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一例限制型心肌病患者因淋巴细胞白血病治疗致右心功能障碍行单心室修复术成功。

Successful One-and-a-Half Ventricle Repair of Right Ventricle Dysfunction Due to Lymphoblastic Leukemia Treatment in a Patient with Restrictive Cardiomyopathy.

机构信息

Department of Congenital Heart Diseases, A.N. Bakoulev National Medical Research Center of Cardiovascular Surgery, Moscow, Russian Federation.

出版信息

Am J Case Rep. 2021 Nov 24;22:e933677. doi: 10.12659/AJCR.933677.

Abstract

BACKGROUND The cardiotoxic effects of chemotherapy in cancer treatment can damage cardiomyocytes. A common link in the pathogenesis is the proliferation of fibroblasts and the increase of collagen synthesis, leading to development of common endomyocardial fibrosis. The walls of ventricles become rigid and their inability to relax prevents them from carrying the required amount of blood. The myocardial contractility gradually decreases and leads to ventricular dysfunction and signs of heart failure. CASE REPORT A 29-year-old woman with reduced exercise tolerance, dyspnea, and heart rhythm disorders was admitted to our hospital. Lymphoblastic leukemia had been diagnosed at the age of 8 years, and she underwent 8 courses of polychemotherapy. She had normal heart anatomy. At the current admission, the diagnostic protocol included echocardiography, computed tomography, cardiac catheterization, and angiocardiography. She was diagnosed with restrictive cardiomyopathy with isolated endomyocardial fibrosis of the right ventricle, and moderate tricuspid valve insufficiency NYHA class III. The patient underwent a right-sided bidirectional cavopulmonary connection with tricuspid valve repair. The early postoperative period was uneventful, and SVCp decreased to 14 mmHg. At discharge, the patient's clinical condition had improved and tricuspid regurgitation was minimal. CONCLUSIONS The one-and-a-half ventricular correction, commonly used in patients with Ebstein's anomaly and RV dysfunction or in patients with congenital heart defects associated with RV hypoplasia, is proposed as the method of choice for cardiomyopathy type RV dysfunction.

摘要

背景

癌症治疗中的化疗会损害心肌细胞,导致常见的心肌纤维化。发病机制中的一个共同环节是成纤维细胞的增殖和胶原合成的增加,导致常见的局限性心肌纤维化。心室壁变得僵硬,无法放松,导致无法携带所需的血量。心肌收缩力逐渐下降,导致心室功能障碍和心力衰竭的迹象。

病例报告

一位 29 岁女性,因运动耐量降低、呼吸困难和心律不齐而入院。她在 8 岁时被诊断出患有淋巴母细胞白血病,并接受了 8 轮化疗。她的心脏解剖结构正常。在此次入院时,诊断方案包括超声心动图、计算机断层扫描、心导管检查和心血管造影。她被诊断为限制性心肌病,伴有孤立性右心室局限性心肌纤维化和中度三尖瓣关闭不全(NYHA 分级 III)。患者接受了右侧双向腔静脉肺动脉连接和三尖瓣修复术。术后早期无并发症,上腔静脉压(SVCp)降至 14mmHg。出院时,患者的临床状况有所改善,三尖瓣反流最小。

结论

对于右心室功能障碍型心肌病,建议采用半心室矫正术,该方法常用于埃布斯坦畸形和右心室功能障碍患者,或用于与右心室发育不良相关的先天性心脏病患者。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/524f/8630555/b83b905828e5/amjcaserep-22-e933677-g001.jpg

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