medwireNews: An updated CHEST guideline for treating pulmonary arterial hypertension (PAH) provides some evidence-based recommendations but also highlights many evidence gaps.
The guideline authors – Joe Ornelas (American College of Chest Physicians, Glenview, Illinois, USA) and colleagues – note that there is little evidence on which to base management recommendations. Of 79 recommendations made, just nine are evidence-based, with the rest based on expert consensus.
The team also stresses that the recommendations only apply to patients with PAH, and not to those with other types of pulmonary hypertension.
The guideline, which is published in Chest, is divided into recommendations based on patients’ World Health Organization (WHO) functional class, although the authors note the limitations of this classification. Indeed, one of their recommendations, for all patients, is that exercise capacity, echocardiographic, laboratory and haemodynamic variables should also be accounted for when making treatment decisions.
For patients at risk of PAH or with untreated WHO stage I PAH, the authors advise monitoring and treatment of conditions that can contribute to pulmonary hypertension, such as sleep apnoea and systemic hypertension.
Graded evidence-based recommendations include ambrisentan or sildenafil to improve 6-minute walk distance (6MWD) in patients with stage II PAH, and ambrisentan, sildenafil or bosentan to improve this outcome in class III patients. For class III patients, bosentan is also suggested to reduce hospitalisations.
Another recommendation is for inhaled treprostinil to improve 6 MWD in patients who remain symptomatic on stable doses of an endothelin receptor antagonist (ETRA) or a phosphodiesterase type 5 (PDE5) inhibitor. The final two evidence-based recommendations are for bosentan to improve 6 MWD in patients who cannot or do not wish to take parenteral prostanoid therapy and inhaled treprostinil to improve 6MWD in patients with stage III or IV disease who remain symptomatic on an ETRA or a PDE5 inhibitor.
Consensus-based advice includes intravenous epoprostenol for patients with WHO stage III disease who have evidence of progression or a poor prognosis, and for treatment-naïve patients with WHO stage IV PAH, for whom simultaneous bosentan is also recommended. The addition of a second and third class of PAH therapy is advised for stage IV patients who continue to deteriorate.
The guidelines also offer consensus-based advice for specific situations, namely pregnancy, altitude and air travel, vaccinations and surgery.
“PAH remains an incurable disease; currently clinicians attempt to manage it with pharmacotherapy”, say the guideline authors. “While we believe the outlook for a newly diagnosed patient with PAH has improved, it remains far from adequate or acceptable.”
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By Eleanor McDermid, Senior medwireNews Reporter