Outcomes of thoracic aortic interventions in Marfan syndrome in the state of Texas over 11 years

Matthew F Mikulski; Andrew Well; Carlos M Mery; Gregory Johnson; Erin A Gottlieb; Charles D Fraser; Ziv Beckerman

doi:10.1093/icvts/ivad128

Outcomes of thoracic aortic interventions in Marfan syndrome in the state of Texas over 11 years

Interdiscip Cardiovasc Thorac Surg. 2023 Aug 3;37(2):ivad128. doi: 10.1093/icvts/ivad128.

Authors

Matthew F Mikulski^{1

2}, Andrew Well^{1

2}, Carlos M Mery^{1

2}, Gregory Johnson^{2

3}, Erin A Gottlieb^{1

2}, Charles D Fraser^{1

2}, Ziv Beckerman^{1

2}

Affiliations

¹ Department of Surgery and Perioperative Care, Dell Medical School, The University of Texas at Austin, Austin, TX, USA.
² Texas Center for Pediatric and Congenital Heart Disease, UT Health Austin and Dell Children's Medical Center, Austin, TX, USA.
³ Department of Pediatrics, Dell Medical School, The University of Texas at Austin, Austin, TX, USA.

Abstract

Objectives: 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 (TAI) in the Marfan syndrome population in the state of Texas from 2009 to 2019.

Methods: A retrospective review of the Texas Inpatient Discharge Dataset from 1 January 2009 to 31 December 2019. Discharges from acute care hospitals with a Marfan syndrome diagnosis by the International Classification of Diseases 9/10 codes and a procedure code for TAI were analysed utilizing descriptive, univariate and multivariable regression statistics.

Results: There were 4641 Marfan syndrome discharges identified, of whom 644 (13.9%) underwent TAI. 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 (ARF), 52 (8.1%) had mechanical ventilation >96 h and the median length of stay was 10 [interquartile range (IQR) 7-16] days. After adjustment, concomitant coronary artery intervention was associated with in-hospital mortality [odds ratio (OR) 3.69 [IQR 1.15-11.90], P = 0.029] and ARF (OR 2.66 [IQR 1.19-5.94], P = 0.017). Aortic dissections/ruptures were associated with ARF (OR 1.73 [IQR 1.14-2.63], P = 0.010), ventilation >96 h (OR 2.19 [IQR 1.21-3.97], P = 0.010), and 15% longer length of stay (95% confidence interval 2.4-29.1%, P = 0.038).

Conclusions: TAI are frequent among 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.

Keywords: Aortic dissections; Marfan syndrome; Outcomes; Thoracic aortic aneurysms.