Pulmonary vein stenosis

Chen Chih J Sun, Thomas Doyle, Richard Ringel

Research output: Contribution to journalArticle

Abstract

Pulmonary vein stenosis (PVS) is a rare disorder. Accurate diagnosis often requires anatomical examination. We report four children with pulmonary vein stenosis. Autopsy showed bilateral lesions in two patients who were thought clinically to have unilateral disease. A diagnosis of PVS was made at autopsy in the third case. Intimal and medial fibromuscular proliferation was noted in extrapulmonary and intrapulmonary veins. Some of the fibromuscular proliferation were eccentric, resembling organized thrombi. In one case a focal organizing thrombus was found in a clinically unobstructed but anatomically narrowed veno-atrial junction. In another case injection of contrast medium into the stenotic pulmonary vein (PV) showed anastomosis between PV and bronchial vessels as well as small pulmonary arteries. Bilateral hypertensive arteriopathy was observed in unilateral and bilateral PVS. Our histological finding of intrapulmonary venous lesions in the lobes in which PVS was not detected clinically suggests that during surgical correction of unilateral PVS multiple biopsies of the opposite lung may help to evaluate possible bilateral disease. Our study also suggests that thrombosis in a stenotic pulmonary vein may further compromise the lumen and contribute to the progression of pulmonary vein obstruction. The possible pathogenesis of bilateral pulmonary hypertensive arteriopathy in unilateral PVS also is discussed.

Original languageEnglish (US)
Pages (from-to)880-886
Number of pages7
JournalHuman Pathology
Volume26
Issue number8
DOIs
StatePublished - 1995
Externally publishedYes

Fingerprint

Pulmonary Veins
Thrombosis
Autopsy
Tunica Intima
Lung
Pulmonary Vein Stenosis
Contrast Media
Pulmonary Artery
Veins
Biopsy
Injections

Keywords

  • bronchopulmonary anastomosis
  • pulmonary hypertension
  • pulmonary hypertensive arteriopathy
  • pulmonary vein stenosis

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

Pulmonary vein stenosis. / Sun, Chen Chih J; Doyle, Thomas; Ringel, Richard.

In: Human Pathology, Vol. 26, No. 8, 1995, p. 880-886.

Research output: Contribution to journalArticle

Sun, Chen Chih J ; Doyle, Thomas ; Ringel, Richard. / Pulmonary vein stenosis. In: Human Pathology. 1995 ; Vol. 26, No. 8. pp. 880-886.
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