Unexplained pulmonary hypertension in older patients: Clinical characteristics, follow-up, and prognosis

The clinical characteristics, follow-up and prognosis of unexplained pulmonary hypertension (UPHT) in 8 older patients, age at diagnosis 62.5 ± 11.6 years (Mean ± 1 SD, range 51 to 82), were evaluated. Seven of the patients were women. Systolic pulmonary artery pressure (PAP) at diagnosis was 87 ± 10 mm Hg (Mean ± 1 SD, range 70 to 100). A majority of the patients (7/8) had advanced disease at diagnosis (New York Heart Association classes 3 and 4) with right heart failure (RHF) as the presenting symptom in 6 of the 8 patients (75%). Following treatment with calcium channel blockers, diuretics, oxygen and anticoagulants, functional capacity and symptoms of RHF, but not PAP, improved in 4 patients (50%). These patients are alive 37 ± 11 months (Mean ± 1 SD, range 23 to 53) after diagnosis. Four patients died after a mean follow-up of 6.8 ± 7.6 months (Mean ± 1 SD, range 2 to 18). One of these patients died suddenly and another 3 died with intractable RHF. Our study shows that most newly diagnosed older patients with UPHT in our institute were women. RHF at diagnosis, a sign that indicates a poor prognosis in younger patients with UPHT, is common in older patients with UPHT and was not associated with a particularly poor prognosis. Clinical response to medical treatment, rather than PAP changes, was associated with improved outcome.