Intravenous prostanoids in systemic sclerosis-associated pulmonary arterial hypertension: a single-centre experience.

OBJECTIVES: The current study evaluates survival rates among SSc-associated pulmonary arterial hypertension (SSc-PAH) patients on i.v. prostanoids, and short-term impact of i.v. prostanoids on clinical and haemodynamic parameters. METHODS: Baseline demographics, invasive and non-invasive data, European Society of Cardiology (ESC) score and REVEAL score of 81 SSc-PAH patients (median age 61 years, interquartile range 54-67 years, 84% females) were prospectively recorded, from November 2006 till November 2020, before initiation of i.v. prostanoids, and at first formal reassessment. Survival data were retrieved from National Health Service Spine and hospital databases. RESULTS: Significant improvements in clinical and haemodynamic parameters in response to i.v. prostanoid therapy were documented. Functional class (FC) (16.6% improved by 1FC, P =0.041), mean pulmonary arterial pressure (-6.5 mmHg, P =0.036), pulmonary vascular resistance (-2.6 WU, P =0.012), cardiac index (Q/m2) (+0.7 l/min/m2, P =0.003) and mixed venous oxygen saturation (SvO2) (+3%, P =0.036) improved. Estimated survival for CTD-PAH patients on i.v. prostanoids was 64%, 31% and 18%, at 1 year, 3 years and 5 years, respectively. Independent baseline predictors of mortality were older age (HR: 1.043, 95% CI: 1.011-1.075, P =0.007), higher N-terminal pro-brain natriuretic peptide levels (HR: 2.191, 95% CI: 1.131-4.243, P =0.020), and lower SvO2 levels (HR: 0.962, 95% CI: 0.926-0.998, P =0.039). High ESC risk or high and very high REVEAL score was associated with significantly worse survival compared with patients with lower risk scores, both at baseline and when reassessed after a median of 6.5 months. CONCLUSIONS: Survival among SSc-PAH patients on i.v. prostanoids remains poor, risk scoring at baseline and after 6.5 months of therapy improves prognostication.

Ambulatory prostanoid therapy: safe reduction in duration of inpatient training.

AIMS: IV prostanoid therapy for advanced pulmonary arterial hypertension requires lifelong, continuous infusion, and extensive self-care. The inpatient training pathway (ITP) ensures patient competency but can be psychologically and physically demanding. Therefore, an alternative Elective Prostanoid Admission Pathway (EPAP) was developed.Compare clinical outcomes and patient experiences for patients following the EPAP vs. the ITP. METHODS AND RESULTS: From 2013, clinically stable patients were trained via the EPAP, which consisted of pre-admission including an outpatient training day, followed by inpatient training. The EPAP patients were followed-up face-to-face/via telephone and could access a Clinical Nurse Specialist-led telephone service between appointments. Very high-risk patients were trained via the ITP, which consisted of pre-therapy counselling and daily ward-based training. Prior to 2013, patients followed the ITP pathway irrespective of clinical status. All were enrolled into the 'IV buddy' scheme and retrospectively asked to complete patient experience surveys. Among EPAP (n = 24) and ITP (n = 54) patients, 17% and 33% discontinued therapy, respectively. Among all, frequent challenges to treatment initiation were: dexterity (43%) and body image (27%). Elective Prostanoid Admission Pathway use reduced inpatient stay duration by 8 days per patient and infection rates remained low. Patient experience surveys [n = 17 (EPAP), n = 10 (ITP)] showed equal patient satisfaction between groups, but the incidence of side effects was numerically lower in EPAP patients, who also reported home practice and having access to the 'IV buddy' scheme as 'very useful' [12/14 (86%) and 10/13 (77%), respectively]. CONCLUSIONS: Elective Prostanoid Admission Pathway implementation improved patient outcomes and has the potential for application to other clinical scenarios where patient self-management is required.

Optical coherence tomography evaluation of pulmonary arterial vasculopathy in Systemic Sclerosis.

Our current understanding of the pathophysiology of pulmonary vascular disease is incomplete, since information about alterations of the pulmonary vasculature in pulmonary arterial hypertension (PAH) is primarily provided by autopsy or tissue specimens. The aim of this study was to compare the distal pulmonary vasculature of <2 mm in diameter in Systemic Sclerosis (SSc) patients with (n = 17) and without (n = 5) associated PAH using Optical Coherence Tomography during Right Heart catheterization. SSc-PAH patients showed significant thickening of Intima Media Thickening Area compared to patients without PAH (27 +/- 5.8% vs. 21 +/- 1.4%, p = 0.024). A good haemodynamic response to previous targeted PAH treatment was associated with a significantly greater number of small pulmonary artery side branches <300 µm per cm vessel (3.8 +/- 1.1 vs. 1.8 +/- 1.1; p = 0.010) and not associated with Intima Media thickening Area (26 +/- 5.4% vs. 28 +/- 6.7%; p = 0.6). Unexpected evidence of pulmonary artery thrombus formation was found in 19% of SSc-PAH patients. This is the first in-vivo study demonstrating a direct link between a structural abnormality of pulmonary arteries and a response to targeted treatment in PAH. Intravascular imaging may identify subgroups that may benefit from anticoagulation.