A rare case of papillary fibroelastoma involving the tricuspid valve. A single center experience over a period of 22 years (1999-2021).

BACKGROUND AND AIM: Papillary fibroelastoma (PFE) represents only 16% of the benign cardiac tumor and approximately 15% of these are located on the 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 but only one case of a tricuspid valve PFE in a 69-year-old patient. Trans-thoracic echocardiography demonstrated a mobile mass (20 × 10 mm), adhering to the atrial side of the septal leaflet of the tricuspid valve of unknown origin. In consideration of the mobility of the mass and the consequent high embolic risk, surgical removal was made. The 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 diagnosis was PFE. CONCLUSIONS: PFEs of the tricuspid valve are rare entities being in most cases found incidentally. In our experience, the incidence of this tumor in this location is 1/10,000 cases of cardiac surgery. Although most patients are asymptomatic, surgical treatment is nevertheless recommended in consideration of the high embolic risk.

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

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

A rare case of acute myocardial infarction related to mobile papillary fibroelastoma of the aortic valve.

We present the case of a 75-year-old woman with typical myocardial infarction but a negative coronary angiogram. Echocardiography identified the rare cause of her chest pain: a mobile mass on the aortic valve that obstructed the coronary ostium. The histopathology revealed a papillary fibroelastoma (PFE), and her chest pain was relieved after surgical resection of the mass.

[Pathology of primary cardiac tumors]. / Pathologie des tumeurs primitives du cœur.

Primary heart neoplasms are rare and poorly known by general pathologists. However, they are not exceptionally encountered in pathology laboratories having a recruitment of cardiac surgery specimens. About 80 % of them are benign tumors and 20 % are malignant tumors. Some tumors are specific to the heart or have characteristics when they grow in the heart, including myxoma and papillary fibroelastoma. The classification currently in use is the 4th edition of the WHO classification of heart tumors published in 2015, which takes into account the evolution of knowledge in the field of sarcomas, but which does not yet recognize cardiac intimal sarcoma. recently individualized in its intracardiac location.

Unusual place unusual number.

Cardiac papillary fibroelastoma is a rare, benign cardiac tumor. It often arises from the valvular endocardium and is usually solitary. Nonvalvular location is rare and even more rare is the presentation as multiple masses. A 71-year-old female patient was referred for echocardiographic evaluation due to progressive fatigue. The presence of multiple left atrial masses was observed on echocardiographic evaluation. The patient was treated surgically for the prevention of embolic complications, and the histologic diagnosis of multiple nonvalvular papillary fibroelastoma was made. This case highlights the need to consider this unusual location and presentation for this type of tumor.

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.

Incidental finding of an asymptomatic pulmonary valve papillary fibroelastoma: A case report.

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.

Histology of Thrombectomy Specimen Reveals Cardiac Tumor Embolus in Cryptogenic Young Stroke.

A 25-year-old woman presented with acute onset of right hemiplegia and global aphasia with National Institutes of Health Stroke Scale score of 19. Computed tomography angiography demonstrated occlusion of the left proximal middle cerebral artery. She was thrombolysed with intravenous recombinant tissue plasminogen activator (0.6 mg/kg) within 3 hours of onset of symptoms and was taken up for mechanical thrombectomy. The retrieved specimen appeared pale white and soft; histopathologic examination revealed a papillary neoplasm composed of papillae with hyalinized cores lined by endothelium, consistent with papillary fibroelastoma of cardiac origin. Transesophageal echocardiography showed no abnormalities, which can be explained by complete embolization of the mass into the cerebral circulation. On follow-up after 5 months, the patient recovered with only minimal aphasia and no cardiac symptoms. Our experience with this case highlights the importance of histopathologic examination of the mechanical thrombectomy specimen, as it provided the only clue to the etiology of stroke.

Incidental tricuspid valve fibroelastoma associated with patent foramen ovale in a young female: Straightforward diagnosis but controversial management.

Papillary fibroelastoma is an uncommon primary cardiac tumor, predominantly associated with left-sided valvular endocardium. Affectation of tricuspid valve leaflets is rare, and management in asymptomatic patients remains controversial. We present a 30-year-old female referred for evaluation prior to bariatric surgery. A routine echocardiogram revealed a mobile cardiac mass attached to the tricuspid valve. A patent foramen ovale was also present. Tumor was surgically removed. Histology confirmed the definite diagnosis of a papillary fibroelastoma. In our case, the presence of a patent foramen ovale associated with the right-sided fibroelastoma was decisive in the decision for surgery.

