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Abstract Number: 2686

In Established Pulmonary Arterial Hypertension the Follow-up Cardiac Catheterization-Derived  Pulmonary Artery Systolic Pressure and Vascular Resistence May be Predicted By Echocardiography- a Longitudinal Study in Two Connective Tissue Disease Cohorts

Vasiliki-Kalliopi Bournia1, Iraklis Tsangaris2, Loukianos Rallidis2, Anastasia Anthi2, Stylianos Orfanos2, Eftychia Demerouti3, Panayiotis Karyofyllis3, Vasilis Voudris3, Stylianos Panopoulos4 and Petros P Sfikakis4,5, 1First Department of Propedeutic and Internal Medicine and Joined Rheumatology Program, National and Kapodistrian University of Athens Medical School, Athens, Greece, 2Pulmonary Hypertension Clinic, National and Kapodistrian University of Athens Medical School, Athens, Greece, 3Cardiology Department, Onassis Cardiac Surgery, Athens, Greece, 4First Department of Propaedeutic and Internal Medicine and Joint Rheumatology Program, National and Kapodistrian University of Athens Medical School, Athens, Greece, 5First Department of Propaedeutic and Internal Medicine and Joint Rheumatology Program, National and Kapodistrian University of Athens Medical School, athens, Greece

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: mixed connective tissue disease (MCTD) and systemic sclerosis

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Session Information

Date: Tuesday, November 7, 2017

Title: Systemic Sclerosis, Fibrosing Syndromes and Raynaud's – Clinical Aspects and Therapeutics Poster III

Session Type: ACR Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose: Pulmonary arterial hypertension (PAH) associated with connective tissue disease should always be diagnosed with the gold-standard method of right heart catheterization (RHC). On the other hand, transthoracic echocardiography (TTE) is well validated for initial assessment when PAH is suspected. Whether TTE has a role in the follow-up of PAH is less known. The purpose of this study was to test the hypothesis that TTE can predict follow-up RHC-derived hemodynamic measurements in connective tissue disease-associated PAH.

Methods: This retrospective study included two independent cohorts of 30 and 14, patients, respectively, with systemic sclerosis (n=34) and mixed connective tissue disease (n=10) (mean age±SD: 59.09±12.16 years, 86% women), in whom PAH had been suggested by baseline TTE and confirmed by RHC [pulmonary artery systolic pressure (PASP): 59.3±16.8, range 25-90mmHg; pulmonary vascular resistance (PVR): 6.2±3.2, range 0.7-14.5 Wood units). All 44 patients underwent a second RHC and TTE at follow-up after 9.6±5.7 (range 4-29) months. Seventeen patients had a 3rd follow-up RHC and TTE 18.7±7.1 (range 10-37) months from baseline, thus producing in all 50 and 28 pairs of baseline/follow-up measurements, in each cohort, respectively. By considering as the gold-standard the follow-up RHC, we examined whether clinically meaningful hemodynamic changes from baseline (i.e. greater than +15% change) in either RHC-derived PASP or PVR could be predicted by the corresponding TTE-derived PASP changes from baseline to follow-up. RHC and TTE were always performed, each, by the same examiner.

Results: In 72% and 93% of comparisons between baseline and follow-up, in the first and second cohort respectively, the latter TTE measurements could safely replace RHC in terms of PASP estimation. Using Mc Nemar’s test we confirmed that the two methods did not differ significantly in either cohort (p=0,79 and p=0,48 for the first and the second cohort, respectively). When in addition to changes in PASP, PVR changes were also considered, follow-up TTE could again safely replace the second RHC in terms of PASP and PVR estimation in 74% and 89% of patients retests (p=0,58 and p=0,25 for the first and the second cohort, respectively). Of note, baseline hemodynamic values or TTE measurements did not differ between patients in whom TTE could predict RHC-derived PASP and PVR and those in whom the results of the two methods at follow-up were divergent.

Conclusion: In a study where operator-dependent methodological errors are limited, we found, in two independent cohorts, that 4 out of 5 patients with connective tissue disease-associated PAH, PASP and PVR can be safely monitored in the long-term non-invasively. Further studies to help identify those patients at need for follow-up RHC are warranted.


Disclosure: V. K. Bournia, None; I. Tsangaris, None; L. Rallidis, None; A. Anthi, None; S. Orfanos, None; E. Demerouti, None; P. Karyofyllis, None; V. Voudris, None; S. Panopoulos, None; P. P. Sfikakis, None.

To cite this abstract in AMA style:

Bournia VK, Tsangaris I, Rallidis L, Anthi A, Orfanos S, Demerouti E, Karyofyllis P, Voudris V, Panopoulos S, Sfikakis PP. In Established Pulmonary Arterial Hypertension the Follow-up Cardiac Catheterization-Derived  Pulmonary Artery Systolic Pressure and Vascular Resistence May be Predicted By Echocardiography- a Longitudinal Study in Two Connective Tissue Disease Cohorts [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/in-established-pulmonary-arterial-hypertension-the-follow-up-cardiac-catheterization-derived-pulmonary-artery-systolic-pressure-and-vascular-resistence-may-be-predicted-by-echocardiography-a-lo/. Accessed .
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