心脏内平滑肌瘤病：临床发现及详细超声心动图特征——一项中国机构的经验

Intracardiac leiomyomatosis: clinical findings and detailed echocardiographic features--a Chinese institutional experience.

作者信息

Gu Xiaoyan, He Yihua, Li Zhian, Chen Jian, Liu Wenxu, Zhang Ye, Nixon J V Ian

机构信息

Department of Ultrasound, Beijing Anzhen Hospital, Capital Medical University, Beijing, China; Pauley Heart Center, Virginia Commonwealth University School of Medicine, Richmond, Virginia.

出版信息

J Am Soc Echocardiogr. 2014 Sep;27(9):1011-6. doi: 10.1016/j.echo.2014.04.018. Epub 2014 Jun 6.

DOI:10.1016/j.echo.2014.04.018

PMID:24909789

Abstract

BACKGROUND

Intravenous leiomyomatosis is a rare, benign, smooth muscle tumor originating in the uterus that may extend through the inferior vena cava into the heart. Intracardiac leiomyomatosis (ICL), present in 10% of patients with intravenous leiomyomatosis, may cause right heart failure, tricuspid valve obstruction, and pulmonary embolism. The imaging characteristics of ICL continue to be reported. The purposes of this study were to characterize the echocardiographic features of ICL and to correlate the clinical findings.

METHODS

Between 1999 and 2012, 12 female patients with suspected ICL underwent cardiac surgery and histologic confirmation of the tumor. The clinical data, echocardiographic findings, and histologic results were retrospectively reviewed.

RESULTS

The ages of the patients with ICL ranged from 40 to 59 years. Ten patients (83%) had undergone myomectomy or hysterectomy, one patient had a uterine fibroid, and one patient had endometriosis. Seven patients (58%) reported dyspnea and/or palpitations, and one patient had syncope; four patients were asymptomatic. Echocardiographic findings included six patients with homogenous right atrial masses, four patients with myxoma-like right atrial masses, and two patients with serpentine, convoluted right atrial masses. In nine patients, the right atrial masses were noted to cross the tricuspid valve. All masses extended from the inferior vena cava. No masses appeared to adhere to the right atrium, right ventricular or pulmonary arterial walls, or tricuspid valve. Tricuspid regurgitation was noted in all patients. No pulmonary emboli were present.

CONCLUSIONS

The echocardiographic features of the ICL tumors varied. Tricuspid regurgitation and tumors emerging from the inferior vena cava were seen in all patients. Cardiac symptoms, including dyspnea, palpitations, and syncope, occurred in 67% of patients; the remaining 33% were asymptomatic.

摘要

背景

静脉内平滑肌瘤病是一种罕见的良性平滑肌肿瘤，起源于子宫，可经下腔静脉延伸至心脏。心内平滑肌瘤病（ICL）见于10%的静脉内平滑肌瘤病患者，可导致右心衰竭、三尖瓣梗阻和肺栓塞。ICL的影像学特征仍在不断报道。本研究的目的是描述ICL的超声心动图特征并关联临床发现。

方法

1999年至2012年间，12例疑似ICL的女性患者接受了心脏手术及肿瘤的组织学确诊。对临床资料、超声心动图结果和组织学结果进行回顾性分析。

结果

ICL患者年龄在40至59岁之间。10例患者（83%）曾接受肌瘤切除术或子宫切除术，1例患者有子宫肌瘤，1例患者有子宫内膜异位症。7例患者（58%）报告有呼吸困难和/或心悸，1例患者有晕厥；4例患者无症状。超声心动图结果包括6例右心房均质肿块患者、4例黏液瘤样右心房肿块患者和2例蜿蜒、盘绕的右心房肿块患者。9例患者右心房肿块穿过三尖瓣。所有肿块均从下腔静脉延伸。未见肿块附着于右心房、右心室或肺动脉壁或三尖瓣。所有患者均有三尖瓣反流。无肺栓塞。