An Update on Pulmonary Arterial Hypertension (original) (raw)
Related papers
2014
Abstract: Pulmonary arterial hypertension (PAH) is a rapidly progressive pulmonary vascular disease with a multifactorial etiopathogenesis that can result in right-sided heart failure and death. Read this review and sign up to receive Therapeutics and Clinical Risk Management journal here: http://www.dovepress.com/articles.php?article\_id=18674
Assessment of Structural and Functional Pulmonary Vascular Disease in Patients with PAH
Pulmonary Hypertension - From Bench Research to Clinical Challenges, 2011
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The Journal for Nurse Practitioners, 2007
Pulmonary arterial hypertension (PAH) is a rare, life-threatening, progressive disease that is characterized by sustained elevations of pulmonary artery pressure with no apparent cause. Because it is often difficult to diagnose, the true incidence of PAH is unknown. The true number of cases has eluded researchers because accurate diagnosing of PAH is complex. The prevalence of PAH is estimated at 1 to 2 million people worldwide, with 500 to 1000 new cases diagnosed in the United States each year. It is important for nurse practitioners who work in both primary and acute care to be familiar with this devastating disease and to consider this condition as a differential diagnosis when caring for patients with dyspnea.
Pulmonary Arterial Hypertension: Evaluation and Management
2007
Pulmonary arterial hypertension (PAH), a rare disease involving the pulmonary vascular circuit, is defined as an elevation in pulmonary arterial pressures and is characterized by symptoms of dyspnea, chest pain, and syncope. If left untreated, the disease carries a high mortality rate, with the most common cause of death being decompensated right heart failure. Over the past 5 years, there have been significant advances in this field in regards to understanding the pathogenesis, diagnosis, and classification of PAH. The availability of newer drugs has resulted in a radical change in the management of this disease with significant improvement in both quality of life and mortality. Ongoing research promises to lead to a more comprehensive understanding of the genetics, etiology, and pathogenesis of pulmonary arterial hypertension, which may ultimately translate into more effective therapeutic options.
Annals of Thoracic Medicine
AIMS: The purpose of this study is to present our center's experience in managing patients with pulmonary arterial hypertension (PAH). The main objective is to describe patients' management profile and treatment outcome. METHODS: This study presents the results from a single pulmonary hypertension (PH) specialized center in Saudi Arabia. Both incidence and prevalence cases are included. We have previously reported the clinical and physiological characteristics at the time of diagnosis for this cohort of patients. In this study, we describe the clinical management and the outcome of therapy in the same cohort, who were prospectively followed for a mean of 22 months. RESULTS: A total of 107 patients were identified as having PAH. At the time of enrollment, 56.1% of patients were in modified New York Heart Association functional class (NYHA FC) III and 16.8% were in IV. Phosphdiesterase-5 inhibitor was the most commonly used target therapy (82.2%) followed by endothelin receptors antagonist (74.4%). Only five patients (4.7%) were candidate to use calcium channel blockers. Seventy-nine patients (73.8 %) received a combination nonparenteral target therapy. Thirty-one patients (28.9%) died during the follow-up period. Modified NYHA FC III and IV patients, portopulmonary hypertension, heritable PAH, and PAH associated with connective tissue diseases had the highest mortality rate (P < 0.001). CONCLUSION: Our patients are detected at advanced stage of the disease, and thus the mortality is still unacceptably high. Advanced functional class at presentation and certain disease subgroups are associated with increased mortality.
Pulmonary arterial hypertension: an update
Netherlands Heart Journal, 2011
Pulmonary arterial hypertension (PAH), defined as group 1 of the World Heart Organisation (WHO) classification of pulmonary hypertension, is an uncommon disorder of the pulmonary vascular system. It is characterised by an increased pulmonary artery pressure, increased pulmonary vascular resistance and specific histological changes. It is a progressive disease finally resulting in right heart failure and premature death. Typical symptoms are dyspnoea at exercise, chest pain and syncope; furthermore clinical signs of right heart failure develop with disease progression. Echocardiography is the key investigation when pulmonary hypertension is suspected, but a reliable diagnosis of PAH and associated conditions requires an intense work-up including invasive measurement by right heart catheterisation. Treatment includes general measures and drugs targeting the pulmonary artery tone and vascular remodelling. This advanced medical therapy has significantly improved morbidity and mortality in patients with PAH in the last decade. Combinations of these drugs are indicated when treatment goals of disease stabilisation are not met. In patients refractory to medical therapy lung transplantation should be considered an option.
Pathogenesis of Pulmonary Arterial Hypertension: The Need for Multiple Hits
Circulation, 2005
ulmonary hypertension can be classified into 4 categories: pulmonary arterial hypertension (PAH), pulmonary venous hypertension, pulmonary hypertension associated with hypoxemia, and pulmonary hypertension due to chronic thrombotic or embolic disease. PAH is a progressive and often fatal condition that predominantly affects women. Approximately 10% of patients diagnosed with PAH without a demonstrable cause have a family history of the disease and are referred to as having familial PAH (FPAH), whereas the remainder are classified as having idiopathic PAH (IPAH).