The First Pulmonary Hypertension Registry in the United Arab Emirates (UAEPH): Clinical Characteristics, Hemodynamic Parameters with Focus on Treatment and Outcomes for Patients with Group 1-PH

Abstract

Background: The aim of this study is to present the first United Arab Emirates pulmonary hypertension registry of patients’ clinical characteristics, hemodynamic parameters and treatment outcomes. Method: This is a retrospective study describing all the adult patients who underwent a right heart catheterization for evaluation of pulmonary hypertension (PH) between January 2015 and December 2021 in a tertiary referral center in Abu Dhabi, United Arab Emirates. Results: A total of 164 consecutive patients were diagnosed with PH during the five years of the study. Eighty-three patients (50.6%) were World Symposium PH Group 1-PH; nineteen patients (11.6%) were Group 2-PH due to left heart disease; twenty-three patients (14.0%) were Group 3-PH due to chronic lung disease; thirty-four patients (20.7%) were Group 4-PH due to chronic thromboembolic lung disease, and five patients (3.0%) were Group 5-PH. Among Group 1-PH, twenty-five (30%) had idiopathic, twenty-seven (33%) had connective tissue disease, twenty-six (31%) had congenital heart disease, and five patients (6%) had porto-pulmonary hypertension. The median follow-up was 55.6 months. Most of the patients were started on dual then sequentially escalated to triple combination therapy. The 1-, 3- and 5-year cumulative probabilities of survival for Group 1-PH were 86% (95% CI, 75–92%), 69% (95% CI, 54–80%) and 69% (95% CI, 54–80%). Conclusions: This is the first registry of Group 1-PH from a single tertiary referral center in the UAE. Our cohort was younger with a higher percentage of patients with congenital heart disease compared to cohorts from Western countries but similar to registries from other Asian countries. Mortality is comparable to other major registries. Adopting the new guideline recommendations and improving the availability and adherence to medications are likely to play a significant role in improving outcomes in the future.

Prescription patterns of ambrisentan in some cities of Colombia

“… 13 , 14 , 15 , 17 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 Arterial hypertension was the most prevalent comorbidity in this report, which is consistent with the literature. 17 , 20 , 26 , 27 , 28 , 29 , 30 , 31 Pulmonary arterial hypertension (Group 1) was the most prevalent, in line with other studies with real‐world evidence, 20 , 22 , 23 , 25 and most of the patients had WHO Functional Class I; this differs from other reports in which the patients presented a more deteriorated functional class, 15 , 20 , 22 , 23 , 25 , 29 but the proportion of cases with home oxygen use was similar (33.3% vs. 21.6–30.2%). 24 , 28 …”

“…The median age of the patients in this study was similar to that found in some studies in Asia (47.0–50.8 years) 20 , 21 and Europe (49.6–52.0 years), 22 , 23 , 24 but in other reports from Europe (61.0–61.6 years), 13 , 14 , 25 North America (62.7–68.1 years) 17 , 26 , 27 , 28 and Oceania (60.0 years), 29 patients were older, 13 , 14 , 17 , 25 , 26 , 27 , 28 , 29 and there was a predominance of women (82.1% vs. 61.0–79.0%). 13 , 14 , 15 , 17 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 Arterial hypertension was the most prevalent comorbidity in this report, which is consistent with the literature. 17 , 20 , 26 , 27 , 28 , 29 , 30 , 31 Pulmonary arterial hypertension (Group 1) was the most prevalent, in line with other studies with real‐world evidence, 20 , 22 , 23 , 25 and most of the patients had WHO Functional Class I; this diffe...…”

Diagnosis and Treatment Patterns of Chronic Thromboembolic Pulmonary Hypertension in Russia, Kazakhstan, Turkey, Lebanon, and Saudi Arabia: A Registry Study

“…The patients in this registry were younger than those in some other registries, including the International CTEPH Registry (Europe and Canada) [ 45 ], the US CTEPH Registry [ 40 ], the worldwide prospective registry [ 39 ], the single-center study in the Netherlands [ 41 ], or the UAE Registry [ 44 ]. They were also younger than other studies, including the Giessen PH registry in Germany [ 46 ], the COMPERA (Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension) registry [ 47 ], a study from Germany which included patients from COMPERA and three German referral centers [ 48 ], and a large cohort study in Canada [ 49 ].…”

“…In the current study, 68% of patients presented with a history of a previous VTE. This was a slightly lower proportion than in the International CTEPH Registry (81% in operable and 72% in non-operable patients) [ 45 ], the German study (76%) [ 48 ], COMPERA (72%) [ 47 ], the single-center study in the Netherlands (78% in clinically inoperable patients; 80% in technically inoperable patients or those with residual PH after PEA) [ 41 ], the UAE Registry (78% of patients had a history of PE) [ 44 ], or the Russian National Registry (53% and 27.2% of patients had a history of deep vein thrombosis or PE, respectively) [ 42 ]. Despite guideline recommendations stating that patients with CTEPH should receive lifelong anticoagulation therapy [ 6 , 15 ], only 79% and 52% of patients were documented as receiving anticoagulation at initial visit and study end, respectively.…”

“…Most patients had advanced disease (World Health Organization functional class III or IV) at baseline, suggesting that diagnosis was often delayed, but patients were diagnosed and managed according to international guidelines, and cumulative probability of survival at 1 year was 96.6%. The first PH registry in the United Arab Emirates (UAE) recently reported the baseline characteristics of 34 patients with CTEPH [ 44 ]. Other than these reports, there are few available registry CTEPH data for other Eastern European countries or the Middle East.…”