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Papillary fibroelastoma is one of the rare benign cardiac tumors; that stays asymptomatic and is usually found unintentionally during routine transthoracal and/or transesophageal echocardiographic (EKO) examinations. Papillary fibroelastomas arising from the left car- diac valves may usually cause systemic embolism and the right sided papillary fibroelastomas may cause recurrent pulmonary embolism. Papillary fibroelastomas arising from the aortic valves may cause coro- nary embolism that leads to myocardial infarction and sudden death. We present in this case study a sudden and unexpected death of a child who ate pears after dinner, fainted, started vomiting and died suddenly and unexpectedly despite all medical interventions. The case was taken to Muğla State Hospital by her family; but lost her life despite all med- ical interventions. In the autopsy of the case performed with the suspi- cion of intoxication the papillary fibroelastoma of the mitral valve was detected in the autopsy and concluded as a cause of death. Papiller fibroelastom nadir görülen benign kardiyak tümörler- dendir. Papiller fibroelastoma, asemptomatik bir seyir izlemekte ve ge- nellikle rastlantısal olarak rutin transtorakal ve/veya transözafagial ekokardiyografik (EKO) incelemede saptanmaktadır. Sol kalp kapak- larındaki papiller fibroelastomlar sistemik embolilere, sağ taraflı pa- piller fibroelastomlar ise tekrarlayan pulmoner embolilere neden olabilmektedir. Aortik kapak kaynaklı papiller fibroelastomlar koroner embolilere sebep olarak myokardiyal enfarkt ve ani ölümlere sebep ola- bilmektedirler. Bu çalışmada, akşam yemeğinden sonra armut yiyen, ardından bayılan, kusmaya başlayan ve ani ve beklenmedik şekilde ölen bir çocuk olguyu sunuyoruz. Olgu ailesi tarafından Muğla Devlet Has- tanesine götürülmüş; ancak yapılan tıbbi müdahaleye rağmen hayatını kaybetmiştir. Olgunun entoksikasyon şüphesi ile yapılan otopsisinde mitral kapağın papiller fibroelastomu saptanmıştır ve ölümün nedeni olduğu düşünülmüştir.

Papillary fibroelastoma (PFE) is a cardiac tumor that is mainly found on the heart valve and the endocardium of the atria and ventricles. Symptoms such as stroke and myocardial infarction are usually caused by embolization of either the tumor itself or associated thrombus. PFE is known to originate mainly from the left side of the heart, and these cases are—in principle—candidates for surgical resection. On the other hand, cases in which PFE originates from the right side of the heart are rare and reports are limited; thus, the surgical indication is unclear. We herein report a case of symptomatic PFE originating from the tricuspid valve of the heart. In this case, contrast enhanced computed tomography did not show pulmonary embolism; however, lung perfusion scintigraphy showed multiple perfusion defects. The patient was treated by anticoagulant therapy followed by surgical resection. Thereafter, the symptoms disappeared and the multiple perfusion defects improved on lung perfusion scintigraphy, demonstrating the efficacy of the anticoagulant therapy and surgical resection for PFE in the right side of the heart. Learning objective Papillary fibroelastoma (PFE) originating from the tricuspid valve of the heart is rare and reports on this topic are limited. Thus, the surgical indication is unclear. In the present case, lung perfusion scintigraphy indicated pulmonary embolism due to PFE, while contrast enhanced computed tomography did not. Anticoagulant therapy and subsequent surgical resection were considered effective for PFE in the right side of the heart.

We present a case of a 75-year-old woman with typical myocardial infarction, however coronary angiogram was negative. Echocardiography identified the rare cause of chest pain, as a mobile mass of aortic valve was found to obstruct the coronary ostium. histopathology revealed a papillary fibroelastoma (PFE). Chest pain was relieved after surgical resection of the mass.

Myxoma and papillary fibroelastoma are the most common benign cardiac tumors accounting for the majority of the benign tumors a surgeon will see. (2) Myxomas occur most commonly in the left atrium but can occur in any cardiac chamber and represent over 50% of the benign tumors. Fibroelastomas are the second most common benign tumors and generally involve the cardiac valves but can occur within the chambers. Surgical resection is the appropriate approach for myxoma and most fibroelastomas and the surgical techniques needed have been well established. This manuscript presents our approach to the evaluation, preparation and surgical techniques required for treating these primary benign cardiac tumors.

