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REVEAL registry: PAH exacerbated by presyncope, syncope

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ISHLT 31st Annual Meeting and Scientific Sessions

SAN DIEGO – For patients with pulmonary arterial hypertension, a history of presyncope and syncope worsened the severity of disease, according to data from the REVEAL registry.

What this indicates, according to researchers, is the importance of validating the prognosis and effect of syncope among patients with pulmonary arterial hypertension (PAH) as treatment guidelines continue to be defined.

The study featured patients (n=70) who had both PAH and a history of either presyncope or syncope at initial prediagnosis of symptoms (IS) and first PAH-clinic visit from the REVEAL registry, which was a 55-center, observational, US-based longitudinal registry. The study’s objective was to both define the significance of presyncope and syncope in newly diagnosed patients with PAH and determine whether it is linked with clinical and or hemodynamic parameters.

Inclusion criteria for the study was that all patients with PAH must be characterized by having each of the following: mean pulmonary artery pressure <25 mm Hg at rest or <30 mm Hg with exercise; pulmonary capillary wedge pressure or left ventricular end-diastolic pressure no greater than 18 mm Hg; and pulmonary vascular resistance of at least 240 dyn/s/cm^-5. Patients were then followed for at least 5 years.

The researchers reported that patients with PAH and a history of presyncope and syncope were younger; had higher mean pulmonary artery pressure and right atrial pressure; had more functional class IV disease; had more right ventricular chamber enlargement; and had higher percent predicted diffusing capacity of carbon dioxide. They also found that 3-year survival was worse for the IS group despite these patients being characterized as younger and having a better capacity for exercise.

“Presyncope/syncope is associated with markers of increased disease severity in newly diagnosed PAH patients. However, it was not predictive of unadjusted survival,” said Rachel Le, MD, with the Mayo Clinic, Rochester, Minn., and investigator on the study, in her presentation.

As directions for future research, Le and colleagues suggested determination of the prognosis of presyncope or syncope as evidence-based PAH treatment guidelines are established; separating out the differences between presyncope or syncope; accessing whether treatment will affect prognosis in these patients; the effect of resolution of these symptoms with therapy; and differentiating pediatric vs. adult patients. – by Brian Ellis

For more information:

• Le R. Abstract #20. Presented at: ISHLT 31st Annual Meeting and Scientific Sessions; April 13-16, 2011; San Diego.

To me, the interesting aspect of this data was validation of something that has been repeatedly mentioned by astute clinicians of yesteryear: the relationship of presyncope and syncope to severity of PAH. It was an elegant analysis of an important registry and raises the question of a pathophysiologic link of syncope/presyncope to worsening PAH and not just something that is a consequence of PAH.

– James Young, MD
Cardiology Today Section Editor

Disclosure: Drs. Le and Young reported no relevant financial disclosures.

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