Papillary Fibroelastoma Research Articles

Lambl's Excrescences: Association with Cerebrovascular Disease and Pathogenesis

Background: Lambl's excrescences (LEx) are detected by transesophageal echocardiography (TEE) and are characterized as thin, elongated, and hypermobile structures located at the leaflets' coaptation point of the heart valves. The association of LEx with cerebrovascular disease (CVD) is still undefined and yet patients with LEx and suspected CVD receive unproven effective antiplatelet or anticoagulant therapy or even undergo valve surgery. Also, the association of LEx with aging and atherogenic, inflammatory, or thrombogenic parameters has not been reported. Methods: Seventy-seven patients with systemic lupus erythematosus (SLE) (71 women, age 37 ± 12 years) and 26 age- and sex-matched healthy controls (22 women, age 34 ± 11 years) prospectively underwent routine history and physical exam, transcranial Doppler, brain MRI, TEE, carotid duplex, and clinical and laboratory evaluations of atherogenesis, inflammation, platelet activity, coagulation, and fibrinolysis. Subjects without stroke/TIA on enrollment (with and without LEx) had a median follow-up of 57 months. Results: On enrollment, 33 (43%) of 77 patients had CVD manifested as acute stroke/TIA (23 patients), cerebromicroembolism by transcranial Doppler (17 patients), or cerebral infarcts by MRI (14 patients). Mitral or aortic valve LEx were equally frequent in healthy controls (46%) as in patients with and without any CVD (39 and 43%), stroke/TIA (35 and 43%), cerebromicroembolism (41 and 42%), or cerebral infarcts (36 and 43%) (all p ≥ 0.72). Also, other mechanisms for CVD other than LEx such as Libman-Sacks vegetations, patent foramen ovale or interatrial septal aneurysm, aortic or carotid atherosclerosis, or thrombogenesis were found in ≥94% of patients with CVD. In addition, 36 subjects with and 44 without LEx had similar low incidence of stroke/TIA (1 (1.3%) and 2 (2.5%), respectively, p = 1.0) during follow-up. Finally, LEx were not associated with aging, atherogenic risk factors, atherosclerosis, inflammation, or thrombogenesis. Conclusions: In this study, LEx are similarly prevalent in healthy controls and SLE patients, are not associated with CVD, and are not associated with pathogenic risk factors. Therefore, the study findings suggest that LEx may not be cardioembolic substrates, may not represent pathologic valve structures, and may not require therapy.

Papillary Fibroelastoma of the Aortic Valve: Analysis of 21 Cases, Including a Presentation with Cardiac Arrest

Cardiac papillary fibroelastoma is a rare, benign tumor, arising predominantly from cardiac valves. This tumor can cause a variety of symptoms due to thromboembolism. We describe our single-center surgical experience with papillary fibroelastoma of the aortic valve. From April 2004 through June 2013, 6,530 patients underwent cardiac surgery. Of those, 6,098 patients were included in the final analysis. Twenty-one patients (0.34%) underwent surgical resection of 30 papillary fibroelastomas of the aortic valve. Most patients (67%) were incidentally diagnosed to have cardiac papillary fibroelastoma. The usual symptom was cerebral infarction (in 5 of 7 symptomatic patients). A rare presentation of papillary fibroelastoma in one patient was cardiac arrest caused by left main coronary artery ostial obstruction. Tumor size was not related to patient age (Pearson correlation coefficient, 0.34; P=0.13). Neither the number of tumors (1.43 ± 0.72 vs 1.43 ± 0.62) nor tumor size (8.14 ± 2.42 vs 8.07 ± 3.31 mm) was significantly different between symptomatic and asymptomatic patients. All lesions were resected by means of the simple shave technique. There were no operative or 30-day deaths. Follow-up echocardiograms showed no tumor recurrence (mean follow-up duration, 17 ± 14 mo). We identified no significant relationship among tumor size, number of tumors, symptoms, or patient age. Because simple shave excision of the tumor can be safely achieved without evidence of tumor recurrence, we conclude that surgical resection can be reasonable in asymptomatic patients.

Pulmonary valve papillary fibroelastoma: a rare tumor and rare location.

