Department of Cardiology, Queen Elizabeth Hospital NHS Foundation Trust, Birmingham, UK.
Curr Opin Support Palliat Care. 2013 Mar;7(1):8-13. doi: 10.1097/SPC.0b013e32835c0707.
There are increasing numbers of adults with congenital heart disease (CHD) and these patients remain at long-term risk of complications and premature death. This review focuses on the changing picture of adult CHD with more complex patients surviving, the challenges of balancing life-prolonging intervention, the barriers to discussing the end-of-life (EOL) issues and draws on the experience of other specialities in managing young patients.
The prevalence of adults with the most severe forms of CHD has increased, especially those with a Fontan circulation. The eventual decline is inevitable with limited treatment options. There should be a parallel palliative care approach in patients who are being considered for high-risk, life-prolonging interventions. Oncologists caring for the young patients with cancer and cystic fibrosis specialists have demonstrated the unique needs of young patients with chronic diseases that may be applicable to adult CHD patients and help with their EOL planning.
These patients require an early and proactive approach to EOL discussions, and the unique needs of young patients should be recognized. Further research is needed to develop local and national guidelines for the palliative care approach in these patients.
先天性心脏病（CHD）患者数量不断增加，这些患者长期存在并发症和过早死亡的风险。本综述重点关注复杂患者存活的成人 CHD 变化情况、平衡延长生命干预的挑战、讨论临终（EOL）问题的障碍，并借鉴其他专科在管理年轻患者方面的经验。
最严重形式的 CHD 成人患者的患病率增加，尤其是那些具有 Fontan 循环的患者。最终的下降是不可避免的，因为治疗选择有限。对于正在考虑高风险、延长生命的干预措施的患者，应该采取平行的姑息治疗方法。照顾患有癌症的年轻患者的肿瘤学家和囊性纤维化专家已经证明了患有慢性疾病的年轻患者的特殊需求可能适用于成人 CHD 患者，并有助于他们的 EOL 规划。
这些患者需要早期和积极主动地进行 EOL 讨论，并且应该认识到年轻患者的独特需求。需要进一步研究，为这些患者的姑息治疗方法制定地方和国家指南。
