Abstract
Cardiac papillary fibroelastomas (CPFE) are exceptional primary benign cardiac tumours affecting the heart valves. We report here the case of a 15-year-old boy in whom echocardiography performed for non-specific chest pain during follow-up for bicuspid aortic valve showed as accidental finding the presence of a round mobile mass without stalk attached on the inferior side of the aortic valve. The mass did not cause any outflow tract obstruction or aortic insufficiency. Electrocardiogram-gated cardiac computed tomography and magnetic resonance imaging allowed to suspect CPFE. Although the patient was asymptomatic, open cardiac surgery with elective surgical resection of the tumour was performed to avoid systematic emboli. Histology confirmed the diagnosis of CPFE. This is an exceptional case of acquired CPFE in a young patient with bicuspid aortic valve. Due to the risk of systemic embolization, aortic or coronary ostium obstruction, elective excision of such lesions is recommended.
Highlights
Cardiac papillary fibroelastomas (CPFE) are exceptional primary cardiac tumours with a reported incidence lower than 0.03% in adults.[1,2,3,4,5] They arise from the cardiac endothelium and principally affect the heart valves.[1]
We report the case of a 15-year-old boy with CPFE on a bicuspid aortic valve, accidentally discovered by echocardiography at follow-up
CPFE are extremely rare in children
Summary
Cardiac papillary fibroelastomas (CPFE) are exceptional primary cardiac tumours with a reported incidence lower than 0.03% in adults.[1,2,3,4,5] They arise from the cardiac endothelium and principally affect the heart valves.[1]. The lesion is well depicted at transthoracic and/or transesophageal echocardiography.[3,6] Cardiac magnetic resonance imaging and/or Electrocardiogram-gated cardiac computed tomography (ECG-gated TDM) may be helpful to precise the diagnosis of CPFE.[6,7] Owing to the high risk of embolization in the pediatric population, prophylactic tumour excision is considered to be the definitive and safe treatment of choice.[1,3]. We report the case of a 15-year-old boy with CPFE on a bicuspid aortic valve, accidentally discovered by echocardiography at follow-up. A former TTE performed 4 years earlier in the context of palpitations had showed a thin bicuspid aortic valve without any stenosis or insufficiency. An ECG-gated cardiac TDM confirmed the presence of a bicuspid aortic valve. Cardiac magnetic resonance imaging (MRI) performed during preoperative assessment confirmed the diagnosis of CPFE (Figure 2B). The anatomopathological analysis confirmed the nature of the lesion (Figure 3). ©Copyright: the Author(s), 2019 Licensee PAGEPress, Italy Clinics and Practice 2019; 9:1135 doi:10.4081/cp.2019.1135
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