pulmonary%20arterial%20hypertension
PULMONARY ARTERIAL HYPERTENSION
Pulmonary arterial hypertension is a syndrome resulting from restricted flow through the pulmonary arterial circulation resulting in increased pulmonary vascular resistance and ultimately leading to right heart failure.
It is a part of the spectrum of pulmonary hypertension, which is hemodynamic and pathophysiological condition defined as an increase in mean pulmonary arterial pressure ≥25 mmHg at rest.
Typical symptoms include progressive dyspnea on exertion, palpitations, fatigue, weakness, angina, syncope and abdominal distention.

Patient Education

Genetic Testing/Counseling

  • Should be offered to relatives of patients with familial pulmonary arterial hypertension (PAH), if available
  • Idiopathic pulmonary arterial hypertension (IPAH) patients should be advised regarding the availability of genetic testing & counseling for their relatives

Physical Activity

  • Physical activity to maintain adequate skeletal muscle conditioning should be limited to a symptom-free level
  • Low-level aerobic exercise (eg walking) as tolerated, is recommended
  • Heavy physical exertion or isometric exercise, exercise after meals or in extreme temp should be avoided

Travel/Altitude

  • Exposure to high altitudes may contribute to hypoxic pulmonary vasoconstriction & may not be well tolerated
  • Avoid mild degrees of hypobaric hypoxia that starts at altitude between 1500-2000 meters
  • Supplemental O2 is needed in commercial airplanes when pre-flight pulse oximetry saturation is <92%

Pregnancy

  • Pregnancy, labor, delivery & postpartum period are potentially devastating with 30-50% mortality in patients with PAH
    • Appropriate method of birth control may be used by women of childbearing potential
    • Barrier contraceptive methods & progesterone-only preparations are safe for PAH patients 
  • Pregnancy should be avoided or terminated in women with cyanotic congenital heart disease, PH & Eisenmenger syndrome
  • During menopause, hormonal therapy may be considered for intolerable menopausal symptoms in conjunction with oral anticoagulation

Psychosocial Support

  • If needed, refer patient to psychiatrist/psychologist to assist in dealing with anxiety &/or depression about their disease state
  • If available, patients & families should be referred to support groups as these are useful in improving the understanding & acceptance of the disease condition
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Stephen Padilla, 21 Nov 2018
Use of triple therapy consisting of a long acting muscarinic antagonist (LAMA), long acting β agonist (LABA) and inhaled corticosteroid (ICS) leads to a reduced rate of moderate or severe exacerbations of chronic obstructive pulmonary disease (COPD) and better lung function and health-related quality of life compared with dual therapy (ICS and LABA or LAMA and LABA) or LAMA monotherapy, according to the results of a meta-analysis.