Tumours of the heart valves.

Cardiac Papillary Fibroelastoma: A Treatable Cause of Transient Ischemic Attack and Ischemic Stroke Detected by Transesophageal Echocardiography

Papillary fibroelastomas (PFEs) are benign cardiac tumors, most commonly arising on left sided heart valves but have been reported on non-valvular structures. Histologically, PFEs are comprised of throngs of an avascular mucopolysaccharide core with an overlying layer of endothelial cells. While PFEs carry no inherent malignant potential, the risk of embolization poses a unique challenge to clinicians. Case series exist describing the anatomical characteristics of PFEs, though as echocardiography has become more ubiquitous and imaging quality has dramatically improved, little contemporary data exists on the patient characteristics, management techniques employed, and outcomes. Our objective was to describe a quaternary center’s experience with PFEs. A single quaternary center, retrospective case review of patients with a diagnosis of cardiac tumor between Janurary 1 2008 and Janurary 30 2024, using ICD codes D15.1 and I42.4, resulted in 410 patients. Using chart review, 80 of the 410 patients had a suspected diagnosis of PFE based on TEE or TTE characteristics. We then performed a retrospective analysis of the 80 patients. Of the 80 patients, 47 underwent surgical resection, 5 roboticly, with no evidence of PFE on subsequent TTE. The median age at time of diagnosis was 67.7 +/- 13.8 years. 35 patients had a diagnosis of stroke any time prior to PFE identification. The most common indication for the diagnostic echocardiogram was arterial emboli. Valvular PFEs were commonly found on the upstream aspect of valves (55.9%) and most commonly located on the aortic valve (63.8%). Of the 27 patients with a diagnosis of stroke and PFE size data, 20 had a PFE >/= 5 mm. In the non-surgical group 7 were on P2Y12 inhibitor or therapeutic anticoagulation for the indication of PFE. Within 3 years there was 1 mortality in the surgical group and 6 in the non-surgical group. In the non-surgical group 15.2% were hospitalized for bleeding at 3 years verse 6.25% in the surgical group. While PFEs have been previously characterized in case series, there remains a scarcity of literature on their clinical presentation and management techniques. This single center case series demonstrates an association between PFEs and arterial embolic events with low three-year mortality. There may be a relation to PFE size and stroke as stroke was more common in those with a PFE >/= 5mm. Additional studies evaluating PFE characteristics and treatment strategies are necessary to guide PFE management.

Papillary fibroelastoma (PFE) is the second most common benign primary cardiac tumor, usually affecting left-sided valves. Pulmonary valve (PV) involvement is extremely rare, representing less than 8% of all PFEs. Although often asymptomatic, PFEs can present with embolic events. We report a 60-year-old man admitted with acute dyspnea, orthopnea, and lower-limb edema. Transthoracic echocardiography revealed severe left ventricular systolic dysfunction (ejection fraction: 15%), severe functional mitral regurgitation, and a mobile echogenic mass (1.0 × 0.8 cm) attached to the arterial surface of the PV consistent with a PFE. Chest computerized tomography (CT) confirmed bilateral segmental pulmonary emboli with infarctions and pulmonary artery dilation. Coronary angiography excluded ischemic disease. Given the patient’s new-onset heart failure with reduced ejection fraction and high operative risk, the heart team opted for initial anticoagulation and optimized heart failure therapy, postponing surgical resection until stabilization. PV PFE is a rare but potentially fatal cause of pulmonary embolism. Multimodality imaging is crucial for diagnosis, and individualized treatment is required to balance embolic risk and surgical mortality.

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. 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.

