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血流动力学查房与临床病理相关性:以影像学、血流动力学及心内膜心肌活检为指导对一名红细胞增多症患者的评估

Hemodynamic rounds and clinical pathology correlation: Evaluation of a polycythemic patient guided by imaging, hemodynamics, and endomyocardial biopsy.

作者信息

Sagar Pramod, Sivakumar Kothandam

机构信息

Department of Pediatric Cardiology, Madras Medical Mission, Institute of Cardio Vascular Diseases, Chennai, Tamil Nadu, India.

出版信息

Ann Pediatr Cardiol. 2021 Oct-Dec;14(4):516-520. doi: 10.4103/apc.apc_247_20. Epub 2022 Mar 25.

Abstract

A 32-year-old male with symptomatic polycythemia was investigated by hematologist for myeloproliferative malignancies and underwent five therapeutic phlebotomies. The identification of hypoxia on pulse oximetry later led to pulmonology evaluation that showed normal lung function tests as well as normal lung parenchyma and airways on advanced imaging. The absence of murmurs and significant precordial findings, normal chest X-ray, and unremarkable findings in electrocardiogram apart from first-degree heart block delayed the cardiac referral. Cardiac imaging showed a significant right to left shunt through a large oval fossa defect, mild hypoplasia of the right ventricle, normal right ventricular function and mild fibrosis within the myocardium. Hemodynamic assessment with test balloon occlusion led finally to a complete closure of the defect, which normalized the hypoxia. A step-by-step clinical approach finally leading to the management is presented in this clinicopathology correlation and hemodynamic rounds.

摘要

一名32岁有症状性红细胞增多症的男性患者,血液科医生对其进行骨髓增殖性恶性肿瘤检查,并接受了5次治疗性放血。后来通过脉搏血氧测定法发现低氧,这促使进行了肺科评估,结果显示肺功能测试正常,高级影像学检查显示肺实质和气道也正常。由于没有杂音和明显的心前区异常发现、胸部X线正常、心电图除一度房室传导阻滞外无异常表现,导致心脏转诊延迟。心脏成像显示通过一个大的卵圆孔未闭存在明显的右向左分流、右心室轻度发育不全、右心室功能正常以及心肌内轻度纤维化。用测试球囊封堵进行血流动力学评估最终使缺损完全闭合,低氧情况得以正常化。本临床病理相关性及血流动力学分析介绍了最终实现治疗的逐步临床方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4b37/9075555/a1082241390e/APC-14-516-g001.jpg

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