An Overview of Pulmonary Arterial Hypertension

Abstract

This article focuses on pulmonary arterial hypertension, including both primary pulmonary hypertension (PPH) and those forms of pulmonary arterial hypertension that are related to other factors, including collagen vascular diseases, congenital shunts, portal hypertension, human immunodeficiency viral infection, and exposure to specific drugs and toxins. Risks for different types of pulmonary arterial hypertension are identified. The common pathogenesis for pulmonary arterial hypertension is discussed, and includes an overview of the role of key vasoactive substances such as nitric oxide, prostacyclin, endothelin, and thromboxane. Typical presenting clinical manifestations, recommendations for screening of patients at risk, and key diagnostic findings are discussed. The mainstay of treatment is identified as pharmacologic, and may include diuretics, digoxin, warfarin, calcium channel antagonists, and prostacyclin analogues such as epoprostenol. Surgical interventions are considered as a last resort, and may include unilateral or bilateral lung transplant or atrial septostomy. Treatment options for patients with pulmonary arterial hypertension hold more hope today than they did a decade ago and are identified so as to guide the advanced practice nurse in recognizing and then facilitating the appropriate management of patients with this rare but disabling disease.

1H NMR-Based Analysis of Serum Metabolites in Monocrotaline-Induced Pulmonary Arterial Hypertensive Rats

“…Pulmonary arterial hypertension (PAH), a chronic and fatal disease with a poor prognosis, is characterized by elevated pulmonary arterial pressure which could lead to right side heart failure [ 1 – 3 ]. The pathophysiological changes of PAH, including migration and proliferation of pulmonary arterial smooth muscle cell (PASMC), adventitial fibroblast (PAAF), and leukocytes infiltration as well as release of inflammatory cytokines, could result in pulmonary endothelial dysfunction and vascular remodeling in the small pulmonary arteries [ 2 , 4 ]. However, the cause and mechanism of these proliferations and antiapoptosis in PAH is unknown.…”

Perceptions of Received Information, Social Support, and Coping in Patients with Pulmonary Arterial Hypertension or Chronic Thromboembolic Pulmonary Hypertension

“… 9 In the initial stages of PAH and CTEPH, symptoms are often vague and unspecific but patients commonly present with unexplained dyspnea, fatigue, and weakness. 4 , 10 Accordingly, many patients see several medical specialists before being diagnosed with PAH. 11 – 13 A key reason for this is the very low prevalence of PAH and CTEPH and the lack of awareness and knowledge about PAH and CTEPH amongst healthcare professionals.…”

Low Complication Rates With Totally Implantable Access Port Use in Epoprostenol Treatment of Pulmonary Hypertension

“…Unexpected interruption of continuous intravenous administration of epoprostenol can be life-threatening. [1][2][3][4] In our series, the TIAP showed a long lifespan and a low device-associated mortality rate.…”

“…[1][2][3][4] Without treatment, the median survival of patients with PAH is 2.8 years. 4 Continuous intravenous administration of epoprostenol, a prostacyclin analog, is an effective therapy for selected patients with this disorder.…”

“…In general, percutaneous tunneled catheters, such as the Hickman or Broviac catheter, are used for continuous administration of epoprostenol. [1][2][3][4][5][6][7][8][9][10][11][12][13][14] Complications related to the use of indwelling catheters represent a substantial risk for these patients. This can result in a significant decline in catheter life and extra hospital admissions, affecting the patient's quality of life.…”