P585Intramural haemangioma of the interventricular septum in a patient with strong family history of sudden cardiac death

Abstract

Background: Cardiac primary tumors are extremely rare and benign forms are the majority; haemangiomas represent 2-3% of cases. We report the case of a 61-year-old man with strong family history of sudden cardiac death (SCD) who was incidentally diagnosed with cardiac haemangioma. Method(s): The patient was referred to our center after a hypertensive crisis. Basal ECG showed sinus rhythm, left ventricular (LV) hypertrophy with QRS widening and repolarization abnormalities (Fig 1a). Maximal cycle ergometric stress test induced pseudonormalization of the T waves during exercise. Due to these results echocardiography and cardiac magnatic resonance (CMR) were planned. Result(s): Echocardiography showed small LV size with severe septal hypertrophy (30 x 55 mm) and enhanced echo-reflectivity at ultrasound multipulse scheme (eSCAR), no regional wall motion abnormalities nor valvular disease (Fig 1b). CMR revealed severe asymmetric thickening of interventricular septum (maximum thickness 27 mm) due to a subendocardial intramural septal elliptic mass (transverse diameter 22 mm, longitudinal diameter 60 mm) protruding into the LV cavity causing tendency to systolic obliteration. The mass showed peripheral calcification, isointense T1 and hyperintense T2 signal intensity patterns (Fig 1c and 1d). Early and late enhancement of the mass emerged at inversion recovery sequences after Gadolinium administration (Fig 1e). Coronary angiography showed tortuous vessels without stenosis and contrast injection in the left main stem caused opacification of the cardiac mass (Fig 1f). The heart team evaluation contraindicated the surgical removal of the mass due to its position and extension. To screen out malignancies total-body computed tomography (CT) was performed and showed a left parietal posterior brain mass suspicious for neuroglial cyst with no surgical indication, then confirmed at the brain MR (Fig 1g). Considered the familiarity for SCD, an invasive electrophysiological study was performed and resulted negative. After review of the literature, patient was treated with propranolol, already used in cases of infantile cutaneous haemangioma. An implantable loop recorder was implanted before discharge. CMR after 6 months and echocardiography after 12-months follow-up showed no significant changes in mass size and morphology (Fig 1h and 1i). The patient is currently stable under pharmacological treatment. Conclusion(s): Even if histological characterization of the mass is not available, the multimodality imaging approach supports the diagnosis of cardiac haemangioma. Interesting in this case is firstly the septal position of haemangioma, that is very uncommon and a hazard for endo-myocardial biopsy. Secondly, the presence of family history of SCD (it has already been reported that LV haemangioma can induce ventricular fibrillation). Thirdly the additional evidence of brain glioependimal cyst is curious because, as cardiac haemangioma, it is rarely reported in literature.