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. 2023 Mar 2;12(5):1996.
doi: 10.3390/jcm12051996.

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

Khaled Saleh  1 , Naureen Khan  2 , Kelly Dougherty  3 , Govinda Bodi  1 , Miriam Michalickova  1 , Samiuddin Mohammed  1 , Theodora Kerenidi  1 , Ziad Sadik  4 , Jihad Mallat  5   6   7 , Samar Farha  8 , Hani Sabbour  9
Affiliations

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

Khaled Saleh et al. J Clin Med. .

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.

Keywords: Riociguat; UAE; ambrisentan; congenital heart disease; connective tissue disease; hemodynamic parameters; idiopathic pulmonary hypertension; iloprost; outcomes; pulmonary hypertension; selexipeg; sildenafil; treprostenil.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
None, mono, dual and triple vasodilator therapies for Group 1-PH subgroups at initial and last visit. CTD, connective tissue disease; CHD, congenital heart disease.
Figure 2
Figure 2
Survival for the overall group 1-PH cumulative.
Figure 3
Figure 3
Survival for the subgroups 1-PH cumulative. CHD, congenital heart disease; CTD, connective tissue disease-related pulmonary hypertension.
See this image and copyright information in PMC

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