Houyel Lucile, To-Dumortier Ngoc-Tram, Lepers Yannick, Petit Jérôme, Roussin Régine, Ly Mohamed, Lebret Emmanuel, Fadel Elie, Hörer Jürgen, Hascoët Sébastien
Service de Chirurgie des Cardiopathies Congénitales, Hôpital Marie-Lannelongue-M3C, Le Plessis-Robinson, France.
Service de Cardiologie, Hôpital Marie-Lannelongue, Le Plessis-Robinson, France.
Arch Cardiovasc Dis. 2017 May;110(5):346-353. doi: 10.1016/j.acvd.2017.01.002. Epub 2017 Feb 22.
With the advances in congenital cardiac surgery and postoperative care, an increasing number of children with complex congenital heart disease now reach adulthood. There are already more adults than children living with a congenital heart defect, including patients with complex congenital heart defects. Among these adults with congenital heart disease, a significant number will develop ventricular dysfunction over time. Heart failure accounts for 26-42% of deaths in adults with congenital heart defects. Heart transplantation, or heart-lung transplantation in Eisenmenger syndrome, then becomes the ultimate therapeutic possibility for these patients. This population is deemed to be at high risk of mortality after heart transplantation, although their long-term survival is similar to that of patients transplanted for other reasons. Indeed, heart transplantation in adults with congenital heart disease is often challenging, because of several potential problems: complex cardiac and vascular anatomy, multiple previous palliative and corrective surgeries, and effects on other organs (kidney, liver, lungs) of long-standing cardiac dysfunction or cyanosis, with frequent elevation of pulmonary vascular resistance. In this review, we focus on the specific problems relating to heart and heart-lung transplantation in this population, revisit the indications/contraindications, and update the long-term outcomes.
随着先天性心脏病外科手术及术后护理的进展，越来越多患有复杂先天性心脏病的儿童现已成年。患有先天性心脏缺陷（包括复杂先天性心脏缺陷患者）的成年人数量已超过儿童。在这些先天性心脏病成年患者中，相当一部分人随着时间推移会出现心室功能障碍。心力衰竭占先天性心脏缺陷成年患者死亡人数的26% - 42%。对于这些患者，心脏移植或艾森曼格综合征患者的心肺移植就成为最终的治疗选择。尽管该人群心脏移植后的长期生存率与因其他原因接受移植的患者相似，但他们被认为在心脏移植后有较高的死亡风险。事实上，先天性心脏病成年患者的心脏移植往往具有挑战性，原因有几个潜在问题：心脏和血管解剖结构复杂、既往多次姑息性和矫正性手术、长期心脏功能障碍或紫绀对其他器官（肾脏、肝脏、肺）的影响以及肺血管阻力频繁升高。在本综述中，我们重点关注该人群心脏和心肺移植相关的具体问题，重新审视适应证/禁忌证，并更新长期预后情况。