Aortic valve replacement for papillary fibroelastoma.

Surgery is indicated for symptomatic patients with papillary fibroelastomas (PFE) on the aortic valve. The valve is commonly spared during tumor excision. Rarely, aortic valve replacement (AVR) is needed. We present a case requiring AVR for an aortic valve PFE and review the literature to determine the risk factors for failure of aortic valve-sparing techniques in patients with PFE.

[Papillary fibroelastoma: retrospective analysis. Clinical presentation and surgical results]. / Fibroelastoma papilar cardíaco: estudio retrospectivo. Presentación clínica y resultados quirúrgicos.

Papillary ibroelastomas are small benign intracardiac tumors known for their embolic potential. Since the introduction of echocardiography with improved resolution and transesophageal imaging techniques, they are being increasingly detected in clinical practice. In recent series, papillary fibroelastoma is considered the most frequent benign tumor of the heart. Our objective was to analyze characteristics and midterm surgical outcome of histologically-confirmed cases of papillary fibroelastoma. We conducted a retrospective study on patients with cardiac tumors submitted to surgical excision between June 1992 and February 2017. Out of 108 patients, 18 had papillary fibroelastomas. Their mean age was 58 years (22-77); 10 were men. The most frequent localizations were the aortic valve (7) and the mitral valve (5). None had significant valvular dysfunction. By transesophageal echocardiography, the tumor size (larger diameter) was 13.33 ± 5.55 mm (6.6-28.0). Two patients, both with tumor in the aortic valve, had suffered a stroke; other two had dyspnoea and atrial flutter, respectively. The remaining 14 patients were asymptomatic and their tumors were incidental findings. In 15 patients the valve was preserved. There was neither surgical mortality nor recurrence after 2.6 years of follow-up. In conclusion, most papillary fibroelastomas can be surgically removed with valve preservation and favorable clinical outcome. However, until the results of randomized trials support the decision, an aggressive surgical approach in asymptomatic patients needs to be defined in the context of surgical expertise.

Non-exophytic Lambl excrescences of aortic valves: a morphological study.

AIMS: To elucidate the histopathological findings of classical Lambl excrescences (LEs) and non-exophytic LEs (non-ex LEs) without excrescent papillary features. METHODS AND RESULTS: We examined 126 aortic valves (AVs) and revealed LEs (non-ex and/or classical), non-ex LEs and classical LEs in 106, 78 and 88 AVs, respectively. The detection of non-ex LEs was challenging, but elastica van Gieson stain highlighted their presence. Non-ex and classical LEs chiefly involved the ventricular regions, favoured posterior cusps and coexisted in the same areas of 31 AVs. A possible transformation of classical LEs into non-ex LEs was suggested histologically in 39 AVs. Non-ex LEs were associated with age of >70 years (P < 0.001) and marked deformity (P = 0.007). Classical LEs were associated inversely with marked deformity (P < 0.001), but not with age of >70 years. Compared with age- and sex-matched control AVs, non-ex LEs and marked deformity in dysfunctional AVs were more common (P = 0.037 and P < 0.001, respectively), but classical LEs were less common (P = 0.021). CONCLUSIONS: Non-ex LEs have subtle features but are a common form of LEs, and seem to develop from classical LEs. AV dysfunction-related marked deformity can promote non-ex LEs.

Resting angina due to papillary fibroelastoma of the right coronary cusp.

A 63-year-old man with chest pain at rest was referred to our hospital. Transthoracic echocardiography showed a mobile ball-like mass at the top of the right coronary cusp. Subsequently, transesophageal echocardiography also showed a mobile mass at the right coronary cusp. Aortic valve replacement with a mechanical valve was performed under general anesthesia. We diagnosed this condition as papillary fibroelastoma based upon the pathological findings with hematoxylin and eosin staining, and Elastica van Gieson staining. Coronary angiography revealed no organic lesions. The operation was successful, and the patient remains asymptomatic. We speculate that the resting chest pain was induced by transient occlusion of the right coronary orifice by the tumor. We describe this rare case in detail including a review of the literature.

Papillary fibroelastoma: clinical and echocardiographic features and initial approach in 54 cases.

