(715) Cardiopulmonary Resuscitation in a Patient With Marfan Syndrome and Pectus Bars: A Case Report

Introduction: Marfan Syndrome (MS) is a connective tissue disorder associated with severe pectus excavatum (PE), progressive aortic root dilation (ARD) and premature death often due to cardiac complications. ARD is more common in the presence of PE and yields significant risk of mortality. Thus, MS patients may require concomitant or staged surgical correction of both morbidities. Importantly, as lifetime risk of premature death is significant, consideration must be given to cardiac resuscitation that may be complicated by pectus (Nuss) bars and timing of surgical corrections. We present a 15 yo male with MS who underwent concomitant aortic root replacement and placement of two Nuss bars 16 days prior to sustaining an out-of-hospital cardiac arrest.

Description: The patient had witnessed cardiac arrest following an acute episode of dyspnea and chest pain. EMS arrived within 10 minutes and began CPR. An AED showed PEA. He remained pulseless on arrival to our hospital, where CPR was continued but noted to be difficult as the presence of Nuss bars were unknown. Echocardiogram showed a large pericardial echodensity that resembled a hematoma or thrombus. Brief periods of ROSC were achieved, but ultimately ECPR was activated. Concurrently, sternotomy with mediastinal exploration was performed with evacuation of the pericardial space. Aortic root dehiscence was found and the patient was emergently transferred to the OR for Nuss bar removal and aortic root repair. His hospital course was complicated by hypoxic-ischemic brain injury, cerebellar tonsillar herniation, AKI and cardiac dysfunction. On hospital day five, life-sustaining support was withdrawn and the patient died.

Discussion: Our case highlights that effective CPR is impaired in the presence of Nuss bars and questions if staged correction of PE followed by cardiac surgery may reduce the risk of ineffective CPR. In 2019, Glithero described the first case of ROSC in a non-MS patient with a single Nuss bar. Stearns developed a model which estimated that one and two Nuss bars require a 226% and 453% increase in force, respectively, as compared to chest compressions over a native sternum. Thus, as MS patients are at risk of premature cardiac death, consideration of earlier pectus correction may decrease future complications associated with adequate CPR.