The AngioVac (Angiodynamics) system is indicated for the removal of right-sided venous soft thrombi and emboli. This is the first report that demonstrates the AngioVac system can be extended to the extraction of right-sided cardiac tumors, in the current case, a pulmonary valve papillary fibroelastoma infected with Streptococcus salivarius and Rothia species. (Level of Difficulty: Advanced.), Graphical abstract

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. Web of Science 164 1 91 84

Papillary fibroelastoma (PFE) is an increasingly recognized cardiac tumor. Despite its prevalence, controversy exists as to whether it represents a reactive or neoplastic process due to histopathologic similarities with Lambl excrescences (LEs), an accepted reactive phenomenon. Recently, KRAS mutations were reported in a small collection of PFEs, but the incidence of mutations and conditions in which they arise in are unknown. Furthermore, the relationship between PFE and LE has yet to be investigated. Institutional archives were queried for cases of PFE (2001-2017). Paraffin-embedded tissue was microdissected for tumor isolation. Prospectively identified LEs (2018) were collected and wholly isolated. Extracted DNA underwent droplet digital polymerase chain reaction analysis of the most common KRAS mutations (codons 12/13 and 61). Relevant clinical information was abstracted from the medical record. Fifty-two PFEs were tested from 50 patients (32 women). The median patient age was 67 years. Seventeen (33%) PFEs harbored pathogenic variants in tested KRAS codons (12 in codons 12/13; 5 in codon 61). Mutations were mutually exclusive. No clinical or pathologic correlates differed significantly from cases without detectable pathogenic variants. No pathogenic mutation were detected in LEs (n=20; P=0.002). Herein, we report on the largest series of PFE tested for KRAS mutations and present the largest cohort of KRAS-mutant PFEs to date, providing evidence in support of the notion that at least a subset of PFEs represents neoplasia. Moreover, the lack of KRAS mutations in LEs provides evidence as to the separate etiology of this accepted reactive lesion.

Papillary fibroelastoma is a rare, benign tumor that affects males more frequently than females and that tends to be diagnosed during the fifth or sixth decade of life. It tends to arise on cardiac valves, with the aortic valve being the most frequent location followed by the mitral valve, the tricuspid valve, and the pulmonary valve. We present the case of a robotic-assisted, totally endoscopic excision of a mitral valve papillary fibroelastoma.

CASE A 67-year-old female presented with an exceedingly rare cardiac neoplasm - papillary fibroelastoma. This is made rarer still as it occurred on the pulmonary valve. The patient complained of a prolonged history of chest discomfort. Magnetic resonance imaging and echocardiography revealed a pulmonary valve papillary fibroelastoma. Surgical excision proved curative and the patient remains asymptomatic to date. DISCUSSION The literature surrounding papillary fibroelastomas is discussed. Primary cardiac tumours are uncommon. Papillary fibroelastomas occurring the right side of the heart comprise less than 0.05% of these. They have a characteristic macroscopic appearance which allow them to be easily identified with echocardiography and at surgical excision. They can present in a variety of ways including classical cardiac symptoms, embolic complications or as an incidental finding. Surgical excision is the definitive treatment.

BACKGROUND Right anterior minithoracotomy (RAMT) is an alternative to full or partial sternotomy for accessing the aortic valve. The adoption of this approach for aortic valve replacement has been limited by its perceived technical complexity. Resection of a mobile aortic valve tumor is a simple procedure that is conventionally done through a sternotomy. AIM The following case describes the resection of an aortic valve papillary fibroelastoma through an RAMT. MATERIALS & METHODS This is a report on a single patient's case. RESULTS The fibroelastoma was safely excised through a RAMT approach. DISCUSSION An aortic valve fibroelastoma offers an ideal starting point for surgeons to begin using a RAMT approach. CONCLUSION The excision of aortic valve masses is a procedure that is well suited to minimally invasive strategies, and we suggest sternum-sparing RAMT is the optimal approach.

Papillary fibroelastomas have a range of clinical presentations. The surgical removal of these tumors should always be considered as best alternative to a conservative approach.

A 61-year-old male presented for an annual exam and received a transthoracic echocardiogram (TTE) which revealed a mobile mass arising from a subaortic membrane. Further investigations with a transesophageal echocardiogram (TEE) and cardiac computerized tomography angiography (CTA) confirmed the presence of a mobile 9 mm×3 mm mass on a subaortic membrane. Cardiothoracic surgery was performed with an open operation removing the mass and subaortic membrane. Upon visual inspection, the mass was likened to a sea anemone and immunohistochemical staining performed pathologically confirmed the diagnosis of cardiac papillary fibroelastoma. This case represents the first reported example of a cardiac papillary fibroelastoma (PFE) arising from a subaortic membrane. Although PFEs are benign cardiac tumors, proper identification and consideration for excision of these lesions may be indicated to prevent thromboembolic complications.

