caa a22 4500 445823860 CHVBK 20180317145253.0 cr unu---uuuuu 170323e20110801xx s 000 0 eng 10.1007/s00246-011-9963-2 doi (NATIONALLICENCE)springer-10.1007/s00246-011-9963-2 Pulmonary Arterial Hypertension: What the Large Pulmonary Arteries Tell Us [Elektronische Daten] [Thomas Kulik, Renee Clark, Babar Hasan, John Keane, Daniel Springmuller, Mary Mullen] The morphology of the large intrapulmonary arteries (PAs) in pulmonary hypertension (PH) has received limited attention. Dilation, pruning, abrupt tapering, and tortuosity of PAs occur, but whether different patients have distinct PA phenotypes is unknown. Pulmonary arteriograms from 41 pediatric patients with PH 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 patients were either T (19) or S (11); 11 were A. The phenotypes were not associated with age. Tortuous patients had higher PA pressure and resistance than the S group and less likely to react to inhaled nitric oxide than S patients (p<0.05). Clinical outcomes were similar for the three groups. Thus, in PH patients 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 PH 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 PH. Springer Science+Business Media, LLC, 2011 Pulmonary hypertension nationallicence Pulmonary angiography nationallicence Vascular tortuosity nationallicence Kulik Thomas The Pulmonary Hypertension Program, Department of Cardiology, Division of Cardiac Critical Care, Department of Nursing/Patient Services, Children's Hospital Boston, 02115, Boston, MA, USA aut Clark Renee The Pulmonary Hypertension Program, Department of Cardiology, Division of Cardiac Critical Care, Department of Nursing/Patient Services, Children's Hospital Boston, 02115, Boston, MA, USA aut Hasan Babar Department of Cardiology, Children's Hospital Boston and Harvard Medical School, 02115, Boston, MA, USA aut Keane John Department of Cardiology, Children's Hospital Boston and Harvard Medical School, 02115, Boston, MA, USA aut Springmuller Daniel Escuela de Medicina Ponticicia Universidad Catolica de Chile, Santiago, Chile aut Mullen Mary The Pulmonary Hypertension Program, Department of Cardiology, Division of Cardiac Critical Care, Department of Nursing/Patient Services, Children's Hospital Boston, 02115, Boston, MA, USA aut Pediatric Cardiology Springer-Verlag 32/6(2011-08-01), 759-765 0172-0643 32:6<759 2011 32 246 https://doi.org/10.1007/s00246-011-9963-2 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s00246-011-9963-2 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Kulik Thomas The Pulmonary Hypertension Program, Department of Cardiology, Division of Cardiac Critical Care, Department of Nursing/Patient Services, Children's Hospital Boston, 02115, Boston, MA, USA aut NATIONALLICENCE 700 1- Clark Renee The Pulmonary Hypertension Program, Department of Cardiology, Division of Cardiac Critical Care, Department of Nursing/Patient Services, Children's Hospital Boston, 02115, Boston, MA, USA aut NATIONALLICENCE 700 1- Hasan Babar Department of Cardiology, Children's Hospital Boston and Harvard Medical School, 02115, Boston, MA, USA aut NATIONALLICENCE 700 1- Keane John Department of Cardiology, Children's Hospital Boston and Harvard Medical School, 02115, Boston, MA, USA aut NATIONALLICENCE 700 1- Springmuller Daniel Escuela de Medicina Ponticicia Universidad Catolica de Chile, Santiago, Chile aut NATIONALLICENCE 700 1- Mullen Mary The Pulmonary Hypertension Program, Department of Cardiology, Division of Cardiac Critical Care, Department of Nursing/Patient Services, Children's Hospital Boston, 02115, Boston, MA, USA aut NATIONALLICENCE 773 0- Pediatric Cardiology Springer-Verlag 32/6(2011-08-01), 759-765 0172-0643 32:6<759 2011 32 246 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer