Tricuspid valve papillary fibroelastoma: an unusual case of dizzy spells

Introduction

Papillary fibroelastomas are rare tumours and generally considered benign ¹ . Although usually asymptomatic, they can be associated with syncope, chest pain, myocardial infarction (MI), stroke, intermittent dyspnoea, and sudden death^2-4. MI or sudden cardiac death may be due to embolization of a portion of the tumour into a coronary artery ⁵ . Furthermore, there have been reports of pulmonary embolization from right-sided papillary fibroelastomas ⁶ . Papillary fibroelastomas account for less than 10% of all primary cardiac tumours ⁷ . We report a case of occasional dizzy spells found to be due to papillary fibroelastoma of the tricuspid valve.

Case report

An 81-year-old Caucasian woman was referred from her local hospital for surgical treatment of a right atrial mass. She presented with a four-month history of occasional dizzy spells, with some breathlessness on exertion, as well as lethargy. Her past medical history was unremarkable and she was not on any medication. Physical examination was unremarkable. All blood tests were unremarkable. Electrocardiography revealed normal sinus rhythm and all laboratory blood results were within normal limits. Chest radiography revealed a prominent hilum on the right side, but the computed tomography (CT) scan did not show any abnormality. Preoperative imaging revealed no significant carotid disease. Left heart catheterization was unremarkable. Preoperative transthoracic echocardiography (TTE) showed a 2.0cm mass in the right atrium (Figure 1- A, B). All other investigations carried out by physicians to delineate other causes for dizzy spells were normal.

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Figure 1.

(A) Transthoracic echocardiography right ventricular inflow view showing the fibroelastoma (arrow) during systole. RV = right ventricle, RA = right atrium.

Perioperative transoesophageal echocardiography (TOE) at the time of surgery demonstrated a spherical, pedunculated mass in the right atrium, with connective tissue reflectance measuring 2x2.5cm arising from the anterior leaflet of the tricuspid valve very near to the commissure between the septal and the anterior leaflets. It was prolapsing in and out of the right ventricle with movement of the valve and there was mild to moderate tricuspid regurgitation. The right atrium was not enlarged and the rest of the heart was structurally normal, with good biventricular function. The patient underwent elective surgery. Cardiopulmonary bypass was established with aorto-bicaval cannulation. The patient was cooled to 28°C and the heart was arrested using antegrade blood cardioplegia. The right atrium was opened and the attachment of the tumour to the anterior leaflet was excised and sent for histology. The defect in the leaflet was closed with a patch of autologous pericardium. As the tricuspid valve appeared to be myxomatous, a size 28mm Carpentier-Edwards annuloplasty ring was inserted. There was another area of what appeared to be abnormal tissue overlying the anterior leaflet of the tricuspid valve which was not excised. The valve was tested and found to be competent. The patient was re-warmed and weaned from CPB without problems. TOE at the time confirmed no tricuspid regurgitation.

Histopathology showed two fragments of soft, whitish, gelatinous tissue, typical of a papillary fibroelastoma with no atypia. Postoperative transthoracic echocardiography (TTE) on day 4 demonstrated complete excision of the mass (Figure 1C). There was good right ventricular function with mild tricuspid valve regurgitation. The postoperative recovery was unremarkable. The patient was discharged on day seven in sinus rhythm; however, she was started on oral anticoagulant therapy for three months. The patient was reviewed in the clinic four weeks later and described that the dizzy spells had completely disappeared since the operation.

Discussion

Primary cardiac tumours have an estimated echocardiographic incidence of 0.019% and an estimated incidence of 0.0017-0.33% in autopsy series ⁸ . Papillary fibroelastomas are usually small tumours arising from the heart valves and constitute 7% to 8% of cardiac tumours in autopsy series ⁹ . Uncommonly, they arise from the endocardial wall ¹⁰ and most often are single, but multiple tumours, especially arising from the left side of the heart, have been reported ¹¹ . They are found with almost equal frequency on both sides of the heart and on all valves ¹² .

Although the etiology of papillary fibroelastomas is unknown, there are theories that they result from mechanical trauma (patients with long-standing heart disease) or that they represent inflammatory nodules or hamartomas ¹³ . The first explanation may be the reason as to why they are found more often in elderly patients rather than in children ¹³ .

They are usually incidental findings when echocardiography is performed, but, rarely, they have been diagnosed after investigation for symptoms such as chest pain and dyspnoea, or due to thromboembolic events^2,4,14,15. They may provide a focus for fibrin and platelet aggregation, leading to embolization. Emboli may take the form of either tumour fragments or thrombi released from deposits on the tumour’s surface and can cause life-threatening complications^16-19.

Echocardiography remains the gold standard of diagnosis for papillary fibroelastomas and TOE is superior in differentiating it from thrombus or vegetation ²⁰ . Intraoperative TOE is extremely useful in demonstrating complete excision, as well as competence of the valve following repair.

In our case, the symptom of dizzy spells that led to the discovery of the tumour has not been reported before in the literature. There are previous reports published in which clinical symptoms and signs were attributed to tricuspid papillary fibroelastomas, including intermittent dyspnoea, pulmonary embolization, cyanotic episodes and congestive heart failure^4-6,21,22.

Although we don’t know the exact aetiology behind the symptoms, we hypothesize that the size of the mass and the location may have compromised the cardiac output, causing an occasional drop and leading to the dizzy spells.

Provided there is no major contraindication to operation, large papillary fibroelastomas may be managed surgically. Surgical resection sometimes requires valve repair or replacement if the valve is critically involved. Prophylactic anticoagulation is advised once the diagnosis has been confirmed and for a short duration postoperatively. Although the incidence of recurrence is unknown, surgical treatment of papillary fibroelastomas has good long-term results. However, careful follow-up with echocardiography is recomended.^4,23

We would like to highlight that physicians should be aware of neurological symptoms such as dizzy spells as a potential symptom of tricuspid valve papillary fibroelastoma.