Background/Purpose: Patients with systemic sclerosis (SSc) complicates variety of cardiopulmonary diseases which can result in pulmonary hypertension (PH). The types of PH are usually classified by the result of right heart catheterization (RHC) based on values of mean pulmonary arterial pressure (mPAP), pulmonary artery wedge pressure (PAWP), and pulmonary vascular resistance (PVR): Group 1 as mPAP >20mmHg, PAWP ≤15mmHg, and PVR ≥3WU; Group 2 as mPAP >20mmHg, PAWP >15mmHg, and PVR < 3WU. However, by this classification, patients with PH having normal PAWP (≤15mmHg) and normal PVR (< 3 WU) are categorized as neither Group 1 PH nor Group 2 PH and have a possible contribution of left heart disease (LHD). We therefore examined whether LHD is contributing among SSc-PH patients with such RHC results.

Methods: We retrospectively investigated 43 patients with SSc who were diagnosed as PH by RHC from 2006 through 2018 at our University hospital. Nice classification updated in 2018 was used in the diagnosis and classification of PH. To identify lesions responsible for PH, inspections with cardiac echocardiography, cardiac MRI, and myocardial scintigraphy were conducted.

Results: The mean age of the enrolled 43 patients was 66 (11) year-old, 88% of them were female. Twenty-one percent of the patients had diffuse cutaneous SSc. Mean follow-up periods were 56 (33) months.  Of the 43, 17 (40%) of the patients had severe interstitial lung disease with % forced vital capacity ≤70% (Group 3). Among the rest of 26 patients, 5 (19%) had PAWP >15mmHg (Group 2). Of the remaining 21 patients, only 10 patients (48%) had PVR ≥3 WU and was classified as Group 1 and another 11 had PAWP ≤15mmHg and PVR < 3WU. Among them, 9 patients (82%) showed less than 7 of diastolic pressure gradient obtained from RHC, indicating the presence of postcapillary PH. Among them, 6 patients (55%) had an elevation of E/e’ and/or left atrial volume index (LAVI), suggesting left diastolic dysfunction by cardiac echocardiography. Myocardial damage was detected by cardiac MRI [n = 6 (55%)] or myocardial scintigraphy [n = 6 (55%)]. Based on the results, 5 patients were diagnosed as precapillary PH and another 5 as postcapillary PH in addition to 5 with PAWP >15mmHg [in total, 10 / 43 (23%) with SSc-PH had LHD-associated PH]. The diagnosis was undetermined in one patient who had mixed characteristics of pre- and postcapillary PH. Pulmonary vasodilators were introduced in 5 patients who were clinically diagnosed as precapillary PH while diuretics and/or antihypertensive drugs were introduced in another 4 diagnosed as postcapillary PH. One patient died from PAH during the mean follow-up.

Conclusion: Since significant number of patients may have LHD, particularly diastolic dysfunction-associated PH even among SSc patients with normal PAWP as well as those with PAWP >15mmHg, careful diagnosis and therapeutic intervention with pulmonary vasodilator are required in this patient population. Longitudinal follow up will be needed to study the impact of the LHD on the survival.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/the-contribution-of-left-heart-disease-in-patients-with-systemic-sclerosis-associated-pulmonary-hypertension-having-normal-pulmonary-artery-wedge-pressure/