According to the research literature, the caseous calcification of the mitral annulus (CCMA) is a rare variant of the mitral annulus calcification (MAC) entity, described mostly in elderly women. The aim of this study was to present the case of a 53-year-old female patient with caseous calcification of the mitral valve annulus and posterior cusp, which was diagnosed as papillary fibroelastoma. An echo-dense and quasi-homogeneous tumoral mass, measuring 1.6/1.4 cm, at the level of the posterior mitral ring was detected by echocardiographic examination, as well as by cardiac magnetic resonance imaging (MRI). Histopathological analysis revealed fibrous connective tissue with myxoid areas, hyaline degeneration with unstructured necrosis and dystrophic calcifications, which was consisting with the operative findings of a “toothpaste tumor”, or caseous calcification of the mitral valve. Differential diagnosis with other cardiac tumors, abscesses, thrombi or fibroelastomas is emphasized.

Introdução: os tumores cardíacos são neoplasias raras, frequentemente assintomáticos e diagnosticados incidentalmente. São classificados como primários e secundários. Suas principais complicações são a embolização sistêmica e o acidente vascular cerebral. Por possuírem clínica inespecífica, é importante um diagnóstico apurado, baseado em exames complementares, a fim de evitar suas possíveis complicações e traçar um plano terapêutico com bom prognóstico. Relato do caso: paciente do sexo masculino procurou atendimento cardiológico ambulatorial por Hipertensão Arterial Sistêmica não controlada mesmo com uso correto das medicações. Submetido a um ecocardiograma transtorácico, que evidenciou uma massa compatível com fibroelastoma papilífero (FEP). Paciente continua em acompanhamento ambulatorial cerca de um ano após o diagnóstico, assintomático e sem complicações. Discussão: o FEP é um tumor que acomete principalmente as válvulas cardíacas, por volta dos 50 anos. Possui clínica inespecífica e seu tratamento definitivo é cirúrgico. O caso relata um paciente octagenário assintomático, diagnosticado com FEP em cordoalha e com tratamento ambulatorial. Conclusão: o FEP é uma condição rara e de difícil diagnóstico. Embora benigno, suas complicações podem ser fatais. Nesse contexto, é importante pesquisar eventos prévios de origem coronariana ou cerebrovasculares e fazer o uso de exames complementares para direcionar ao diagnóstico correto, evitando possíveis complicações.

Introduction Papillary fibroelastomas (PFEs) are a rare subtype of benign primary cardiac tumors, which are most commonly found on the aortic valve (AV). They have the potential to cause severe adverse clinical consequences, thus prompting surgical excision in the majority of cases. In this article, we report on a series of 5 PFEs resected from the AV using a robotic-assisted sternal-sparing approach, and review of the literature on this approach. To our knowledge, this is the largest reported series of robotic-assisted sternal-sparing AV-PFE resections. Materials and methods From May 2016 until November 2018, 5 patients at our institution underwent removal of a papillary fibroelastoma from the aortic valve using a totally endoscopic robotic-assisted approach. After obtaining institutional IRB approval, we retrospectively reviewed their data for this report. We additionally contacted the patients to acquire a last clinical follow up for this study. Results In this series of 5 patients who underwent robotic totally endoscopic excision of AV PFE, all patients had successful removal of their tumor with no significant morbidity or mortality. All 5 patients were seen at a 30-day follow-up office visit, at which time they had recovered from surgery and were back to full activity. At a mean of 24 months all patients were doing well and free of symptoms with no evidence of tumor recurrence on repeat echo evaluation. Discussion This is a series of 5 patients with AV-associated PFEs who underwent robotic totally-endoscopic excision without rib-spreading or conversion to sternotomy. This report demonstrated the successful application of robotic-assisted technology in aortic valve pathology. All PFEs were successfully removed without valve repair or replacement. We were able to offer a safe, curative, minimally-invasive surgical excision option for this group of patients. Conclusion Our patients in this cohort demonstrated the well-established benefits of robotic sternal-sparing cardiac surgery, including excellent intra and postoperative outcomes and accelerated recovery.

We present a rare case of papillary fibroelastoma (PFE) of the aortic valve diagnosed after being referred from a pre-anesthesia clinic. This patient presented in preanesthesia clinic for assessment prior to right total knee replacement. Along with other investigations, echocardiography was ordered as the patient had a previous history of ischemic heart disease with angioplasty. There was no previous echocardiogram (ECHO) in the patient records. An incidental finding of a sclerotic aortic valve with highly mobile mass was seen attached to the right coronary cusp on the aortic side with same echogenicity as the valve. Based on this rare finding, the patient was referred to an interventional cardiac center prior to an elective orthopedic surgery.Citation: Abdullah A, Fouad A, Esmat AM, Elhefnawy A, Adeel S. Pre-anesthesia clinic: skip it or not? A case report. Anaesth. pain & intensive care 2019;23(2):221-224

