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Asymptomatic papillary fibroelastoma of the aortic valve in a young woman - a case report.

ABSTRACT: Echocardiography represents an invaluable diagnostic tool for the detection of intracardiac masses while simultaneously provides information about their size, location, mobility and attachment site as well as the presence and extent of any consequent hemodynamic derangement. A 29-year-old asymptomatic young woman with incidental transthoracic echocardiographic (TTE) discovery of an aortic valve mass is presented. The 2-dimensional TTE showed a mobile, pedunculated mass, attached by a thin stalk to the aortic surface of the right coronary aortic cusp at the junction of its base with the anterior aortic wall. The importance of valve sparing tumour resection even in asymptomatic patients is emphasised.

SUBMITTER: Parthenakis F

PROVIDER: S-EPMC2749026 | biostudies-literature | 2009 Sep

REPOSITORIES: biostudies-literature

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Asymptomatic papillary fibroelastoma of the aortic valve in a young woman - a case report.

Parthenakis Fragiskos F Nyktari Evangelia E Patrianakos Alexandros A Pitsis Antonis A Asimaki Anthoula A Vardas Panos P

Cardiovascular ultrasound 20090902

Echocardiography represents an invaluable diagnostic tool for the detection of intracardiac masses while simultaneously provides information about their size, location, mobility and attachment site as well as the presence and extent of any consequent hemodynamic derangement. A 29-year-old asymptomatic young woman with incidental transthoracic echocardiographic (TTE) discovery of an aortic valve mass is presented. The 2-dimensional TTE showed a mobile, pedunculated mass, attached by a thin stalk ...[more]

PMID: 19725951

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Native aortic valve thrombosis resembling papillary fibroelastoma.

Project description:The differential diagnosis of cardiac mass is important in determining the therapeutic plan and avoiding unnecessary surgical intervention. Non-invasive imaging methods would be useful in the diagnosis of suspected cardiac mass, because they may provide earlier diagnosis and more accurate assessment of cardiac mass. Native aortic valve thrombosis is a rare disorder and difficult to differentiate from a tumor, and in particular, a papillary fibroelastoma. Thus, the clinical decision making with imaging modalities should be performed cautiously. We recently met a female patient who had a aortic valve mass resembling papillary fibroelastoma in normal native valve. The patient underwent a surgical resection and the pathologic finding showed an organized thrombus with no evidence of papillary fibroelastoma.

| S-EPMC4192414 | biostudies-literature

An atypical cause of myocardial infarction: case report of an obstructing papillary fibroelastoma of the aortic valve.

Project description:Papillary fibroelastomas are rare primary cardiac tumours with a prevalence of 0.01% at autopsy. They are histologically benign tumours but have been demonstrated through case series to confer an increased risk of thrombo-embolism resulting in: transient ischaemic attack, stroke, myocardial infarction, and pulmonary and systemic embolization. A 54-year-old woman presented with central chest pain radiating to her left arm. At presentation there was a significant troponin rise; initial high-sensitivity troponin-I (hsTn-I) 660 pg/mL increased to 3340 pg/mL at 6 h. Coronary angiogram did not reveal any obstructing coronary artery disease. Echocardiography revealed a rounded, mobile mass on the left coronary cusp of the aortic valve suspicious for papillary fibroelastoma. The patient underwent shave excision of the lesion. Intra-operatively it was noted that the mass intermittently sat within the ostium of the left main resulting in its occlusion. Histology confirmed a papillary fibroelastoma. Primary cardiac tumours are rare but can cause life-threatening complications such as stroke, myocardial infarction, and cardiac arrest. In the literature, the mechanism of these complications is mainly attributed to thrombo-embolism. This case demonstrates the utility of echocardiogram in investigating and diagnosing a rare cause of myocardial infarction and highlights an unusual mechanism, that is tumour causing obstruction of the coronary ostium.

| S-EPMC6601164 | biostudies-literature

Aortic valve papillary fibroelastoma: a rare cause of angina.

