[Show abstract][Hide abstract] ABSTRACT: We sought to determine the factors associated with long-term survival in patients with primary pulmonary hypertension (PPH) treated with continuous epoprostenol infusion.
Epoprostenol improves survival in patients with PPH in New York Heart Association (NYHA) functional class III or IV. However, some patients do not benefit from epoprostenol and must be considered for lung transplantation. The best timing for listing these patients on a lung transplantation program is currently unknown.
Between December 1992 and January 2001, 178 patients with PPH in NYHA functional class III or IV were treated with epoprostenol. The 6-min walk test (WT) and right-sided heart catheterization were performed at baseline, after three months on epoprostenol and thereafter once a year.
Overall survival rates at one, two, three, and five years were 85%, 70%, 63%, and 55%, respectively. On univariate analysis, the baseline variables associated with a poor outcome were a history of right-sided heart failure, NYHA functional class IV, 6-min WT <or=250 m (median value), right atrial pressure >or=12 mm Hg, and mean pulmonary artery pressure <65 mm Hg. On multivariate analysis, including both baseline variables and those measured after three months on epoprostenol, a history of right-sided heart failure, persistence of NYHA functional class III or IV at three months, and the absence of a fall in total pulmonary resistance of >30%, relative to baseline, were associated with poor survival.
Survival of patients with PPH treated with epoprostenol depends on the severity at baseline, as well as the three-month response to therapy. These findings suggest that lung transplantation should be considered in a subset of patients who remain in NYHA functional class III or IV or in those who cannot achieve a significant hemodynamic improvement after three months of epoprostenol therapy, or both.
Journal of the American College of Cardiology 08/2002; 40(4):780-8. DOI:10.1016/S0735-1097(02)02012-0 · 15.34 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: In patients with severe pulmonary hypertension (PH), right ventricular function is a main determinant of clinical stability and outcome. Supraventricular tachyarrhythmias (SVTs) may compromise cardiac function and threaten prognosis in patients with PH, but the incidence and clinical relevance of SVTs in PH and chronic right ventricular failure have not been evaluated.
In a 6-year retrospective single-center analysis, 231 consecutive patients followed for pulmonary arterial hypertension, or inoperable chronic thromboembolic PH were studied for SVTs. Analysis included incidence, clinical consequences, treatment, and outcome.
Thirty-one episodes of SVT were observed in 27 of 231 patients (cumulative incidence 11.7%, annual risk 2.8% per patient), including atrial flutter (n = 15), atrial fibrillation (n = 13), and AV nodal reentry tachycardia (n = 3). Supraventricular tachyarrhythmia onset was almost invariably associated with marked clinical deterioration and right ventricular failure (84% of SVT episodes). Outcome was strongly associated with the type of SVT and restoration of sinus rhythm. During follow-up, cumulative mortality was low (6.3%, follow-up 26 +/- 23 months) when sinus rhythm was restored (all cases of AV nodal reentry tachycardia and atrial flutter). In contrast, 9 of 11 patients with sustained atrial fibrillation died from right ventricular failure (cumulative mortality 82%, follow-up 11 +/- 8 months).
In patients with PH, SVTs constitute a relevant problem, often resulting in clinical deterioration. Sustained atrial fibrillation may be associated with a high risk of death from right ventricular failure.
American heart journal 02/2007; 153(1):127-32. DOI:10.1016/j.ahj.2006.09.008 · 4.56 Impact Factor
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