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 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