Characteristics of Sarcoid Associated Pulmonary Hypertension in Ireland

Title: Characteristics of Sarcoid Associated Pulmonary Hypertension in Ireland
Author(s): M Higgins S Cullivan B McCullagh S Gaine
Institution: Mater Misericordiae University Hospital
Poster: Click to view poster
Category: Miscellaneous
Abstract: Sarcoidosis is a multisystemic disease of uncertain aetiology, that can affect the pulmonary circulation and right heart. Sarcoid associated pulmonary hypertension (SAPH) is an important and under-recognised complication of sarcoidosis, which carries significant morbidity and mortality. This is the first study describing the characteristics of SAPH in the National Pulmonary Hypertension Unit (NPHU) in Ireland.

This retrospective single centre study identified cases of SAPH, confirmed by right heart catheterisation, diagnosed between 2010 and 2020. Nineteen cases of SAPH were identified in this period and baseline characteristics are highlighted in table 1.

The nature of SAPH was multifactorial and included a sarcoid related pulmonary arterial vasculopathy, left heart disease, hypoxic lung disease due to interstitial lung disease and extrinsic pulmonary artery (PA) compression due to progressive massive fibrosis. At SAPH diagnosis, 13 (68%) patients were prescribed sarcoid directed therapy. Pulmonary hypertension specific therapy was prescribed in 14 cases (74%) and comprised of monotherapy in 10 (53%) and double combination therapy in 2 (21%) cases. One patient required PA stent insertion for extrinsic compression and 1 patient underwent double lung transplantation.

This study described the phenotypes of SAPH in the NPHU and characterises the clinical and treatment characteristics of this cohort.

Baseline characteristics
Patients, n=19
Sex: male n(%), 13(68)
Age (years) at SAPH diagnosis: mean ± SD, 54 ± 12
Interval (months) between sarcoid diagnosis and SAPH: mean ± SD, 184 ± 177
WHO functional class (FC), (n) I/II/III/IV, 0/4/14/1
BNP (ng/L): mean ± SD, 266 ± 419
6-minute walk distance (meters): mean ± SD, 312 ± 82
Risk stratification (ESC/ERS): n(%)
- Low risk: 1(5)
- Intermediate risk:12 (63)
- High risk: 6 (32)
Right heart catheterization
- mRAP (mmHg), 6.7 ± 5.8
- mPAP (mmHg), 39 ± 12
- PAWP (mmHg), 12.5 ± 7
- CO (L/min), 4.9 ± 1.5
- PVR (WU), 6.9 ± 5.2
Pulmonary function tests
- FEV1 (%), 50 ± 18
- FVC (%), 71 ± 24
- FEV1/FVC, 64 ± 14
- DLCO, 38 ± 16

Table 1: Highlights the characteristics of patients with sarcoid associated pulmonary hypertension in this study. Abbreviations: SAPH: Sarcoid associated pulmonary hypertension; SD: Standard deviation; WHO: world health organisation; BNP: B-type natriuretic peptide: mRAP: mean right atrial pressure; mPAP: mean pulmonary artery pressure; PAWP: pulmonary artery wedge pressure; CO: cardiac output; PVR: pulmonary vascular resistance; FEV1: forced expiratory volume in 1 second; FVC: forced vital capacity; DLCO: diffusing capacity for carbon monoxide