Case presentation: In December 2022, A 32-y old women presented to the emergency department of our institution for pain and acrocyanosis of the 3rd and 4th finger of the right hand, compared abruptly 10 hours before. Physical examination was otherwise normal, she was apyretic. Her previous medical history was unremarkable, she did not take any medication and was a light smoker. Blood samples including blood count, platelets, coagulation tests, liver and kidney function, and inflammatory markers were normal. An echocardiogram was prescribed to rule out significant cardiovascular disease. The exam showed: normal left and right ventricular dimensions and systolic function, and absence of significant valvular disease. A highly mobile, iso-echoic mass was noted inside the left ventricle, with a major diameter of 8 mm; it was attached to the antero-lateral papillary muscle, not involving the mitral valve leaflets, and it had a frond-like appearance. Findings were consistent with a fibroelastoma (PFE; figure 1, 2). Doppler examination excluded pathology of ascending aorta, right subclavian artery, and demonstrated patency of arteries of the right arm and right hand. The case was compatible with peripheral embolization from the cardiac mass. A cardiac CT scan confirmed the echo findings and ruled out significant coronary disease. The patient underwent cardiac surgery two days later. With median sternotomy and cardiopulmonary bypass, the left ventricle was reached through aortotomy. A multilobular yellowish mass was seen attached to the antero-lateral papillary muscle, and was completely resected (figure 3); chordae tendineae were preserved. Macroscopic examination (figure 3) and histological findings confirmed the diagnosis of PFE. Post operative course was unremarkable.
Discussion: PFEs are benign tumors most commonly involving aortic valve, followed by mitral valve; atypical origins including mitral subvalvular apparatus, as in our case, has seldom been described. Most cases of PFE are asymptomatic, limited in dimensions and discovered incidentally. Interestingly, Raynaud phenomenon was at first suspected as cause of acrocyanosis in our patient, but it was inconsistent with biochemical findings, so that an echocardiogram was prescribed. Echo and CT aspects of the mass were typical for PFE, and it appeared at high embolic risk being pedunculated and highly mobile. Given the peripheral ischemic event occurred, prompt surgical resection was performed successfully.