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Clinical Characteristics, Haemodynamics and Treatment of Pulmonary Hypertension in Sarcoidosis in a Single Centre, and Meta-Analysis of the Published Data

https://doi.org/10.1016/j.amjcard.2012.09.031Get rights and content

Pulmonary hypertension (PH) in sarcoidosis is associated with bad outcomes. Although there is interest in using pulmonary vasodilators (PVs) for PH in sarcoidosis, there are few data to support their use. In this study, a retrospective review of a cohort of patients with PH and sarcoidosis was conducted, focusing on those treated with PVs, and a meta-analysis of published reports indexed in MEDLINE was performed. Twenty-four patients were found. The rate of mortality or transplantation rate was 41.2%. Median survival without transplantation was 5.3 years. More patients who died or underwent transplantation during follow-up had moderate or severe lung fibrosis (66.7% vs 15.4%), had right ventricular dysfunction (80% vs 7.7%), and were in World Health Organization class IV (66.7% vs 30.8%). Body surface areas were lower in patients with events, as was cardiac output. Mortality was not different between patients treated with PVs and those not treated (54.5% vs 38.5%, p = 0.44) despite the treated patients' having more right ventricular dysfunction and worse hemodynamics. In a Cox regression survival model, lower body surface area, right ventricular dysfunction, and the presence of moderate or severe lung fibrosis were predictors of worse outcomes, but not treatment with PVs. PV-treated patients (n = 11) showed improved 6-minute walk distances and decreased N-terminal pro–B-type natriuretic peptide levels during follow-up. There was a trend toward improvement in hemodynamic profile. Four studies plus the data from this study were included in the meta-analysis. Six-minute walk distance improved by 30.64 m after treatment. Hemodynamics improved, with a reduction in mean pulmonary arterial pressure of 8.03 mm Hg and a decrease in pulmonary vascular resistance of 4.23 Wood units. In conclusion, PH in sarcoidosis is associated with adverse outcomes, particularly when accompanied by right ventricular dysfunction and/or moderate or severe lung fibrosis. Treating selected patients can improve hemodynamics and functional parameters.

Section snippets

Methods

We performed a retrospective review of patients with sarcoidosis referred to the National Pulmonary Hypertension Unit of the Royal Free Hospital in London from 1999 to 2011. We identified all patients who underwent right-sided cardiac catheterization and then cross-checked individual medical records. We included patients with sarcoidosis who had PH, defined as a mean pulmonary artery pressure ≥25 mm Hg, confirmed by right-sided cardiac catheterization. We recorded the use of any PV therapy,

Results

From 1999 to December 2011, 24 patients (Table 1) with sarcoidosis and evidence on right-sided cardiac catheterization of PH were seen in the National Pulmonary Hypertension Unit of the Royal Free Hospital in London. The median follow-up duration was 22.6 months. The mean age of patients at diagnosis of PH was 57.83 ± 13.05 years. There was a small predominance of women (54.2%), and almost 90% of patients were in functional class III or IV, with 42% in functional class IV. During follow-up, 9

Discussion

The rate of PH in sarcoidosis has been reported to be about 10%11 and up to 70% in patients referred for lung transplantation. PH has been demonstrated to be associated with dramatically increased mortality,3, 11, 12 with a reported mortality after 2 years of the diagnosis of PH of 35%. Despite this prevalence, there are no recommendations for screening.11 The lack of information with respect of the natural history and the lack of evidence supporting therapeutic intervention may underlie this

Disclosures

Dr. Dobarro has received a grant from Eli Lilly & Company, Indianapolis, Indiana. Dr. Schreiber has received speaker's fees from Actelion Pharmaceuticals, Allschwil, Switzerland, and speaker's fees and travel awards from GlaxoSmithKline, London, United Kingdom. Dr. Denton has received consultancy fees or honoraria from Actelion Pharmaceuticals; Encysive Pharmaceuticals, Houston, Texas; Pfizer, Inc., New York, New York; GlaxoSmithKline; Biovitrum AB, Stockholm, Sweden; Aspreva Pharmaceuticals,

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