Mikulski Matthew F, Well Andrew, Mery Carlos M, Johnson Gregory, Gottlieb Erin A, Fraser Charles D, Beckerman Ziv
Department of Surgery and Perioperative Care, Dell Medical School, The University of Texas at Austin, Austin, TX, USA.
Texas Center for Pediatric and Congenital Heart Disease, UT Health Austin and Dell Children's Medical Center, Austin, TX, USA.
Interdiscip Cardiovasc Thorac Surg. 2023 Aug 3;37(2). doi: 10.1093/icvts/ivad128.
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.
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.
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).
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.
马凡综合征是一种遗传性结缔组织疾病，伴有严重的主动脉病变，具有较高的心血管疾病发病率。本研究描述了2009年至2019年德克萨斯州马凡综合征患者胸主动脉介入治疗（TAI）的死亡率和发病率。
对2009年1月1日至2019年12月31日的德克萨斯州住院患者出院数据集进行回顾性分析。利用描述性、单变量和多变量回归统计分析急性护理医院中通过国际疾病分类9/10编码诊断为马凡综合征且有TAI程序编码的出院病例。
共识别出4641例马凡综合征出院病例，其中644例（13.9%）接受了TAI。223例（34.6%）出现胸主动脉或胸腹主动脉夹层或破裂。33例（5.1%）同时进行了冠状动脉介入治疗。住院死亡30例（4.7%），诊断为急性肾衰竭（ARF）126例（19.6%），机械通气>96小时52例（8.1%），中位住院时间为10天[四分位间距（IQR）7 - 16天]。调整后，同时进行冠状动脉介入治疗与住院死亡率相关[比值比（OR）3.69 [IQR 1.15 - 11.90]，P = 0.029]和ARF相关（OR 2.66 [IQR 1.19 - 5.94]，P = 0.017）。主动脉夹层/破裂与ARF相关（OR 1.73 [IQR 1.14 - 2.63]，P = 0.010）、通气>96小时相关（OR 2.19 [IQR 1.21 - 3.97]，P = 0.010）以及住院时间延长15%相关（95%置信区间2.4 - 29.1%，P = 0.038）。
TAI在住院的马凡综合征患者中很常见。同时进行冠状动脉介入治疗与死亡风险增加相关，主动脉夹层/破裂与发病率增加相关。主动脉夹层/破裂的高患病率指出了改善影像监测、遵循治疗指南以及马凡综合征主动脉病变预防性管理的潜在目标。