INTRODUCTION: Papillary fibroelastoma (PFE) is a benign cardiac tumor that is currently detected more often due to the technological improvements in echocardiography. OBJECTIVES: To describe the echocardiographic features of PFE and correlate them with the clinical presentation and initial treatment. MATERIALS AND METHODS: A prospective analysis of patients with a diagnosis of PFE was conducted between 2000 and 2015. We assessed the clinical history, symptoms at the time of diagnosis, echocardiographic features, and initial treatment. RESULTS: Fifty-four patients with a diagnosis of PFE by echocardiography were included. The incidence was 0.038%. Mean age was 62±16 years; 50% were male. Forty-six percent of patients had symptoms at the time of diagnosis, the most frequent of which was transient ischemic accident (TIA). Embolic episodes occurred in 31% of patients, mainly to the brain. PFEs were valvular in 70.4% of cases and multiple in 13%. Mean maximum length was 1.18±0.58 cm, and 44% were mobile. PFEs >1.5 cm were most often found in the heart valves (56.8% vs 10.8%; P=.045). There was no significant relation between size, mobility, location and number of tumors, and the presence of embolism or symptoms. Most frequent treatment was oral anticoagulation in 48.6%, followed by simple tumor resection in 42.8% of cases. CONCLUSIONS: PFE is a small tumor, predominantly valvular and benign, but entails a high incidence of cerebral embolism. The initial approach should be individualized according to clinical manifestations, comorbidities, and the experience of the surgical center.

Papillary fibroelastoma of the pulmonary valve--a systematic review.

The pulmonary valve is the least affected site for valvular papillary fibroelastoma. With increasing use of routine echocardiography and other modalities of imaging, pulmonary valve papillary fibroelastomas (PVPFE) are being recognized more frequently. PVPFE is more often an incidental diagnosis and symptomatic patients usually present with shortness of breath. Embolic phenomena and right ventricular outflow tract obstruction are the most serious complications of PVPFE. Since PVPFE is rare, the purpose of this systematic review is to address demographic characteristics, the clinical presentation, management, and outcome of this benign tumor of the pulmonary valve.

Stroke and cardiac papillary fibroelastoma: mechanical thrombectomy after thrombolytic therapy.

We describe a case of a 34-year-old man with a sudden development of right hemiparesis and aphasia because of infarction of the left middle cerebral artery that was submitted to intravenous (IV) recombinant tissue plasminogen activator and mechanical thrombectomy. Transesophageal echocardiogram showed a small mass on the anterior leaflet of the mitral valve. Cardiac surgery was performed, and histological examination of the removed material was consistent with cardiac papillary fibroelastoma (CPF). Experience in using IV thrombolysis for the treatment of embolic stroke because of CPF is limited. To the best of our knowledge, only 3 patients are reported in literature in whom acute ischemic stroke and associated CPF were treated with thrombolytic therapy. A discussion of the efficacy of IV thrombolysis and the possible superiority of mechanical thrombectomy is included.

Papillary fibroelastoma complicated by Streptococcus sanguinis bacteremia: a rare case of cardiac tumor with embolic events.

Fibroelastoma is a rare cardiac tumor that can cause embolization, stroke, myocardial infarction, heart failure, and cardiac arrest. Here, we report the case of a 45-year-old male who presented with right-sided weakness and fever. He was diagnosed with acute right frontal infarction and was found to have Streptococcus sanguinis bacteremia. Upon confirmation of a positive blood culture after 24 hours, treatment for endocarditis was initiated. Transesophageal echocardiography revealed findings highly suggestive of a papillary fibroelastoma (PFE). PFE ought to be regarded as a potential differential diagnosis in individuals who exhibit symptoms of fever, thromboembolism, and persistent bacteremia. Non-invasive imaging such as echocardiography is of great value in the diagnosis of PFE, while surgical resection remains the best treatment modality to overcome current and future associated complications.

Characteristics of Lambl excrescences of aortic and pulmonary valves in healthy hearts.

Lambl excrescences (LEs) were initially described in the mid-1800s during autopsies of human hearts, and their significance and biology have been debated ever since. LEs are typically found on aortic and pulmonary valve (semilunar) cusps. There is debate concerning whether LEs are a significant cause of thromboembolic events, or whether they are harmless growths. However, there have not been many reports discussing LEs, and fewer still have examined the prevalence and characteristics of LEs in healthy human hearts. Those who have examined LE prevalence have reported a very high incidence of LEs (85-90%). Herein, we examine LE prevalence and characteristics (size, location, number) in 403 healthy human hearts across all age groups. We find that the prevalence of LEs in healthy hearts is far lower than previously reported.