Disease characteristics and clinical outcome over two decades from the Swiss pulmonary hypertension registry

Abstract

Pulmonary hypertension (PH), especially pulmonary arterial and chronic thromboembolic pulmonary hypertension (PAH/CTEPH), are rare and progressive conditions. Despite recent advances in treatment and prognosis, PH is still associated with impaired quality of life and survival. Long‐term PH‐registry data provide information on the changing PH‐epidemiology and may help to direct resources to patient's needs. This retrospective analysis of the Swiss Pulmonary Hypertension Registry includes patients newly diagnosed with PH (mainly PAH/CTEPH) registered from January 2001 to June 2019 at 13 Swiss hospitals. Patient characteristics (age, body mass index, gender, diagnosis), hemodynamics at baseline, treatment, days of follow‐up, and events (death, transplantation, pulmonary endarterectomy, or loss to follow‐up) at last visit were analyzed. Patients were stratified into four time periods according to their date of diagnosis. Survival was analyzed overall and separately for PAH/CTEPH and time periods. 1427 PH patients were included (thereof 560 PAH, 383 CTEPH). Over the years, age at baseline (mean ± SD) significantly increased from 59 ± 14 years in 2001–2005 to 66 ± 14 years in 2016–2019 (p < 0.001) while the gender distribution tended toward equality. Mean pulmonary artery pressure and pulmonary vascular resistance significantly decreased over time (from 46 ± 15 to 41 ± 11 mmHg, respectively, 9 ± 5 to 7 ± 4 WU, p < 0.001). Three‐year survival substantially increased over consecutive periods from 69% to 91% (for PAH 63%–95%, for CTEPH 86%–93%) and was poorer in PAH than CTEPH independently of time period (p < 0.001). Most patients were treated with mono‐ or combination therapy and an increasing number of CTEPH underwent pulmonary endarterectomy (40% 2016–2019 vs. 15% 2001–2005). This long‐term PH registry reveals that over two decades of observation, newly diagnosed patients are older, less predominantly female, have less impaired hemodynamics and a better survival.

Real-World Management of Patients with Pulmonary Arterial Hypertension: Insights from EXPOSURE

“…The 1-year survival estimate observed here for incident patients (88%) is similar to that seen for incident patients in disease registries (86%–95%) [ 3 – 5 , 11 , 12 , 14 , 19 ]. In the past decade, analyses from national registries [ 15 , 25 ] have shown that populations of incident patients tend to have worse survival than prevalent patients, which could reflect a survival bias among the prevalent patients [ 26 ].…”

Long-term outcome of patients with combined post- and pre-capillary pulmonary hypertension

“…This finding is consistent with not only long-term follow-up data from patients with PH due to valvular heart disease, but also registry data from patients with PAH, including the Swiss PH Registry. 4 , 10 Furthermore, a female predominance in patients with PH due to heart failure with preserved ejection fraction has been previously reported. 11 The impact of specific aetiologies of chronic LHD leading to CpcPH should be further investigated.…”

“…The design of the Swiss PH Registry has been described previously. 4–6 All participants gave written informed consent to have their data registered and used for research. The clarification of responsibility conducted by the Cantonal Ethics Review Board concluded that no further ethical approval is needed (BASEC-No.…”

Pulmonary arterial wedge pressure increase during exercise in patients diagnosed with pulmonary arterial or chronic thromboembolic pulmonary hypertension

“…The main findings are mainly driven by patients ≥50 years, which is due to the large sample size in this age group (119 versus 24). The haemodynamic limitations of included patients were generally slightly milder compared with the latest Swiss PH registry data, which revealed an mPAP of 41±11 mmHg along with a PVR of 7±4 WU for the years 2016–2019 [ 16 ]. This was most probably because we chose to study patients with relatively mild disease who were able to perform exercise RHC.…”