Background: To date, lung or heart-lung transplantation remains the only definitive treatment for most adults with severe primary pulmonary hypertension or Eisenmenger syndrome. Although the hemodynamic derangements and clinical history of adults with severe primary pulmonary hypertension have been well documented, those of adults with Eisenmenger syndrome have not.
Methods: We evaluated hemodynamics and nontransplantation survival of 100 adults with severe pulmonary hypertension (34 +/- 9 years, 73 women and 27 men; 57 with primary pulmonary hypertension, 37 with Eisenmenger syndrome, and six with a previously repaired congenital heart defect) followed up by the lung transplant or adult congenital heart services at Washington University.
Results: Despite a trend toward greater pulmonary artery pressures (107 +/- 20 versus 97 +/- 21 mm Hg, p = 0.06), patients with Eisenmenger syndrome had greater systemic cardiac indexes (2.7 +/- 0.6 versus 2.2 +/- 0.8 L/min/m2, p < 0.05) and lower mean right atrial pressures (5 +/- 2 versus 12 +/- 5 mm Hg, p < 0.0001) than patients with primary pulmonary hypertension. Four (11%) patients with Eisenmenger syndrome died and eight (22%) received transplants during the follow-up interval; 13 (23%) patients with primary pulmonary hypertension died, and 31 (54%) received transplants over the same interval. Actuarial survival of patients who did not receive transplants was 97% at 1 year, 89% at 2 years, and 77% at 3 years for patients with Eisenmenger syndrome and 77%, 69%, and 35%, respectively, for patients with primary pulmonary hypertension. Data on hemodynamics from a small number of patients with a previously repaired heart defect and severe pulmonary hypertension were similar to those from patients with primary pulmonary hypertension.
Conclusions: Our data suggest that adults with Eisenmenger syndrome have a more favorable hemodynamic profile and prognosis than adults with primary pulmonary hypertension.