一名患有严重肺动脉高压和红细胞增多症的成年患者的大型动脉导管未闭：伴有严重肺动脉高压的动脉导管未闭
Large Patent Ductus Arteriosus in an Adult With Severe Pulmonary Hypertension and Polycythemia: PDA With Severe Pulmonary Hypertension.
作者信息
Langenfeld Tyler L, Sati Maram, Bocks Martin L, Agarwal Arpit K
机构信息
Department of Pediatrics, Rainbow Babies and Children's Hospital, Cleveland, Ohio, USA.
Department of Pediatric Cardiology, Rainbow Babies and Children's Hospital, Cleveland, Ohio, USA.
出版信息
JACC Case Rep. 2025 Aug 20;30(24):104677. doi: 10.1016/j.jaccas.2025.104677.
BACKGROUND
Patent ductus arteriosus (PDA) is one of the most common cardiac abnormalities in children, but in rare cases it can go undiagnosed into adulthood until symptoms arise.
CASE SUMMARY
We present a unique case of a 55-year-old male patient with severe pulmonary hypertension and polycythemia vera requiring frequent phlebotomy who was found to have a PDA on cardiac computed tomography. Owing to the severity of his pulmonary hypertension, he was not a candidate for closure and was therefore treated with medical management.
DISCUSSION
PDA diagnosed in adulthood is rare and carries significant morbidity, with findings such as congestive heart failure, infectious endarteritis, or severe pulmonary hypertension. Our patient had multiple severe and rare sequelae of a very large unrepaired PDA, including Eisenmenger syndrome and polycythemia vera, that required frequent phlebotomy. Despite the large size of the PDA, it was not visualized on echocardiography.
背景
动脉导管未闭（PDA）是儿童最常见的心脏异常之一，但在极少数情况下，它可能直到成年出现症状才被诊断出来。
病例摘要
我们报告了一例独特的病例，一名55岁男性患者，患有严重的肺动脉高压和真性红细胞增多症，需要频繁进行放血治疗，心脏计算机断层扫描发现其患有动脉导管未闭。由于其肺动脉高压的严重程度，他不适合进行封堵手术，因此接受了药物治疗。
讨论
成年期诊断出的动脉导管未闭很罕见，且具有显著的发病率，会出现诸如充血性心力衰竭、感染性心内膜炎或严重肺动脉高压等症状。我们的患者患有一个非常大的未修复动脉导管未闭的多种严重且罕见的后遗症，包括艾森曼格综合征和真性红细胞增多症，需要频繁进行放血治疗。尽管动脉导管未闭尺寸很大，但在超声心动图上并未显示出来。
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