COMPARISON OF THE HEMODYNAMICS AND SURVIVAL OF ADULTS WITH SEVERE PRIMARY PULMONARY-HYPERTENSION OR EISENMENGER SYNDROME

Background: To date, lung or heart-lung transplantation remains the on ly definitive treatment for most adults with severe primary pulmonary hypertension or Eisenmenger syndrome. Although the hemodynamic derange ments and clinical history of adults with severe primary pulmonary hyp ertension have been well documented, those of adults with Eisenmenger syndrome have not. Methods: We evaluated hemodynamics and nontransplan tation survival of 100 adults with severe pulmonary hypertension (34 /- 9 years, 73 women and 27 men; 57 with primary pulmonary hypertensio n, 37 with Eisenmenger syndrome, and six with a previously repaired co ngenital heart defect) followed up by the lung transplant or adult con genital heart services at Washington University. Results: Despite a tr end 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/m(2), 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%) r eceived transplants during the follow-up interval; 13 (23%) patients w ith primary pulmonary hypertension died, and 31 (54%) received transpl ants over the same interval. Actuarial survival of patients who did no t 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%, re spectively, for patients with primary pulmonary hypertension. Data on hemodynamics from a small number of patients with a previously repaire d heart defect and severe pulmonary hypertension were similar to those from patients with primary pulmonary hypertension. Conclusions: Our d ata suggest that adults with Eisenmenger syndrome have a more favorabl e hemodynamic profile and prognosis than adults with primary pulmonary hypertension.