Long-term outcomes after repair for anomalous right coronary artery from the pulmonary artery.
Long-term outcomes after repair for anomalous right coronary artery from the pulmonary artery.
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DOI：
10.1017/s1047951122000373
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发表时间：
2022-02-18
影响因子：
1
通讯作者：
Aggarwal, Varun
中科院分区：
文献类型：
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作者：
Tran, Andrew;Kochilas, Lazaros;Thomas, Amanda S.;Aggarwal, Varun
异常右冠状动脉起源于肺动脉（ARCAPA）是一种罕见的先天性心脏病，可导致冠状动脉灌注异常，需要手术修复。在这里，我们报告了儿科心脏护理联盟（PCCC）内接受ARCAPA手术的患者的结果，该联盟是北美儿科心脏病干预登记处。我们查询了1982年至2003年期间接受ARCAPA手术修复的18岁以下患者的PCCC。结果来自PCCC，并与国家死亡指数（NDI）和器官获取和移植网络（OPTN）联系到2019年。确定了24例患者（男性：15例）接受ARCAPA手术，中位年龄为5.8（IQR 2.7-10.3）岁。其中，23例被认为是“简单”（没有重大心内疾病）和一个“复杂”（并存法洛四联症）。5例患者出现症状[胸痛（1例）、运动时呼吸困难（2例）或晕厥史（2例）];其余19例患者转诊评价杂音或共存CHD。手术修复后无院内死亡。14例患者有足够的NDI/OPTN关联识别符;其中，在中位随访19.4年（IQR：18-24.6）内，仅1例死亡发生于无关的非心脏原因。再植入25年后的结局非常好，进一步的纵向监测对于了解既存冠状动脉病变与老化效应的相互作用非常重要。
Anomalous right coronary artery from pulmonary artery (ARCAPA) is a rare congenital heart disease that can lead to abnormal coronary perfusion and a need for surgical repair. Here, we report the outcomes of patients who underwent ARCAPA surgery within the Pediatric Cardiac Care Consortium (PCCC), a North American registry of interventions for paediatric heart diseases. We queried the PCCC for patients undergoing surgical repair for ARCAPA at <18 years of age between 1982 and 2003. Outcomes were obtained from the PCCC and after linkage with the National Death Index (NDI) and the Organ Procurement and Transplantation Network (OPTN) through 2019. Twenty-four patients (males: 15) were identified having surgery for ARCAPA at a median age of 5.8 (IQR 2.7–10.3) years. Of them, 23 cases were considered “simple” (without major intracardiac disease) and one “complex” (co-existing with tetralogy of Fallot). Five patients presented with symptoms [chest pain (1), dyspnoea on exertion (2) or history of syncope (2)]; while the remaining 19 patients were referred for evaluation of either murmur or co-existing CHD. There was no in-hospital mortality after the surgical repair. Fourteen patients had sufficient identifiers for NDI/OPTN linkage; among them, only one death occurred from unrelated non-cardiac causes within a median period of 19.4 years of follow-up (IQR: 18–24.6). Outcomes were excellent after reimplantation up to 25 years later and further longitudinal monitoring is important to understand the interaction of pre-existing coronary pathology with the effects of ageing.