06 July 2020

News from SAUDIPH registry

The PVRI Eastern Mediterranean & Saudi Arabia Task Force has recently published two articles, which we are delighted to introduce below. This work represents the first data analysis and results from the Systematic Prospective follow-up for better understanding of clinical characteristics of patients with pulmonary hypertension disease (SAUDIPH) registry, which is currently a single centre registry for the pulmonary hypertension programme at the King Faisal Specialist Hospital & Research Centre in Riyadh, Saudi Arabia.

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Chronic thromboembolic pulmonary hypertension in Saudi Arabia: preliminary results from the SAUDIPH registry

Abdullah M. Aldalaan, Sarfraz A. Saleemi, Ihab Weheba, Abeer Abdelsayed, Pekka Hämmäinen, Maha M. Aleid, Fatima Alzubi, Hamdeia Zaytoun, Nadeen Alharbi

Abstract
Background Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare, progressive vascular disease with poor prognosis if left untreated. This study aims to assess the patient characteristics, treatment approach and clinical and survival outcomes for CTEPH patients enrolled in the Systematic Prospective Follow Up for Better Understanding of Clinical Characteristics of Patients with Pulmonary Hypertension Disease (SAUDIPH) registry.

Methods
This study presents a subanalysis of CTEPH patients enrolled in the SAUDIPH registry. This registry enrolled patients with pulmonary hypertension, established through right heart catheterisation, under clinical management at a specialised tertiary care centre. Patients received standard care during the period of the registry.

Results
At the time of this analysis, 64 CTEPH patients were enrolled in the registry. Mean age at diagnosis was 39.7 years and there was a female predominance (67.6%). At baseline, most patients were in World Health Organization functional classes III or IV (70.1%). At the last follow-up visit, most patients (63.2%) had undergone endarterectomy, showing significant improvement in disease severity from baseline. Patients who underwent endarterectomy showed numerically higher (p=0.126) probability of survival at 1 year (97.5%) versus those who did not undergo endarterectomy (94.4%).

Conclusion
Patients were diagnosed at relatively young age, but still showed high disease severity, suggesting delay in diagnosis. Patients who underwent surgical treatment showed substantial improvements in clinical and haemodynamic parameters, while the remaining patients tended to show disease progression. The 96.6% 1-year cumulative probability of survival was high compared to previous studies.

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

Abdullah M. Aldalaan, Sarfraz A. Saleemi, Ihab Weheba, Abeer Abdelsayed, Maha M. Aleid, Fatima Alzubi, Hamdeia Zaytoun, Nadeen Alharbi


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.


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