Tricuspid Papillary Fibroelastoma: A Rare Tumor in an Uncommon Location.

Papillary fibroelastomas (PFEs) are rare benign cardiac tumors typically arising from the valvular endocardium, often affecting the aortic and mitral valves. They can range from asymptomatic to causing severe thromboembolic complications like stroke. This article presents a case of a tricuspid valve PFE in an 81-year-old patient with severe multi-vessel coronary artery disease. Transthoracic echocardiography revealed a mass on the tricuspid valve, confirmed by cardiac MRI. The patient underwent surgical excision, and histopathology confirmed the PFE diagnosis. The case highlights the importance of multimodal imaging in diagnosis and individualized treatment strategies for valvular heart tumors.

Rheumatic heart disease of the mitral valve alongside the papillary fibroelastoma of the aortic valve: A case report.

Key Clinical Message: Besides thromboses, it's crucial to also consider the rare possibility of tumors like papillary fibroelastomas when evaluating worsening cardiopulmonary symptoms in patients with severe rheumatic mitral stenosis and atrial fibrillation. Abstract: Cardiac papillary fibroelastoma is a rare and benign endocardial tumor typically found on the aortic valve. The simultaneous occurrence of rheumatic heart disease affecting the mitral valve and papillary fibroelastoma on the aortic valve is infrequent, with limited documented instances. This unique case can enhance our understanding of the clinical presentation, diagnostic approaches, management options, and implications for patient outcomes in these two conditions. We present the case of a 47-year-old woman who was admitted to the hospital due to worsening dyspnea and fatigue, during which time she discovered an aortic valve papillary fibroelastoma. Further investigations revealed two thrombi in her left atrium and left atrial appendage, along with significant rheumatic mitral valve stenosis. The patient underwent thrombectomy, mitral valve replacement, and valve-sparing aortic valve tumor resection. Following surgery, the patient's recovery was unremarkable. This case report emphasizes the need for a comprehensive evaluation in patients with rheumatic mitral stenosis, considering all possible etiologies. While thrombi are typical in mitral stenosis and atrial fibrillation, the rare presence of tumors like papillary fibroelastomas should be recognized, underscoring the importance of further assessment when suspicion arises. Importantly, individuals with asymptomatic cardiac papillary fibroelastomas should undergo surgical treatment to minimize the potential risk of tumoral embolization.

Unusual Location of a Papillary Fibroelastoma Causing a Multi-territorial Stroke.

A 56-year-old male presented with a multi-territorial stroke without traditional cerebrovascular risk factors. A transesophageal echocardiogram revealed an intracardiac lesion attached to the lateral wall of the left atria, consistent with an atrial myxoma. Surgical excision of the lesion was performed and revealed that lesion was in fact a papillary fibroelastoma with thrombus attached, which demonstrates a novel mechanism by which intracardiac masses can cause cerebral events.

Papillary fibroelastoma complicated by Streptococcus sanguinis bacteremia: a rare case of cardiac tumor with embolic events.

Fibroelastoma is a rare cardiac tumor that can cause embolization, stroke, myocardial infarction, heart failure, and cardiac arrest. Here, we report the case of a 45-year-old male who presented with right-sided weakness and fever. He was diagnosed with acute right frontal infarction and was found to have Streptococcus sanguinis bacteremia. Upon confirmation of a positive blood culture after 24 hours, treatment for endocarditis was initiated. Transesophageal echocardiography revealed findings highly suggestive of a papillary fibroelastoma (PFE). PFE ought to be regarded as a potential differential diagnosis in individuals who exhibit symptoms of fever, thromboembolism, and persistent bacteremia. Non-invasive imaging such as echocardiography is of great value in the diagnosis of PFE, while surgical resection remains the best treatment modality to overcome current and future associated complications.

Prenatal diagnosis of cardiac papillary fibroelastoma of the aortic valve: A report of two cases.

In our perinatology service, we observed two cases of cardiac papillary fibroelastoma in fetuses. This case-report focused on assessing the prenatal diagnosis and outcome of these two fetal cases in which cardiac fibroelastoma was initially identified via fetal echocardiography and subsequently confirmed by histopathological analysis.

Aortic valve fibroelastoma presenting with myocardial infarction with non-obstructive coronary arteries (MINOCA): A case report and review of the literature.

