1Westpfalz-Klinikum GmbH Klinik für Innere Medizin 2 Kaiserslautern, Deutschland
Background
Compared with secondary masses, the prevalence of primary cardiac tumors is 1:2000. The most common benign primary cardiac tumor are myxomas, cardiac hemangiomas are found in only 2.8% of cases. Most common is an intramural growth, only 25% demonstrate an intracavitary atrial or ventricular growth. Clinical presentation depends on the localization of the mass with decreased exercise tolerance as the most common symptom. Intracavitary masses growing in the interventricular septum may lead to conduction disturbances or sudden cardiac death. This case report describes a 32-year-old patient who was admitted to the hospital due to severe and acute chest pain and dyspnoe.
Case Presentation
Electrocardiography showed unspecific ST-segment elevations in the inferior and posterior leads. The immediately performed coronary angiography could exclude coronary artery disease, but showed a cardiac mass with blood supply from the left anterior descending (LAD) (Figure 1).
Figure 1: RAO30°/caudal30° (left) and LAO0°/caudal35° views (right). Stars indicating the cardiac structure.
Laboratory analysis showed markedly elevated cardiac biomarkers with Troponin T at 16.668 ng/ml (reference: <0.057 ng/ml) and CK 880 U/l (reference: <190 U/l). On bedside echocardiography a cardiac mass in the basal interventricular septum was described. Computertomography revealed an intramyocardial, clearly delineated, homogeneous structure (40 x 17 mm) next to the LAD and ruled out aortic dissection. Cardiac magnetic resonance (CMR) demonstrated a normal sized left ventricle (LVEDVi: 77 ml/m2; reference: 119 ml/m2) with normal systolic LV-function (LVEF: 62%; reference: >53%). In the basal anteroseptal region an isointense cardiac mass (27 x 29 mm) was described. TIRM sequences showed an increased signal intensity of the mass. The mass was supplied by the contrast agent earlier and more intensely. The structure demonstrated an inhomogeneous late gadolinium enhancement (LGE) (Figure 2). The remaining myocardial tissue showed a subepicardial, also intramyocardial LGE lateral/inferior/inferoseptal midventricular towards the apex. In addition, a patchy intramyocardial LGE was found in the septal midventricular region. The working diagnosis after the CMR was hemangioma/hemangiosarcoma.
Figure 2:
Stars indicating the structure in 2-chamber (A, C) and basal short axis (B, D) cine image and PSIR image respectively.
We subsequently performed PET-CT for staging, which did not demonstrate lymphadenopathy or metastases. The thoracic-oncological tumour board recommended surgical resection. Epicardial macroscopic examination could not precisely identify the structure. Following left-sided ventriculotomy, the cardiac mass was localized, and resection of larger parts of the basal septum including the tumor was performed. On histological examination, the mass could be identified as intramyocardial hemangioma.
Conclusion
In summary, this case report demonstrated an uncommon localization of an intramural cardiac hemangioma. The clinical presentation occurred acutely due to the unfavorable site and blood supply by the LAD. CMR was a very sensitive modality to identify, locate and describe the cardiac mass. The hypervascularity was described by CMR, but the blood supply could only be definitely demonstrated by coronary angiography supporting a combined diagnostic approach to these patients.