XXXX : what the large pulmonary arteries tell us.

@entity1 @entity842 @entity120
The morphology of the large intrapulmonary arteries (PAs) in @entity120 ( @entity120 ) has received limited attention. Dilation, pruning, abrupt tapering, and tortuosity of PAs occur, but whether different @entity1 have distinct PA phenotypes is unknown. Pulmonary arteriograms from 41 pediatric @entity1 with @entity120 were blindly reviewed by four experts who assigned each angiogram one of three designations: straight (S), tortuous (T), or ambiguous (A). Hemodynamic variables and outcomes were compared to the phenotypes. Thirty @entity1 were either T (19) or S (11); 11 were A. The phenotypes were not associated with age. Tortuous @entity1 had higher PA pressure and resistance than the S group and less likely to react to inhaled @entity842 than S @entity1 (p < 0.05). Clinical outcomes were similar for the three groups. Thus, in @entity120 @entity1 two subtypes of PA morphology can often be discerned, a reflection of variability in PA tortuosity. These morphological subtypes have differing hemodynamic characteristics. The mechanism(s) underlying these differences is unknown, but neither hydrodynamic factors nor duration of @entity120 are fully explanatory. Because PA morphology might reveal information regarding the biology of pathological remodeling, it might prove enlightening to assess the large PA phenotype in future studies of @entity120 .