Primary cardiac tumors are rare, but papillary fibroelastoma (PFE) is reportedly the most common form, which usually occurs on the left-side valves of the heart. However, PFE involving the tricuspid and pulmonary valves has also been documented. Although PFE is benign and seldom associated with valvular dysfunction, the associated embolic complications may lead to serious consequences. Most patients with PFE lack specific clinical symptoms and the diagnosis is incidental. Surgical resection is the mainstay treatment for PFE in order to prevent the occurrence of embolic complications. In this report, we present a case of a rare asymptomatic PFE of the pulmonary valve, which was incidentally noted during a routine examination with transthoracic echocardiography (TEE). There was neither valvular dysfunction nor hemodynamic change. The PFE was surgically removed, and the diagnosis was further confirmed with histopathology.

Introduction: Cardiac tumors constitute only 0.2% of all tumors. Primary cardiac tumors are extremely rare with an incidence of 0.0017 to 0.19. Cardiac myxomas are the most common benign neoplasm of the heart. The prevalence is rare and incidence is 0.001% to 0.03%. Objective: To review our experiences with this rare entity and highlight the various aspects of myxoma presentation, histopathology and diagnosis in Western Maharastrian Population. Methodology: A total of 18 cases were accrued from the histopathology files of MH Cardiothoracic centre from 2009-2019 and studied. Results: A total 18 cases were included in the analysis. Mean age was 45 years, with equal male to female ratio (50%). Patients ranged from 01 months to 84 years old. Echocardiography was the diagnostic technique employed. The tumor was demonstrated by angiocardiography prior to surgery. Hemodynamically, left atrial myxomas revealed moderately severe pulmonary hypertension and right atrial myxomas, with right atrial hypertension. There were 12 myxomas in the left atrium, 3 in the right atrium, 1 in left ventricle, 1 in the Aortic valve and one case of papillary fibroelastoma in left atrium was reported. Eighteen patients underwent open-heart operation with removal of the myxoma. The size of myxoma varied from friable centimetric fragments to globular mass measuring 8.5cm in greatest dimension. The mean dimension of myxoma measured on echocardiography was 3.69 cm. 77.7% percent had left sided and 16.6% had right sided myxoma. Conclusion: Our results showed that the prevalence of cardiac myxoma in Western Maharashtra is comparable with national and global estimates however the female to male ratio was same, no gender predominance was noted. The mean age of presentation was 45 years, slightly lower than global pattern. Mean greatest dimension was 3.69cm. Prevalence of right atrial myxoma was slightly higher in our sample compare to other national and international published literature.

The prevalence of primary cardiac tumors varies from 0.02% to 0.45%.1 Cardiac papillary fibroelastoma (PFE) is a benign endocardial papilloma. It is considered the second most prevalent primary cardiac tumor after myxoma and the most prevalent valvular tumor.2 The typical clinical presentation is either cardiac or neurologic, including transient ischemic attack, stroke, ischemic heart disease, sudden death, ventricular arrhythmia, heart failure, syncope, and blindness.3 We report a rare case of PFE on the aortic valve in a patient with no previous cardiac history who presented with multiple cardiac arrests from electrical storm despite intravenous antiarrhythmic drugs that resolved after surgical removal of the tumor.

Papillary fibroelastoma (PFE) is a benign cardiac tumor usually incidentally found on cardiac imaging. They are typically located on the left-sided heart valves and are concerning primarily due to their risk for embolization into the periphery. Right-sided PFE, however, is very rare and their management is not well known. We present a 66-year-old male with a past medical history of HIV on antiretroviral therapy presenting with new exertional dyspnea. Upon workup, he was found to have a mass on the tricuspid valve seen on echocardiography which was ultimately resected and found to be a tricuspid fibroelastoma. The clinical management of right-sided PFE is poorly documented. Treatment of PFE in an asymptomatic is dependent on characteristics such as location, mobility, and risk of embolization. Echocardiography has made the incidental diagnosis of PFE a common issue in asymptomatic patients such as ours.

Background and aim of the study Robotic technology provides excellent visualization and surgical precision and it is reaching maturity in cardiac surgery, although mostly confined to mitral surgery and coronary revascularization. Robotic aortic valve surgery (rAVS) has not been sufficiently developed, and experience is extremely scarce. Methods We present a robotic resection of a papillary fibroelastoma on the aortic valve using a totally thoracoscopic right lateral approach. Results This technique provides excellent exposure, facilitates patient recovery and improves cosmesis. Conclusions rAVS has tremendous potential and many patients may benefit in the future. The lateral approach used in our case may offers advantages over others previously attempted and may also facilitate adoption of rAVS by teams currently performing robotic mitral surgery.