Cardiac papillary fibroelastomas (CPFs) are rare benign cardiac tumors more often involving the left-sided valves and related with threatening embolic complications. We report the case of a 35-year-old woman presenting with relapsing-remitting chest pain and elevated cardiac troponins. After a negative coronary angiography, an integrated imaging assessment based on echocardiography and cardiac magnetic resonance showed a pedunculated mass on the aortic valve causing an intermittent obstructive engagement of the right coronary ostium. A tailored surgical treatment was performed and the histopathological examination of the specimen revealed mesenchymal tissue with the characteristics of CPF.

A Case of Rapidly Growing Tricuspid Valve Papillary Fibroelastoma Presenting With Syncope.

Cardio-oncology, at the intersection of cardiovascular diseases, oncological conditions, and treatments, presents unique challenges in medical care. This abstract highlights a case involving a 60-year-old male presenting with syncope at work; the workup revealed a rapidly growing tricuspid valve papillary fibroelastoma (PFE), emphasizing diagnostic approaches, management strategies, and clinical implications. The diagnostic investigation, including blood cultures, transthoracic echocardiogram, transesophageal echocardiogram, and cardiac MRI, confirmed the diagnosis of tricuspid valve PFE. A multidisciplinary approach led to a shared decision with the patient to opt for serial monitoring. Syncope was attributed to dehydration. This case underscores the complexities of managing cardiovascular conditions in the context of oncology and the importance of collaborative decision-making in patient care.

Pulmonic valve fibroelastoma-A rare incidental finding.

Primary cardiac neoplasms are a rare, commonly benign, tumor with an approximate incidence rate of 0.02%. Papillary fibroelastoma (PFE), a common form of primary cardiac neoplasms, typically present as a mass on the aortic and mitral valves, while rarely presenting as a pulmonary valve tumor. The majority of PFEs are asymptomatic, however valvular masses can pose a significant health hazard due to their potential to fragment into the bloodstream, facilitate thrombus formation, and restrict blood flow. Due to these risks, careful resection of the mass is recommended for symptomatic patients and asymptomatic patients if the tumor is large (>1 cm), mobile, or on left-sided valves. Here we present a case of an incidental finding of a pulmonic valve papillary fibroelastoma in a 65-year-old man by transesophageal echocardiography during a coronary artery bypass graft procedure.

The mass that should not be there: Multi-modality imaging shapes diagnostic reasoning.

A rare case of pulmonary artery fibroelastoma that demonstrates the importance of multimodality imaging and serial scans in reducing diagnostic uncertainty.

Papillary fibroelastoma originating from the atrial septum touching the mitral valve leading to infective endocarditis: a case report.

BACKGROUND: Cardiac papillary fibroelastoma is a rare benign tumor, which is often mistaken for a vegetation. Predominantly asymptomatic, it can cause life-threatening complications. Although rare, mobile papillary fibroelastoma movement between affected valves may hamper valve closure and damage the valve, leading to valvular regurgitation. Endothelial damage increases the risk of developing infective endocarditis. We report a rare case of a highly mobile papillary fibroelastoma originating from the atrial septum touching the mitral valve, leading to mitral regurgitation and, eventually, infective endocarditis. CASE PRESENTATION: A 26-year-old woman with suspected infective endocarditis was referred to us from a previous hospital after having experienced intermittent fever for a month. Before the fever, she had been experiencing exertional dyspnea. In addition, she had undergone a cesarean section two weeks before this admission. A transthoracic echocardiogram showed a mobile mass originating from the atrial septum touching the mitral valve with severe mitral regurgitation. Computed tomography revealed an occluded right profunda femoris artery with an embolus. Infective endocarditis associated with a mobile vegetation with high embolic risk was diagnosed, and urgent surgery was performed. Following the surgery, examinations revealed papillary fibroelastoma originating from the atrial septum and infective endocarditis of the mitral valve. The histopathological examination confirmed that a mass initially thought to be a mobile vegetation was a papillary fibroelastoma. The postoperative course was uneventful except for pericarditis. There has been no recurrence of infective endocarditis or papillary fibroelastoma. CONCLUSIONS: The highly mobile papillary fibroelastoma was thought to have caused both chronic mitral regurgitation and infective endocarditis. Mobile papillary fibroelastomas can cause endothelial damage to nearby valves and predispose patients to infective endocarditis.