Introduction Primary cardiac tumors including papillary fibroelastomas are rare, with an incidence as low as 0.1%, but they pose considerable embolic risk. When untreated, cardiac fibroelastomas can present with embolic stroke in about 30% of patients. While larger, mobile fibroelastomas may be thought to carry a greater risk of cerebral embolism, observational data indicate no single echocardiographic feature to be sufficiently predictive of stroke. We present a case of a young female who presented with recurrent strokes in the setting of a small, untreated papillary fibroelastoma. Case presentation A 41‐year‐old Black female with past medical history significant for three ischemic strokes (2020, 2021) without residual deficits, presented in June 2024 to our institution with new left‐sided weakness and dysarthria over 24 hours. NIHSS was 7 for left facial droop, dysarthria, and left‐sided weakness. Initial imaging (CT head, CTA head/neck, CT perfusion) demonstrated chronic, known cortical infarcts without large‐vessel occlusion or perfusion deficit. MRI brain without contrast demonstrated multiple small, acute infarcts in the right perirolandic region and FLAIR changes consistent with prior infarcts in the left MCA, right occipital and right cerebellar regions. A transthoracic echocardiogram demonstrated no structural abnormalities, normal left ventricular ejection fraction, and normal left atrial volume index. However, a transesophageal echocardiogram demonstrated a rounded mobile echodensity measuring 0.48cm x 0.13cm attached to the commissure of left and right cusps on the aortic valve, consistent with a papillary fibroelastoma. Transthoracic echocardiogram in 2020 during her first stroke admission demonstrated a similarly sized, mobile echodensity (~0.3 times 0.2cm) of the aortic valve concerning for fibroelastoma. At the time, this fibroelastoma was thought to be too small to be causative of her ischemic stroke, therefore therapeutic anticoagulation or surgical intervention was deferred. She was discharged with aspirin 81mg daily, and was switched to clopidogrel 75mg daily after her second stroke in 2021. Again, surgery was deferred. Hypercoagulability workup (anticardiolipin, beta‐2 glycoprotein, protein C, protein S, homocysteine, Factor V Leiden, antithrombin 3) was normal. There was no evidence of atrial fibrillation during any of her four inpatient stroke admissions. Given that this was her fourth ischemic stroke without an alternative mechanism of stroke, aggressive management of papillary fibroelastoma was pursued. She was started on therapeutic anticoagulation with apixaban and evaluated by cardiothoracic surgery for surgical removal, which took place in August 2024. Discussion Cardiac fibroelastomas are an underrecognized cause of stroke and carry a high risk of morbidity and mortality if untreated, even when the size is small. This case illustrates the importance of early and aggressive intervention of papillary fibroelastomas when associated with otherwise cryptogenic embolic events. The gold‐standard treatment for management of embolic events in the setting of cardiac fibroelastomas is surgical excision, which can leave the valve intact for >95% of patients and carries a low risk of recurrent stroke. In this case, the patient was started on antiplatelet agents however recurrent strokes occurred while adherent to these therapies. In patients unable to undergo surgical excision, initiation of anticoagulation may be considered, but is of uncertain benefit.

Surgical Treatment of Benign Cardiac Tumors

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.

Papillary fibroelastoma is a rare benign cardiac tumor, second after cardiac myxoma as more frequent cardiac valvular tumor. The therapy is usually based on surgery according to size, mobility, symptoms, and risks of surgery. We report the case of a 67-year old female with transient ischemic attack. Echocardiography showed the presence of a round mobile formation localized on the atrial side of septal leaflets of tricuspid valve, first identified as an endocarditis vegetation. Cardiac magnetic resonance (cMRI) confirmed the presence of the mass and showed an isointense signal in T1-turbo spin echo sequences, hypointense in cine steady-state free precession and in first-pass sequences, and hyperintense in T2 and phase-sensitive inversion recovery with central hypointense core, with a suspected diagnosis of fibroelastoma. The patient underwent cardiac surgery and histology confirmed the presence of fibroelastic tissue with papillary extroflexions compatible with diagnosis of fibroelastoma. The use of cMRI may be useful in the evaluation of the exact position, dimensions, and nature of cardiac masses and fibroelastomas, diagnostic workup, and preliminary assessment before surgery.

