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This study\u2019s objective was to test the hypothesis of a loss of pulmonary capillaries as an underlying mechanism by comparison to other patient groups with and without pulmonary hypertension (PH).<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Materials and methods<\/jats:title>\n            <jats:p>Between March 2019 and June 2023, patients of four groups were recruited for this observational study: IPAH with preserved (1) and low <jats:italic>D<\/jats:italic>\n              <jats:sub>LCO<\/jats:sub> (2), combined pulmonary fibrosis and emphysema with PH (3), and emphysema without PH (4). Patients underwent clinical CT and <jats:sup>129<\/jats:sup>Xe MRI including dissolved-phase imaging yielding the ratio of <jats:sup>129<\/jats:sup>Xe in red blood cells and membrane tissues (RBC-M), chemical shift saturation recovery for determining RBC fraction \u03b7 and diffusion-weighted imaging yielding surface-volume ratio. Kruskal\u2013Wallis tests were used for statistical analysis.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Results<\/jats:title>\n            <jats:p>Twenty-nine participants were recruited, of which 22 (age 64\u2009\u00b1\u200910, 11 male, 5\/5\/7\/5 for the individual groups) could be included in the analysis. RBC-M and \u03b7 were reduced in IPAH with low versus preserved <jats:italic>D<\/jats:italic>\n              <jats:sub>LCO<\/jats:sub> and emphysema groups (<jats:italic>p<\/jats:italic>\u2009\u2264\u20090.01). CT low-attenuation area percentage was not increased in IPAH with low <jats:italic>D<\/jats:italic>\n              <jats:sub>LCO<\/jats:sub> compared to any group. <jats:sup>129<\/jats:sup>Xe MRI-derived surface-volume ratio was reduced in IPAH with low versus preserved <jats:italic>D<\/jats:italic>\n              <jats:sub>LCO<\/jats:sub> (<jats:italic>p<\/jats:italic>\u2009=\u20090.04).<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Conclusion<\/jats:title>\n            <jats:p>Results are consistent with a loss of pulmonary capillaries in patients with IPAH and low <jats:italic>D<\/jats:italic>\n              <jats:sub>LCO<\/jats:sub> along with destruction of alveolar tissue, likely due to early diffuse emphysema.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Key Points<\/jats:title>\n            <jats:p>\n              <jats:bold>\n                <jats:italic>Question<\/jats:italic>\n              <\/jats:bold>\n              <jats:italic>A loss of pulmonary capillaries has been suggested in patients with IPAH and low diffusion capacity without clinically significant emphysema on CT<\/jats:italic>.<\/jats:p>\n            <jats:p>\n              <jats:bold>\n                <jats:italic>Findings<\/jats:italic>\n              <\/jats:bold>\n              <jats:sup>\n                <jats:italic>129<\/jats:italic>\n              <\/jats:sup>\n              <jats:italic>Xe uptake in red blood cells and lung surface-volume ratio were reduced in IPAH patients with low compared to preserved diffusion capacity<\/jats:italic>.<\/jats:p>\n            <jats:p>\n              <jats:bold>\n                <jats:italic>Clinical relevance<\/jats:italic>\n              <\/jats:bold>\n              <jats:italic>This study furthers the understanding of the underlying pathological mechanisms in IPAH with low diffusion capacity, providing evidence that loss of pulmonary capillaries is accompanied by alveolar tissue destruction despite near-normal CT<\/jats:italic>.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1007\/s00330-024-11209-1","type":"journal-article","created":{"date-parts":[[2024,12,8]],"date-time":"2024-12-08T07:42:18Z","timestamp":1733643738000},"page":"3010-3020","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Loss of pulmonary capillaries in idiopathic pulmonary arterial hypertension with low diffusion capacity is accompanied by early diffuse emphysema detected by 129Xe MRI"],"prefix":"10.1007","volume":"35","author":[{"given":"Agilo Luitger","family":"Kern","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Da-Hee","family":"Park","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan","family":"Fuge","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jens M.","family":"Hohlfeld","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frank","family":"Wacker","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marius M.","family":"Hoeper","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karen M.","family":"Olsson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5595-6948","authenticated-orcid":false,"given":"Jens","family":"Vogel-Claussen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,12,8]]},"reference":[{"key":"11209_CR1","doi-asserted-by":"publisher","first-page":"3618","DOI":"10.1093\/eurheartj\/ehac237","volume":"43","author":"M Humbert","year":"2022","unstructured":"Humbert M, Kovacs G, Hoeper MM et al (2022) 2022 ESC\/ERS guidelines for the diagnosis and treatment of pulmonary hypertension. 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