{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,12]],"date-time":"2025-10-12T03:49:50Z","timestamp":1760240990007,"version":"build-2065373602"},"reference-count":20,"publisher":"MDPI AG","issue":"11","license":[{"start":{"date-parts":[[2019,11,2]],"date-time":"2019-11-02T00:00:00Z","timestamp":1572652800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Objective: Marfan syndrome (MFS) is a connective tissue disorder associated with severe cardiovascular morbidity and mortality. It is unknown if aorta complications in MFS are associated with progressive pulmonary artery (PA) dilatation. Methods: We measured the PA diameter on routine magnetic resonance imaging in a population of MFS patients seen in our specialised centre with follow up of diameters as well as the outcome. Results: PA dilatation was defined as an increase in diameter of 2 mm or more, and 71 patients (44%) of our total cohort (n = 162) met this criterion; mean follow up between two scans was 8.6 years (standard deviation (SD) \u00b1 2.7 years). Furthermore, 28 patients suffered from dissections, of which 14 had a type A dissection, 10 had a type B dissection, and 4 patients suffered from both. Of those who suffered from dissection, 64% (18 out of 28) had a dilatation of the PA, versus 39% (53 out of 134) in the patient group without a dissection (p &lt; 0.05). There was a significant association between type B dissection and descending aorta diameter (OR 1.14; 95% CI 1.05\u20131.24 p &lt; 0.01) and PA dilatation (OR 1.69; 95% CI 1.03\u20132.77 p = 0.04). In the multivariable analysis the final model for type B dissection, only systolic blood pressure (OR 1.06; 95% CI 1.01\u20131.11 p = 0.02) and PA dilatation were statistically significant (OR 1.85; 95% CI 1.10\u20133.12 p = 0.02) while descending aorta diameter was not. Conclusions: We report an association between progressive PA dilatation and type B dissection. Our findings encourage a renewed interest in PA dimensions in MFS.<\/jats:p>","DOI":"10.3390\/jcm8111848","type":"journal-article","created":{"date-parts":[[2019,11,4]],"date-time":"2019-11-04T04:13:08Z","timestamp":1572840788000},"page":"1848","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Progressive Pulmonary Artery Dilatation is Associated with Type B Aortic Dissection in Patients with Marfan Syndrome"],"prefix":"10.3390","volume":"8","author":[{"given":"Christel","family":"Brouwer","sequence":"first","affiliation":[{"name":"Department of Radiology and Nucleair Imaging, Radboud University Medical Center, Nijmegen 6500 HB, the Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Haldun","family":"Bulut","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Radboud University Medical Center, Nijmegen 6500 HB, the Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1554-3254","authenticated-orcid":false,"given":"Willemijn","family":"van Gemert","sequence":"additional","affiliation":[{"name":"Department of Radiology and Nucleair Imaging, Radboud University Medical Center, Nijmegen 6500 HB, the Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alexander HJ","family":"Staal","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Radboud University Medical Center, Nijmegen 6500 HB, the Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kim","family":"Cortenbach","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Radboud University Medical Center, Nijmegen 6500 HB, the Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7613-5433","authenticated-orcid":false,"given":"Miranda","family":"Snoeren","sequence":"additional","affiliation":[{"name":"Department of Radiology and Nucleair Imaging, Radboud University Medical Center, Nijmegen 6500 HB, the Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robin","family":"Nijveldt","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Radboud University Medical Center, Nijmegen 6500 HB, the Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5064-0143","authenticated-orcid":false,"given":"Anthonie","family":"Duijnhouwer","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Radboud University Medical Center, Nijmegen 6500 HB, the Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bart L","family":"Loeys","sequence":"additional","affiliation":[{"name":"Department of Clinical Genetics, Radboud University Medical Center, Nijmegen 6500 HB, the Netherlands"},{"name":"Center for Medical Genetics, Antwerp University Hospital &amp; University of Antwerp, Antwerp 2000, Belgium"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Niels","family":"van Royen","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Radboud University Medical Center, Nijmegen 6500 HB, the Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Janneke","family":"Timmermans","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Radboud University Medical Center, Nijmegen 6500 HB, the Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Roland RJ","family":"van Kimmenade","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Radboud University Medical Center, Nijmegen 6500 HB, the Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2019,11,2]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1965","DOI":"10.1016\/S0140-6736(05)67789-6","article-title":"Marfan\u2019s syndrome","volume":"366","author":"Judge","year":"2005","journal-title":"Lancet"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"S1877","DOI":"10.1016\/S0003-4975(02)04147-4","article-title":"Natural history of thoracic aortic aneurysms: Indications for surgery, and surgical versus nonsurgical risks","volume":"74","author":"Elefteriades","year":"2002","journal-title":"Ann. Thorac. Surg."