Introduction
Primary cardiac tumours are rare. They can be asymptomatic and identified incidentally or present with non-specific symptoms including arrhythmia, syncope, chest tightness, or heart-failure-related symptoms based on their location and the electro-mechanical effects. Reference Koçak, Ozyazicioğlu, Gündoğdu and Sevimli1 We report a patient with right ventricular cavernous haemangioma, in which non-invasive cardiovascular imaging including echocardiography, and MRI helped in the diagnosis with a high pretest probability.
Case report
A 12-year-old boy was referred to our paediatric cardiology clinic for the evaluation of palpitations that he reported over five months prior. These episodes were not associated with syncope, dizziness, dyspnoea, or chest pain. He was active, played football and baseball, and was otherwise healthy. His clinical examination including vital signs was normal.
At the clinic, the patient had a 12-lead electrocardiogram that showed sinus bradycardia (heart rate 50 bpm) without preexcitation. A 14-day extended Holter monitor showed several narrow and wide complex tachycardia episodes at 180–195 bpm, likely supraventricular tachycardia with aberrancy. He had a transthoracic echocardiogram (Philips Healthcare, Andover, MA, USA) that showed a large mass measuring 2.1 x 2.9 x 3.4 cm attached to the anterolateral wall of the right ventricular (Figure 1 and Video 1). The mass did not appear to cause any right ventricular inflow or outflow obstruction, and there was no evidence of blood flow within the mass by colour Doppler (Video 2).
2D TTE. Apical four-chamber view shows a large mass measuring 2.1 x 3.4 cm attached to the anterolateral aspect of the RV. RA = right atrium; RV = right ventricle.
The patient underwent a cardiovascular MRI study on a 1.5 T scanner (Siemens Healthineers, Erlangen, Germany) for tissue characterisation of the mass. Cardiovascular MRI confirmed the presence and the location of the mass by steady-state free-precession cine sequence (Video 3). The mass was hyperintense in T2-weighted imaging with fat suppression; T1 (ShMOLLI) and T2 mapping demonstrated prolonged times at 1567 and 72 msec, respectively (Figure 2). First-pass perfusion imaging showed an uptake of the contrast within the mass (Video 4), which gave the impression that it was well-vascularised. It was enhanced on late gadolinium enhancement imaging and did not have hypo-intensity on long TI imaging (Figure 3), which excluded the presence of thrombus within the mass. These findings suggested that the mass was of vascular origin. Differential diagnoses included haemangioma and other vascular tumours.
CMR. panel (a). Dark-blood T2-weighted turbo spin echo (TSE) with fat-suppression sequence, shows the mass (arrow) as hyperintense. panel (b): T1 mapping shMOLLI and panel (c): T2 mapping show the mass (arrowheads) has prolonged T1 and T times at 1567 and 72 msec, respectively. LV = left ventricle, RV = right ventricle.
CMR. panel (a). Long TI shows the mass (arrow) is hyperintense with no hypodensity within it. panel (b). LGE shows the mass (arrow) enhanced. LV = left ventricle; RV: right ventricle.
After a multidisciplinary discussion, the patient underwent an electrophysiological study, which showed inducible atrioventricular nodal reentrant tachycardia which was unrelated to the mass (Figure 4). He had successful cryoablation of the slow pathway region. Subsequently, he underwent surgical resection of the right ventricular mass (measured 4 x 3.6 x 1.5 cm). Histopathological examination showed a poorly circumscribed lesion containing predominantly thin-walled capillary structures insinuating between the native cardiac myocytes and collections of pericytes without atypia or necrosis (Figure 5). Immunoperoxidase stains were performed with appropriate controls. CD31 and CD34 highlighted the tumour endothelial cells and smooth muscle actin highlighted the pericytes. The morphologic and immunohistochemical features were indicative of an intramural cavernous haemangioma. His postoperative course was uneventful, and he had a 24-month follow-up in the outpatient clinic without reporting any complaints and the transthoracic echocardiogram did not show a recurrence of the mass.
