Pulmonary hypertension in Saudi Arabia: First data from the SAUDIPH registry with a focus on pulmonary arterial hypertension

Abstract

Background and objective This study presents the first results of ‘SAUDIPH’ registry, aiming to assess patient characteristics, treatment approach and clinical and survival outcomes in patients with PAH. Methods The registry enrolled patients with Group 1 and Group 4 PH under clinical management in a specialized tertiary care centre from 2004 to 2018. Changes from baseline to last follow‐up visit were assessed. Results A total of 222 patients were enrolled, and Group 1 PH was the most frequent aetiology (57.7%). Mean age at diagnosis was 32 years. mPAP was 55.0 mm Hg and was higher for Group 1 PH (59.0 mm Hg, P < 0.001). At the last visit, most patients were on specific therapy (83.7%) and 30% shifted from FC III/IV to FC I/II. NT‐proBNP improved by 29.2% in the overall population. The 1‐, 3‐ and 5‐year cumulative probabilities of survival were 95.6% (95% CI: 91.5–99.9%), 89.2% (95% CI: 82.1–96.9%) and 74.6% (95% CI: 59.4–93.7%), respectively. CHD‐PAH demonstrated the best survival among Group 1 PAH with 1‐, 3‐ and 5‐year cumulative probability of 100%, 100%, and 75.0% (95% CI: 42.6–100), respectively. Conclusion PAH was the most frequent aetiology and patients were younger at diagnosis compared to other cohorts. Most patients showed improvement in FC and NT‐proBNP. The estimated 1‐year survival was better than previous studies, possibly reflecting wider use of combination therapy and the high prevalence of CHD‐PAH.

Genetic basis of pulmonary arterial hypertension: a prospective study from a highly inbred population

“…In our study, we could not confirm previous findings that evidenced very young ages at diagnosis, in PAH patients with positive gene mutations, except for those with EIF2AK4 mutations 14 . The median age at diagnosis of the patients with gene mutations (29.5 years) is in agreement with previous reports on the Saudi Arabia PAH population 24 …”

“…14 The median age at diagnosis of the patients with gene mutations (29.5 years) is in agreement with previous reports on the Saudi Arabia PAH population. 24 Regarding disease severity, our results showed a tendency for higher disease severity in patients with positive mutations, mainly in those with mutations in EIF2AK4. In fact, even though our patients with mutations were equally distributed between WHO FC II and III/IV, 6MWD, NT-proBNP, and hemodynamic parameters (cardiac index (CI), mean pulmonary arterial pressure (mPAP) and mean right atrial pressure (mRAP)) are indicative of a uncontrolled disease, as reported by Pfarr et al 14 Mutations were identified in 30% of the IPAH patients and in 100% of the PVOD patients (n ¼ 2).…”

“… 14 The median age at diagnosis of the patients with gene mutations (29.5 years) is in agreement with previous reports on the Saudi Arabia PAH population. 24 …”

“…This prospective, non-randomized study included patients from our ongoing SystemAtic prospective follow Up for better unDerstanding of clInical characteristics of patients with Pulmonary Hypertension disease (SAUDIPH) registry. 24 Forty-eight Saudi Arabian PAH patients were enrolled, with full-informed consent, under an Institutional Review Board-approved protocol (RAC# 2161062). The study was approved by Hospital Research Advisory Council and Ethical Committee.…”

Characteristics, goal‐oriented treatments and survival of pulmonary arterial hypertension in China: Insights from a national multicentre prospective registry

“…than 6 months before enrolment, while prevalent cases were regarded as those who had already received a diagnosis of PAH and started targeted therapy more than 6 months prior to enrolment. 15,16 Initial combination therapy was defined as initiating two or more PAH-targeted drugs at the start of targeted treatment in newly diagnosed and treatment-naive patients. 8 Diagnostic interval was calculated as the period between symptom onset and diagnosis of PAH by RHC.…”