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.

Pulmonary%20arterial%20hypertension Patient Education

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 temperature 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
Digital Edition
Asia's trusted medical magazine for healthcare professionals. Get your MIMS Respirology - Malaysia digital copy today!
Sign In To Download
Editor's Recommendations
Most Read Articles
5 days ago
Routinely used for treating cardiovascular diseases, statins have been shown to benefit other conditions, and new evidence suggests that using the drug at high intensity reduces the risk of hip or knee replacement, an effect that may be specific to rheumatoid arthritis.
Jairia Dela Cruz, 5 days ago
Following vegan and vegetarian diets, which offer plenty of what is good for health, has been reported to have a downside: an increased risk of depression and anxiety, especially for younger adults.
Pearl Toh, 29 Jun 2020
Having migraine during midlife appears to be associated with a higher risk of developing dementia in later life, according to a large population-based longitudinal Danish study presented at the AHS* 2020 Virtual Meeting, indicating that migraine may be a risk factor for dementia.
Roshini Claire Anthony, 3 days ago

Upadacitinib may be a suitable treatment for patients with active psoriatic arthritis (PsA) who have insufficient response to non-biologic disease-modifying anti-rheumatic drugs (non-bDMARDs), according to results of the phase III SELECT-PsA-1* trial presented at EULAR 2020.