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
Which medications for PAH?
CCBs. Four options in this class have shown utility, notably in patients who have had a positive vasoreactivity test (see “How best to approach evaluation and diagnosis?”):
- Nifedipine is started at 10 mg tid; target dosage is 20 to 60 mg, bid or tid.
- Diltiazem is started at 60 mg bid; target dosage is 120 to 360 mg bid.
- Amlodipine is started at 5 mg/d; target dosage is 15 to 30 mg/d.
- Felodipine is started at 5 mg/d; target dosage is 15 to 30 mg/d.
Felodipine and amlodipine have longer half-lives than other CCBs and are well tolerated.
ERA. Used as vasodilators are ambrinsentan (starting dosage, 5 mg/d; target dosage, 10 mg/d), macitentan (starting and target dosage, 10 mg/d), and bosentan (starting dosage, 62.5 mg bid; target dosage, 125 mg bid).
Nitric oxide–cyclic guanosine monophosphate enhancers. These are the PDE5 inhibitors sildenafil (starting and target dosages, 20 mg tid) and tadalafil (starting dosage, 20 or 40 mg/d; target dosage, 40 mg/d), and the guanylate cyclase stimulant riociguat (starting dosage, 1 mg tid; target dosage, 2.5 mg tid). All 3 agents enhance production of the potent vasodilator nitric oxide, production of which is impaired in PH.
Prostanoids. Several options are available:
- Beraprost sodium. For this oral prostacyclin analogue, starting dosage is 20 μg tid; target dosage is the maximum tolerated dosage (as high as 40 μg tid).
- Extended-release beraprost. Starting dosage is 60 μg bid; target dosage is the maximum tolerated dosage (as high as 180 μg bid).
- Oral treprostinil. Starting dosage is 0.25 mg bid or 0.125 mg tid; target dosage is the maximum tolerated dosage.
- Inhaled iloprost. Starting dosage of this prostacyclin analogue is 2.5 μg, 6 to 9 times per day; target dosage is 5 μg, 6 to 9 times per day.
- Inhaled treprostinil. Starting dosage is 18 μg qid; target dosage is 54 to 72 μg qid.
- Eproprostenol is administered by continuous IV infusion, at a starting dosage of 2 ng/kg/min; target dosage is determined by tolerability and effectiveness (typically, 30 ng/kg/min).
- IV treprostinil. Starting dosage 1.25 ng/kg/min; target dosage is determined by tolerability and effectiveness, with a typical dosage of 60 ng/kg/min.
Combination treatment with the agents listed above is often utilized.
Selexipag. This oral selective nonprostainoid prostacyclin receptor agonist is started at 200 μg bid; target dosage is the maximum tolerated, as high as 1600 μg bid.
Continue to: Supportive therapy