Project description: Not available

| S-EPMC3147204 | biostudies-literature

Multimodality imaging of a giant aortic valve papillary fibroelastoma.

Project description:Papillary fibroelastomas (PFEs) are benign cardiac tumors arising from endocardium. They are commonly found on valvular surfaces and average 1.0-1.5 cm in size. Though often asymptomatic, PFEs can lead to potentially severe complications, primarily due to their embolic potential. Surgical resection is recommended for all symptomatic or large PFEs. We report the case of a patient presenting with cardiovascular symptoms who was found to have a very large aortic valve PFE, as diagnosed by histopathologic examination following surgical resection. Multimodality cardiovascular imaging demonstrates the classic morphologic findings, including a pedunculated appearance and oscillating "frond-like" surface projections.

| S-EPMC3745854 | biostudies-literature

Role of imaging for diagnosis and management of aortic valve papillary fibroelastoma and cardiac amyloid light chain amyloidosis: a case report.

Project description:BackgroundWe report the case of a patient who presented with concomitant aortic valve papillary fibroelastoma (PFE) and cardiac amyloidosis. Although histologically benign, PFE confers an increased thromboembolic risk, and surgical excision is often indicated. However, outcomes of cardiac surgery are poor in patients with cardiac amyloidosis.Case summaryA 61-year-old man with complaints of dyspnoea and weight loss of 10 kg developing over the past 5 months was evaluated in the cardiology clinic. Echocardiography revealed sessile aortic valve PFE and was also highly suggestive of cardiac amyloidosis. The diagnosis of amyloid light chain amyloidosis secondary to indolent multiple myeloma was eventually confirmed. Therapy with daratumumab, bortezomib, cyclophosphamide, and dexamethasone allowed full remission over a 6-month period and resulted in marked improvement in symptoms and cardiac function as evaluated by global longitudinal strain. Further workup with cerebral magnetic resonance revealed multiple vascular sequelae. Surgical removal of the aortic fibroelastoma with bioprosthetic aortic valve replacement was performed successfully and the patient had an uneventful recovery.DiscussionPapillary fibroelastoma and cardiac amyloidosis are rare and most likely unrelated entities. Concomitant presentation of both conditions in the same patient presents a unique therapeutic challenge. By allowing cardiac function to be monitored during chemotherapy, speckle-tracking echocardiography can prove instrumental in determining the optimal timing of surgical intervention.

| S-EPMC8422351 | biostudies-literature

A papillary fibroelastoma with myxoma camouflage: a case report.

Project description:BackgroundBenign cardiac tumours are infrequent in clinical practice and, of these, cardiac myxoma is the one with the highest incidence. Given that a left intraventricular presentation is rare, other differential diagnoses such as papillary fibroelastoma should be considered.Case summaryA 73-year-old woman patient with cardiac mass detected in transthoracic echocardiography (TTE) after a transient ischaemic attack. At TTE 2D-3D, a left intraventricular mass anchored at the level of the anterolateral papillary muscle was detected. Subsequently, cardiac magnetic resonance (CMR) was performed for mass characterization. This revealed behaviour in T1 (isointense with respect to myocardium), T2 (hyperintense), very prolonged T1-mapping (1848 msg), and T2-mapping (161 msg) values, without gadolinium uptake in the first-pass perfusion sequence, but with intense uptake in late enhancement sequences. Previous findings were compatible with a diagnosis of papillary fibroelastoma. The mass was resected intraoperatively and, although its macroscopic appearance pointed to a diagnosis of cardiac myxoma, it was finally confirmed to be a papillary fibroelastoma by pathological anatomy.DiscussionIn cases where the size of the mass and its mobility allow tissue characterization by CMR, a diagnosis of papillary fibroelastoma and its differentiation with cardiac myxoma are feasible by this cardiac imaging technique.

| S-EPMC9351726 | biostudies-literature

Case report: Echocardiographic diagnosis of double orifice mitral valve in an asymptomatic woman.