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"I357","DOI":"10.1161\/CIRCULATIONAHA.105.000620","article-title":"Role and results of surgery in acute type B aortic dissection: Insights from the International Registry of Acute Aortic Dissection (IRAD)","volume":"114","author":"Trimarchi","year":"2006","journal-title":"Circulation"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"2226","DOI":"10.1161\/CIRCULATIONAHA.106.622340","article-title":"Long-term survival in patients presenting with type B acute aortic dissection: Insights from the International Registry of Acute Aortic Dissection","volume":"114","author":"Tsai","year":"2006","journal-title":"Circulation"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"476","DOI":"10.1136\/jmg.2009.072785","article-title":"The revised Ghent nosology for the Marfan syndrome","volume":"47","author":"Loeys","year":"2010","journal-title":"J. Med. Genet."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"487","DOI":"10.1136\/heart.87.5.487","article-title":"Images in cardiology. Development of the human heart: Days 15\u201321","volume":"87","author":"Schleich","year":"2002","journal-title":"Heart"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"528","DOI":"10.1038\/gim.2012.172","article-title":"A clinical appraisal of different Z-score equations for aortic root assessment in the diagnostic evaluation of Marfan syndrome","volume":"15","author":"Kempers","year":"2013","journal-title":"Genet. Med."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"581","DOI":"10.1002\/ajmg.1320290316","article-title":"International Nosology of Heritable Disorders of Connective Tissue, Berlin, 1986","volume":"29","author":"Beighton","year":"1988","journal-title":"Am. J. Med. Genet."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"417","DOI":"10.1002\/(SICI)1096-8628(19960424)62:4<417::AID-AJMG15>3.0.CO;2-R","article-title":"Revised diagnostic criteria for the Marfan syndrome","volume":"62","author":"Devereux","year":"1996","journal-title":"Am. J. Med. Genet."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"499","DOI":"10.1136\/hrt.82.4.499","article-title":"Survival and complication free survival in Marfan\u2019s syndrome: Implications of current guidelines","volume":"82","author":"Groenink","year":"1999","journal-title":"Heart"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1016\/S0002-9149(00)80066-1","article-title":"Life expectancy in the Marfan syndrome","volume":"75","author":"Silverman","year":"1995","journal-title":"Am. J. Cardiol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"2873","DOI":"10.1093\/eurheartj\/ehu281","article-title":"2014 ESC Guidelines on the diagnosis and treatment of aortic diseases: Document covering acute and chronic aortic diseases of the thoracic and abdominal aorta of the adult. The Task Force for the Diagnosis and Treatment of Aortic Diseases of the European Society of Cardiology (ESC)","volume":"35","author":"Erbel","year":"2014","journal-title":"Eur. Heart J."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"2779","DOI":"10.1093\/eurheartj\/ehv178","article-title":"Corrigendum to: 2014 ESC Guidelines on the diagnosis and treatment of aortic diseases","volume":"36","author":"Erbel","year":"2015","journal-title":"Eur. Heart J."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"e101","DOI":"10.1016\/j.jtcvs.2010.12.032","article-title":"Descending aortic diameter of 5.5 cm or greater is not an accurate predictor of acute type B aortic dissection","volume":"142","author":"Trimarchi","year":"2011","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1016\/j.jcmg.2012.07.018","article-title":"Predicting the risk for acute type B aortic dissection in hypertensive patients using anatomic variables","volume":"6","author":"Shirali","year":"2013","journal-title":"JACC Cardiovasc. Imaging"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"246","DOI":"10.1016\/j.jacc.2014.10.050","article-title":"The risk for type B aortic dissection in Marfan syndrome","volume":"65","author":"Franken","year":"2015","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"470","DOI":"10.1136\/heart.87.5.470","article-title":"Pulmonary artery root dilatation in Marfan syndrome: Quantitative assessment of an unknown criterion","volume":"87","author":"Nollen","year":"2002","journal-title":"Heart"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"203","DOI":"10.1186\/s13023-014-0203-8","article-title":"The main pulmonary artery in adults: A controlled multicenter study with assessment of echocardiographic reference values, and the frequency of dilatation and aneurysm in Marfan syndrome","volume":"9","author":"Sheikhzadeh","year":"2014","journal-title":"Orphanet. J. Rare Dis."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"922","DOI":"10.1038\/gim.2012.82","article-title":"The pulmonary artery in patients with Marfan syndrome: A cross-sectional study","volume":"14","author":"Lundby","year":"2012","journal-title":"Genet. Med."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"1194","DOI":"10.1007\/s00246-018-1880-1","article-title":"The Pulmonary Artery in Pediatric Patients with Marfan Syndrome: An Underestimated Aspect of the Disease","volume":"39","author":"Stark","year":"2018","journal-title":"Pediatr. Cardiol."}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/8\/11\/1848\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T13:31:29Z","timestamp":1760189489000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/8\/11\/1848"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,11,2]]},"references-count":20,"journal-issue":{"issue":"11","published-online":{"date-parts":[[2019,11]]}},"alternative-id":["jcm8111848"],"URL":"https:\/\/doi.org\/10.3390\/jcm8111848","relation":{},"ISSN":["2077-0383"],"issn-type":[{"type":"electronic","value":"2077-0383"}],"subject":[],"published":{"date-parts":[[2019,11,2]]}}}