Although rare, papillary fibroelastomas (PFEs) are considered the third most common cause of primary cardiac neoplasm. They are usually asymptomatic and are found during routine echocardiography. PFEs of the pulmonary valve are extremely rare. They are usually benign; however, because of their potential to embolize to the pulmonary circulation, it is often recommended that they be removed. This article reviews a case of an asymptomatic PFE of the pulmonary valve, incidentally found in a patient who presented with three-vessel coronary artery disease.

Non-valvular left ventricular papillary fibroelastoma in a patient with recurrent stroke.

A 69-year-old gentleman presented with sudden-onset dizziness and diplopia. Clinical examination findings were suggestive of posterior circulation stroke. He was found to be in atrial flutter on admission, reverting spontaneously to sinus rhythm. Three weeks before admission, he was admitted to a different hospital with anterior circulation stroke, which left him with minor residual weakness. He was found to be in sinus rhythm during that admission and was discharged home on dual-anti platelet therapy. Transthoracic echocardiogram (TTE) performed to exclude a cardiac source of emboli showed a highly mobile echogenic structure in the left ventricular outflow tract (LVOT) attached to the interventricular septum (Fig. 1A and Video 1). Subsequent transoesophageal echocardiogram (TOE) confirmed a highly mobile mass in the LVOT, which appears to be prolapsing through the aortic valve (Fig. 1B, C and Videos 2, 3). The left atrium and appendage are free of spontaneous contrast or thrombus and there are no atherosclerotic plaques noted in the aorta. Given the history of recurrent stroke, the highly mobile nature of the mass and its position in the LVOT, the patient was referred for urgent surgery. The patient underwent complete surgical excision of the mass (Fig. 1D), which was then confirmed to be benign papillary fibroelastoma (PFE) on histopathology. PFE is the second most common cardiac tumour which usually originate from the valvular endothelium. PFE arising from this non-valvular location is exceptionally rare. When present in such a location, they are often mistaken for myxoma from TTE and usually warrants further imaging such as TOE or cardiac MRI to confirm diagnosis. Surgery is curative in most cases and prompt referral should be considered (1). Open in a separate window Figure 1 (A) Transthoracic echocardiogram (PLAX view) showing the mass in the LVOT attached to the inter-ventricular septum. (B) Transoesophageal echocardiogram confirming the mass measuring 1.1×1.1 cm in the LVOT attached to the inter-ventricular septum. (C) Transoesophageal echocardiogram showing the mass prolapsing through the aortic valve. (D) Intraoperative picture showing the jelly-like mass attached to the inter-ventricular septum.

Lambl's Excrescence, Migrainous Headaches, and “Tiger Stripes”: Puzzling Findings in One Patient

Lambl's excrescences are mobile, thin, filiform structures that occur at sites of valve closure. Even though many clinicians consider them to be part of the normal aging process, evidence suggests an association between Lambl's excrescences and ischemic stroke, migrainous headaches, and acute coronary syndromes. As a thin filamentous structure, a Lambl's excrescence is better detected and characterized with transesophageal than with transthoracic echocardiography. Intracardiac oscillatory structures can also be seen as "tiger stripes" on spectral pulsed Doppler echocardiographic recordings. Herein, we present the case of a 68-year-old woman who had 3 concurrent enigmatic findings with unclear correlation: migrainous headaches, Lambl's excrescences, and the uncommon finding of "tiger stripes" on spectral Doppler echocardiography. We discuss the possible correlation between these 3 findings and review the available literature on Lambl's excrescences and tiger stripes.

Papillary fibroelastoma located on tricuspid valve

Papillary fibroelastoma located on tricuspid valve

Symptomatic Papillary Fibroelastoma- Case Report and Literature Review

Primary cardiac tumors represent a small subset of cardiac neoplasms. The papillary fibroelastoma (PFE) is a benign tumor that typically affects the cardiac valvular system and is second in prevalence only to myxomas. Though typically asymptomatic and diagnosed incidentally, few patients with PFE may also experience neurologic and cardiac symptoms that prompt further diagnostic investigation. We present a case of a patient with transient monocular vision loss with a later occurrence of bilateral lower extremity weakness and confusion over a short period of time. The alarming symptomatology eventually led to diagnosis by transesophageal echocardiogram of a PFE involving the aortic valve. We include a review of the current literature on PFE to further Review Article Joshi et al.; BJMMR, 8(9): 741-747, 2015; Article no.BJMMR.2015.501 742 elucidate etiology of this neoplasm, symptomology, prognosis, and treatment. Currently, the role of anticoagulation in PFE remains unclear. Due to the embolic nature of this neoplasm as evidenced by the case presentation, surgical resection, anticoagulation or a combination of both provides patients with maximal prevention of stroke.

