Multiple papillary fibroelastomas in the right heart

Abstract

Background:

Cardiac multiple papillary fibroelastonas are rare.

Methods and Results:

A 70-year-old woman presented with an exertional dyspnea. A trans-thoracic echocardiogram showed a moving mass near the inferior vena cava in the right atrium. She underwent an operation for a cardiac tumor. A trans-esophageal echocardiogram during the operation showed a mass in the right ventricle, which had echo properties similar to the mass in the right atrium. The mass in the atrium was resected including the attached intima, and the mass in the ventricle was resected by the shave method. Her postoperative course was uneventful. A pathological examination showed that the two tumors were papillary fibroelastomas.

Conclusion:

A precise examination should be performed when a cardiac tumor is diagnosed to investigate the possibility of multiple lesions.

Introduction

Myxoma has the highest prevalence among benign tumors, followed by lipoma, papillary fibroelastoma, and others. Papillary fibroelastomas usually develop on a left-sided valve leaflet, and they can appear in the right heart. Basically, papillary fibroelastoma is a solitary lesion, and multiple fibroelastomas are rarely recognized. Herein, we report a case of multiple fibroelastomas in the right heart.

Case

A 70-year-old woman presented with an exertional dyspnea. She received radiotherapy associated with chemotherapy for esophageal cancer 10 years ago and had suffered from autoimmune hepatitis for several years. On examination, her blood pressure was 112/50 mmHg without arrhythmias. Normal heart sounds and normal vesicular sounds were audible.

Laboratory findings showed a white blood cell count of 7800 (μlL-(1 and a slight elevation in levels of serum aspartate aminotransferase (32  mU mL⁻¹), γ-glutamyltransferase (2 mU mL⁻¹), alkaline phosphatase (544 mU mL⁻¹), and lactate dehydrogenase (355 mU mL⁻¹). A chest X-ray showed a slightly enlarged cardiothoracic ratio of 60% without pleural effusion. An electrocardiogram revealed a normal sinus rhythm. A trans-thoracic echocardiogram showed a moving mass near the inferior vena cava in the right atrium (Supplementary Video 1). The patient underwent an operation for a cardiac tumor. A trans-esophageal echocardiogram during operation showed a mass in the right ventricle, which had echo properties similar to the mass in the right atrium (Figure 1; Supplementary Video 2). The operation was performed under the standard pulmonary cardiopulmonary bypass and cardiac arrest. Two tumors were resected via the right atriotomy and the trans-tricuspid approach. The mass in the atrial free wall was resected including the attached intima, and the mass located laterally to the muscle of Lancisi in the ventricle was resected by a shave excision technique.

Figure 1.

Trans-esophageal echocardiography during the operation: (a) a mass (arrows) is visible on the free wall near the inferior vena cava in the right atrium, and it shows mobility and (b) a mass with echo properties similar to that in the right atrium (arrows) is found in the right ventricle.

Her postoperative course was uneventful. A pathological examination showed that the two tumors were papillary fibroelastomas. Histological findings demonstrate distinctive clusters of hair-like projections on the surface of the two tumors (Figure 2). The patient was discharged 10 days after operation. She is doing well 1 year after operation without any signs and symptoms of the tumor.

Figure 2.

Microscopic finding of the resected masses (hematoxylin and eosin stain). Histological findings demonstrate distinctive clusters of hair-like projections on the surface of the two tumors.

Discussion

Myxoma has the highest prevalence among benign tumors, followed by lipoma, papillary fibroelastoma, and rhabdomyoma. Papillary fibroelastomas comprise approximately 7% of all cardiac tumors. Like myxomas, they constitute a curable and identifiable cause of strokes and other embolic events. Papillary fibroelastomas usually develop on a left-sided valve leaflet^1,2 and can appear in the right heart.^3–6

Diagnostic procedures for papillary fibroelastomas include trans-thoracic and trans-esophageal echocardiographic techniques, cardiac magnetic resonance imaging, and computed tomography. Differential diagnosis of papillary fibroelastoma is not easy. Buttan et al. ⁷ showed that homogeneous hyperechogenicity is a characteristic finding of papillary fibroelastoma, and a lobulated appearance is a characteristic one of myxoma. Our patient showed no typical lobulated appearance, thus we suspected papillary fibroelastoma.

Papillary fibroelastoma is a solitary lesion, and multiple fibroelastomas are rarely recognized.^1,2 Alozie et al. ² found multiple masses on the left, right, and non-coronary cusps during surgery, and postoperative histopathological analysis confirmed the diagnosis of multiple cardiac papillary fibroelastomas. Lin et al. also reported a case with multiple fibroelastomas. A preoperative three-dimensional trans-esophageal echocardiogram showed multiple masses attached to the aortic and tricuspid valves, and both of them were pathologically diagnosed as papillary fibroelastomas. ⁸

A benign tumor has no possibility of recurrence after complete resection, and it can be resected with adjacent myocardial intima, so-called a shave excision technique.^1,9 Pathological findings of the tumor are very characteristic. It shows multiple slender fronds under water, giving the appearance of a sea anemone, and its microscopic feature shows dense elastin coated by collagen at the core of each frond. ¹⁰

Fibroelastomas in our case originated from the right atrial and ventricular walls apart from the valve cusps. An intraoperative trans-esophageal echocardiogram contributed to obtaining the diagnosis of the mass in the right ventricle. A detailed examination should be performed when a cardiac tumor is diagnosed to determine the possibility of multiple lesions.