR) were seen. Importantly severe aortic valve stenosis with a peak/mean gradient of 137/83mmHg and a dimensionless index (DVI) of 0.09 was evident. Coronaries were normal on left heart catheterization. (Figure 2 a and b) A few hours before surgery she developed increasing shortness of breath with possible pulmonary edema and had to be emergently intubated. She was a known difficult airway (per previous records) and a 7.5mm Parker Tube was passed via a fibreoptic bronchoscope with the patient awake. In the operating room, immediately prior to induction, a brief period of tachycardia led to extreme hemodynamic instability. The pre-procedure 2+ MR increased to a wide-open ischemic 4+ MR. LV dysfunction and poor LV wall motion was also evident on the pre-bypass echo. (Figure 3 a and b). She was emergently put on cardio-pulmonary bypass. Intraoperatively, a Mitral valve repair with a Duran band #27, AV repair Duran Ancore annuloplasty band #27, Aortic root replacement with a 21-mm aortic homograft were successfully performed. Post-pump echocardiography demonstrated a well-seated aortic homograft without regurgitation and no residual mitral regurgitation. Maximum velocity in the aortic valve was148.0 cm/s. The aortic valve peak/mean gradient was 9/4mmHg. (Figure 4 a and b). The patient was transported to the ICU with an open-sternal wound. Some evidence of biventricular dysfunction persisted for the next 12 hours. However, she showed an excellent response to resuscitation and the vasopressor support was gradually weaned off in the next 48 hours at which stage she was taken back to the operating room for a chest closure, that she tolerated well. She was subsequently discharged to the step down and back home within the next ten days. At discharge she was back to her neurological baseline status and was doing well from a cardiovascular standpoint. Discussion: The hemodynamic instability due to severe aortic valve disease complicated by the ischemic mitral regurgitation, the difficult airway as a part of the syndrome and the emergent nature of the cardio-pulmonary bypass with the need for aggressive post-bypass resuscitation were a combined challenge for the cardiothoracic anesthesiology, critical care and surgical teams of our institute. William's patients with corrected supravalvular disease, presenting for redo surgery remains a perioperative challenge as intense as those presenting for first time surgery.