Advanced pulmonary vasodilators linked to greater survival in Eisenmenger syndrome
Treatment with advanced pulmonary vasodilator therapy (AT) appears to confer survival benefits in patients with Eisenmenger syndrome (ES) whose illness severity may be greater prior to treatment, according to a study.
Researchers followed 253 adult ES patients (mean age 31 years; 60 percent female) from 12 adult congenital heart disease centres across Australia and New Zealand. Demographic, medical and outcome data were collected and analysed prospectively and retrospectively.
At diagnosis of ES, 64 percent had World Health Organization (WHO) functional class ≥3. Ventricular septal defect was the most common underlying lesion, identified in 33 percent of patients, including 21 percent with “complex” anatomy.
During a median follow-up of 9.1 years, majority of patients (72 percent) were prescribed at least one AT (49 percent single agent), with bosentan mostly (66 percent; n=168). The mean time on AT was 6 years.
Compared with nonexposed patients, those with AT exposure were more functionally impaired at presentation (WHO ≥3, 69 vs 51 percent; p=0.007) and more likely to have been prescribed anticoagulation (47 vs 27 percent; p=0.003). The risk of death/transplant per year was 4.8 percent in the AT-exposed group vs 8.4 percent in the never-exposed group.
Multivariable Cox proportional hazards analysis found AT exposure to be independently associated with greater survival (hazard ratio [HR] for survival, 2.27; 95 percent CI, 1.49 to 3.45; p<0.001), whereas WHO ≥3 at presentation was associated with a worse prognosis (HR for mortality, 1.82; 1.19 to 2.78; p=0.006).
“ES occurs in patients with a structurally abnormal heart where intracardiac or extracardiac systemic to pulmonary communications result in severe pulmonary vascular disease thence right to left shunting, leading to chronic cyanosis,” researchers said.
“The finding of greater long-term survival in those treated with AT as compared with those untreated may be instructive for both therapeutic algorithms in [patients] with ES and in the design of future randomized trials to confirm this association,” they added.