Long-Term Data from the Swiss Pulmonary Hypertension Registry

Abstract

Results: From 996 (53% female) PH patients, 549 had pulmonary arterial hypertension (PAH), 36 PH due to left heart disease, 127 due to lung disease, 249 to chronic thromboembolic PH (CTEPH) and 35 to miscellaneous PH. Age and BMI significantly increased over time, whereas hemodynamic severity decreased. Overall, event-free survival was 84, 72, 64 and 58% for the years 1-4 and similar for time periods since 2000, but better during the more recent periods for PAH and CTEPH. Of all PAH cases, 89% had target medical therapy and 43% combination therapy. Of CTEPH patients, 14 and 2% underwent pulmonary endarterectomy or transplantation, respectively; 87% were treated with PAH target therapy. Conclusion: Since 2000, the incident Swiss PH patients registered were older, hemodynamically better and mostly treated with PAH target therapies. Survival has been better for PAH and CTEPH diagnosed since 2008 compared with earlier diagnosis or other classifications. © 2015 S. Karger AG, Basel Key WordsRegistry · Pulmonary hypertension · Pulmonary arterial hypertension · Chronic thromboembolic pulmonary hypertension Abstract Background: Registries are important for real-life epidemiology on different pulmonary hypertension (PH) groups. Objective: To provide long-term data of the Swiss PH registry of 1998-2012. Methods: PH patients have been classified into 5 groups and registered upon written informed consent at 5 university and 8 associated hospitals since 1998. New York Heart Association (NYHA) class, 6-min walk distance, hemodynamics and therapy were registered at baseline. Patients were regularly followed, and therapy and events (death, transplantation, endarterectomy or loss to follow-up) registered. The data were stratified according to the time of diagnosis into prevalent before 2000 and incident during 2000-

Prevalence of Anxiety and Depression in Pulmonary Hypertension and Changes during Therapy

“…All patients with PAH, CTEPH or other types of PH seen and diagnosed at our clinic gave their written informed consent to have their data registered [17]. The local database of the PH center at the University Hospital of Zurich was reviewed for patients with PH who had filled in HADS questionnaires.…”

“…In rare diseases, such as PAH or CTEPH, registries provide valuable information on the baseline characteristics and outcomes of the disease [17]. We have built a PH database in our daily practice, which includes classification according to international guidelines [18], and have prospectively collected data according to standardized procedures, which allows long-term observation.…”

“…We have built a PH database in our daily practice, which includes classification according to international guidelines [18], and have prospectively collected data according to standardized procedures, which allows long-term observation. In this database, we have also included several instruments for assessing health-related quality of life (HRQoL) and emotional disorders by the Hospital Anxiety and Depression Scale (HADS) [17]. …”

Chronic thromboembolic pulmonary hypertension - a diagnostic and therapeutic update

“…CTEPH is less frequent than pulmonary artery hypertension (PAH). In a recently published report from the Swiss Registry, CTEPH was diagnosed roughly half as frequently as PAH (in 249 vs. 549 patients or 45 %); interestingly, the quote increased when comparing the diagnoses in the years from 2000 to 2004 with those from 2009 to 2012 (from 35 % to 58 %, p = 0.01), which could be the result of a qualitatively improved diagnostic work-up by better adherence to guidelines recommending V/Q scanning [10]. Symptoms of dyspnoea, fatigue, chest pain, syncope, peripheral oedema, and palpitations are unspecifi c, and diagnosis is often delayed.…”

Risk Factor Profiles Achieved with Medical Therapy in Prevalent Patients with Pulmonary Arterial and Distal Chronic Thromboembolic Pulmonary Hypertension

“…The efficacy of combination therapy in distal CTEPH is less clear due to lack of studies. However, from a pathophysiological perspective, distal CTEPH might be expected to respond comparably to PAH [13] and thus an increasing number of PH centres also use combination therapy in patients with distal CTEPH [3, 18]. …”