In:
European Respiratory Journal, European Respiratory Society (ERS), Vol. 54, No. 5 ( 2019-11), p. 1900612-
Abstract:
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.
Type of Medium:
Online Resource
ISSN:
0903-1936
,
1399-3003
DOI:
10.1183/13993003.00612-2019
DOI:
10.1183/13993003.00612-2019.Shareable1
Language:
English
Publisher:
European Respiratory Society (ERS)
Publication Date:
2019
detail.hit.zdb_id:
2834928-3
detail.hit.zdb_id:
1499101-9
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