Prenatal diagnosis of isolated coronary artery fistula: systematic review, analysis of perinatal prognostic factors and case report

Isolated coronary artery fistula (CAF) is a rare entity in which evidence for both prognosis and need for perinatal treatment is lacking. We aim to evaluate the characteristics, evolution and perinatal outcomes of reported cases, including one from our center. We performed a systematic review in Medline, Pubmed, and Embase databases for cohort studies or case series related to prenatally diagnosed isolated congenital CAF according to PRISMA guidelines. The search was restricted to articles published until January 2022, including a case report from our center. A descriptive analysis was performed, and perinatal characteristics were dichotomized by outcome (development of symptoms, as well as the need for surgery during the neonatal period). Strength of association between prenatal variables and outcome was evaluated through Odds Ratio. Only 27 cases of prenatal diagnosis of isolated CAF have been published, including our patient. Most had their origin in the right coronary artery (63%) and drained in the right ventricle (55.6%). Most cases (72%) developed progressive intrauterine dilation of the fistulous tract, which was usually associated with symptoms of cardiac overload, such as cardiomegaly (57.7%). Up to two-thirds of prenatally diagnosed patients developed heart failure symptoms in the neonatal period, and 84% required postnatal intervention. Prenatal diagnosis of both cardiomegaly and diastolic steal is associated with an OR of 52 and 41 of developing postnatal symptoms. Prenatal diagnosis of isolated CAF can be achieved with adequate tools and trained sonographers. The development of cardiomegaly and diastolic steal significantly increases the risk of developing postnatal symptoms.

孤立性冠状动脉瘘（isolated coronary artery fistula，CAF）是一种罕见病症，目前关于其预后及围产期治疗的必要性均缺乏相关证据。本研究旨在评估已报道病例的特征、转归及围产期结局，其中包含本中心的1例病例。我们按照PRISMA指南，对Medline、Pubmed及Embase数据库开展系统综述，检索针对产前诊断的孤立性先天性CAF的队列研究或病例系列研究，检索时限限定为2022年1月之前发表的文献，同时纳入本中心的1例病例报告。本研究采用描述性分析方法，根据结局（症状发生情况以及新生儿期手术需求）对围产期特征进行二分法分类，并通过比值比（Odds Ratio，OR）评估产前变量与结局之间的关联强度。目前已发表的产前诊断孤立性CAF病例仅27例，包括本中心的患者。其中多数瘘管起源于右冠状动脉（63%），并引流至右心室（55.6%）。72%的病例出现瘘管进行性宫内扩张，该表现通常与心脏负荷过重的症状相关，例如心脏增大（57.7%）。多达三分之二的产前诊断患者在新生儿期出现心力衰竭症状，84%的患者需要接受产后干预。产前诊断发现心脏增大及舒张期窃血（diastolic steal）的患者，其产后出现症状的比值比分别为52和41。产前诊断孤立性CAF可通过合适的检查工具及训练有素的超声医师实现。心脏增大与舒张期窃血的出现，会显著增加产后出现症状的风险。