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.

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.

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.

Cardiac Papillary Fibroelastoma: Atypical Presentation Mimicking Infective Endocarditis With False Positive Commensal Blood Cultures.

Cardiac papillary fibroelastomas (CPFs) are rare benign cardiac neoplasms that carry a high risk of embolization if not diagnosed and managed in a timely manner. As most patients are asymptomatic, CPF may be incidentally detected on transthoracic echocardiography (TTE) when performed for other indications. Management of incidental CPF in asymptomatic patients is debatable. We report an unusual case of an incidental CPF in an asymptomatic patient admitted to the hospital for presumed infective endocarditis (IE). Two weeks following laser resection of laryngeal cancer (LC), a new pansystolic murmur was audible during a routine cardiology visit. Outpatient TTE revealed a "vegetation-like" lesion on the mitral valve (MV). Blood cultures (BC) with Gram-positive cocci in clusters (GPC) were reported within 24 hours. This prompted hospital admission for empiric antibiotics. A transesophageal echocardiogram (TEE) confirmed the lesion to be an echogenic mass attached to the MV consistent with CPF. Repeat BC, prior to empiric antibiotic initiation, were all negative. In the absence of all other signs and symptoms of IE, it was determined that the initial BC was false positive and IE was ruled out. Surgical resection was performed due to the potential risk of embolization. The pathology confirmed the diagnosis of CPF with negative tissue cultures.

Diagnostic and Therapeutic Challenges Associated With Left Ventricular Tumors.

Cardiac tumors are uncommon. Sometimes it is challenging to differentiate non-invasively between different kinds of cardiac tumors and thrombi, which is critical to dictate the subsequent treatment. In addition, not all high-risk cardiac tumors are amenable to surgical resection posing a therapeutic challenge. We report a case of cardiac papillary fibroelastoma in the left ventricular cavity with a 10-year follow-up, with no embolic complications.

Cardiac papillary fibroelastoma as a cause of acute coronary syndrome.

Cardiac papillary fibroelastoma (CPF) is a benign primary cardiac neoplasm, commonly found in men and above 40 years old. The clinical presentation of CPF ranges from asymptomatic to embolism-related complications such as stroke, myocardial ischemia, infarction, or ventricular fibrillation. Acute coronary syndrome is a rare complication of CPF, which was reported only in a few cases in medical literature. Hence, we report a case of a 50-year-old female with a CPF on the right coronary cusp of the aortic valve diagnosed with multi-modality imaging with definitive diagnosis through histopathologic confirmation. The patient presented with acute onset of fatigue, diaphoresis, and vomiting. Initial electrocardiogram (ECG) demonstrated T wave inversion in aVL. Repeated ECG two hours later showed persistent T wave inversion in aVL with new T wave inversions in lead I and ST depression in V2-V6. Troponin levels were elevated from 3.6 ng/L to 1503 ng/L but the patient did not report chest pain, abdominal pain, or dyspnea. Computed tomography coronary angiography did not show any significant coronary stenosis but revealed a low attenuation node with 7 × 6 mm in dimension attached to the right coronary cusp of the aortic valve. Treatment was discussed among a multidisciplinary team and the CPF was surgically removed. Learning objective: Acute coronary syndrome is a rare, but potentially fatal complication of cardiac papillary fibroelastoma (CPF). Multi-modality imaging is valuable in delineating the evaluation of exact position, dimensions, nature of cardiac masses, diagnostic workup, and preliminary assessment before the surgery. There are no clear guidelines for the treatment of CPF.

Case report: Minimally invasive excision of multifocal cardiac papillary fibroelastomas involving right atrium and aortic valve.

Background: Cardiac papillary fibroelastomas (CPFs) are rare benign cardiac tumors most commonly found on left-sided cardiac valves. Right atrial CPFs are extremely rare, accounting for only 2% of all CPFs. Median sternotomy is a typical approach for surgical excision of CPFs in most cases. Herein, we report an extremely rare case of multifocal CPFs involving the right atrium and aortic valve that were surgically excised via minimally invasive right anterolateral thoracotomy. Case Summary: A 59-year-old Chinese man was admitted because of an incidental finding of a right atrial mass on transthoracic echocardiography during a routine check-up. The mass was initially diagnosed as a myxoma, and the patient was scheduled for minimally invasive excision via right anterolateral thoracotomy. An additional mass on the non-coronary cusp of the aortic valve was identified using intraoperative transesophageal echocardiography. The patient still underwent complete tumor excision via right anterolateral thoracotomy. Both neoplasms were pathologically diagnosed as CPFs. Conclusions: This case highlights the need for a comprehensive cardiac evaluation of cardiac tumors because CPFs can manifest as multifocal lesions. Moreover, minimally invasive surgery is highly feasible as the CPF can be easily excised, and the valve can usually be preserved.

