scholarly journals Novel Treatment Pathways in Pulmonary Arterial Hypertension

2021 ◽  
Vol 17 (2) ◽  
pp. 106-114
Author(s):  
Kanza N Qaiser ◽  
Adriano R Tonelli
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Disease Process ◽  
Ongoing Research ◽  
Treatment Pathways ◽  
Pulmonary Vascular Remodeling ◽  
Pulmonary Arterial ◽  
Long Term Prognosis ◽  
New Treatments

Pulmonary arterial hypertension (PAH) is a severe and progressive vascular disease characterized by pulmonary vascular remodeling, proliferation, and inflammation. Despite the availability of effective treatments, PAH may culminate in right ventricular failure and death. Currently approved medications act through three well-characterized pathways: the nitric oxide, endothelin, and prostacyclin pathways. Ongoing research efforts continue to expand our understanding of the molecular pathogenesis of this complex and multifactorial disease. Based on recent discoveries in the pathobiology of PAH, several new treatments are being developed and tested with the goal of modifying the disease process and ultimately improving the long-term prognosis.

TAPSE/sPAP predicts the long-term prognosis in pulmonary arterial hypertension patients

2021 ◽  
Author(s):  
Begum Uygur ◽  
Mustafa Yildiz ◽  
Ali Riza Demir ◽  
Serkan Kahraman ◽  
Kadriye Memic Sancar ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Pulmonary Arterial ◽  
Long Term Prognosis

scholarly journals Physicians’ and patients’ expectations of therapies for pulmonary arterial hypertension: where do they meet?

2014 ◽  
Vol 23 (134) ◽  
pp. 458-468 ◽  
Author(s):  
Luke S. Howard ◽  
Pisana Ferrari ◽  
Sanjay Mehta
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Emotional Support ◽  
Multidisciplinary Approach ◽  
Slow Disease Progression ◽  
Social And Emotional ◽  
Pulmonary Arterial ◽  
Long Term Prognosis

In recent years, many new, effective therapies for pulmonary arterial hypertension (PAH) have become available and are widely used, yet the long-term prognosis for patients with PAH remains poor. In the absence of a cure, physicians’ expectations of PAH-specific therapies are to: 1) improve patients’ symptoms and functional capacity; 2) slow disease progression; and 3) improve survival. However, patients with PAH may prioritise other more tangible needs, such as improvements in their ability to carry out their daily tasks and increase their quality of life. Patients with PAH have also called out for social and emotional support from their physicians, caregivers, families and patient associations. Therefore, it is necessary that clinical trials of PAH-specific treatments include end-points that are meaningful to both patients and physicians, and that a multidisciplinary approach to the management of patients with PAH takes into consideration the broader aspects of patients’ and caregivers’ needs and wishes beyond simple physiological measurements.

scholarly journals The clinical characteristics and long-term prognosis of pulmonary arterial hypertension associated with hereditary hemorrhagic telangiectasia

2018 ◽  
Vol 8 (2) ◽  
pp. 204589401875991 ◽  
Author(s):  
Wen Li ◽  
Chang-ming Xiong ◽  
Qing Gu ◽  
Xiao-tong Wang ◽  
Xiao-ling Cheng ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Cardiac Index ◽  
Arterial Hypertension ◽  
Hereditary Hemorrhagic Telangiectasia ◽  
Clinical Characteristics ◽  
Total Bilirubin ◽  
World Health ◽  
Pulmonary Arterial ◽  
Long Term Prognosis

Pulmonary arterial hypertension (PAH) is a severe complication of hereditary hemorrhagic telangiectasia (HHT); however, little is known about its clinical characteristics and prognosis. Nine newly diagnosed HHT-PAH patients were prospectively recruited between October 2007 and January 2016 and were followed up every half-year. Eighteen idiopathic pulmonary arterial hypertension (IPAH) patients, matched with HHT-PAH patients on mean pulmonary arterial pressure, pulmonary capillary wedge pressure, pulmonary vascular resistance, cardiac index, and World Health Organization (WHO) functional class (FC), were recruited. The clinical characteristics of HHT-PAH patients were described and the prognosis of these two cohorts were compared. Of HHT-PAH patients, 55.56% were WHO FC III. Kaplan–Meier survival analysis showed one- and three-year survival rates of HHT-PAH patients were 77.8% and 53.3% respectively, which were worse than matched IPAH patients (log rank: P = 0.047). HHT-PAH patients had higher red cell distribution width (14.88 ± 2.93% versus 13.19 ± 0.83%, P = 0.031), larger right ventricular anteroposterior diameter (34.67 ± 6.67 mm versus 28.56 ± 6.35 mm, P = 0.029), and lower mean corpuscular hemoglobin concentration (317.38 ± 17.71 g/L versus 335.72 ± 14.68 g/L, P = 0.011) than matched IPAH patients. Multivariate Cox proportional hazards regression analyses showed baseline total bilirubin independently predicted the mortality of HHT-PAH after adjusting by age, cardiac index, mixed venous oxygen saturation, or serum uric acid. HHT-PAH patients may have a worse prognosis than matched IPAH patients. Baseline total bilirubin may be a promising predictor for the long-term prognosis in HHT-PAH patients.

scholarly journals Advances in the management of pulmonary arterial hypertension

2021 ◽  
Vol 69 (7) ◽  
pp. 1270-1280
Author(s):  
Himanshu Deshwal ◽  
Tatiana Weinstein ◽  
Roxana Sulica
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Survival Rates ◽  
Patient Centered ◽  
Term Survival ◽  
Pulmonary Vascular Remodeling ◽  
Centered Care ◽  
Genetic Level ◽  
Pulmonary Arterial

The management of pulmonary arterial hypertension (PAH) has significantly evolved over the last decades in the wake of more sensitive diagnostics and specialized clinical programs that can provide focused medical care. In the current era of PAH care, 1-year survival rates have increased to 86%–90% from 65% in the 1980s, and average long-term survival has increased to 6 years from 2.8 years. The heterogeneity in the etiology and disease course has opened doors to focusing research in phenotyping the disease and understanding the pathophysiology at a cellular and genetic level. This may eventually lead to precision medicine and the development of medications that may prevent or reverse pulmonary vascular remodeling. With more insight, clinical trial designs and primary end-points may change to identify the true survival benefit of pharmacotherapy. Identifying responders from non-responders to therapy may help provide individualized patient-centered care rather than an algorithm-based approach. The purpose of this review is to highlight the latest advances in screening, diagnosis, and management of PAH.

Sign in / Sign up

Export Citation Format

Share Document