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.

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.

Strategies for optimizing intravenous prostacyclin-analog therapy in patients with pulmonary arterial hypertension.

INTRODUCTION: Intravenous prostacyclin-analogs (PCA, e.g. epoprostenol, treprostinil, iloprost) have become an essential part in the therapy of patients with pulmonary hypertension (PH), mainly pulmonary arterial hypertension (PAH). They show considerable differences in pharmacology. A combination therapy including intravenous drugs is regarded as the 'gold standard' in most of PAH patients. AREAS COVERED: This review discusses and summarizes the studies and concepts on which this therapy is based. To date, intravenous prostacyclin-analogs are mainly administered when standard therapy fails to improve patients to low-risk status. However, preliminary data from uncontrolled studies suggest that an 'upfront triple' therapy including intravenous or subcutaneous prostacyclin-analogs could be preferable in selected patients. EXPERT OPINION: Various IV PCA have been evaluated in the treatment of patients with PAH. Today, combination therapy is the 'gold standard' for the majority of patients. Intravenous PCA is recommended from functional class III onwards. Timing of its initiation is still a point of discussion. An escalation of therapy to IV or SC PCA is always necessary if a low-risk status cannot be achieved with other targeted therapies. Preliminary data suggest that selected patients could benefit from an 'upfront triple' therapy. Controlled studies on which such recommendation could be based are lacking.

Pulmonary thromboendarterectomy after treatment with treprostenil in a chronic thromboembolic pulmonary hypertension patient: a case report.

In recent years, there has been a major advance in the treatment of pulmonary hypertension. New medications are continually added to the therapeutic arsenal. The prostanoids are among the first agents used to treat pulmonary hypertension and are currently considered the most effective. This case study describes a 63-year-old man who was diagnosed with chronic thromboembolic pulmonary hypertension and successfully treated with subcutaneously administered treprostenil for 6 months before a successful pulmonary thromboendarterectomy. Treatment of chronic thromboembolic pulmonary hypertension often requires a multidisciplinary approach before surgery. Further evaluation of prostanoids is needed to define their role and time of initiation of medical therapy in these patients.