Aortic Dissection in Hospitalized Children and Young Adults: A Multiinstitutional Study

Abstract

AD is rare in children and young adults but most commonly occurs in CHD and CTD and in males. AD is associated with high inpatient mortality.

Impact of Aortic Aneurysm on Hospitalizations in Patients with Marfan Syndrome: A Multi-Institutional Study

“…Further study into this phenomenon is warranted and would require detailed familial pedigree and genetic analysis. Aortic dissection and/or rupture were uncommon in the present study, a finding consistent with the reports of other authors [2,11,15]. Unexpectedly, the mortality rate was low for patients with AAD, as only one death occurred during the study period.…”

“…Prior authors have reported the mortality rate for AAD, whether with or without MFS, to range from 18-38 % in pediatric and adult groups [4,5,10,15]. Using the PHIS database, Shamszad et al [11] reported that AAD was rare in pediatric patients with connective tissue diseases, including MFS, Ehlers-Danlos syndrome, and others, and mortality was 22.2 %. However, focused analysis of those patients with MFS was not undertaken in that report, which likely explains the difference in mortality rate compared to the present study.…”

Thoracic aortic dissection and rupture in conotruncal cardiac defects: A population-based study

“…As a previous study showed a bimodal distribution of aortic dissection in the young with a peak in infancy and no other study has demonstrated a non-iatrogenic TAD in an infant with CHD, infants younger than 1 year old were excluded from the analysis [19,23,24].…”

“…The ICD-9 code 746.4, the code for BAV and for congenital aortic valve insufficiency, was used to represent BAV as per prior studies that used ICD-9 codes [25][26][27]. The diagnosis of ventricular septal defect (VSD) was defined to exclude truncus arteriosus and TOF, and the diagnosis of atrial septal defect (ASD) was defined to exclude hypoplastic left heart syndrome, TOF, truncus arteriosus, and D-TGA.…”

“…There has been one case report of TAD in D-TGA (42 year old female status post atrial switch with a Mustard with aortic dimension of 70 mm); and one case report of TAD in Fontan palliation (26 year old male status post lateral tunnel Fontan with aortic dimension of 78 mm) [16][17][18]. A previous multiinstitution study using the Pediatric Health Information System (PHIS) database in patients less than 30 years old found six additional cases TAD in patients with conotruncal heart defects (3 D-TGA, 2 TOF, 1 double outlet right ventricle) [19]. The clarification of whether conotruncal CHD is associated with TAD is important, as the adult population with CHD is increasing, and the question of how to manage these patients is still unclear.…”