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Published on: February 17, 2018
A complex clinical case of intracardiac leiomyomatosis
Yuanzhi Li1, Yidan Li1, Min Liang2
1Department of Ultrasound, Beijing Chao Yang Hospital, Capital Medical University, No. 8 Gongren Tiyuchang Nanlu, Chaoyang District, Beijing, 100020, China.
Insights
Intracardiac leiomyomatosis (ICL) is a rare condition where tumors invade the heart. Echocardiography is key for diagnosis, with CT and MRI aiding in assessing tumor extent and prognosis.
Area of Science:
- Cardiovascular Medicine
- Oncology
- Radiology
Background:
- Intracardiac leiomyomatosis (ICL) is a rare condition involving intravascular tissue proliferation and tumor emboli.
- Tumor invasion of cardiac structures poses a life-threatening risk.
- Diagnosis of ICL is complex and challenging.
Purpose of the Study:
- To present a case of intravenous leiomyomatosis (IVL) with cardiac involvement.
- To highlight the diagnostic utility of various imaging modalities in ICL.
Main Methods:
- A case report of a 38-year-old female patient with suspected ICL.
- Pulmonary artery computed tomography (CT) identified intrapulmonary artery structures.
- Echocardiography revealed intracardiac and pulmonary artery masses.
- Magnetic resonance imaging (MRI) delineated the pelvic mass and vascular extension.
- Histopathological analysis confirmed IVL post-surgery.
Main Results:
- CT showed low-density structures in pulmonary artery branches.
- Echocardiography revealed mobile tumors in the right heart and pulmonary trunks, with ventricular septal thickening.
- MRI identified a uterine mass extending into the inferior vena cava (IVC) and right iliac vein.
- Histopathology confirmed intravenous leiomyomatosis.
Conclusions:
- Echocardiography is the primary diagnostic tool for IVL affecting the heart.
- CT and MRI are crucial for determining tumor location, extent, and prognosis.
- Early and accurate diagnosis through integrated imaging is vital for patient management.
Background:
Intracardiac leiomyomatosis (ICL) is an uncommon condition characterized by the proliferation of intravascular tissue within the veins, leading to the development of tumor emboli. This can pose a significant threat to life when the tumor invades cardiac structures. The diagnostic process for this condition is complex and presents considerable challenges.
Case Presentation:
We report a case of a 38-year-old female patient whose pulmonary artery computed tomography (CT) revealed low density structure in the branches of the pulmonary artery. Echocardiography revealed a mobile tumor within the right heart chambers and pulmonary trunks as well as characteristic thickening of the ventricular septum consistent with hypertrophic cardiomyopathy (HCM). Magnetic resonance imaging (MRI) revealed a mass in the right anterior uterine wall, extending to the inferior vena cava (IVC) and right iliac vein. Post-surgery histopathological analysis confirmed a diagnosis of intravenous leiomyomatosis (IVL).
Conclusions:
When IVL affects the heart, echocardiography is the best diagnostic tool for detecting the disease. CT and MRI are essential in identifying the location and extent of the tumor, as well as in evaluating prognosis.
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