Typical slow/fast atrioventricular nodal reentrant tachycardia.
Panel (a). Gross specimen after surgical resection of the RV mass. panel (b). Histopathology shows thin-walled capillary structures between the native cardiac myocytes and collections of pericytes without atypia or necrosis.
Discussion
About 75% of cardiac tumours are benign and cardiac haemangioma represents 2–9 % of these tumours. Reference Koçak, Ozyazicioğlu, Gündoğdu and Sevimli1–Reference Padalino, Vida and Boccuzzo3 Although intracardiac haemangiomas have been previously reported, we shed light on the role of cardiac imaging in anatomical delineation, hemodynamic effects, and tissue characterisation of the tumour.
Transthoracic echocardiogram is usually the first approach modality to diagnose intracardiac tumours. Reference Patel, Lim and Saric4 It can provide an accurate delineation of the anatomy and the hemodynamic effects of the tumour; however, it has limitations, especially in older populations including poor acoustic window and decreased ability to characterise the tissue. The use of contrast and perfusion echocardiography can increase the accuracy of the study. Reference Abdelmoneim5 Transesophageal echocardiography on the other hand has a higher special resolution. Reference Calderone, Tuck, Gray, Porter and Rais-Bahrami6
Cardiac CT has the highest spatial resolution and a higher capability of defining the anatomy including the extracardiac structure, but the disadvantages include radiation exposure and iodine-based contrast, which can predispose to kidney injuries or trigger allergies. Reference Lopez-Mattei, Yang, Ferencik, Baldassarre, Dent and Budoff7 Measuring the density of tumours in Hounsfield units can give an idea about tissue composition. In addition, delayed enhancement imaging can be useful to exclude blood clots within the tumour. Reference Tyebally, Chen and Bhattacharyya8
Cardiovascular MRI can provide a precise delineation of anatomy, volumetrics, and function. Besides, it has a wide field of view and numerous planes of imaging. Reference Cavalcante, Lalude and Schoenhagen9 It can discriminate multiple tissue compositions based on various imaging sequences (Table 1). Characteristics of vascular tumours including haemangioma on cardiovascular MRI include clearly positive (iso- or hyper-intense) first-pass perfusion sequence, variable, often weak enhancement (iso- or mildly hyper-intense) on myocardial delayed enhancement imaging and variable location. Reference Beroukhim, Prakash and Buechel10 In a paediatric series that characterised cardiac tumours by cardiovascular MRI, age at presentation for vascular tumours ranged from birth to 13 years, 44% of patients were male, and 55% were diagnosed prenatally. Reference Beroukhim, Prakash and Buechel10 The largest dimension of haemangiomas ranged from 1.4 to 4.4 cm, all were single, 90% had heterogenous appearance, 80% were associated with either inflow or outflow obstruction or valve regurgitation related to the tumour, and 20% were associated with pericardial effusion. Reference Beroukhim, Prakash and Buechel10
MRI-based characteristics of different types of intracardiac masses
The patient also had atrioventricular nodal reentrant tachycardia, which was an incidental finding that prompted cardiac evaluation but was not related to the tumour.
Conclusion
Primary cardiac tumours are rare and can present with non-specific presentations. Although biopsy is the gold standard for diagnosis, thanks to advancements in cardiovascular imaging modalities, we can define them and characterise the tissue with a high pretest probability.
Supplementary material
The supplementary material for this article can be found at https://doi.org/10.1017/S1047951125101261.
Data availability statement
Majority of data associated with this study are provided in text and figures. Additional data will be made available upon request.
Funding statement
None.
Competing interests
All authors have reported that they have no relationships relevant to the contents of this paper to disclose.
Ethical standard
Informed written consent was obtained from the patient or their parents as applicable, as per West Virginia University IRB guidelines.
