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Deposition of collagen in the alveolar wall of lungs from patients with tetralogy of Fallot and pulmonary atresia with major aortopulmonary collateral arteries—an ultrastructural study

Published online by Cambridge University Press:  19 August 2008

Hikaru Matsuda ,
Tohru Kuratani ,
Yasuhisa Shimazaki ,
Keishi Kadoba ,
Jyunjiro Kobayashi ,
Shigeaki Ohtake ,
Masahiro Nakayama  and
Takeshi Nakada
Hikaru Matsuda*
Affiliation:
From the First Department of Surgery, Osaka University Medical School and the Divisions of Cardiovascular Surgery and Pathology, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka
Tohru Kuratani
Affiliation:
From the First Department of Surgery, Osaka University Medical School and the Divisions of Cardiovascular Surgery and Pathology, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka
Yasuhisa Shimazaki
Affiliation:
From the First Department of Surgery, Osaka University Medical School and the Divisions of Cardiovascular Surgery and Pathology, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka
Keishi Kadoba
Affiliation:
From the First Department of Surgery, Osaka University Medical School and the Divisions of Cardiovascular Surgery and Pathology, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka
Jyunjiro Kobayashi
Affiliation:
From the First Department of Surgery, Osaka University Medical School and the Divisions of Cardiovascular Surgery and Pathology, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka
Shigeaki Ohtake
Affiliation:
From the First Department of Surgery, Osaka University Medical School and the Divisions of Cardiovascular Surgery and Pathology, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka
Masahiro Nakayama
Affiliation:
From the First Department of Surgery, Osaka University Medical School and the Divisions of Cardiovascular Surgery and Pathology, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka
Takeshi Nakada
Affiliation:
From the First Department of Surgery, Osaka University Medical School and the Divisions of Cardiovascular Surgery and Pathology, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka
*
Dr. Hikaru Matsuda, First Department of Surgery, Osaka University Medical School, Yamada-oka 2-2, Suita, Osaka 565, Japan. Tel. 6-879-3150; Fax.81-6-879-3159.
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Abstract

Abstract Lung biopsies were taken at surgery from five patients (age 2–13, average 7.6 years) with tetralogy of Fallot and pulmonary atresia with major aortopulmonary collateral arteries. The biopsies were then processed for ultrastructural study, comparing paired samples taken, on the one hand, from a segment connected to central pulmonary arteries and, on the other hand, from a segment supplied directly by collateral arteries. Specimens from patients with isolated ventricular septal defect, and from those without cardiac disease, were used as controls. In the patients with tetralogy of Fallot and pulmonary atresia, all biopsies taken from segments supplied by the major collateral arteries showed marked deposition of collagen in the alveolar wall, with an increase in the thickness of the basement membrane (3.6±1.2 µm, mean±SD) greater than seen in those taken from segments connected to central pulmonary arteries (0.9±0.6, p<0.05). The proportional fibrosis of the alveolar interstitial space was also significantly greater in the biopsies from the segments supplied by collateral arteries (30±9%) compared to those from segments fed by central pulmonary arteries (15±8%, p<0.05). No significant differences were found in these indices between the biopsies from segments connected to the central pulmonary arteries in patients with tetralogy of Fallot and pulmonary atresia and those from patients with isolated ventricular septal defect or normal controls. There was no apparent relation to the pulmonary arterial pressure in these findings. The results suggest that the pulmonary segments fed directly by major aortopulmonary collateral arteries in patients with tetralogy of Fallot and pulmonary atresia seem likely to be afflicted by alveolar wall fibrosis, although the etiology and clinical implications of this finding remain unclear.

Keywords

Tetralogy of Fallot with pulmonary atresiaalveolar wall fibrosisaortopulmonary collateral arteriesultrastructure of the lung
Type
Original Articles
Information
Cardiology in the Young , Volume 4 , Issue 3 , July 1994 , pp. 277 - 284
Copyright
Copyright © Cambridge University Press 1994

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