Fibroelastoma, uma Doença Incidentaloma? - Imagem de Casos de Fibroelastomas como Achados Incidentais em Quatro Pacientes, Quatro Valvas Diferentes / Fibroelastoma, an Incidentaloma Disease? - Image Cases of Fibroelastomas as Incidental Findings in Four Patients, Four Different Valves

Resumo Os fibroelastomas são o segundo tumor cardíaco benigno mais comum. São estruturas pequenas, avasculares, com uma dimensão média de 9mm, podendo atingir até 70mm, habitualmente aderentes à superfície das válvulas cardíacas (válvulas aórtica e mitral são as mais comumente afetadas, seguidas das válvulas tricúspide e pulmonar). A etiologia não é clara, sendo a hipótese de formação de microtrombos nas margens de coaptação das válvulas a mais aceite. Na ecocardiografia apresentam aspeto pediculado, móvel, com superfície filamentosa, tipicamente com uma aparência pontilhada nas margens e ecolucente. Do ponto de vista clínico, podem estar associados a fenómenos embólicos, no entanto, na maioria dos casos o diagnóstico é incidental. Apresentamos de seguida quatro casos de diagnóstico incidental de fibroelastomas nas quatro válvulas cardíacas, diagnosticados por ecocardiograma transtorácico (ETT) (Vídeo 1; Figura 1). Vídeo 1 Da esquerda para a direita, de cima para baixo: fibroelastomas no folheto anterior da válvula tricúspide, folheto anterior da válvula mitral, cúspide esquerda da válvula pulmonar e cúspide esquerda da válvula aórtica, cada um correspondendo a um doente diferente. Em: http://abccardiol.org/supplementary-material/2024/12102/2023-0222_IM_video01.mp4 Figura 1 Da esquerda para a direita, de cima para baixo: fibroelastomas no folheto anterior da válvula tricúspide, folheto anterior da válvula mitral, cúspide esquerda da válvula pulmonar e cúspide esquerda da válvula aórtica, cada um correspondendo a um doente diferente.

Abstract Fibroelastomas are the second most common benign cardiac tumor1. They are small avascular structures with a mean size of 9mm, ranging up to 70mm, usually attached to the heart valves' surface (aortic and mitral are the most affected, followed by tricuspid and pulmonary valves). Their etiology is unclear, but the hypothesis of coalescence of microthrombus at the coaptation margins of valves is the most widely accepted theory. On echocardiography, they are pedicled, mobile, with a filamentous surface, and usually have a speckled appearance with echolucencies and a stippled pattern near the edges. Clinically, they may be associated with embolic phenomena; however, in most cases, the diagnosis is incidental. We present a series of four clinical cases with an incidental diagnosis of fibroelastomas across the four cardiac valves as assessed by transthoracic echocardiography (Video 1; Figure 1). Video 1 From left to right and top to bottom: fibroelastomas of the anterior leaflet of the tricuspid valve, anterior leaflet of the mitral valve, left cusp of the pulmonary valve and left cuspid of the aortic valve, each corresponding to a different patient. Link: http://abccardiol.org/supplementary-material/2024/12102/2023-0222_IM_video01.mp4 Figure 1 From left to right and top to bottom: fibroelastomas of the anterior leaflet of the tricuspid valve, anterior leaflet of the mitral valve, left cusp of the pulmonary valve and left cuspid of the aortic valve, each corresponding to a different patient.

Characteristics of Lambl excrescences of aortic and pulmonary valves in healthy hearts.

Lambl excrescences (LEs) were initially described in the mid-1800s during autopsies of human hearts, and their significance and biology have been debated ever since. LEs are typically found on aortic and pulmonary valve (semilunar) cusps. There is debate concerning whether LEs are a significant cause of thromboembolic events, or whether they are harmless growths. However, there have not been many reports discussing LEs, and fewer still have examined the prevalence and characteristics of LEs in healthy human hearts. Those who have examined LE prevalence have reported a very high incidence of LEs (85-90%). Herein, we examine LE prevalence and characteristics (size, location, number) in 403 healthy human hearts across all age groups. We find that the prevalence of LEs in healthy hearts is far lower than previously reported.

Removal of an infected pulmonary artery fibroelastoma disguised as a presentation of pulmonary embolism using a percutaneous suction thrombectomy device.

Papillary fibroelastoma (PFE) is a rare, often benign, tumor originating typically in the endocardium and valves, with a preference for the left side of the heart. Although PFEs can appear asymptomatic, in the setting of embolization, they can lead to stroke, acute limb ischemia, and/or mesenteric ischemia. Rarely, PFEs can originate from the pulmonary valve, with the potential for embolic showering into the pulmonary artery, leading to potential right-sided heart outflow obstruction. Treatment has been open surgery in most cases, although treatment of right-sided heart masses with extracorporeal circulatory support extraction systems have been described. Recently, large bore suction thrombectomy devices have become available, typically used for cases of venous thromboembolism. In the present report, we describe a case of a symptomatic infected PFE treated by percutaneous suction thrombectomy using the Inari FlowTriever system (Inari Medical).

