Pulmonary hypertension after bone marrow transplantation in children.
Eur Respir J
; 54(5)2019 11.
Article
en En
| MEDLINE
| ID: mdl-31649064
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 0.5-252â months; median 20.3â months). During the post-HSCT course, echocardiography scans motivated by respiratory symptoms identified 31 patients with elevated tricuspid regurgitation velocity (>2.8â 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±10â mmHg (range 28-62â mmHg) and pulmonary vascular resistance 17.3±9.2â Wood Units (range 8-42â 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±2.3â years (range 2-10â years). All survivors could be weaned off pulmonary hypertension treatment after a median follow-up of 5â 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±23â days; median 30â days) than in survivors (mean±sd 7±3â 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.
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Complicaciones Posoperatorias
/
Trasplante de Médula Ósea
/
Trasplante de Células Madre Hematopoyéticas
/
Hipertensión Pulmonar
Tipo de estudio:
Observational_studies
/
Risk_factors_studies
Límite:
Adolescent
/
Adult
/
Child
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Child, preschool
/
Humans
/
Infant
/
Newborn
Idioma:
En
Revista:
Eur Respir J
Año:
2019
Tipo del documento:
Article
País de afiliación:
Francia