CARDIOVASCULAR JOURNAL OF AFRICA • Volume 35, No 1, January – April 2024 66 AFRICA The patient recovered well after surgery and was discharged on postoperative day eight. Echocardiography performed at three-month intervals postoperatively did not reveal any evidence of recurrence and the patient remains asymptomatic. Discussion Cardiac fibromas are uncommon, benign, solitary tumours that occur in children and adults. These tumours account for 12–16% of primary cardiac tumours in children but for less than 5% of all cardiac tumours in adults. The majority of cardiac fibromas are found in the left (60%) or right ventricle (30%), with a minority in the right atrium (10%).2,7 In the gross specimen, a cardiac fibroma is firm, well restricted but not encapsulated, grey-white in colour, and typically does not grow to a large size. Calcification of the central portion is an important characteristic of a cardiac fibroma that distinguishes this tumour from a rhabdomyoma and indicates a poor blood supply. The myocardium can interdigitate with a cardiac fibroma at the tumour border and replace functioning muscle, leading to intractable congestive heart failure. Histopathological examination of a cardiac fibroma reveals fibroblasts embedded in collagen and varying numbers of elastic fibres.4 Sometimes, necrosis and cystic degeneration may be observed.3,4 In our case, the cardiac fibroma originated from the myocardium of the right ventricle and received its blood supply via the distal left anterior descending artery. The tumour was calcified and firm with a smooth, yellowish surface. Echocardiography is the preferred imaging method in view of its ready accessibility and non-invasive nature.2 Furthermore, computed tomography and magnetic resonance imaging (MRI) can localise the tumour and characterise the tissue and surrounding structure.2,3 MRI can also provide additional functional data, reveal the underlying pathological mechanisms, and evaluate the haemodynamic effects of this tumour.5 The clinical manifestations of cardiac fibroma vary in severity according to the position and size of the tumour.4 Although many patients with this condition are asymptomatic, about 50% present with symptoms, including inflowandoutflowobstruction, angina, arrhythmias and conduction disorders. Heart failure is the most common clinical manifestation, followed by chest pain, syncope and sudden death.5,8-10 Our patient presented with chest tightness and nausea because the calcified mass was compressing part of the myocardium and causing cardiac ischaemia. Cardiac fibroma rarely resolves spontaneously and surgical resection should be considered in symptomatic patients.5-10 Asymptomatic individuals require long-term follow up and may need surgery to avoid complications.5 Complete removal is the first goal of surgery for a cardiac fibroma. However, although this tumour was benign and had an easily distinguishable excision plane, partial excision might have been preferable if complete resection was not possible, because of a high risk of damage to essential cardiac structures, despite the higher risk of recurrence. The option of heart transplantation remains available if irreparable cardiac damage is suspected during tumour resection. Cardiopulmonary bypass is usually required intra-operatively to remove a cardiac fibroma without causing unnecessary injury to surrounding structures when the tumour is located within a ventricle. In our patient, who was symptomatic, cardiopulmonary bypass was not required for complete excision of the tumour because it was located on the surface of the right ventricular wall and there was a clear border between the tumour and the myocardial tissue, and the right ventricular wall was preserved intact. Conclusions This report describes an unusual calcified fibroma in the right ventricle in an adult. Surgical excision is an effective way of improving the prognosis in a symptomatic patient. The study was supported by a grant from the Zhejiang Provincial Education Department Scientific Research Fund (number Y201738814). The authors thank Liwen Bianji (Edanz) (www.liwenbianji.cn) for editing the English text of a draft of this manuscript. References 1. Burke A, Virmani R. Pediatric heart tumours. Cardiovasc Pathol 2008; 17: 193–198. 2. Ünsal H, Ekici E. Conservative management of a left ventricle cardiac fibroma in an asymptomatic child patient. Turk Kardiyol Dern Ars 2015; 43: 481–483. 3. Tao TY, Yahyavi-Firouz-Abadi N, Singh GK, et al. Pediatric cardiac tumors: clinical and imaging features. Radiographics 2014; 34: 1031– 1046. 4. Burke A, Tavora F. The 2015 WHO classification of tumors of the heart and pericardium. J Thorac Oncol 2016; 11: 441–452. 5. Zheng XJ, Song B. Left ventricle primary cardiac fibroma in an adult: A case report. Oncol Lett 2018; 16: 5463–5465. 6. Gowdamarajan A, Michler R. Therapy for primary cardiac tumors: is there a role for heart transplantation? Curr Opin Cardiol 2000; 15: 121–126. 7. De Montpreville VT, Serraf A, Aznag H, et al. Fibroma and inflammatory myofibroblastic tumor of the heart. Ann Diagn Pathol 2001; 5: 335–342. 8. Burke AP, Rosado-de-Christenson M, Templeton PA, et al. Cardiac fibroma: clinicopathologic correlates and surgical treatment. J Thorac Cardiovasc Surg 1994; 108: 862–870. 9. Darwazah AK, Shoeb J, Eissa SS. Pedunculated endocardial left ventricular fibroma presenting with cerebral and bilateral peripheral embolization. Ann Thorac Surg 2010; 89(3): 965–967. 10. Cho JM, Danielson GK, Puga FJ, et al. Surgical resection of ventricular cardiac fibromas: early and late results. Ann Thorac Surg 2003; 76: 1929–1934.
RkJQdWJsaXNoZXIy NDIzNzc=