Abstract 13510: Management of Pulmonary Hypertension in Association with Bronchopulmonary Dysplasia: Medium Term Follow-up.
Background: Pulmonary Hypertension (PH) significantly worsens outcome in infants with bronchopulmonary dysplasia (BPD). Current treatment strategies include early use of pulmonary vasodilators, gentler ventilation strategies, and early shunt closure, but data on long term impact is scarce.
Methods: 27 children (23–27 weeks GA at birth), with BPD and severe PH treated with pulmonary vasodilators, high frequency ventilation and early shunt closure were followed over a 0.5–7 year period. Baseline pulmonary vascular reactivity was assessed by echocardiography alone (20) or echo with cardiac catheterization (7). Treatment included sildenafil (12), sildenafil+bosentan (11), +iloprost (4). All received inhaled nitric oxide (iNO) initially. Serial echo estimated PH and RV function. 10/27 infants underwent cardiac cath after 6–9 months; PDAs persistently left to right on oral therapy alone (6) were closed.
Results: Responses to therapy included improvement in symptoms (25/27), oxygenation (25/27), hemodynamics on catheterization (10/27) and decreased PH on echo (25/27). Five patients with initial R-L shunts were older when the oral medications were approved and had already spent 1.5–2.5 years in hospital on a ventilator. Their cardiac shunts were closed at 0.8–3 years of treatment (1VSD, 1ASD, 3PDA). They have ≤ ½ systemic PH and still require medication. 6 infants had PDA closure within 4–6 weeks of oral therapy. 11 could be weaned off O2 and medication at 0.9–3 (m=1.9) years of treatment. The rest have lower O2 requirements. One infant with alveolar-capillary dysplasia and one with surfactant protein C deficiency died. All living patients (25/27) have improvement in functional class (Ross Class 4 to class 1–2) despite lung disease being unchanged. All patients (11/27) (p<0.05) who could be taken off vasodilator medications and oxygen had pulmonary vasodilators initiated early and any L-R PDAs closed within 6 months of age as compared to those treated later, and who still are on medications despite significant improvement in clinical status and PH.
Conclusion: Early specific management of PH improves the prognosis, morbidity and mortality in infants with BPD and PH. Periodic surveillance echo for PH in chronically ventilated infants with BPD is recommended.
- © 2010 by American Heart Association, Inc.