Fibromuscular hyperplasia of the pulmonary artery in sudden infant and perinatal unexpected death.
BACKGROUND
The purpose of this study was to describe cases presenting with fibromuscular hyperplasia of the pulmonary arteries that could belong to the group of sudden infant death syndrome (SIDS) and sudden unexpected perinatal death "gray zone" or borderline cases.


METHODS
In a total of 12 cases, eight females and four males, ranging in age from 39 gestational weeks to 93 postnatal days, dying suddenly and unexpectedly, a fibromuscular hyperplasia of the pulmonary artery was detected. Postmortem examinations were requested with a clinical SIDS or sudden unexpected perinatal death. A complete autopsy was performed, including close examination of the brainstem and cardiac conduction system.


RESULTS
Histological examination showed the presence of various degrees of fibromuscular hyperplasia with fibrosis of the right (six cases), left (five cases) or both (one case) pulmonary arteries.


CONCLUSIONS
In our cases, fibromuscular hyperplasia of the pulmonary artery alone might or might not have accounted for the sudden deaths, if it had not been for the concomitant presence of hypoplasia of the arcuate nucleus in the brainstem and/or cardiac conduction system abnormalities. Therefore, they were classified as SIDS/sudden unexpected perinatal death gray zone or borderline cases. Necropsy studies of sudden infant and perinatal death should always include an accurate gross and histological examination of the pulmonary arteries, as well as of the brainstem and cardiac conduction system.
Ottaviani
Giulia
G
Lino Rossi Research Center for the Study and Prevention of Unexpected Perinatal Death and SIDS, University of Milan, Milan, Italy. giulia.ottaviani@unimi.it
Lavezzi
Anna Maria
AM
Matturri
Luigi
L
D001344:Autopsy; D001933:Brain Stem / Q000473:pathology; D003645:Death, Sudden / Q000473:pathology*; D005260:Female; D005313:Fetal Death / Q000473:pathology*; D005865:Gestational Age; D006329:Heart Conduction System / Q000473:pathology; D006801:Humans; D006965:Hyperplasia; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009131:Muscle, Smooth, Vascular / Q000473:pathology*; D011651:Pulmonary Artery / Q000473:pathology*; D013398:Sudden Infant Death / Q000473:pathology*