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.

Tumores cardíacos benignos operados en un Centro de Cirugía Cardiovascular Regional 2015 - 2021 / Benign cardiac tumors operated on at a regional cardiovascular center (2015 - 2021)

Introducción: Los tumores cardíacos primarios se caracterizan por su baja prevalencia, son principalmente mixomas y se presentan frecuentemente de forma asintomática. Objetivos: Identificar el tipo histológico más común, edad de presentación, tipo de cirugías y sobrevida de un grupo de pacientes tratados por Tumores Cardíacos Primarios (TCP) en el Hospital Regional de Temuco. Métodos: Revisión de fichas clínicas de 14 pacientes portadores de TCP entre marzo 2015 y diciembre 2021. Resultados: El tipo histológico más común fue el mixoma (85,7%), seguido por el fibroelastomas papilar (14,3%). La edad promedio fue 62 años (39-85), 9 fueron mujeres y 5 hombres. Los antecedentes mórbidos más comunes fueron: Insuficiencia Cardíaca Congestiva (ICC), Hipertensión Arterial (HTA) y Accidente Vascular Encefálico (AVE). La localización anatómica más común fue la Aurícula izquierda (92%). El tratamiento en el 92% de los casos fue resección aislada y en el 7% restante resección y reparación con parche. Conclusiones: Nuestros resultados son concordantes con la literatura.

Background: primary cardiac tumors are characterized by a low prevalence. Most of them are myxomas and asymptomatic. Aim: To describe the most common histological type, the age of presentation, type of surgery performed and survival of a group of patients operated on for Primary Cardiac Tumors (PCT) in the Hospital Regional de Temuco (Chile). Methods: Review of clinical records of 14 patients with PCT operated on between March 2015 and December 2021. Results: By far the most common histological type was a myxoma (85.7%), followed by a papillary fibroelastoma (14.3%). Mean age was 62 years (39-85), 9 were women and 5 men. The most common associated medical conditions were Congestive Heart Failure (CHF), Arterial Hypertension and Stroke The usual anatomical location was the left atrium (92%). Surgical treatment was isolated resection in 92% of cases and along with a patch repair in the remaining patient. Conclusion: good results were obtained, similar to those described in the literature.

CARDIAC PAPILLARY FIBROELASTOMA: A CASE WITH UNPRECEDENTED SPREAD.

INTRODUCTION: Papillary fibroelastomas (PFE) are a rare primary cardiac neoplasm, out of which multifocal PFE constitute a small minority of cases. These benign masses are commonly found on valvular surfaces, particularly the aortic valve. CLINICAL CASE: We present a patient with a history of embolic stroke and intra-cardiac masses. Multimodal imaging revealed multiple nodules with extensive intra-cardiac distribution. All nodules were successfully removed without valve dysfunction. CONCLUSION: This is a unique case of multiple PFE involving intracardiac cavities as well as all valvular structures.

Cardiac Papillary Fibroelastoma Result in Acute Left Main Coronary Artery Disease: A Case Report.

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.

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

BACKGROUND: Papillary 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 PRESENTATION: We 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. CONCLUSION: Differential 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.

Long-term follow-up of resection of primary benign right ventricular tumours: a 10-year surgical experience.

INTRODUCTION: Primary benign right ventricular tumours are rare. They can cause significant mortality without appropriate and timely treatment. We investigated surgical treatment and survival characteristics for right ventricular tumours. MATERIALS AND METHODS: From 2007 to 2017, 21 patients with primary benign right ventricular tumours who underwent tumour resection were retrospectively reviewed. Clinical findings and follow-up results were analysed. RESULTS: Thirteen men and eight women were enrolled, with a mean age of 42.3 ± 15.3 years. The most frequent histotypes were myxoma, haemangioma and papillary fibroelastoma. Eight patients underwent concomitant tricuspid valvuloplasty and one had tricuspid valve replacement. No major adverse events or death occurred during the perioperative period. One patient with haemangioma underwent partial tumour resection; however, the tumour regressed gradually during follow-up. Within the 10-year follow-up period (mean 4.8 ± 2.6 years), the recurrence-free and overall survival rates were 81.0% and 85.7%, respectively. CONCLUSIONS: Tumour resection for primary benign right ventricular tumours is safe and effective, and has a good prognosis. Tricuspid valvuloplasty or tricuspid valve replacement may be necessary for the resection of right ventricular tumours to improve the haemodynamics. Haemangiomas naturally undergo spontaneous regression.

[Aortic valve papillary fibroelastoma and anomalous origin of the right coronary artery]. / Fibroelastoma papillare della valvola aortica e origine anomala dell'arteria coronaria destra.

We report a case of a 48-year-old man who complained episodes of angina at rest. Instrumental investigation demonstrated a mass involving the ventricular side of the aortic valve suggestive for papillary fibroelastoma; an anomalous origin of the right coronary artery from the left sinus of Valsalva and no significant coronary artery disease were shown as well. The patient underwent open heart surgery, the tumor was excised and the aortic valve was spared. Pathological examination confirmed the diagnostic hypothesis of papillary fibroelastoma. The resolution of anginal symptoms after surgery suggests the possibility of papillary fibroelastoma causing these symptoms.

Cardiac papillary fibroelastoma originating from the coumadin ridge and review of literature.

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.

A rare case of a pulmonary 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.

Double valve involvement: papillary fibroelastoma in a patient with severe mitral and aortic valve regurgitation.

Cardiac papillary fibroelastoma is a benign neoplasm that arises in the endocardium. It commonly presents as an incidental finding on transthoracic echocardiography or as emboli to the coronary, cerebral or pulmonary vasculature. Clinical manifestations described in the literature have generally been related to a sequelae of the associated embolic phenomenon of these lesions. Valve regurgitation is less common with papillary fibroelastoma and when found, it is not known to cause severe regurgitation requiring valve replacement. We report a case of papillary fibroelastoma in a patient with severe mitral and aortic valve regurgitation in association with mobile masses requiring double valve replacement. This patient managed initially as infective endocarditis with severe double valve regurgitation, was found to have valvular masses concernng for papillary fibroelastoma and subsequently confirmed on pathology.

Papillary Fibroelastoma of the Right Ventricle in the Setting of Multifocal Pulmonary Hemorrhages and Pulmonary Embolization in a 39-Year-Old Man: A Case Report.

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.

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.