Papillary Fibroelastoma Incidentally Found on Left Atrial Wall During Minimally Invasive Aortic Valve Replacement.

A 60-year-old man was about to undergo minimally invasive aortic valve replacement when transesophageal echocardiography revealed an intracardiac mass on the left atrial free wall. Multimodal images from 5 months earlier had shown no mass. We converted the procedure to open surgery. The excised mass resembled a cardiac myxoma but was determined to be a papillary fibroelastoma. This case illustrates that papillary fibroelastomas can form and grow rapidly, warranting alertness for their unexpected discovery before and during cardiac surgical procedures.

Left Atrial Fibroelastoma as a Cause of Stroke: A Case Report.

Cardiac papillary fibroelastoma is a benign and rare primary tumor of the heart that is most frequently located in the aortic or the mitral valves. Papillary fibroelastoma arising from the left atrium is exceedingly rare, comprising less than 7% of all cardiac papillary fibroelastomas. Tumors in this location could be a source of cardioembolic stroke, often in the anterior circulation of the cerebrum. A 66-year-old female presenting with right hemiparesis, central facial palsy, homonymous hemianopia, and dysarthria received intravenous thrombolysis for stroke without apparent improvement. Magnetic resonance imaging of the brain revealed ischemic infarction in the territories of the left middle and posterior cerebral arteries. A tumor with a maximal diameter of 2.3 cm was disclosed during workup for possible cardioembolic stroke with transthoracic echocardiography and computed tomography of the heart. The clinical course was complicated by stroke-in-evolution and hemorrhagic transformation. The patient underwent left atrial tumor excision and left atrium appendage closure. In-patient stroke rehabilitation programs were also initiated after the conditions stabilized. No clinically significant complications developed after the operation. Neurological functions improved and the patient was able to perform most basic daily living activities with some assistance. An exhaustive search for the cause of cardioembolic stroke is paramount, as management strategies may differ from patients with thrombotic stroke.

New insights into the pathogenesis of cardiac papillary fibroelastomas.

INTRODUCTION: Cardiac papillary fibroelastomas (CPFs) are rare benign cardiac tumors typically found on the heart valves. The previously published data on the CPF focused on its clinical presentation, optimal management, and prognosis. However, histogenesis of these lesions remains controversial. Accordingly, the aim of this study was to establish the role of endocardial endothelium (EE) in CPF formation. MATERIALS AND METHODS: Four CPF tumors removed from the right atrioventricular valves were analyzed using hematoxylin & eosin, orcein, and Masson trichrome staining together with immunochemistry for CD-34, CD-68, vimentin, vWF and a-SMA. Moreover, conventional transmission electron microscopy was used for morphological analysis and a-SMA presence confirmation. RESULTS: Ultrastructural morphology, immunohisto- and immunocytochemical analyses indicated that cells covering collagenous core have an endothelial origin. Some endocardial endothelium cells have the potential to undergo a transition to mesenchymal cells. Moreover, the abundant presence of extracellular vesicles may indicate an active intercellular communication. Within the intermediate translucent zone, amorphous substances with monocytes/macrophage-like cells and fibroblastic cells were found. Finally, within collagenous core activated (myo)fibroblasts were observed. CONCLUSIONS: Our study demonstrated that the endocardial endothelium of the CPF was "double-sided", i.e., it presented both endothelial and mesenchymal cell characteristics. Another finding was the presence of monocytes, and macrophages which were integrated into CPF core and displayed features of a fibroblast that have been shown to contribute to extracellular matrix production. This could be interpreted as being attributed to the CPF histogenesis.

A rare cause of left ventricular outflow tract mass in infancy: Cardiac papillary fibroelastoma.

Primary cardiac tumors are one of the rare causes of intracardiac masses in pediatric population. Cardiac papillary fibroelastomas are benign cardiac tumors with predilection for valvular endocardium, and the presentation is dictated by the cardiac structure involved and the tumor characteristics. We report an infant with cardiac papillary fibroelastoma manifesting as an asymptomatic left ventricular outflow tract mass. This case is being presented to emphasize the rare occurrence of cardiac papillary fibroelastoma in pediatric population and to be cognizant of this entity when considering differential diagnosis of intracardiac masses in this subgroup.

