title: Impact of aortic aneurysm on hospitalizations in patients with marfan syndrome: a multi-institutional study.
abstract: Marfan syndrome (MFS) is an autosomal dominant connective tissue disorder affecting 1 in 3,000 people. Cardiovascular involvement is a prominent feature of MFS, with aortic dissection and/or rupture being the leading cause of death. Advances in the medical and surgical care of patients with MFS have improved survival. Hospital resource utilization and outcomes have not been evaluated in a large population of patients with MFS. We sought to analyze pediatric hospital resource utilization and outcomes in patients with MFS. Nationally distributed data from 43 pediatric hospitals in the 2004-2011 Pediatric Health Information System database were used to identify patients admitted to the hospital with International Classification of Diseases-9th Revision codes for a diagnosis of MFS. Aortic aneurysm (AA) with or without dissection, length of stay (LOS), and hospital charges were determined. During the study period, there were 1,978 admissions in 1,228 patients with MFS. AA was present in 217 (11%) admissions in 188 (15%) patients (63% male). Mean age of patients with AA was 13.8 ± 5.9 years. Aortic dissection or rupture was present in 15 (7% with AA) admissions in 15 (8% with AA) patients (mean age 15.7 ± 5.2 years). Other cardiac diagnoses occurred more commonly in the AA cohort (p < 0.0001), regardless of the reason for admission. Cardiothoracic surgical procedures were performed in 116 AA admissions (53%). Mean LOS, hospital charges per admission, and charges per day were significantly higher in AA cohort compared to those without AA. In-hospital mortality for AA was 2%. The presence of AA in patients with MFS increases hospital resource utilization. Cardiothoracic surgeries are commonly performed in this cohort. Other cardiovascular diagnoses are more prevalent in patients with AA suggesting a more severe phenotype.
authors.affiliation: Department of Pediatrics, The University of Arkansas for Medical Sciences College of Medicine, Little Rock, AR, USA, rtcollins@uams.edu.
authors.forename: R Thomas
authors.initials: RT
authors.lastname: Collins
authors.forename: Venusa
authors.initials: V
authors.lastname: Phomakay
authors.forename: Yuri A
authors.initials: YA
authors.lastname: Zarate
authors.forename: Xinyu
authors.initials: X
authors.lastname: Tang
mesh_terms: D000293:Adolescent; D000328:Adult; D001014:Aortic Aneurysm / Q000209:etiology* / Q000601:surgery*; D001131:Arkansas; D006348:Cardiac Surgical Procedures; D002648:Child; D002675:Child, Preschool; D005260:Female; D017722:Hospital Charges; D017052:Hospital Mortality; D006760:Hospitalization / Q000706:statistics & numerical data*; D006776:Hospitals, Pediatric; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007902:Length of Stay / Q000706:statistics & numerical data; D008297:Male; D008382:Marfan Syndrome / Q000150:complications* / Q000601:surgery*
references.citation: Am J Dis Child. 1987 Nov;141(11):1179-82
references.pmid: 3673967
references.citation: Am J Cardiol. 1995 Jan 15;75(2):157-60
references.pmid: 7810492
references.citation: N Engl J Med. 1972 Apr 13;286(15):804-8
references.pmid: 5011789
references.citation: Isr Med Assoc J. 2008 Mar;10(3):189-93
references.pmid: 18494230
references.citation: J Am Coll Cardiol. 1989 Aug;14(2):422-8; discussion 429-31
references.pmid: 2526834
references.citation: Am Heart J. 2013 May;165(5):828-835.e3
references.pmid: 23622922
references.citation: Clin Cardiol. 2009 Jun;32(6):E23-6
references.pmid: 19322890
references.citation: Arch Dis Child. 2001 Feb;84(2):129-37
references.pmid: 11159287
references.citation: Am J Cardiol. 2004 Aug 1;94(3):400-2
references.pmid: 15276119
references.citation: Circulation. 2008 May 27;117(21):2802-13
references.pmid: 18506019
references.citation: Congenit Heart Dis. 2014 Jan-Feb;9(1):54-62
references.pmid: 23682693
references.citation: Circulation. 2004 Jun 22;109(24):3014-21
references.pmid: 15197151
references.citation: Cardiol Young. 2003 Aug;13(4):341-4
references.pmid: 14694954
references.citation: J Am Coll Cardiol. 2004 Feb 18;43(4):665-9
references.pmid: 14975480
references.citation: Nature. 1991 Jul 25;352(6333):337-9
references.pmid: 1852208