Pulmonary Arterial Hypertension

Abstract

Pulmonary arterial hypertension (PAH) is a rare and debilitating disease characterized by abnormal proliferation and contraction of pulmonary vascular smooth muscle cells. The resulting increase in pressure and pulmonary vascular resistance results in progressive right heart failure, low cardiac output, and ultimately death if left untreated. PAH is defined by a persistent elevation in pulmonary artery pressure with normal left-sided pressures, differentiating it from left-sided heart disease. Symptoms progress from shortness of breath and decreasing exercise tolerance to right heart failure, with peripheral edema and marked functional limitation. Exercise-induced syncope, worsening symptoms at rest, and intractable right heart failure indicate critical disease. PAH may be idiopathic with no identifiable cause or associated with collagen vascular diseases, drugs, HIV, liver disease, and/or congenital heart disease. Familial or genetically mediated PAH accounts for a small percentage of cases. Advances in the understanding of pathobiological pathways that contribute to vascular proliferation and remodeling have resulted in new therapies that improve quality of life and survival. Emerging therapies focus on the nitric oxide, prostacyclin, and endothelin pathways. Nursing interventions are critical to ensure patients' success with these expensive and complex treatments and their optimal adjustment to living with PAH.

Symptom Experience of Pulmonary Arterial Hypertension Patients

“…Diagnosis can be difficult as patients often present with vague symptoms and these are often attributed to stress, anxiety, obesity, and or asthma. Right heart catheterization is the definitive tool for diagnosis of PAH (Traiger, 2007), although an electrocardiogram (ECG) to assess for P pulmonale (P wave >.25 mV in lead II) is an indicator of increased right sided heart pressure and is readily obtainable (Henkens, Scherptong, van Kralingen, Said, & Vliegen, 2008). Transthoracic echocardiography is also a beneficial screening tool (Traiger, 2007).…”

Inspiratory Muscle Training in Pulmonary Arterial Hypertension

“…3 These hemodynamic findings impair exercise capacity in patients with PAH. Symptoms of PAH include syncope, dyspnea, and excessive fatigue and limit daily life and physical function.…”

Human Responses to Pulmonary Arterial Hypertension

“…Symptoms are sometimes vague and may be attributed to deconditioning, causing a delay of 2 to 3 years from symptom presentation to diagnosis, especially in women. 8 The echocardiogram is a screening tool, but diagnosis is confirmed by performing a right heart catheterization.…”

“…These pathways have been used as targets for the development of PAH treatments. 8 Endothelin is a potent vasoconstrictor that affects cell growth in vascular smooth muscle, leading to fibrosis and inflammation. Endothelin antagonists act to block these effects and vasodilate.…”

“…Epoprostenol has a very short half-life (3 minutes) and requires intravenous (IV) administration. 8 For those patients whose functional status does not improve or improves and declines again, lung transplantation is another treatment option. Generally, a double lung transplant is performed.…”