作者@article {Levy1900612 ={征税,Marilyned Moshous, Despina and Szezepanski, Isabelle and Galmiche, Louise and Castelle, Martin and Lesage, Fabrice and Dupic, Laurent and Neven, B{\'e}n{\'e}dicte and Fischer, Alain and Blanche, St{\'e}phane and Bonnet, Damien}, title = {Pulmonary hypertension after bone marrow transplantation in children}, volume = {54}, number = {5}, elocation-id = {1900612}, year = {2019}, doi = {10.1183/13993003.00612-2019}, publisher = {European Respiratory Society}, abstract = {Introduction 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.Methods Between January 2008 and December 2015, we retrospectively analysed 366 children who underwent HSCT (age range 0.5{\textendash}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{\textperiodcentered}s-1), confirmed when possible by right heart catheterisation (RHC).Results 22 patients had confirmed pulmonary hypertension with mean{\textpm}sd pulmonary arterial pressure 40.1{\textpm}10 mmHg (range 28{\textendash}62 mmHg) and pulmonary vascular resistance 17.3{\textpm}9.2 Wood Units (range 8{\textendash}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{\textpm}sd follow-up of 6.5{\textpm}2.3 years (range 2{\textendash}10 years). All survivors could be weaned off pulmonary hypertension treatment after a median follow-up of 5 months (range 3{\textendash}16). The delay between clinical symptoms and initiation of pulmonary hypertension therapy was significantly longer in patients who subsequently died (mean{\textpm}sd 33.5{\textpm}23 days; median 30 days) than in survivors (mean{\textpm}sd 7{\textpm}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.Pulmonary hypertension is a rare and severe complication of haematopoietic stem cell transplantation in children, with a high mortality if misdiagnosed. Early diagnosis and treatment allow rapid clinical improvement with normalisation of PAP in most cases. http://bit.ly/2KXyhNN}, issn = {0903-1936}, URL = {//www.qdcxjkg.com/content/54/5/1900612}, eprint = {//www.qdcxjkg.com/content/54/5/1900612.full.pdf}, journal = {European Respiratory Journal} }