Pulmonary%20arterial%20hypertension 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 and 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 and 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 and 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 and progesterone-only preparations are safe for PAH patients 

• Pregnancy should be avoided or terminated in women with cyanotic congenital heart disease, PH and 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 and/or depression about their disease state
• If available, patients and families should be referred to support groups as these are useful in improving the understanding and acceptance of the disease condition