RT Journal Article
SR Electronic
T1 Pulmonary hypertension after bone marrow transplantation in children
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP 1900612
DO 10.1183/13993003.00612-2019
VO 54
IS 5
A1 Marilyne Levy
A1 Despina Moshous
A1 Isabelle Szezepanski
A1 Louise Galmiche
A1 Martin Castelle
A1 Fabrice Lesage
A1 Laurent Dupic
A1 Bénédicte Neven
A1 Alain Fischer
A1 Stéphane Blanche
A1 Damien Bonnet
YR 2019
UL http://erj.ersjournals.com/content/54/5/1900612.abstract
AB 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 0.5–252 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−1), confirmed when possible by right heart catheterisation (RHC).Results 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).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