Listen to the Heart – Rare Cardiac Tumor in an Adolescent

We are reporting about a 13 year old male patient presenting with acute self-relieving chest pain, located midsternally. His medical history revealed an afebrile pharyngitis 2 weeks ago without further complaints like dyspnea or fatigue. There is no cardiovascular disease present among the patientʼs family history. Aside a 2/6 systolic heart murmur located over the 2^nd and 3^rd intercostal space at the left sternal border the physical examination was remarkable. ECG showed no pathologic findings. Performing echocardiography we discovered a 1.1×1.4×1.8 cm mass with a stalk attachment to the endocardium of the right ventricular outflow tract (RVOT), restricting half the diameter of the RVOT and leading to a mild stenosis of RVOT with a maximum gradient of 15 mmHg ([Fig. 1]). The pulmonary valve (PV) was found unremarkable without any impairment of flow pattern across the valve and into the main pulmonary artery. Biventricular function was unimpaired (i. e. LVEF: 66%, tricuspid annular plane systolic excursion: 25 mm, both within age appropriate normal range) and echocardiography revealed no further pathologies. A cardiac magnetic resonance imaging showed no signs of malignancy and allowed for exclusion of thrombus (normal late enhancement pattern) and myxoma (no hyperintensity in T2 sequence) as differential diagnosis and presented papillary fibroelastoma (PFE) to be the most likely diagnosis ([Fig. 2]). Initially, the patient declined surgery and we started him on oral aspirin to prevent grafted thrombus formation. Due to growth of the mass throughout follow-up, surgery using a transvalvular approach necessitating cardiopulmonary bypass was performed. A subsequent histological study revealed a hemangioma.

Fig. 1 echocardiography shows a mass in the right ventricular outflow tract.

Fig. 2 MRI of the cardiac tumor.