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BACKGROUND: We evaluated long-term prognosis according to improvement of pulmonary hypertension (PH) and left ventricular ejection fraction (LVEF) in patients with heart failure with reduced ejection fraction (HFrEF) and PH. METHODS: We included all consecutive patients with HFrEF and PH who had a baseline and follow-up echocardiographic examinations from September 2011 to March 2017. PH was defined as maximal velocity of tricuspid regurgitation (TR Vmax) over 3.0 m/s, and LVEF improvement was defined as LVEF change ≥ 15% from the baseline echocardiography. Primary outcome was 5-year major adverse cardio-cerebrovascular events (MACCE). RESULTS: We analyzed 271 patients. Mean LVEF was 28±8% and TR Vmax was 3.4±0.4 m/s. On follow-up, 183 (68%) showed improvement of LVEF, and 165 (61%) demonstrated improvement of PH. We classified patients into 4 groups according to improvement of PH and LVEF; group 1 (both improvement, 134 patients), group 2 (PH improvement only, 31 patients), group 3 (LVEF improvement only, 49 patients) and group 4 (no improvement, 57 patients). Group 4 had older age, higher incidence of myocardial infarction and aggravation of pre-existing HF. During the follow-up (31±20 months), 27% died and 40.8% experienced MACCE. Group 4 had the worst survival (HR=4.332, 95% CI=2.396-7.833, p<0.001), and group 3 had increased MACCE rate (HR=2.030, 95% CI=1.060-3.888, p=0.033) compared with group 1. Group 2 had similar long-term clinical events (HR=1.085, 95% CI=0.458-2.571, p=0.853) to group 1. CONCLUSIONS: In patients with HFrEF and PH, persistence of PH and no LVEF improvement was associated with the worst long-term outcome.