Pulmonary hypertension after bone marrow transplantation in children.

Pulmonary hypertension is a rare but important cause of mortality after haematopoietic stem cell transplantation (HSCT) in children. This complication is poorly characterised in the literature. We report here a series of children who developed pulmonary hypertension after HSCT. Between January 2008 and December 2015, we retrospectively analysed 366 children who underwent HSCT (age range 0.5-252 months; median 20.3 months). During the post-HSCT course, echocardiography scans motivated by respiratory symptoms identified 31 patients with elevated tricuspid regurgitation velocity (>2.8 m·s 22 patients had confirmed pulmonary hypertension with mean±sd pulmonary arterial pressure 40.1±10 mmHg (range 28-62 mmHg) and pulmonary vascular resistance 17.3±9.2 Wood Units (range 8-42 Wood Units). Among the 13 responders at reactivity test, only one patient responded to calcium channel blockers. Seven patients (32%) died. 15 pulmonary hypertension patients were alive after a mean±sd follow-up of 6.5±2.3 years (range 2-10 years). All survivors could be weaned off pulmonary hypertension treatment after a median follow-up of 5 months (range 3-16). The delay between clinical symptoms and initiation of pulmonary hypertension therapy was significantly longer in patients who subsequently died (mean±sd 33.5±23 days; median 30 days) than in survivors (mean±sd 7±3 days) (p<0.001). Pulmonary hypertension is a severe complication of HSCT with an underestimated incidence and high mortality. Aggressive and timely up-front combination therapy allowed normalisation of pulmonary pressure and improved survival.

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Déclaration de conflit d'intérêts

Conflict of interest: M. Levy has nothing to disclose. Conflict of interest: D. Moshous has nothing to disclose. Conflict of interest: I. Szezepanski has nothing to disclose. Conflict of interest: L. Galmiche has nothing to disclose. Conflict of interest: M. Castelle has nothing to disclose. Conflict of interest: F. Lesage has nothing to disclose. Conflict of interest: L. Dupic has nothing to disclose. Conflict of interest: B. Neven has nothing to disclose. Conflict of interest: A. Fischer has nothing to disclose. Conflict of interest: S. Blanche has nothing to disclose. Conflict of interest: D. Bonnet reports personal fees from Actelion, Pfizer, Novartis and Bayer, outside the submitted work.