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

Abstract

BackgroundChronic 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.MethodsThis 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.ResultsAt 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%).ConclusionPatients 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.

Clinical and physiological characteristics of, medically treated, chronic thromboembolic pulmonary hypertension patients in Saudi Arabia: A single center experience

“…Consistent with previous studies,[ 9 12 19 20 ] VTE (with or without concomitant risk factors, such as antiphospholipid syndrome or SCD) was the major risk factor identified in our CTEPH cohort (65%). Although only one of our patients had no evidence of a previous symptomatic thromboembolic event, up to 25% of patients may have no prior history of VTE,[ 12 ] which makes an early diagnosis of CTEPH challenging.…”

“…[ 12 17 18 19 ] However, our findings are in line with previously reported data of local pulmonary arterial hypertension and CTEPH population. [ 20 21 ]…”

“…Both international and local studies have associated diagnostic delay with worse hemodynamic profile and shorter survival. [ 11 12 20 21 ]…”

Prospective clinical assessment of patients with pulmonary arterial hypertension switched from bosentan to macitentan (POTENT)

“…The demographic characteristics of the study population are in agreement with those reported in previous studies on the Saudi PAH population 21,27 . As usual in PH literature, we could notice a female predominance in our cohort 28 .…”

Results From the United States Chronic Thromboembolic Pulmonary Hypertension Registry

“…comparison. Data will be changing over time, because CTEPH populations from Russia, 6 Japan, 7 and Middle Eastern populations 8 will emerge in the literature. At this moment in time, the data from the United States clearly are shaded in the color of PEA, which promotes a treatment strategy that has changed the lives of thousands of patients from severe right heart failure back to near-normal but currently is challenged by percutaneous balloon pulmonary angioplasty.…”