Papillary Fibroelastoma Research Articles

Left ventricular chord masquerading as an aortic valve papillary fibroelastoma

Abstract Background Characterization of cardiac masses and anomalies is challenging and usually requires multimodality imaging. Herein, we present the case of a left ventricular chord masquerading as an aortic valve papillary fibroelastoma. Case summary In this case, a 62-year-old woman presented with acute right foot weakness. Brain imaging showed multiple areas of acute infarction concerning for a cardioembolic stroke. Transesophageal echocardiography revealed a mass, which was thought to be a papillary fibroelastoma, attached to the non-coronary cusp of the aortic valve. The patient underwent surgical excision of the mass which turned out to be a left ventricular chord crossing the aortic valve and attaching to the left aortic sinus of Valsalva. Conclusion This case underlines the importance of close examination of the aortic subvalvular apparatus when assessing aortic valve masses.

Abstract P172: Pulmonary Valve Papillary Fibroelastoma In A Patient With Chronic Chest Pain

Primary cardiac tumors are rare entity, and the most common benign cardiac neoplasms include myxomas, lipomas and papillary fibroelastomas (PFE) with PFE accounting for 8% of the tumors. A 53-year-old female presented with chronic chest pain and exertional shortness of breath. She first sought evaluation four years ago, when her pulmonologist referred her to thoracic surgery after an incidental finding of a 3.9 cm anterior mediastinal mass on CT-scan (Figure 1A-B). The patient described the pain as a constant pressure and squeezing sensation in her chest and worsening shortness of breath while laying on her sides, relieved only when a pillow was propped up under her chest. The pain did not significantly reduce her mobility or capacity to work. Initial evaluation, including transthoracic echocardiogram, stress testing, carotid ultrasound, and pulmonary function testing - was performed and found to be unremarkable. Further evaluations revealed a hiatal hernia, leading her symptoms to be attributed to GERD as causing her persistent non-exertional chest pain. The patient remained in good health despite her symptoms persisting. She later re-sought attention for chest pain on exertion and hoarseness three years later. She again underwent a full cardiopulmonary workup, yielding benign findings on EKG, troponins, and echocardiogram. Due to no alarming symptoms at the time, she was subsequently discharged. The patient continued to endorse chest pain and a follow-up MRI revealed a now 5 cm anterior mediastinal mass, thought to be a thymic cyst. Over the next few months, the patient continued to endorse worsening chest pain and exertional shortness of breath. She followed up with her cardiologist and a subsequent TEE was significant for a pulmonic valvular mass. She was admitted to the hospital for further investigation. During her hospital stay, the patient underwent surgery to resect the anterior mediastinal mass likely to be a thymic cyst and the pulmonary artery mass which was determined to be a pulmonary valve papillary fibroelastoma in the histopathology exam (Figure 2). The surgery was successful with no complications postoperatively. The patient was discharged with no further chest pain and with improvement of the exertional shortness of breath.

A Case of Multiple Cardiac Papillary Fibroelastoma in the Aortic Valve

A Case of Multiple Cardiac Papillary Fibroelastoma in the Aortic Valve

Robotic resection of an aortic valve fibroelastoma using a right lateral approach.

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. We present a robotic resection of a papillary fibroelastoma on the aortic valve using a totally thoracoscopic right lateral approach. This technique provides excellent exposure, facilitates patient recovery and improves cosmesis. 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.

ECHOGENIC MASS ATTACHED TO TRICUSPID VALVE

SUMMARY
 A 5-day old female child with complaints of tachypnea, had TTE which revealed an echogenic mass on septal leaflet of Tricuspid valve. Based on echocardiographic features, it was diagnosed as Papillary fibroelastoma.
 CASE DESCRIPTION
 A 5-day old female child was referred for transthoracic echocardiographic study due to respiratory difficulty. The child was afebrile and had a respiratory rate of 60/min, heart rate 130 bpm, and blood pressure 80/50 mmHg. No murmur or added sound heard on CVS examination. TTE revealed a dense mass (Figure 1) attached to right atrial side of septal leaflet of Tricuspid valve by a small pedicle. The mass was mobile, prolapsing into right ventricle during diastole (Clips) without causing obstruction with size of 0.8 X 0.74 cm. Intracardiac masses attached to valves are usually Papillary fibroelastomas. Other possible differential diagnoses could be myxoma, vegetation, Lambl’s excrescence or secondary tumors etc. Based on the location and echo features of the mass (mobility, attachment to downstream side of valve, and frond like speckled appearance) the diagnosis of Papillary fibroelastoma was made.
 LEARNING POINTS
 