Background and aim Papillary fibroelastoma (PFE) represents only 16% of the benign cardiac tumor and approximately 15% of these are located on tricuspid valve. Materials and Methods Over a period of 22 years (1999-2021) we observed 75 pts with cardiac tumors at our Center over 9650 pts operated on. Most of them were mixoma but in 10 cases histology showed a PFE. We describe a rare case (1/75 of cardiac tumors) of a tricuspid valve PFE in a 69-year-old patient. Trans-thoracic echocardiography demonstrated a mobile mass (20 x 10 mm), adhering to the atrial side of the septal leaflet of the tricuspid valve. In consideration of the mobility of the mass and the consequent high embolic risk, surgical removal was made. Patient underwent surgery through a median sternotomy on CPBP. A “gelatinous” mass adhering to the tricuspid leaflet was found and completely removed. The postoperative course was uneventful. The pathological evaluation confirmed the diagnosis of PFE. Conclusions PFE of the tricuspid valve is rare entities being in most cases found incidentally. In our experience the incidence of this tumor in this location is 1/10000 cases of cardiac surgery. Although most patients are asymptomatic, surgical treatment is nevertheless recommended in consideration of the high embolic risk.

Difficult diagnosis is due to rarity of the case. TT or TE echocardiography is sufficient to make a correct diagnosis. The risk of embolism or coronary ostia occlusion should guide the decision for surgery.

Background: Cardiac papillary fibroelastoma is a rare, benign primary cardiac tumor that remains asymptomatic. Severe complications have been reported in some cases. The only effective therapy is surgical excision. Case presentation: Here, we report a case of cardiac papillary fibroelastoma with the initial symptom of chest pain and a first diagnosis of acute left main coronary artery disease. This patient eventually underwent tumor excision surgery and recovered well. Conclusion: For patients with symptomatic cardiac papillary fibroelastoma, we provide a series of comprehensive data from before, during, and after surgery. This might be helpful for the future diagnosis and treatment of these tumors.

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.

Papillary fibroelastomas (PFE) are benign neoplasms, mostly located on valvular surfaces with high embolic potential. This study presents a 27-year single institutional experience on surgical treatment of PFE in an adult patient- cohort with long-term follow-up. This study was approved by the institutional review board. Date and number of IRB approval: 11/23/2017, Institutional Review Board approval number A2014-0149. The need for individual patient consent was waived. We retrospectively evaluated all patients who underwent cardiac surgery for suspected space-occupying lesions in the observation period between June 1991 and June 2018 at our hospital. Clinicopathological features, imaging characteristics, surgical procedures and disease outcome were analyzed. 120 patients were diagnosed with various primary/secondary cardiac tumors and histology confirmed 21 PFEs were found in 16 patients. There was no significant age difference between patients with valvular vs nonvalvular PFEs (P = 0.26). Valvular lesions were found in aortic valve (n = 6), mitral valve (n = 2) and tricuspid valve (n = 1). Nonvalvular PFEs were found in right atrium (n = 2), left ventricle (n = 2), left atrial appendage (n = 2) and aortic wall (n = 1). Valvular lesions were significantly smaller in size compared to non-valvular lesions (P = 0.0013). Left-side PFEs were associated with a high embolization episodes (10/13 patients, 77%) not related to the size. One patient died in-hospital. All other patients were discharged out of the hospital postoperative. Follow-up was performed regularly for a median of 2.8 years (range 0.1–11 years) postoperative. Nonvalvular PFE tended to be larger in size and at least when located on the left sided heart had equally high propensity to embolize compared to valvular PFE. We strongly advocate surgical excision in all left-sided PFE.

Funding Acknowledgements Type of funding sources: None. Background Papillary fibroelastoma (PFE) is now regarded as the most common primary tumor of the heart. Although benign, they are clinically significant for their high risk of embolization. They are most commonly found on cardiac valves but can also be present on non-valvular endocardial surfaces. The aim of this study was to better characterize patients with left-sided non-valvular PFE and its clinical sequelae. Methods We retrospectively identified patients with pathology-proven PFEs at a single center between January 1995 and December 2018 (n = 279). Patients with left-sided non-valvular PFE were analyzed. Medical records were retrospectively reviewed for clinical characteristics and outcomes. In addition, intra-operative transesophageal echocardiograms were manually reviewed to estimate overall size and location. Results During the study period, we identified 37 patients with left-sided non-valvular PFE (mean age 61 ± 14 years; 62% females) (Table). PFEs were located on the left ventricle in 41%, left atrium in 35%, and left ventricular outflow tract in 24% of patients. Around a quarter of patients (27%) had a diagnosis of hypertrophic cardiomyopathy, 19% had prior cardiac surgery, and 27% had cancer diagnosed prior to PFE diagnosis. Transient ischemic attack or stroke was the presenting symptom in 22% of patients, myocardial infarction in 6% and peripheral embolization in 6%. Median maximal length for PFE on the left ventricle was 11.1 mm [3;18], on the left atrium 9 mm [2;25], and left ventricular outflow tract 8 mm [6;13]. A minority of patients (9/37 [24%]) had associated valvular PFE on the mitral valve and/or aortic valve (1 patient had both mitral valve and aortic valve PFE, 7 had aortic valve PFE and 1 had Mitral valve PFE). Only 13 patients had follow up transthoracic/transesophageal echocardiogram 1 year after PFE removal; 4/13 (31%) had documented PFE recurrence (3 PFE recurred in the same location as the original; 1 in a different location). Conclusion Left-sided non-valvular PFE is associated with thromboembolic events and at least in those that had follow-up echocardiograms, had a high recurrence rate. More studies are needed to evaluate the management of patients with asymptomatic PFE. Abstract Figure. Baseline Characteristics