The role of aortic valve Lambl's excrescence (LEs) in determining ischemic events has not been well clarified, but they can represent a potential embolic source during procedures with catheter/device manipulation through the aortic valve. Aim of our study was to assess the prevalence of LEs and the rate of embolism in patients with aortic valve stenosis scheduled for transcatheter aortic valve implantation (TAVI). Our population was divided into two groups, named LEs and no-LEs. In each group, the rate of cerebral embolic events was assessed, as well as other TAVI-related complications. In our study population 28 patients (37%) had aortic strands and 48 (63%) did not have them. A cerebral protection device was used in four patients of LEs group (14% vs. 0, P=0.03). The mean procedural time was similar in the two groups: 50±19 and 55±26 minutes (P=0.38) in LEs and no-LEs groups, respectively. The device success was achieved in 96% of LEs and 88% of no-LEs patients (P=0.37). Two patients of no-LEs group died during the procedure. Major complications were observed in both groups without any significant differences. Only one case of stroke occurred in the population without LEs (0 LEs vs. 2% no-LEs, 0.78). No cases of peripheral embolism were observed. In our population the observation of LEs is not uncommon. Despite the presumed high risk of embolism, we have not observed an increase in the rate of cerebral ischemic events or other TAVI related complications in patients with LEs.

In our population the observation of LEs is not uncommon. Despite the presumed high risk of embolism, we have not observed an increase in the rate of cerebral ischemic events or other TAVI related complications in patients with LEs.

Primary Malignant Cardiac Tumors: A Rare Disease With an Adventurous Journey

A rare nonvalvular left ventricular papillary fibroelastoma: A case report

A healthy 41-year-old female experienced sudden onset of right hand weakness, right facial droop and garbled speech. The symptoms lasted for several minutes and then resolved spontaneously. Neurologic exam and head CT were unremarkable. Brain magnetic resonance (MR) imaging demonstrated an infarct in the left frontoparietal operculum; head and neck MR angiogram was unremarkable. A trans-thoracic echocardiogram (TTE) was obtained to evaluate for a patent foramen ovale (PFO) as a cause of paradoxical embolus. Although there was no evidence of PFO, the TTE revealed a 7 × 8 mm pedunculated mass attached to the aortic valve. Trans-esophageal echocardiogram (TEE) confirmed the finding of a highly mobile, echo lucent mass most suggestive of a papillary fibroelastoma (PFE). However, valvular vegetation, thrombus and other primary intra-cardiac tumor could not be excluded. A cardiac MR scan was obtained to further characterize the mass. T2-weighted images demonstrated increased signal within the core of the mass, making tumor more likely than infectious vegetation. The mass also more than doubled in signal intensity after intravenous administration of gadolinium confirming the presence of a vascular source and making thrombus unlikely. The results of non-invasive imaging strongly suggested PFE. In the absence of other stroke source, surgical resection was recommended. Histology of the mass demonstrated a typical budding pattern of fibrous and elastic tissue, confirming the diagnosis of PFE. Papillary fibroelastomas are rare, accounting for less than 10% of primary cardiac tumors; however, they are the most common tumor associated with heart valves. Most PFE are asymptomatic and discovered incidentally. However, due to the risk of embolism, surgical resection is recommended for symptomatic or large left-sided tumors. This case illustrates the importance of a broad differential diagnosis when evaluating a common syndrome (ischemic stroke) in an uncommon circumstance (young patient). It also demonstrates the complimentary nature of echocardiography and cardiac MR in confirming the diagnosis of a cardiac mass prior to invasive therapy. As such, multi-modality imaging has become integral in the evaluation and management of cardiovascular disease.

Transaortic resection of an apical left ventricular fibroelastoma facilitated by a thoracoscope

Small Cardiac Hemangioma: A Challenge for Diagnosis and Dilemma for Management