Project description:Double orifice mitral valve (DOMV) is a rare congenital anomaly that is often associated with cardiac malformation. Valve dysfunction usually presents in childhood; therefore, most cases are diagnosed with DOMV in childhood. Its prevalence and prognostic relevance in adulthood are unknown. Here, we report a case of a 38-year-old woman who presented to the outpatient clinic with an abnormal electrocardiogram and was found to have an isolated double orifice mitral valve malformation on transthoracic echocardiography. Echocardiography is the diagnostic tool of choice for patients with double orifice mitral valves. We should familiarize ourselves with the echocardiographic features of DOMV in order to improve the detection.

| S-EPMC9875288 | biostudies-literature

Acute obstruction of a mechanical aortic valve in a young woman: case report and review of the literature.

Project description:Acute aortic valve obstruction is a medical and surgical emergency necessitating intensive care unit admission. The differential diagnosis includes thrombosis, pannus formation or vegetations. The diagnosis should be obtained as soon as possible, with possible orientation towards the cause. Different diagnostic modalities exist nowadays. Notably, the transesophageal echocardiography (TEE) offers a diagnosis and a guide for management. Surgical treatment remains of choice despite growing evidence about a benefit of combined thrombolytic and anticoagulation line of management.Acute management and resuscitation of acute valvular obstruction.Differential diagnosis and role of echo in the diagnosis.Management options.

| S-EPMC4676487 | biostudies-other

Papillary fibroelastoma causing severe aortic stenosis.

Project description:Herein, we present a rare case of papillary fibroelastoma of the aortic valve, which caused severe aortic stenosis. The papillary fibroelastoma developed on the left ventricular side of the non-coronary cusp, immobilizing the cusp motion and obstructing the left ventricular outflow tract, resulting in severe aortic stenosis that brought on acute heart failure. The patient underwent an urgent surgical treatment, which resulted in a successful outcome. To the best of our knowledge, this is the first case of papillary fibroelastoma in which aortic stenosis was so severe as to cause congestive heart failure.Learning objectivePapillary fibroelastoma (PFE) is the most commonly observed primary cardiac tumor in adults that commonly involves left heart chambers. While PFEs often cause embolisms, they rarely cause valvular dysfunction. In a case presented herein, a PFE developed on the left ventricular side of the non-coronary cusp, immobilizing the cusp motion and obstructing the left ventricular outflow tract, resulting in severe aortic stenosis that brought on acute heart failure.

| S-EPMC9995653 | biostudies-literature

A rare nonvalvular left ventricular papillary fibroelastoma: A case report.

Project description:Papillary fibroelastomas are benign cardiac tumors with high embolic potential typically found on the valvular surfaces of the heart. Nonvalvular papillary fibroelastomas are exceedingly rare. We report the case of a 66-year-old Caucasian male with acute bilateral basal ganglia infarctions found to have a mass adherent to the left ventricular septum by transesophageal echocardiography. The mass was identified as a rare nonvalvular cardiac papillary fibroelastoma based on echogenicity, pedunculated nature, and typical motion. Tissue characterization by cardiac magnetic resonance imaging demonstrated homogeneously hypo-intense signal on T2 weighted imaging and signal hyperintensity after administration of gadolinium contrast, confirming the fibroelastic nature of the mass. Surgical excision was performed via ventriculotomy and histopathologic examination was pathognomonic of a papillary fibroelastoma. We conclude that transesophageal echocardiography provides high diagnostic certainty in patients with cardiac papillary fibroelastomas and can reliably identify atypical locations of these tumors on nonvalvular surfaces. A multimodality imaging approach is not necessarily indicated in all patients with this condition.Learning objectivePapillary fibroelastomas are benign cardiac tumors with high embolic potential typically found on the valvular surfaces of the heart. Nonvalvular papillary fibroelastomas are exceedingly rare. Transesophageal echocardiography readily identifies nonvalvular papillary fibroelastomas based on echogenicity, pedunculated nature, and characteristic motion, and reliably differentiates them from other cardiac masses. A multimodality imaging approach is not indicated in all patients with this condition.

| S-EPMC3981096 | biostudies-literature

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