Objective: To review the current literature on the state of the art of evaluation and management of cardiac papillary fibroelastoma (PFE). Methods: A literature review was performed on the topic of evaluation and management of PFE. PubWeb, Web of Science, Google Scholar, Scopus search engines were used. Key search terms included: “Cardiac papillary fibroelastoma,” “papillary fibroelastoma,” “Surgical resection of papillary fibroelastoma,” “Minimally invasive resection of papillary fibroelastoma,” “Thoracoscopy and PFE.” Relevant articles were archived and synthesized in an EndNote database. Particular attention was focused on identifying cases of minimally invasive resection of PFE and the use of thoracoscopic adjuncts to assist with cavitary PFE removal. Institutional Review Board approval was waived. Results: A total of 119 articles were identified and archived. Forty seven articles were used to compose this paper. 8 articles detailed minimally invasive resection of PFE. The largest series of minimally invasive PFE resection summed 4 patients. There are currently no guidelines to direct care of patients with PFE. General consensus is that the presence of left sided PFE should prompt evaluation for surgery. Conclusions: PFE is a rare tumor, more common in children than adults. Lesions are typically left sided and are at risk for embolization. Minimally invasive surgery is feasible and safe. Long-term outcomes with complete resection are very good.

Papillary fibroelastoma (PFE) is one of the most common tumour of heart valves and found to be second common primary cardiac tumour. Only less than 50 cases were reported in the literature( Remadi et al., 2004) . 1 Due to the advancement in 3D echocardiography, its diagnosis in living patient is become easy. The PFE could be found on both ventricles, valvular leaflets, and chordae tendinea. We present an interesting case of the CPF originating from the posterior mitral leaflet confusing with vegetation or annular calcification. Successful surgical excision was done of the papillary fibroelastoma.

Background: Papillary fibroelastoma (PFE) is the most common benign cardiac tumor. Because PFEs have the potential to embolize, they typically are surgically excised. Prior studies have suggested that postoperative recurrence of PFE is rare or does not occur. Objectives: To determine the rate at which PFEs recurred after surgical removal and to identify any risk factors associated with recurrence. Methods: Patients with pathologically proven PFE were retrospectively identified from a single center. Patients were included in the study if they had an echocardiographic examination at least 1 year after surgery. Echocardiographic images obtained intraoperatively (after excision) and at dismissal were compared with those of the most recent examination to assess the possibility of PFE recurrence. Results: Ninety eight patients were identified. The mean (SD) duration of follow-up was 5.4 (3.7) years (range, 1-17 years). Twelve patients (12.2%) had echocardiographically proven PFE recurrence. Three patients had the recurrent lesion surgically re-excised, and pathologic analysis showed that 2 were recurrent PFEs and 1 was a Lambl excrescence. Clinical presentation of stroke or TIA was more common for the recurrence patients (for the first PFE) than for the nonrecurrence patients (83% vs 26%; P Conclusions: Contrary to published data, PFEs do recur after surgical excision. This emphasizes the importance of postoperative follow-up with transesophageal echocardiography for identifying recurrent masses. Table. Characteristics of Patients With Recurrent PFE Abbreviations: AV, aortic valve; LA, left atrium; LE, Lambl excrescence; LVOT, left ventricular outflow tract; MV, mitral valve; PV, pulmonary valve; RA, right atrium; RC, right cusp; TV, tricuspid valve. a Corresponds to the same location. b Corresponds to a different location .