 Cardiac tumors are rare and mostly benign.
 The three most common primary tumors of heart, in order of frequency, are myxomas, lipomas and papillary fibroelastomas respectively. PFE constitute 7% - 9% of all.1
 PFE usually arise from valves of the heart and are mostly left-sided.2
 They are mobile, range from 0.5 – 2 cm in size and have a frond-like appearance. On echo, they are dense with a speckled interior and shimmering surface.3
 Being prone to embolization and thrombosis, their clinical course may not be benign, especially left sided.
 
 Clips
 
 Subcostal Clip: https://youtu.be/_xTZmx--WcA
 PS SAX View: https://youtu.be/lLh6D9Vn4Fw
 A4C View: https://youtu.be/_xZZvTIh0S8
 A4C RV Directed View: https://youtu.be/vJRyHrevftA
 
 QUESTIONS WITH ANSWERS
 
 Question 1: What is the most common complication of papillary fibroelastoma?
 Question 2: The behavior of right and left sided papillary fibroelastomas could be markedly different, Y/N?
 Question 3: What is the most important feature of papillary fibroelastoma for embolization?
 Question 4: PFE preferentially affects females, Y/N?
 
 Answers
 
 Question 1: Cerebral embolism
 Question 2: Yes
 Question 3: Mobility
 Question 4: No
 
 References
 
 Bouhzam N, Kurtz B, Doguet F, Eltchaninoff H, Bauer F. Incidental papillary fibroelastoma multimodal: imaging and surgical decisions in 2 patients. Tex Heart Inst J. 2012;39(5):731-5.
 Eidem BW, Cetta F, O’ Leary PW. Echocardiography in pediatric and adult congenital heart disease. Lippincott, Williams and Wilkins, Wolters Kluwer. 2010 p 355-361.
 Klarich KW, Enriquez-Sarano M, Gura GM, Edwards WD, Tajik AJ, Seward JB. Papillary fibroelastoma: echocardiographic characteristics for diagnosis and pathologic correlation. J Am Coll Cardiol. 1997;30(3):784-90.

Large papillary fibroelastoma of right atrium, an unusual case of respiratory distress in a young man

BackgroundPapillary fibroelastomas are rare but benign cardiac tumour that are often found on cardiac valvular surfaces. Their clinical manifestations ranging from clinically asymptomatic to substantial complications that are usually secondary to systemic embolism. Multiple theories have been proposed to explain the pathophysiology of its formation.Case presentationWe reported a rare case of large papillary fibroelastoma in the right atrium of a young gentleman which was complicated with pulmonary embolism. Transthoracic echocardiography identified a large pedunculated mass measuring 3.4cmX3.4cmX2cm in right atrium with stalk attached to interatrial septum. The intracardiac mass was resected surgically, which revealed papillary fibroelastoma in histology examination.ConclusionDifferential diagnosis of intracardiac masses requires clinical information, laboratory tests and imaging modalities including echocardiography. Incidentally discovered papillary fibroelastomas are treated on the basis of their sizes, site, mobility and potential embolic complications. Due to the embolic risk inherent to intraacardiac masses, surgical resection represents an effective curative protocol in treating both symptomatic and asymptomatic right sided and left sided papillary fibroelastomas, with excellent long term postoperative prognosis.

Cardiac papillary fibroelastoma.

Papillary fibroelastoma (PFE) is a primary, histologically benign endocardial neoplasm. Though PFE has long been reported as the second most common primary cardiac neoplasm, it has since pulled ahead of cardiac myxomas, largely due to evolving cardiac imaging modalities. While PFEs are benign histologically, they have the potential for devastating clinical consequences, transient ischemic attack, stroke, myocardial infarction, syncope, pulmonary, and peripheral embolism. Despite increased detection rate, there remains uncertainty regarding etiology, exact prevalence, and clinical management of PFEs. This paucity of information is reflected by the lack of official guidelines on this matter. In this article, we aim to summarize the current state of understanding regarding PFE and discuss areas of ongoing controversy.

Surgery for Valvular and Nonvalvular Papillary Fibroelastomas

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.

