Intrapulmonary vascular shunt pathways in alveolar capillary dysplasia with misalignment of pulmonary veins.
Thorax
; 70(1): 84-5, 2015 Jan.
Article
en En
| MEDLINE
| ID: mdl-25052575
ABSTRACT
Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a lethal neonatal lung disease characterised by severe pulmonary hypertension, abnormal vasculature and intractable hypoxaemia. Mechanisms linking abnormal lung vasculature with severe hypoxaemia in ACD/MPV are unknown. We investigated whether bronchopulmonary anastomoses form right-to-left shunt pathways in ACD/MVP. We studied 2 infants who died of ACD/MPV postmortem with direct injections of coloured ink into the pulmonary artery, bronchial artery and pulmonary veins. Extensive histological evaluations included serial sectioning, immunostaining and 3-dimensional reconstruction demonstrated striking intrapulmonary vascular pathways linking the systemic and pulmonary circulations that bypass the alveolar capillary bed. These data support the role of prominent right-to-left intrapulmonary vascular shunt pathways in the pathophysiology of ACD/MPV.
Palabras clave
Texto completo:
1
Bases de datos:
MEDLINE
Asunto principal:
Síndrome de Circulación Fetal Persistente
/
Alveolos Pulmonares
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Arteria Pulmonar
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Venas Pulmonares
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Capilares
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Circulación Pulmonar
Límite:
Humans
/
Infant
/
Newborn
Idioma:
En
Revista:
Thorax
Año:
2015
Tipo del documento:
Article
País de afiliación:
Estados Unidos