P029: SETTING THE BAR - INTRAOPERATIVE IMAGING AND MANAGEMENT IN THE REPAIR OF SEVERE PECTUS EXCAVATUM WITH RIGHT CORONARY DISTORTION
Gregory Mickus, MD, Archer Martin, MD; Mayo Clinic Florida
INTRODUCTION: Pectus excavatum is a congenital chest wall deformity occurring in 1 out of every 400-1000 live births, predominantly males. For patients the primary concern may be aesthetics and the accompanying psychological component.1 Clinically however, the emphasis is placed on cardiopulmonary impingement and the deleterious consequences that ensue. The following case provides an example of anesthetic management for severe pectus excavatum in the setting of severe diastolic impairment.
CASE REPORT: A 21-year-old white male with known congenital pectus excavatum presented with recent-onset exertional dyspnea. Symptoms began several months prior, associated with moderate exertion, episodic palpitations, occasional left-sided chest pain, and feeling as if “my heart is moving to the left side” gradually over time. No other relevant past medical, surgical, or social history, and review of systems otherwise negative. On physical exam, symmetric chest visibly with severe pectus excavatum deformity, moderate chest wall restriction, and leftward displacement of the point of maximal impulse. No cardiopulmonary abnormalities appreciated, and physical exam otherwise within normal limits. Pulmonary function testing revealed mild airway obstruction with FEV1 72%, FEV1/FVC 72.1%, DLCO 73%, RV 164%, and RV/TLC 166%. Chest CT scan results shown in figure 1. Preoperatively the patient received an 18g PIV, premedication with midazolam 2mg, fentanyl 100mcg, and a thoracic T5-6 epidural for postoperative analgesia. After standard ASA monitors were placed, induction was performed with midazolam 0.1mg/kg, fentanyl 4mcg/kg, propofol 3mg/kg, ketamine 0.3mg/kg, and vecuronium 0.15mg/kg for rapid-onset paralysis. A left double-lumen ETT was placed under direct visualization, confirmed with fiberoptic exam, and then a left radial arterial line and 14g PIV were placed. One-lung ventilation was obtained with subsequent limited right thoracotomy without complication. Transesophageal echocardiography (TEE) revealed EF 55%, RV compression from the presumed sternal tip, compression at the RA/RV junction approaching the TV annulus, mild TVR, flow acceleration at the RV inflow, distorted right coronary artery course due to external compression, and a patent foramen ovale. A Nuss pectus bar was placed with removal of bilateral T4-6 costal cartilages, bone graft placement, and sterna osteotomy. Post-bar placement TEE showed complete relief of RA/RV external compression and mild decrease in TV regurgitation. Procedural analgesia was obtained with an additional fentanyl 4mcg/kg, ketamine 1mg/kg, hydromorphone 2mg, IV acetaminophen 1000mg, and a ropivacaine 0.2% 9mL epidural bolus prior to emergence. The patient was extubated at the end of the procedure, had an uncomplicated postoperative course with pain scores for non-movement ranging 0-4/10 and movement 5-6/10. Patient was discharged on postoperative day 6, and at 21-day follow-up reported resolution of chest pain, exertional dyspnea, and palpitations.
CONCLUSIONS: Severe pectus excavatum presents a unique challenge for anesthesiologists given the anatomical alteration and resultant hemodynamic irregularities. Preoperative imaging is useful in planning for intraoperative management. Additionally, transesophageal echocardiography is an invaluable tool to assess the extent of hemodynamic compromise in real-time, guide intraoperative management, and quantify improvement in cardiac function post-repair.
REFERENCES: Fokin AA, Steuerwald NM, Ahrens WA, et al. Anatomical, Histologic, and Genetic Characteristics of Congenital Chest Wall Deformities. Semin Thorac Cardiovasc Surg. 2009;21(1):44.