Recurrence of Pathologically Proven Papillary Fibroelastoma

BackgroundPapillary 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. MethodsWe 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. ResultsWe included 98 patients in the study. The mean duration of follow-up was 5.4 (SD 3.7) years (range, 1 to 17); the median duration of follow-up was 4.3 years (interquartile range, 1.9 to 7.7). Twelve patients (12.2%) had echocardiographically supported PFE recurrence. Three patients had the recurrent lesion surgically reexcised, and pathologic analysis showed that two were recurrent PFEs and one 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 < .001). ConclusionsContrary 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.

A Case of a Tricuspid Valve Papillary Fibroelastoma and Nonspecific Cardiac Symptoms

A 66-year-old woman was referred for investigation of syncope, dyspnea, and mild chest discomfort. A transthoracic echocardiogram revealed a mass attached to the atrial aspect of the septal leaflet of the tricuspid valve. Blood cultures were immediately taken to rule out a differential diagnosis of infective endocarditis. The patient underwent successful surgery to excise the mass and preserve the tricuspid apparatus. Surgical pathology confirmed the diagnosis of a papillary fibroelastoma. Surgical intervention should be considered in these cases due to the risk of embolization and the possibility of malignant tumors. This case emphasis the importance of a conducting an echocardiogram and including the cardiac valves.

Giant and multiple cardiac papillary fibroelastomas masquerading as acute cerebrovascular events.

Cardiac papillary fibroelastomas are rare benign cardiac tumors. Their clinical significance results from their very high propensity for embolization. We present two such cases, which are rare regarding the size and multiplicity of these tumors.

Lambl's excrescences in transcatheter aortic valve implantation: a word of caution on the way to tailored and individualized approach.

article: Lambl’s excrescences in transcatheter aortic valve implantation: a word of caution on the way to tailored and individualized approach - Minerva Cardiology and Angiology 2022 February;70(1):5-7 - Minerva Medica - Journals

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.

Papillary fibroelastoma: an unexpected finding on the aortic valve.

Papillary fibroelastoma: an unexpected finding on the aortic valve.

Clinical and echocardiographic characteristics of patients with pathology proven left-sided non-valvular papillary fibroelastoma

Abstract 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

Valvular fibroelastomas complicated by Coxiella burnetti endocarditis

Cardiac papillary fibroelastoma (CPF) is a valvular tumor that may be mistaken for infective endocarditis (IE). We describe a case of CPF complicated by Coxiella burnetti IE. According to Duke's criteria, a diagnosis of IE was repeatedly considered as excluded or established during the clinical course, highlighting the criteria limitations.

Teleost fish: a new spontaneous model for the study of Lambl's excrescences.

Lambl's excrescences (LE) are fibrous strands typically occurring at coaptation lines of cardiac valves. Although they have been widely reported in human medicine, information on LE occurrence in veterinary medicine is still scarce. The present study aimed to investigate the presence of LE in different freshwater fish and in swordfish. A total of 185 hearts were collected from different fish species, and samples of different cardiac areas (sinus venosus, atrial wall with sinoatrial valves, ventricular wall with atrioventricular valves and bulb with bulboventricular valves) were submitted to histopathological evaluation. LE were detected in 6 of 103 freshwater fish (5.8%) and 19 of 82 swordfish (23.2%). LE developed in atrioventricular, sinoatrial and bulboventricular valves. All affected valves also showed endocardiosis. Based on the results of the present work, teleost fish, specifically swordfish, could be proposed as a novel spontaneous model for the study of LE pathogenesis.

Surgical Treatment of Benign Cardiac Tumors

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. 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.

TAVR for severe aortic stenosis and papillary fibroelastoma in a high-risk setting.

Papillary fibroelastomas (PFE) are rare benign cardiac tumours mainly originating on aortic and mitral valvular surfaces. Management is individualised, but most recommend surgical excision due to thromboembolic risk. We report a 75-year-old man with symptomatic severe aortic stenosis compounded by PFE. Redo sternotomy aortic valve replacement was deferred in favour of the trans-apical (TAVR) approach. This report highlights, for the first time, the application of TAVR as a strategy for aortic valve stenosis and PFE to mitigate risk posed by injury to patent internal mammary arterial graft in close proximity to the manubrium, and complications due to the patient's multiple comorbidities.

A case of recurrent multiple cardioembolic stroke due to papillary fibroelastoma

A case of recurrent multiple cardioembolic stroke due to papillary fibroelastoma