Pulmonary hypertension: An update of Dx and Tx guidelines
Here is how to reduce risk factors that can lead to pulmonary hypertension; play a pivotal role in diagnosis; and know when disease requires a referral.
PRACTICE RECOMMENDATIONS
› Employ echocardiography as the first-line diagnostic test when pulmonary hypertension (PH) is suspected. C
› Order a ventilation– perfusion scan in patients with unexplained PH to exclude chronic thromboembolic PH. C
› Order lung function testing with diffusion capacity for carbon monoxide as part of the initial evaluation of PH. C
› Use right heart catheterization to confirm the diagnosis of pulmonary arterial hypertension. C
Strength of recommendation (SOR)
A Good-quality patient-oriented evidence
B Inconsistent or limited-quality patient-oriented evidence
C Consensus, usual practice, opinion, disease-oriented evidence, case series
Supportive therapy
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
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
Continue to: Patients with severe PH...
