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【medical-news】儿童肺动脉高压的治疗和预后：英
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 children with idiopathic pulmonary arterial hypertension (IPAH) and associated pulmonary arterial hypertension (APAH) 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, 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, postoperative 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.
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Sustained pulmonary arterial hypertension is associated with the development of pulmonary vascular disease, which when advanced leads to right heart failure and death. Pulmonary arterial hypertension is subdivided into idiopathic pulmonary arterial hypertension (IPAH) and associated pulmonary arterial hypertension (APAH) when present with other disorders.1 Left untreated, children with IPAH fare less well than adults. The predicted survival after diagnosis is less than a year compared with 2.8 years in adults.2 The introduction of pulmonary hypertension specific therapies has improved the prognosis in both adults and children. Before the definitive trial of intravenous epoprostenol in adults in 1996 the only medications available were calcium channel antagonists, which are efficacious in only a minority. The beneficial effect of epoprostenol in children with IPAH was demonstrated three years later.3 Anticoagulation was, and is, also considered helpful in adults with IPAH.4 5 The oral drug bosentan, a dual endothelin receptor antagonist, was found to be efficacious in adults in 2002,6 and later we and others showed that it was also efficacious in children.7 8 The phosphodiesterase V (PDE-5) inhibitor sildenafil was shown to be efficacious in a 12-week trial in adults with IPAH.9 A trial is in progress to study the effect of the drug in children.
We established a clinical network for the care of all children with significant pulmonary hypertension in the United Kingdom to try to optimise the treatment of this patient population using new and emerging therapies. This network was designated by the National Specialist Advisory Group of the Department of Health in 2002. It was commissioned as a funded service by the Specialist Commissioning Group in April 2007.
The UK Pulmonary Hypertension Service for Children was organised on a hub-and-spoke principle, the hub being at Great Ormond Street Hospital for Children (GOSHC). The spokes were six major centres of paediatric cardiology in the UK—namely, the Freeman Hospital, Newcastle upon Tyne, Leeds General Infirmary, Bristol Children’s Hospital, Birmingham Children’s Hospital, Yorkhill Hospital, Glasgow and the Royal Belfast Children’s Hospital. Joint clinics were held at these hospitals by SGH with the local paediatric cardiologist. We hope that all children with IPAH are now referred to the service. Children who are referred with APAH generally have severe pulmonary hypertension. Cases of mild PAH are not usually referred to the service. Children with mild postoperative PAH, for example, are usually managed locally by their own physicians. Pulmonary hypertension within the first month of life is specifically excluded from the service, with few exceptions such as a documented or suspected family history of IPAH.
We present a retrospective analysis of the treatment given and the response to treatment in terms of survival in children treated by this service for the first 5-year period.
Heart 2009;95:312-317 [标签:content1][标签:content2]
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作者:admin@医学,生命科学 2011-02-19 17:11
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