RESUMEN
(1) Pulmonary vein stenosis (PVS) can be a severe, progressive disease with lung involvement. We aimed to characterize findings by computed tomography (CT) and identify factors associated with death; (2) Veins and lung segments were classified into five locations: right upper, middle, and lower; and left upper and lower. Severity of vein stenosis (0-4 = no disease-atresia) and lung segments (0-3 = unaffected-severe) were scored. A PVS severity score (sum of all veins + 2 if bilateral disease; maximum = 22) and a total lung severity score (sum of all lung segments; maximum = 15) were reported; (3) Of 43 CT examinations (median age 21 months), 63% had bilateral disease. There was 30% mortality by 4 years after CT. Individual-vein PVS severity was associated with its corresponding lung segment severity (p < 0.001). By univariate analysis, PVS severity score >11, lung cysts, and total lung severity score >6 had higher hazard of death; and perihilar induration had lower hazard of death; (4) Multiple CT-derived variables of PVS severity and lung disease have prognostic significance. PVS severity correlates with lung disease severity.
RESUMEN
Superior vena cava (SVC) obstruction is associated with the gradual development of venous collaterals. We present a rare form of systemic-to-pulmonary subpleural collateral pathway that developed in the bridging subpleural pulmonary veins in a 54-year-old woman with complete SVC obstruction. This uncommon collateral pathway represents a rare form of acquired right-to-left shunt due to previous pleural adhesions with an increased risk of stroke due to right-to-left venous shunting, which requires lifelong anticoagulation.
Asunto(s)
Circulación Colateral , Venas Pulmonares/diagnóstico por imagen , Síndrome de la Vena Cava Superior/diagnóstico por imagen , Circulación Colateral/fisiología , Femenino , Humanos , Persona de Mediana Edad , Tomografía Computarizada Multidetector , Flebografía/métodos , Venas Pulmonares/fisiopatología , Accidente Cerebrovascular/complicaciones , Síndrome de la Vena Cava Superior/fisiopatología , Venas/fisiopatologíaRESUMEN
Superior vena cava (SVC) obstruction is associated with the gradual development of venous collaterals. We present a rare form of systemic-to-pulmonary subpleural collateral pathway that developed in the bridging subpleural pulmonary veins in a 54-year-old woman with complete SVC obstruction. This uncommon collateral pathway represents a rare form of acquired right-to-left shunt due to previous pleural adhesions with an increased risk of stroke due to right-to-left venous shunting, which requires lifelong anticoagulation.