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Pulmonary hypertension after bone marrow transplantation in children

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
Marilyne Levy
1Université Paris Descartes, Sorbonne Paris Cité, Paris, France
2M3C-Unité Médico-Chirurgicale de Cardiologie Pédiatrique, Hôpital Necker-Enfants malades, AP-HP, Paris, France
3UE3C-Unité d'Explorations Cardiologiques-Cardiopathies Congénitales, Paris, France
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  • For correspondence: marilyne.levy@ue3c.fr
Despina Moshous
1Université Paris Descartes, Sorbonne Paris Cité, Paris, France
4Pediatric Hematology-Immunology and Rheumatology Unit, Hôpital Necker-Enfants malades, Assistance Publique Hôpitaux de Paris, France
5INSERM U1163 and Institut Imagine, Paris, France
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Isabelle Szezepanski
2M3C-Unité Médico-Chirurgicale de Cardiologie Pédiatrique, Hôpital Necker-Enfants malades, AP-HP, Paris, France
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Louise Galmiche
6Service d'anatomopathologie, hôpital Necker Enfants-malades, Paris, France
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Martin Castelle
1Université Paris Descartes, Sorbonne Paris Cité, Paris, France
4Pediatric Hematology-Immunology and Rheumatology Unit, Hôpital Necker-Enfants malades, Assistance Publique Hôpitaux de Paris, France
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Fabrice Lesage
1Université Paris Descartes, Sorbonne Paris Cité, Paris, France
7Pediatric Intensive Care Unit, Hôpital Necker-Enfants malades, Assistance Publique Hôpitaux de Paris
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Laurent Dupic
1Université Paris Descartes, Sorbonne Paris Cité, Paris, France
7Pediatric Intensive Care Unit, Hôpital Necker-Enfants malades, Assistance Publique Hôpitaux de Paris
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Bénédicte Neven
1Université Paris Descartes, Sorbonne Paris Cité, Paris, France
4Pediatric Hematology-Immunology and Rheumatology Unit, Hôpital Necker-Enfants malades, Assistance Publique Hôpitaux de Paris, France
5INSERM U1163 and Institut Imagine, Paris, France
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Alain Fischer
1Université Paris Descartes, Sorbonne Paris Cité, Paris, France
4Pediatric Hematology-Immunology and Rheumatology Unit, Hôpital Necker-Enfants malades, Assistance Publique Hôpitaux de Paris, France
5INSERM U1163 and Institut Imagine, Paris, France
8Collège de France, Paris, France
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Stéphane Blanche
1Université Paris Descartes, Sorbonne Paris Cité, Paris, France
4Pediatric Hematology-Immunology and Rheumatology Unit, Hôpital Necker-Enfants malades, Assistance Publique Hôpitaux de Paris, France
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Damien Bonnet
1Université Paris Descartes, Sorbonne Paris Cité, Paris, France
2M3C-Unité Médico-Chirurgicale de Cardiologie Pédiatrique, Hôpital Necker-Enfants malades, AP-HP, Paris, France
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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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Pulmonary hypertension after bone marrow transplantation in children
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 Jan 2019, 1900612; DOI: 10.1183/13993003.00612-2019

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Pulmonary hypertension after bone marrow transplantation in children
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 Jan 2019, 1900612; DOI: 10.1183/13993003.00612-2019
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