PO:12:180 | Echocardiographic screening of pulmonary hypertension in a multidisciplinary cohort of patients with interstitial lung disease

Lorenzo Mattia Bianchessi¹, Veronica Piccin¹, Federica Sabatelli¹, Lorenzo Cavagna¹, Giovanni Zanframundo¹, Domenicafederica Briganti², Mauro Acquaro³,  Stefano Ghio³, Domenico Sorace¹, Valentina Vertui², Ilaria Giana², Andrea Patanè³, Veronica Codullo¹ | ¹UOC Reumatologia, Fondazione IRCCS Policlinico S. Matteo, Pavia; ²Centro Trapianti, Fondazione IRCCS Policlinico San Matteo, Pavia; ³UOC Cardiologia, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy

Background. Pulmonary hypertension (PH) may complicate parenchymal lung diseases, and current international guidelines recommend echocardiographic study of patients with suspected PH. Right heart catheterization (RHC) is recommended if hemodynamic results can modify the therapeutic approach. The objective of the study is to verify, by means of echocardiography, the prevalence of PH and/or right ventricular dysfunction in a cohort of patients with ILD (Interstitial Lung Disease) followed in a multidisciplinary pneumo-rheumatological outpatient clinic, and to evaluate any associated clinical, serological, or radiological findings.

Materials and Methods. Consecutive patients with ILD attending the multidisciplinary outpatient clinic of a third-level national reference center for lung transplantation, excluding patients with already known PH. All patients underwent a Standard 2-D and Doppler echocardiogram with a Vivid E9 or Vivid E80 machine (GE Medical Systems, Norway); clinical, laboratory, and radiographic data were collected. When required by clinical suspicion or for inclusion in the lung transplant list, RHC was performed, and information was collected. Statistical analysis was conducted using SPSS (IBM for Mac (v13)).

Results. 113 patients were included, 66 women and 47 men. The mean age was 67.94 15 15.62 years, and the follow-up was 9.6 (3-13) years. Diagnoses were distributed as follows: 60 (53%) ILD in Systemic Sclerosis (SSc), 19 (17%) with anti-synthetase syndrome (ASSD), 8 (7%) interstitial pneumonia with autoimmune features (IPAF), 4 (3.5%) with rheumatoid arthritis (RA), 22 (19.5%) with idiopathic pulmonary fibrosis (IPF). 53/113 (47%) patients were smokers/ex-smokers, 28 (25%) had chronic respiratory failure requiring oxygen therapy, 35 (31%) desaturated during the six-minute walk test, with a mean distance of $395.8 \pm 87.6$ m, 29 (26%) were taking an antifibrotic agent. Mean respiratory functional parameters were(%) 106,8±19,7, TLC (%) 72,8±21,8, IT 80,21±7,3, DLCO (%) 58,9±21,2 BNP or NTproBNP were alterati (>50 o >300 pg/mL) in 32 (28.3%) patients. On echocardiographic evaluation, mean FEVs was 59,45±4,75% mean E/A was 0,86±0,08, mean PAPs was 26,5±10,6 mmHg, mean TAPSE was 22.5±3.6 mm; ACT (acceleration time) was reduced in 60 (53%), and a notch was present in the pulmonary artery flow in 28 (26%). During follow-up, 9 (8%) patients underwent RHC: 3/9 (33%) had PAPm (mean Pulmonary Artery Pressure) >20 mmHg and in 9/9, the PAWP (Pulmonary Artery Wedge Pressure) was <15 mmHg. Mean PAPm was 21.42 ±12.1 mmHg, median PVR (Pulmonary Vascular Resistance) was 3.7 (2.18-2.99) WU. The patients who developed PH already showed significant baseline differences in FVC% (60.4 ±23.4 vs 87.5 ±22.4, p<0.01), DLCO ($9.7 ± 60.4 p<0.01), PAPs (42.6 ± 5.6 vs 25.2 ± 9.8, p<0.01), % of notch in PA flow (75%$vs 21.8%, p<0.05). Radiographic pattern and specific autoantibodies did not show significant associations with PH.

Conclusions. Echocardiographic screening of patients with ILD demonstrates that the development of pulmonary hypertension is a frequent complication and is associated with specific clinical, functional, and respiratory characteristics already at baseline, which are capable of distinguishing subjects at higher risk who should be subjected to diagnostic confirmation with RHC.