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多囊肾病患者的二尖瓣脱垂

Mitral Valve Prolapse in a Patient With Polycystic Kidney Disease.

作者信息

Santos Leticia, Monteiro Filipa, Gomes Ana C, Fazendas Paula S, Pereira Helder H

机构信息

Internal Medicine, Hospital Garcia de Orta, Almada, PRT.

Cardiology, Hospital Garcia de Orta, Almada, PRT.

出版信息

Cureus. 2024 Nov 3;16(11):e72931. doi: 10.7759/cureus.72931. eCollection 2024 Nov.

DOI:10.7759/cureus.72931
PMID:39628750
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11614357/
Abstract

Autosomal dominant polycystic kidney disease (ADPKD) is a multisystemic heterogeneous disease characterized by the presence of cysts in several organs leading to progressive dysfunction. The cardiovascular manifestations of ADPKD include hypertension, left ventricular hypertrophy, and valvular heart disease, predominantly mitral valve abnormalities. We present the case of a 30-year-old male with a past medical history of ADPKD who was admitted to the emergency department due to sudden chest pain and signs of congestive heart failure for weeks. Echocardiography in the emergency department showed lateral wall hypokinesis and severe mitral regurgitation. Coronary angiography revealed a small collateral branch occlusion unsuitable for revascularization. On the first day of hospitalization, the patient developed an acute ischemia of the left lower limb, for which he underwent revascularization surgery. After the thrombectomy, the patient presented with fever, for which prophylactic antibiotics were started while awaiting investigation into surgical and cystic complications, such as infection, which were ruled out. Once stabilized, a second transthoracic echocardiogram confirmed the severe mitral regurgitation and prolapse due to posterior mitral valve flail, suggesting long-term primary mitral valve disease as the underlying mechanism for regurgitation. The patient underwent surgical mitral valve repair, which was complicated by suture dehiscence.  The severe mitral valve regurgitation was attributed to ADPKD, given the patient's family history, age, and typical cardiovascular findings and multiple renal and hepatic cysts observed. Further investigation into primary mitral valve disorders, such as soft connective tissue diseases like Marfan syndrome, was not deemed necessary.

摘要

常染色体显性多囊肾病(ADPKD)是一种多系统异质性疾病,其特征是多个器官出现囊肿,导致功能逐渐衰退。ADPKD的心血管表现包括高血压、左心室肥厚和瓣膜性心脏病,主要是二尖瓣异常。我们报告一例30岁男性患者,有ADPKD病史,因突发胸痛和数周的充血性心力衰竭体征而入住急诊科。急诊科的超声心动图显示侧壁运动减弱和严重二尖瓣反流。冠状动脉造影显示一个小的侧支血管闭塞,不适于血运重建。住院第一天,患者出现左下肢急性缺血,为此他接受了血运重建手术。血栓切除术后,患者出现发热,在等待调查手术和囊肿并发症(如感染,已排除)期间开始使用预防性抗生素。病情稳定后,第二次经胸超声心动图证实由于二尖瓣后叶连枷导致严重二尖瓣反流和脱垂,提示长期原发性二尖瓣疾病是反流的潜在机制。患者接受了二尖瓣修复手术,但出现了缝线裂开的并发症。鉴于患者的家族史、年龄、典型的心血管表现以及观察到的多个肾囊肿和肝囊肿,严重二尖瓣反流归因于ADPKD。未认为有必要进一步调查原发性二尖瓣疾病,如马凡综合征等软性结缔组织疾病。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7775/11614357/fb03ebf92c92/cureus-0016-00000072931-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7775/11614357/64da1bf0d7b0/cureus-0016-00000072931-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7775/11614357/fec044c83bdf/cureus-0016-00000072931-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7775/11614357/d2561fb855fb/cureus-0016-00000072931-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7775/11614357/fb03ebf92c92/cureus-0016-00000072931-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7775/11614357/64da1bf0d7b0/cureus-0016-00000072931-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7775/11614357/fec044c83bdf/cureus-0016-00000072931-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7775/11614357/d2561fb855fb/cureus-0016-00000072931-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7775/11614357/fb03ebf92c92/cureus-0016-00000072931-i04.jpg

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