Berita Kesehatan
Pulmonary hypertension after bone marrow transplantation in children
Rabu, 06 Nov 2019 17:04:02

Marilyne Levy, Despina Moshous, Isabelle Szezepanski, Louise Galmiche, Martin Castelle, Fabrice Lesage, Laurent Dupic, Bénédicte Neven, Alain Fischer, Stéphane Blanche, Damien Bonnet

European Respiratory Journal 2019; DOI: 10.1183/13993003.00612-2019

Abstract

Pulmonary hypertension (PH) is a rare but important cause of mortality after hematopoietic stem cell transplantation (HSCT) in children. This complication is poorly characterised in the literature. We report here a series of children who developed PH after HSCT.

Methods 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, cardiac echo scans motivated by respiratory symptoms identified 31 patients with elevated tricuspid regurgitation velocity (>2.8 m·s−1), confirmed when possible by right heart catheterisation (RHC).

Results Twenty-two patients had confirmed PH with a mean pulmonary arterial pressure (PAP) 40.1±10 mmHg (range 28–62 mmHg), and pulmonary vascular resistance (PVR) (17.3±9.2 WU/range 8–42). Among the 13 responders at reactivity test only one patient responded to calcium channel blockers. Seven patients (32%) died. Fifteen PH patients are alive after a mean follow-up of 6.5±2.3 years (range 2–10 years). All survivors could be weaned of PH treatment after a median follow-up of 5 months (range 3–16). The delay between clinical symptoms and initiation of PH therapy was significantly longer in patients who subsequently died (33.5±23 days-median 30) than in survivors (7±3 days) (p<0.001).

Conclusion 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.

Footnotes

This manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.

Conflict of interest: Dr. Levy has nothing to disclose.

Conflict of interest: Dr. Moshous has nothing to disclose.

Conflict of interest: Dr. Szezepanski has nothing to disclose.

Conflict of interest: Dr. Galmiche has nothing to disclose.

Conflict of interest: Dr. Castelle has nothing to disclose.

Conflict of interest: Dr. Lesage has nothing to disclose.

Conflict of interest: Dr. DUPIC has nothing to disclose.

Conflict of interest: Dr. Neven has nothing to disclose.

Conflict of interest: Dr. Fischer has nothing to disclose.

Conflict of interest: Dr. Blanche has nothing to disclose.

Conflict of interest: Dr. Bonnet reports personal fees from Actelion, personal fees from Pfizer, personal fees from Novartis, personal fees from Bayer, outside the submitted work.

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