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.
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.