Outcomes of Thoracic Aortic Interventions in Marfan Syndrome in the State of Texas Over Eleven Years.

Abstract

Marfan Syndrome is a heritable connective tissue disorder with significant aortopathy and conveys substantial cardiovascular morbidity. This study characterizes the mortality and morbidities of thoracic aortic interventions in the Marfan Syndrome population in the state of Texas from 2009-2019. A retrospective review of the Texas Inpatient Discharge Dataset from 1/1/2009-31/12/2019. Discharges from acute care hospitals with a Marfan Syndrome diagnosis by International Classification of Diseases 9/10 codes and a procedure code for thoracic aortic intervention were analyzed utilizing descriptive, univariate, and multivariable regression statistics. There were 4,641 Marfan Syndrome discharges identified, of whom 644(13.9%) underwent thoracic aortic intervention. Thoracic or thoraco-abdominal aortic dissection or rupture was noted in 223(34.6%). Thirty-three(5.1%) had a concomitant coronary artery intervention. There were 30(4.7%) in-hospital mortalities, 126(19.6%) diagnoses of acute renal failure, 52(8.1%) had mechanical ventilation >96 hours, and the median length of stay was 10[IQR : 7-16] days. After adjustment, concomitant coronary artery intervention was associated with in-hospital mortality (OR : 3.69[IQR : 1.15-11.90], p = 0.029) and acute renal failure (OR : 2.66[IQR : 1.19-5.94], p = 0.017). Aortic dissections/ruptures were associated with acute renal failure (OR : 1.73[IQR : 1.14-2.63], p = 0.010), ventilation >96 hours (OR : 2.19[IQR : 1.21-3.97], p = 0.010), and 15% longer length of stay (95% CI : 2.4-29.1%, p = 0.038). Thoracic aortic interventions are frequent amongst the hospitalized Marfan Syndrome population. Concomitant coronary intervention is associated with increased risk of death and aortic dissections/ruptures are associated with increased morbidity. The high prevalence of aortic dissections/ruptures points to a potential target for improving imaging surveillance, adherence to treatment guidelines, and preventative management of Marfan Syndrome aortopathy.