The need for oxygen should be addressed in patients with hypoxia in any setting—resting, exercise induced, and nocturnal.24 Patients with an arterial blood oxygen pressure < 60 mm Hg (SaO2 < 90 mm Hg) should be on long-term oxygen therapy.6
Common physical findings in pulmonary hypertension include an increased P2 heart sound, high-pitched holosystolic murmur from tricuspid regurgitation, and pulmonic insufficiency murmur.
Diuretics are beneficial in patients with chronic fluid retention from PH that is related to right ventricular failure.24
Pulmonary rehabilitation and exercise. Contrary to common belief that exercise training is contraindicated in patients with PH, exercise training has emerged in the past decade as an effective tool to improve exercise capacity, ventilatory efficiency, and quality of life. While a patient is training, oxygen saturation, measured by pulse oximetry, should be maintained at > 90% throughout the exercise session to avoid hypoxic pulmonary artery vasoconstriction.25
A patient who does not qualify for pulmonary or cardiac rehabilitation should be referred for physical therapy.24
Ongoing follow-up in primary care
Instruct patients not to abruptly discontinue medications that have been prescribed for PH. Ongoing follow-up and monitoring involves assessing right heart function, exercise tolerance, and resting and ambulatory oximetry. Testing for the level of BNP provides prognostic information and allows assessment of treatment response.15 The frequency of 6MWT, echocardiography, and RHC is decided on a case-by-case basis.
Other considerations
Pregnancy. PAH often affects patients of childbearing age. Because PAH-associated maternal mortality and the risk to the fetus during pregnancy are high, pregnancy is not recommended for patients with PAH. After a diagnosis of PAH in a patient of childbearing age, counseling should be offered at an expert center. Advice on effective contraception methods should be given early on.10,26-29
Surgery. Every patient with clinically significant PH is at increased risk of perioperative morbidity and death.30,31 Guidelines recommend that these patients avoid nonessential surgery; if surgery is necessary, care should be provided at a PH expert center.10
