Incidental finding of a giant intracardiac angioma infiltrating both ventricles in a 35-year-old woman: a case report
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Primary cardiac tumors are rare and often asymptomatic or present with unspecific symptoms. Benign cardiac tumors of vascular origin are especially rare, with only few existing data in the literature.
A 35-year-old Caucasian female patient presented to our department with an asymptomatic giant intracardiac angioma infiltrating both ventricles. Evaluation of this tumor involved electrocardiography, echocardiography, cardiac magnetic resonance imaging, coronary angiography, an open myocardial biopsy, and histological examination of the resected specimen. Because our patient was asymptomatic, she was managed conservatively with regular follow-up. We discuss the treatment options available in comparison with similar cases.
Diagnosis and therapy of benign cardiac tumors, especially of asymptomatic lesions, can be a challenge. There is no evidence available to help in the management of such patients. An extensive evaluation is needed with different imaging modalities, and case-specific decisions should be made that involve experts in cardiology, cardio-oncology, and heart surgery.
KeywordsCMR Cardiac tumor Echocardiography Intracardiac angioma
cluster of differentiation 31, also known as platelet endothelial cell adhesion molecule
cluster of differentiation 34, also known as hematopoietic progenitor cell antigen
cardiac magnetic resonance (imaging)
Primary cardiac tumors are rare and their incidence ranges from 0.0017 to 0.28 % as reported in autopsy studies . The most common benign tumors of the heart are myxomas, followed by lipoma, papillary fibroelastoma, angioma, fibroma, hemangioma, rhabdomyoma, and teratoma. Only about 5 % of all benign cardiac tumors are angiomas , and diagnosing them is often difficult. Many of the primary cardiac tumors are asymptomatic and are detected postmortem. If these tumors are symptomatic, embolization, obstruction, and arrhythmogenesis are the major modes of presentation .
Discussion and Conclusions
Primary cardiac neoplasms are rare and have an incidence range of 0.0017–0.28 % as reported in autopsy studies . Only about 5 % of all benign cardiac tumors are angiomas [1, 2]. On histology, hemangiomas can be classified into cavernous type, capillary type, and arteriovenous type. The most common location for a cardiac hemangioma is the left ventricle, followed by the right atrium, right ventricle, and left atrium. Hemangiomas can present at any age in both genders .
We report the case of a 35-year-old Caucasian female patient with an incidental finding of a large intracardiac angioma that represented a challenge for diagnosis and therapy. Echocardiography and CMR imaging are the most important diagnostic modalities for the evaluation of intracardiac masses [5, 6]. In addition, coronary angiography is helpful for further characterization of the tumor (for example, the degree of vascularization and relationship to the coronary arteries). Our patient was evaluated extensively by all the methods mentioned above in order to make the best treatment decision.
There are very limited data with respect to the treatment of patients with these extremely rare benign intracardiac vascular tumors . Total surgical excision of benign angiomas is not routinely undertaken because of the highly vascular nature of these tumors, unless there are severe symptoms like ventricular tachycardia or cardiac tamponade . There are some reports describing severe complications like ventricular arrhythmias or sudden cardiac death . Only a few surgical resections of right ventricular hemangiomas have been reported in the literature; most of them were symptomatic . Treatment of an asymptomatic benign intracardiac angioma remains controversial, because the evolution of such lesions is unpredictable—it may be constant in size, but can proliferate and grow uncontrollably. Fan et al. recently reported the total surgical resection of an incidentally discovered large hemangioma attached to the left ventricle with good results after 20 months of follow-up . Alternatively, other authors suggest conservative management, especially when the patient is asymptomatic, the tumor is not compressing any major structures, the patient is not hemodynamically compromised, and when surgical risk is high [4, 11]. For example, Gribaa et al. reported a case of a left-sided cardiac hemangioma in an adult woman that was managed conservatively for 11 years without major complications .
The choice between surgical and conservative management should be made on an individual basis for each patient. Malign arrhythmias, congestive heart failure, ventricular outflow tract obstruction, coronary insufficiency, and higher grade atrioventricular blocks are especially alarming symptoms that may promote a surgical approach. Interdisciplinary discussion involving the cardiologist, cardiac surgeon, and oncologist should be undertaken in such cases.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
Short axis multislice on cardiovascular magnetic resonance imaging (AVI 35056 kb)
Three-chamber view on cardiovascular magnetic resonance imaging (AVI 30355 kb)
Perfusion in four-chamber view on cardiovascular magnetic resonance imaging (AVI 15915 kb)
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