TY - JOUR AU - Kolonics-Farkas, Abigél Margit AU - Ágg, Bence AU - Benke, Kálmán AU - Odler, Balázs AU - Bohács, Anikó AU - Jáky-Kováts, Zsuzsanna Ágnes AU - Szabolcs, Zoltán AU - Müller, Veronika TI - Lung Function Changes are More Common in Marfan Patients Who Need Major Thoracic Surgery JF - LUNG J2 - LUNG VL - 197 PY - 2019 IS - 4 SP - 465 EP - 472 PG - 8 SN - 0341-2040 DO - 10.1007/s00408-019-00236-1 UR - https://m2.mtmt.hu/api/publication/30678816 ID - 30678816 N1 - Department of Pulmonology, Semmelweis University, Budapest, Hungary Heart and Vascular Centre, Semmelweis University, Budapest, Hungary Hungarian Marfan Foundation, Budapest, Hungary Department of Pharmacology and Pharmacotherapy, Semmelweis University, Budapest, Hungary Clinical Division of Nephrology, Department of Internal Medicine, Medical University of Graz, Graz, Austria Export Date: 9 October 2021 CODEN: LUNGD Correspondence Address: Kolonics-Farkas, A.M.; Department of Pulmonology, Hungary; email: kolonics-farkas.abigel@med.semmelweis-univ.hu AB - Marfan syndrome is a genetic disorder affecting the connective tissue. Changes in lung tissue might influence respiratory function; however, a detailed respiratory functional assessment according to the need for major thoracic surgery is missing.Comprehensive pulmonary examinations were performed in 55 Marfan patients including respiratory symptoms, lung function (LF) testing using European Coal and Steel Community (ECSC) reference values, TLCO and quality of life measurements. Groups included patients who did not need surgery (Mf, n = 32) and those who underwent major thoracic surgery (Mfop, n = 23).Respiratory symptoms affected 20% of patients. Scoliosis was significantly more frequent in the Mfop group. LF demonstrated in all Marfan patients a tendency towards airway obstruction (FEV1/FVC = 0.77 ± 0.10), more prominent in Mfop patients (0.74 ± 0.08 vs. Mf: 0.80 ± 0.11; p = 0.03). Correction of LF values using a standing height modification by arm span (Hcorrected) revealed additional changes in FVC and FEV1. TLCO and quality of life did not differ between groups.Marfan syndrome is associated with airway obstruction, especially in patients who have undergone major thoracic surgery, indicative of more severe connective tissue malfunction. The use of arm span for height correction is suitable to evaluate LF changes in this special patient group including patients with significant scoliosis. LA - English DB - MTMT ER -