Cardiac Papillary Fibroelastoma in Left Ventricular Trabeculation as a Potential Cause of Cerebral Infarction: A Case Report.

Cardiac papillary fibroelastoma (CPF) is the second or third most common primary cardiac tumor. Although histologically benign, it can cause serious symptoms depending on its location of occurrence, size, and motility. Herein, we report CPF in the left ventricular trabeculation as a potential cause of cerebral infarction.

Cardiac papillary fibroelastomas: A 10-year single-center surgical experience and long-term echocardiographic follow-up study.

AIMS: Limited contemporary data are available on the clinical and echocardiographic outcomes after surgery for cardiac papillary fibroelastoma (CPF). The aim of this study was to review the clinical manifestations, pathological characteristics, surgical management, and prognoses of patients with histologically verified CPF, who underwent surgery at our cardiac surgery center from 2008 to 2018. METHODS AND RESULTS: Twelve patients of median age 62 years (28 to 77 years) were treated. Embolic stroke or transient ischemic attack (five patients, 42%) were the only CPF clinical manifestations. Eleven (92%) tumors were localized on the valves, with the aortic valve being the most common tumor site (seven patients; 58%). Multiple factor analysis revealed no independent predictor of CPF-related embolization. Simple shave tumor excision was sufficient in most patients (10 patients, 83%). No operative or tumor-related late mortality during the median follow-up period of 4.7 years (1.1 to 10.2 years) was recorded. Asymptomatic metachronous valve tumor recurrence (in a location different from that of the original tumor) was revealed in two patients (17%) by transesophageal echocardiography (TEE), not detected by transthoracic echocardiography (TTE). One of these two patients underwent repeated surgery for CPF but later suffered a recurrent embolic stroke, due to another tumor recurrence. CONCLUSION: CPF can be safely and effectively treated surgically. TEE is superior to the TTE option in CPF post-operative recurrence detection. There is a clear need for a prospective study to determine criteria for embolization risk stratification and optimum management in patients with CPF.

Cardiac papillary fibroelastoma of a bicuspid aortic valve in an adolescent: A case report.

Cardiac papillary fibroelastomas (CPFE) are exceptional primary benign cardiac tumours affecting the heart valves. We report here the case of a 15-year-old boy in whom echocardiography performed for non-specific chest pain during follow-up for bicuspid aortic valve showed as accidental finding the presence of a round mobile mass without stalk attached on the inferior side of the aortic valve. The mass did not cause any outflow tract obstruction or aortic insufficiency. Electrocardiogram-gated cardiac computed tomography and magnetic resonance imaging allowed to suspect CPFE. Although the patient was asymptomatic, open cardiac surgery with elective surgical resection of the tumour was performed to avoid systematic emboli. Histology confirmed the diagnosis of CPFE. This is an exceptional case of acquired CPFE in a young patient with bicuspid aortic valve. Due to the risk of systemic embolization, aortic or coronary ostium obstruction, elective excision of such lesions is recommended.

Brainstem stroke caused by left atrial cardiac papillary fibroelastoma: an increasingly recognized rare cause of stroke.

Cardiac papillary fibroelastoma is a rare but increasingly recognized cause of embolic stroke that is prevalent in the older population and requires prompt surgical management. We report an unusual case of left atrial appendage cardiac fibroelastoma in a 76-year-old gentleman who presented with left internuclear ophthalmoplegia and ataxia, with corresponding diffusion-weighted imaging on magnetic resonance imaging of the brain. This case illustrates the importance of echocardiographic imaging in the workup of cardioembolic stroke in the older adult population in the acute setting.

Incidental Discovery of an Atypical Cardiac Tumor.

Primary cardiac tumors are rare, present in roughly 0.05% of the population. Cardiac papillary fibroelastoma (CPF) is the second most common, accounting for 10% of primary cardiac tumors.[1] Most cases of CPFs are discovered incidentally on autopsy; however, they may present clinically with systemic embolization or heart failure symptoms. The recommended treatment for symptomatic CPF patients is surgical resection.[1] Treatment in asymptomatic patients remains somewhat controversial with incidentally discovered tumors presenting a clinical dilemma. We present a case of an atypically located CPF that was discovered incidentally on intraoperative transesophageal echocardiography (TEE) during a routine coronary artery bypass graft operation. This case highlights several important points for cardiac anesthesiologists. The first is the importance of performing a comprehensive intraoperative TEE. Next, this case reinforces the broad utility of TEE for evaluation of intracardiac tumors. Finally, this case demonstrates the importance of precise localization of intracardiac tumors.