Background Papillary fibroelastoma (PFE) is the most common primary benign cardiac tumor. Because PFEs have the potential to embolize, they often are surgically excised. Prior studies have suggested that postoperative recurrence of PFE is rare or does not occur. We aimed to determine the rate at which PFEs recurred after surgical removal and to identify any risk factors associated with recurrence. Methods We retrospectively identified all patients from a single center with pathologically proven PFE, treated from January 1995 through December 2018. Patients were included in the study if they had an echocardiographic examination at least 1 year after surgery. We compared echocardiographic images obtained intraoperatively (after excision) and at dismissal with those of the most recent examination to assess the possibility of PFE recurrence. Results We included 98 patients in the study. The mean (SD) duration of follow-up was 5.4 (3.7) years (range, 1-17 years); the median duration of follow-up was 4.3 years (interquartile range, 1.9-7.7 years). Twelve patients (12.2%) had echocardiographically supported PFE recurrence. Three patients had the recurrent lesion surgically re-excised, and pathologic analysis showed that 2 were recurrent PFEs and 1 was a Lambl excrescence. Initial clinical presentation of stroke or transient ischemic attack was more common for the recurrence group (for the first PFE) than for the nonrecurrence group (83% vs 26%; P Conclusions Contrary to findings from previous studies, PFEs do recur after surgical excision. These findings emphasize the importance of postoperative follow-up with transesophageal echocardiography for identifying recurrent masses.

Cardiac tumors, which are rare cardiac diseases, can be classified into primary cardiac tumors and secondary cardiac tumors. In 2004, the WHO classified cardiac tumors into benign tumors, tumor-like lesions, and malignant tumors [1]. 75% of primary cardiac tumors are benign, and the other 25% are malignant tumors. According to previous reports, three-fourths of all cardiac tumors are benign tumors, nearly half of which are myxomas, with the other half being lipomas, papillary fibroelastoma, and rhabdomyomas; most malignant tumors were undifferentiated sarcomas, followed by angiosarcoma, rhabdomyosarcoma, lymphoma, and so on [2].

Papillary fibroelastomas represent the second most common benign cardiac tumour, secondary only to cardiac myxoma. A majority of patients are asymptomatic on presentation. The most common clinical manifestations include stroke, transient ischaemic attack, myocardial infarction and angina. Echocardiography remains the primary imaging modality for identification of these tumours. The majority of papillary fibroelastomas arise from the valves. Simple surgical excision is the mainstay of treatment, carrying an excellent prognosis. We present an unusual case of cardiac papillary fibroelastoma originating from the coumadin ridge (CR) in a 70-year-old woman. The patient exhibited increasing paroxysms of her atrial fibrillation and was pursuing a MAZE procedure. Preoperatively, a transesophageal echocardiogram revealed a 0.7×1 cm intracardiac mass that had echocardiographic appearance of a fibroelastoma. Surgical resection and MAZE procedures were performed. The gross specimen and histopathology findings were consistent with papillary fibroelastoma. This case reports the seventh documented case of fibroelastoma originating from the CR.

Patient: Male, 39-year-old Final Diagnosis: Papillary fibroelastoma Symptoms: Chest pain • fever • hemoptysis • weight loss Medication: — Clinical Procedure: — Specialty: Cardiac Surgery • Cardiology • Critical Care Medicine • Pulmonology Objective: Rare disease Background: Papillary fibroelastoma accounts for approximately 24% of all primary cardiac tumors and is the third most common primary cardiac neoplasm. It mostly involves the cardiac valves; however, there have been cases of involvement of the endocardium. Papillary fibroelastoma, although typically benign, can cause a plethora of complications, including systemic embolization, myocardial infarction, arrhythmia, and sudden cardiac death. In this article we present a unique case of a man diagnosed with a papillary fibroelastoma of the right ventricle in the setting of multifocal pulmonary hemorrhages and pulmonary embolization. Case Report: A 39-year-old man presented with hemoptysis and dyspnea due to bilateral lower-lobe pneumonia and a left pleural effusion. Further imaging throughout his hospitalization revealed worsening multifocal consolidations, pulmonary hemorrhages, and a pulmonary embolus. A mobile cardiac mass measuring 30.2×20 mm, detected by echocardiography, was found in the right ventricle, partially fixed to the intraventricular septum via a 14.4-mm stalk, which was surgically excised and was found to be consistent with papillary fibroelastoma. He underwent an IVC filter and was discharged on warfarin, which he tolerated well. Conclusions: Papillary fibroelastomas are benign infrequent findings but carry a high risk of systemic complications, as demonstrated in our patient. He had no known cardiac disease or risk factor for tumor growth, yet developed a papillary fibroelastoma of the right ventricle, which is rare. Given the potential of fatal outcomes, it is imperative that patients with unexplained embolic phenomena undergo early diagnosis by echocardiography and early surgical treatment.

