The Fontan circula on is a surgical pallia on for pa ents with a func onally univentricular heart, resul ng in a chronic non-âpulsa le pulmonary flow. The Fontan.
Adverse Pulmonary Vascular Remodeling in the Fontan CirculaKon
F.J.S. Ridderbos BSc 1 • D. Wolff BSc1 • A. Timmer MD PhD2 • J. van Melle MD PhD3 • T. Ebels MD PhD4 • M.G. Dickinson MD1 • W. Timens MD PhD2 • R.M.F. Berger MD PhD1 1Center for Congenital Heart Diseases, Beatrix Children's Hospital, Department of Pediatric Cardiology, 2Department of Pathology, 3Department of Cardiology, 4Department of Cardiothoracic Surgery, University Medical Center Groningen, University of Groningen, The Netherlands
Purpose Purpose
The Fontan circulaKon is a surgical palliaKon for paKents with a funcKonally univentricular heart, resulKng in a chronic non-‐pulsaKle pulmonary flow. The Fontan circulaKon is characterized by gradual aSriKon over Kme. An increase of the pulmonary vascular resistance could be a key factor in the long term failure of the Fontan circulaKon. The current study aimed to invesKgate the presence of pulmonary vascular remodeling in paKents with a Fontan circulaKon.
Methods
Pulmonary vascular histomorphometric analysis and immunohistochemistry were performed in lung Kssue obtained at autopsy from 12 Fontan paKents. The Fontan paKents had died either peri-‐operaKvely (group A: death during or < 15 days aXer Fontan compleKon; N=5) or at mid-‐ to long term follow up (group B: death > 5 years aXer Fontan compleKon; N=7). Two age-‐matched control groups (N=10 and N=14, respecKvely) were included.
Figure 1. Wall thickness small pulmonary vessels group A (peri-‐operaKve death)
Wall thickness
Figure 3. Wall thickness and muscularizaKon of small pulmonary vessels in group B (long term)
Figure 4. InKma fibrosis of small pulmonary vessels in group B (long term)
Control
Wall thickness
Figure 6. CorrelaKon age at death and vascular remodeling in group B (long term)
Total wall thickness One control vessel and four typical examples of eccentric acellular inHma fibrosis in the intra-‐acinar pulmonary vessels of group B (long term) Fontan paHents. Note the acellular thickened vessel wall and the difference with the control vessel in lumen area. Verhoeff staining, scale bar = 50 µm
Results
Figure 5. Histology of small pulmonary vessels in group B (long term)
p=0.028 for total wall thickness Figure 2. Media hypertrophy in group A (peri-‐operaKve death)
p=0.002 for total wall thickness; p=0.028 for media thickness and p
