Pulmonary hypertension after bone marrow transplantation in children

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, cardiac echo 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 Twenty-two patients had confirmed PH with a mean pulmonary arterial pressure (PAP) 40.1±10 mmHg (range 28–62 mmHg), and pulmonary vascular resistance (PVR) (17.3±9.2 WU/range 8–42). Among the 13 responders at reactivity test only one patient responded to calcium channel blockers. Seven patients (32%) died. Fifteen PH patients are alive after a mean follow-up of 6.5±2.3 years (range 2–10 years). All survivors could be weaned of PH treatment after a median follow-up of 5 months (range 3–16). The delay between clinical symptoms and initiation of PH therapy was significantly longer in patients who subsequently died (33.5±23 days-median 30) than in survivors (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.