Related Experiment Video
Updated: Jan 5, 2026
Development of Obliterative Bronchiolitis in a Murine Model of Orthotopic Lung Transplantation
Published on: July 10, 2012
Pulmonary hypertension after bone marrow transplantation in children
Marilyne Levy1,2,3, Despina Moshous4,5,6, Isabelle Szezepanski2
1Université Paris Descartes, Sorbonne Paris Cité, Paris, France marilyne.levy@ue3c.fr.
Insights
Pulmonary hypertension after pediatric stem cell transplant is a serious complication. Early treatment significantly improves survival rates in children.
Area of Science:
- Pediatric Hematology
- Cardiology
- Critical Care Medicine
Background:
- Pulmonary hypertension (PH) is a rare but significant cause of mortality in children post-hematopoietic stem cell transplantation (HSCT).
- The characteristics and outcomes of PH following HSCT in pediatric populations are not well-defined in existing literature.
Purpose of the Study:
- To investigate the incidence, clinical features, and outcomes of pulmonary hypertension in children undergoing HSCT.
- To identify factors associated with mortality and survival in this patient cohort.
Main Methods:
- Retrospective analysis of 366 children who underwent HSCT between January 2008 and December 2015.
- Echocardiography and, where possible, right heart catheterization (RHC) were used to diagnose PH based on elevated tricuspid regurgitation velocity and pulmonary arterial pressure.
Main Results:
- Twenty-two patients were diagnosed with confirmed PH (mean PAP 40.1±10 mmHg).
- Seven patients (32%) died, while 15 survived with improved outcomes, with survivors weaned off PH treatment.
- A significantly shorter delay between symptom onset and treatment initiation was observed in survivors compared to non-survivors (7±3 days vs. 33.5±23 days, p<0.001).
Conclusions:
- Pulmonary hypertension is a severe complication of HSCT in children, with a higher incidence and mortality than previously underestimated.
- Timely and aggressive upfront combination therapy is crucial for normalizing pulmonary pressure and improving survival rates in pediatric HSCT recipients with PH.
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.
More Related Videos
Related Concept Videos
01:22Bone Marrow Sampling and Transplants
The transplant begins with high doses of chemotherapy and radiation treatment, which aim to destroy...
01:30Pulmonary Hypertension: Classification and Pathogenesis
There are various classifications for PH, each relating to different underlying causes and also...
01:16Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure
Oxygen therapy is vital in increasing and maintaining blood oxygen levels in PAH patients. As a result, it aids in reducing fatigue,...
01:26Treatment for Pulmonary Arterial Hypertension: Receptor Tyrosine Kinase Inhibitors and Calcium Channel Blockers
TKIs, such as imatinib (Gleevec), are particularly effective in tackling the growth and mitogenic factors that become upregulated in PAH patients. These factors contribute to the...