Chest. 2012 Mar 29. [Epub ahead of print]
Pulmonary Langerhans Cell Histiocytosis associated pulmonary hypertension: clinical characteristics and impact of pulmonary arterial hypertension therapies.
Le Pavec J, Lorillon G, Jaïs X, Tcherakian C, Feuillet S, Dorfmüller P, Simonneau G, Humbert M, Tazi A.
Centre National de Référence de l'Histiocytose Langerhansienne; Univ Paris Diderot, Sorbonne Cité; Assistance Publique Hôpitaux de Paris; Service de Pneumologie, Hôpital Saint Louis, Paris, France.
Abstract
BACKGROUND: Precapillary pulmonary hypertension (PH) is a complication of pulmonary Langerhans cell histiocytosis (PLCH) associated with increased mortality. However, outcomes and efficacy of pulmonary arterial hypertension (PAH) therapies in patients with PH complicating PLCH (PLCH-PH) remain unknown.
METHODS: Consecutive PLCH patients with PH confirmed by right heart catheterization were included in the study. Characteristics at baseline and during follow-up as well as survival were analyzed.
RESULTS: 29 patients were studied. Baseline characteristics of PLCH-PH patients were: 83% of patients in World Health Organization (WHO) functional class III-IV, mean 6-min walk distance of 355 ± 95 m, mean pulmonary arterial pressure (mPAP) of 45 ± 14 mm Hg, cardiac index of 3.2 ± 0.9 L.min(-1).m(-2) and pulmonary vascular resistance (PVR) of 555 ± 253 dyn.s.cm(-5). Use of PAH therapy in 12 patients was followed by improvement in mPAP (56 ± 14 and 45 ± 12 mm Hg, P = 0.03) and PVR (701 ± 239 and 469 ± 210 dyn.s.cm(-5), P = 0.01) between baseline and follow up evaluations. No significant oxygen worsening was observed in the treated group. The 1-, 3- and 5-year survival estimates of the 29 patients were 96%, 92% and 73%, respectively. Except a trend toward a better survival associated with the use of PAH therapy, WHO functional class was the only variable significantly associated with death.
CONCLUSIONS: In this group of patients, PAH therapies improved hemodynamic without oxygen worsening or pulmonary edema. WHO functional class was the only prognostic factor identified. Prospective clinical trials focusing on this population of patients are warranted.
PMID: 22459770