Histopathological evidence of aortopathy in newborns and infants with Tetralogy of Fallot at the time of the surgical repair

Ondrej Fabian; Roman Gebauer; Miroslav Koblizek; Ludmila Hornofova; Jan Janousek

doi:10.1016/j.carpath.2019.02.004

Histopathological evidence of aortopathy in newborns and infants with Tetralogy of Fallot at the time of the surgical repair

Cardiovasc Pathol. 2019 May-Jun:40:59-64. doi: 10.1016/j.carpath.2019.02.004. Epub 2019 Feb 28.

Authors

Ondrej Fabian¹, Roman Gebauer², Miroslav Koblizek³, Ludmila Hornofova³, Jan Janousek²

Affiliations

¹ Department of Pathology and Molecular Medicine, 2nd Faculty of Medicine, Charles University and Motol University Hospital, V Uvalu 84, Prague 5, 150 06, Czech Republic. Electronic address: Ondrej.Fabian2@fnmotol.cz.
² Children's Heart Centre, 2nd Faculty of Medicine, Charles University and Motol University Hospital, V Uvalu 84, Prague 5, 150 06, Czech Republic.
³ Department of Pathology and Molecular Medicine, 2nd Faculty of Medicine, Charles University and Motol University Hospital, V Uvalu 84, Prague 5, 150 06, Czech Republic.

PMID: 30904751
DOI: 10.1016/j.carpath.2019.02.004

Abstract

Objectives: The purpose of this study was to evaluate the microscopic structural abnormalities of the ascending aorta in infants with Tetralogy of Fallot (ToF) and compare them with aortic samples from control group of small children that died of other diseases. We aimed at identification of the specific histopathological changes associated with ToF and correlation of the severity of these changes with time to surgery and mean levels of saturation in the ToF group, and age at death in control group.

Methods: The full-thickness ascending aortic wall sample was taken from 23 children with ToF at the time of surgical reconstruction (age spread 2 to 19 months) and evaluated by light microscopy. Corresponding samples were taken from 16 cadaverous cases of children with other diseases (0-76 months). The assessed morphological variables included elastic fiber fragmentation/loss, thinning and disorganisation, presence of laminar medial necrosis, intralamellar and translamellar mucoid extracellular matrix accumulations, smooth muscle cell disorganisation, presence of fibrosis, calcifications and neovascularisation and finally grade of overall medial degeneration.

Results: No difference was found between the two groups in the individual morphological variables. However, there was a difference in the distribution of the grades of the overall medial degeneration (P = .016). ToF group showed uniform mild degenerative changes, whereas control group harboured spectrum of changes ranging from normal to moderate. The presence of the given histopathological changes and their severity were associated neither with age at surgery or mean levels of saturation in ToF group, nor with the age at death in the control group.

Conclusions: This study emphasizes the histopathological assessment of the bioptic samples of the ascending aorta during the surgical repair of ToF, since the patients demonstrating moderate or severe degenerative changes already in the early childhood may be in increased risk of the subsequent late complications.

Keywords: Histopathology; Medial degeneration; Tetralogy of Fallot.

MeSH terms

Age Factors
Aorta / pathology*
Aortic Diseases / mortality
Aortic Diseases / pathology*
Cardiac Surgical Procedures* / adverse effects
Cardiac Surgical Procedures* / mortality
Case-Control Studies
Child
Child, Preschool
Female
Humans
Infant
Infant, Newborn
Male
Prognosis
Prospective Studies
Risk Factors
Severity of Illness Index
Tetralogy of Fallot / mortality
Tetralogy of Fallot / pathology
Tetralogy of Fallot / surgery*