Embolic stroke secondary to cardiac tumors is uncommon. However, 25-30% of cardiac tumors may cause systemic emboli. We report a 29-year-old male consulting for a sudden episode of aphasia and right hemiparesis, compatible with infarct of the left middle cerebral artery territory. Transthoracic echocardiography reported an ovoid tumor of 8 × 7 × 7 mm in relation to the sub valvular apparatus of the mitral valve. After neurologic stabilization, surgical treatment was performed. Approached by median sternotomy and in cardiopulmonary bypass, the mitral valve was explored. A macroscopic tumor consistent with a papillary fibroelastoma curled in sub valvular chordae was found. It was deployed and resected from its base, while the anterior mitral leaflet was preserved intact. Histopathological examination confirmed the intraoperative macroscopic diagnosis. The patient recovered uneventfully postoperatively and was discharged on the fifth day after surgery. He currently is in functional capacity I without cardiovascular symptoms at five years follow-up.

Cardiac masses are rare, but remain an important component of cardio-oncology practice. These include benign tumors, malignant tumors (primary and secondary) and tumor-like conditions (e.g., thrombus, Lambl’s excrescences, and pericardial cyst). The advent of multimodality imaging has enabled identification of the etiology of cardiac masses in many cases, especially in conjunction with information from clinical settings. This paper provides a comprehensive review of the epidemiology, clinical presentation, imaging, diagnosis, management, and outcomes of cardiac masses., Central Illustration, Highlights • Cardiac tumors are rare and should be considered as part of the differential diagnosis of any space-occupying mass noted on cardiovascular and/or thoracic imaging modalities. • It may be possible to get close to a diagnosis without biopsy using a structured imaging approach. • The prognosis and treatment of each tumor is different, although early diagnosis is usually associated with a better outcome.

Papillary fibroelastoma (PF) of the right atrium accounts for 2% of nonvalvular cases, and right appendage (RAA) PF was described only once in literature. We present three cases of RAA PF in patients with unrelated symptoms undergoing 3-dimensional transesophageal echocardiographic examination (3D-TEE) scheduled for conventional indications. Key to diagnosis in routine practice resides in systematic examination of the right atrium and RAA in live 3D-TEE imaging with backward and forward navigation of the real-time pyramidal data. A review of literature is provided. Our experience demonstrates that systematic imaging of all cardiac structures with 3D-TEE allows refining PF nosology.

Background Trial data indicate an increased risk of VTE with JAK inhibition. The EMA and FDA have given regulatory advice for their use as a result. To understand the context of any association, we need to understand VTE risk in the background population. Our objective was to describe the changing epidemiology of VTE risk including a breakdown of DVT and PE in the general population in England, over a 20-year period. Methods We utilised hospital episodes statistics data to study all patients presenting for hospital care in England between 1 January 1998 and 31 December 2017. We identified VTE events using ICD10 codes I26.0, I26.9, I80.1, I80.2 and I80.3. To estimate annualised event rates, we used the number of finished consultant episodes for admitted care where the primary diagnosis was VTE for the numerator; the denominator was the whole population estimate, from the office for National Statistics, for the relevant year for England. Rates were calculated per 100,000 population, and the Cochrane Armitage test was used to evaluate statistical significance of trends over time. Linear regression allowed modelling of estimates over time, with model assumptions tested using residual versus fitted plots. To explore non-linearity, we used a cubic spline model. Results The average VTE rate across the 20 years was 127/100,000. VTE rates have increased over time, climbing from 108/100,000 in 1998, to 151/100,000 in 2017. The relative frequency of DVTs and PEs has changed over this time: in 1998 DVT was more frequent than PE (rates 68/100,000 and 40/100,000 respectively). By 2018 this ratio had reversed (DVT 52/100,000: PE 98/100,000). This change was statistically significant (p < 0.0001). DVT rates declined in a linear manner over time. PE rates increased with a non-linear pattern, with a sharp rise apparent between 2008 and 2010. Conclusion The inversion in the frequency of DVTs and PEs is consistent with previous findings using European data. One possible explanation is changing diagnostics (CTPA replacing V/Q), capturing previously subclinical PE events. Limitations include our ecologic design as well as lack of linkage to mortality records. The presumed VTE risk associated with JAK inhibition is small. It is plausible that future shifts in population wide patterns of VTE could obscure or amplify any effect attributable to JAK inhibitors. As longer-term observational data become available, it is crucial they are interpreted in the context of background population trends. Disclosures S. Baroncini: None. M. Yates: None. K. Bechman: None. S. Patel: None. A. Rutherford: None. A. Kleymann: None. J. Galloway: None.

We present a case of a 70-year-old woman with exertional shortness of breath. A transthoracic echocardiogram showed a large mass on the ventricular side of the pulmonary valve. The anatomy of the mass was additionally investigated with a transesophageal echocardiogram, which delineated the anatomical details of the structure. The mass was surgically excised, and histopathology confirmed a cardiac papillary fibroelastoma.