Objective: A retrospective study of the UK Pulmonary Hypertension Service for Children for the first 5 year period of its existence.
Design and Patients: Records of 216 cases with Idiopathic Pulmonary Arterial Hypertension and Associated Pulmonary Arterial Hypertension (IPAH and APAH respectively) were reviewed. Kaplan Meier survival curves were constructed for different diagnostic groups and for different therapies.
Results: At cardiac catheterisation only 7.4% of those with IPAH and 6% of those with APAH responded positively to vasodilator testing and so were treated with nifedipine. Others needing treatment were given continuous intravenous epoprostenol, bosentan or sildenafil singly or in combination. For IPAH survival rates were 85.6%, 79.9% and 71.9% at 1, 3 and 5 years respectively as compared with a survival time of less than a year in historical untreated controls. A combination of intravenous epoprostenol with either bosentan or sildenafil or both appeared to achieve the best outcome. Six children underwent lung transplantation. In APAH survival rates were 92.3%, 83.8% and 56.9% at 1, 3 and 5 years respectively, post-operative congenital heart disease with severe pulmonary hypertension having the worst outcome.
Conclusion: New pulmonary hypertension specific medicines have improved survival in children as in adults. Outcome in this series compares favourably with international outcome data.