Concomitant minimally invasive surgery for tricuspid valve papillary fibroelastoma and right lung cancer in an elderly patient: a case report and review of the literature.

BACKGROUND: It is very common for patients with newly diagnosed lung masses to have heart disease. However, papillary fibroelastomas (PFEs) of the tricuspid valve (TV) combined with lung cancer are rarely reported. It is thus unclear whether a two-stage surgery or concomitant surgery is optimal. CASE PRESENTATION: We report the case of a 73-year-old Chinese male who was diagnosed with PFEs on the TV by transthoracic echocardiography (TTE) examination while being evaluated to undergo video-assisted thoracic surgery (VATS) for a right lower lung nodule. We resected both the PFEs and the lung nodule via right minithoracotomy. The surgery was uneventful, and histopathology reports confirmed PFEs of the TV and moderately to poorly differentiated squamous cell carcinoma. The patient recovered uneventfully, and there was no sign of tumor recurrence during 15 months of follow-up. CONCLUSIONS: We suggest that after careful evaluation, concomitant minimally invasive radical resection of primary lung cancer after cardiac PFE removal is an acceptable and safe treatment strategy and should be performed as soon as possible.

Cardiac papillary fibroelastoma: a rare cause of ST-segment elevation myocardial infarction: a case report.

Introduction and importance: Primary tumors of the heart are extremely rare occurrences. Among them, cardiac papillary fibroelastoma (CPF) is the second most common type. Although these tumors are usually benign, they can pose a risk of embolization, which may lead to severe complications like sudden death or embolization affecting the neurological, systemic, or coronary vasculature. Such complications can be life-threatening. Case presentation: In this report, the authors present the case of a 68-year-old woman who experienced ST-segment elevation myocardial infarction due to embolization from a large papillary fibroelastoma. To address the issue, the authors performed a minimally invasive surgical removal and resection of the aortic valve, followed by a histological examination to confirm the diagnosis. Clinical discussion: This case report discusses a rare occurrence of myocardial infarction caused by tumor embolization from a CPF. The patient presented with complete blockage of a coronary artery in the absence of atherosclerotic disease. Through a comprehensive workup, including transesophageal echocardiography, the CPF was identified as the source of embolization. Surgical resection of CPFs is curative, and recurrence has not been documented. Clinicians should consider CPFs in cases of coronary artery occlusion without atherosclerotic disease and employ transesophageal echocardiography for diagnosis. Prompt surgical intervention leads to an excellent prognosis and prevents recurrent embolization. Conclusion: This report emphasizes the importance of recognizing the potential complications associated with papillary fibroelastoma-induced embolization to the coronary arteries and highlights the need to mitigate the risk of such complications occurring.

Clinical presentation and neurovascular manifestations of cardiac myxomas and papillary fibroelastomas: a retrospective single-institution cohort study.

Background: Primary cardiac tumors are often benign and commonly present as cardiac myxomas (CMs) or papillary fibroelastomas (CPFEs). There is a paucity of prognostic indicators for tumor burden or potential for embolic cerebrovascular events (CVEs). This study was performed to address these gaps. Methods: Medical records at the University of Florida Health Shands Hospital between 1996 and 2021 were screened to identify patients with CMs or CPFEs. Clinical features, echocardiographic reports, and CVE outcomes were quantitatively assessed. Results: A total of 55 patients were included in the study: 28 CM (50.9%) and 27 CPFE (49.1%) patients. Baseline patient characteristics were similar among patients. The neutrophil-lymphocyte ratio was correlated (p < 0.005 in all cases) to three metrics of tumor size in both CM (r = 64-67%) and CPFE (r = 56-59%). CVEs were the presenting symptom in 30 (54.5%) patients. CVE recurrence was high; the 5-year CVE recurrence rate in patients with tumor resection was 24.0% compared to 60.0% without resection. No baseline patient characteristics or tumor features were associated with an initial presentation of CVEs compared to any other indication. Univariate analysis indicated that prolonged duration to surgical resection, left atrial enlargement, male sex, and a neutrophil-lymphocyte ratio >3.0 at the follow-up were significantly associated with 5-year CVE recurrence. Left atrial enlargement and a neutrophil-lymphocyte ratio >3.0 at the follow-up remained significantly associated with 5-year CVE recurrence in multivariate analysis. Conclusion: The neutrophil-lymphocyte ratio may prognosticate tumor size and recurrence of neurologic events. An increased risk of CVE within 5 years of mass resection is almost exclusive to patients initially presenting with CVEs.