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Aim: to assess the prognostic impact of pulmonary hypertension at echo-Doppler evaluation in a population of cardiac amyloidosis. Methods and results: we retrospectively evaluated a series of 50 patients (66% males, mean age 64±12 years, NYHA III-IV 44%, left ventricular (LV) systolic dysfunction 18%, significant mitral regurgitation 10%, significant tricuspid regurgitation (TR) 16%) with a bioptic diagnosis of systemic amyloidosis and cardiac involvement documented at echocardiography, consecutively observed in our Department from 1991 to 2009. All patients performed a complete clinical and echo-Doppler evaluation. Presence of pulmonary hypertension (PH) was assessed as usually by simplified Bernoulli formula applied to continuous Doppler curve of TR. Our population was divided in 2 groups: group 1, patients with PH (pulmonary artery systolic pressure (sPAP) ≥ 34 mmHg (19 patients/38%) and group 2, patients without PH (sPAP < 34 mmHg: 31 patients/62%). No differences about severity of heart failure (NYHA III-IV), degree of LV wall thickening and frequency of diastolic and systolic LV dysfunction were observed between the two groups. Patients with PH showed more frequently significant mitral (21% vs 3%, p=0.04) and tricuspid (35% vs 4%, p= 0.006) regurgitation and right ventricular dysfunction (53% vs 16%, p=0.006). During a mean follow-up of 26±31 months 33 patients (66%) died; survival rates were 16% vs 45% in group 1 and in group 2, respectively (p=0.036). Conclusions: PH detected at echo-Doppler evaluation at diagnosis is relatively frequent in cardiac amyloidosis. Its presence is apparently unrelated to systolic and diastolic LV dysfunction and identifies a subgroup of patients with a particularly poor prognosis.