Background: A high prevalence of asplenia has been observed in patients with unexplained pulmonary hypertension.
Objective: To describe the clinical and histopathologic characteristics of patients with postsplenectomy pulmonary hypertension and to compare the prevalence of surgical asplenia in patients with idiopathic pulmonary hypertension and patients with other pulmonary diseases.
Design: Case series and case–control study.
Setting: University hospital in Hannover, Germany.
Patients: 61 patients with pulmonary hypertension and 151 lung transplant recipients.
Results: The prevalence of asplenia in patients with pulmonary hypertension was 11.5% (95% CI, 4.7% to 22.2%) compared with 0% (CI, 0% to 3.2%) in those without pulmonary hypertension (P < 0.001). Histopathologic examination of lung specimens from patients with postsplenectomy pulmonary hypertension showed intimal fibrosis, plexiform lesions, and abundant thrombotic lesions.
Conclusion: Patients who have had splenectomy may be at increased risk for developing pulmonary hypertension.