Valvular Excrescences: A Possible Transient Phenomenon.

The thrombogenic potential of Lambl excrescences (LE) is minimal unlike the benign tumour fibroelastoma wherefrom thrombi often originate. We present a patient with multiple strokes within a six-year period. A possible locus on the aortic valve was found and diagnosed as fibroelastoma. Before aortic valve substitution prior finding could not be visualized and surgery was aborted. Due to review of earlier Transesophageal Echocardiography (TEE) and the transient component, LE was accepted as the most plausible diagnosis. This illustrates the need for TEE just before surgery.

Multiple papillary fibroelastomas in the right heart

Background: Cardiac multiple papillary fibroelastonas are rare. Methods and Results: A 70-year-old woman presented with an exertional dyspnea. A trans-thoracic echocardiogram showed a moving mass near the inferior vena cava in the right atrium. She underwent an operation for a cardiac tumor. A trans-esophageal echocardiogram during the operation showed a mass in the right ventricle, which had echo properties similar to the mass in the right atrium. The mass in the atrium was resected including the attached intima, and the mass in the ventricle was resected by the shave method. Her postoperative course was uneventful. A pathological examination showed that the two tumors were papillary fibroelastomas. Conclusion: A precise examination should be performed when a cardiac tumor is diagnosed to investigate the possibility of multiple lesions.

Coronry artery fistula with papillary fibroelastomas

Coronry artery fistula with papillary fibroelastomas

三尖弁腱索より発生した乳頭状弾性線維腫の1例

乳頭状弾性線維腫は心臓良性腫瘍のなかでは粘液腫についで多く，左心系に多いとされている．今回われわれは，三尖弁腱索より発生した乳頭状弾性線維腫の1例を経験したので報告する．症例は65歳，女性．検診にて心雑音を指摘され，精査したところ心臓腫瘍を認め当科紹介となった．心臓超音波検査と造影CT検査にて，右室内に約1 cmの可動性腫瘍を認め，手術適応と診断された．人工心肺を確立後，心停止下に右房を切開し，経三尖弁的に右室を観察した．前尖腱索より約9 mmの黄色イソギンチャク状の腫瘍を認めたため，腱索を含めて腫瘍を切除し三尖弁形成術を施行した．術後経過は良好で，第19病日に退院となった．病理組織学的所見では，密な線維組織と脂肪組織が混在し，乳頭状弾性線維腫と診断された．乳頭状弾性線維腫（Papillary Fibroelastoma : PFE）は原発性心臓腫瘍の約8%に認められる比較的稀な疾患である．PFEの多くは左心系に発生し，脳梗塞や心筋梗塞などの塞栓症状を引き起こすとされている．一方，小さな腫瘍や右心系に発生した場合などは，臨床的に無症状であり，検診の心臓超音波検査などで偶然発見される症例も多い．今回われわれは心雑音の精査を契機に診断された，三尖弁腱索より発生するPFEを経験したので，若干の文献的考察を加え報告する．

A Case of Non-pedunculated Papillary Fibroelastoma in the Right Ventricular Outflow Tract Stenosis Complicated with Ventricular Septum Defect

A Case of Non-pedunculated Papillary Fibroelastoma in the Right Ventricular Outflow Tract Stenosis Complicated with Ventricular Septum Defect

Pulmonary valve papillary fibroelastoma: A case report and review of the literature of a rare cardiac tumour

Pulmonary valve papillary fibroelastoma: A case report and review of the literature of a rare cardiac tumour

Des conséquences insolites d’une sclérothérapie à la mousse

Neurological disorders are rare complications of foam sclerotherapy. Visual disturbances and headache are the most commonly reported events and are thought to be equivalent to migraine with aura. Exceptionally, strokes have been reported. Papillary fibroelastoma is a rare cardiac tumor, which may embolize in cerebral arteries. We report the case of a patient in whom neurological disorders occurred during a session of foam sclerotherapy, and led to the discovery of a cardiac fibroelastoma.

Diagnosis and surgical treatment of heart valve non-mixoma tumors

Aim. To study the clinical, diagnostic, surgical and morphological features of non-mixoma primary tumors of the heart valves, the possibilities of their early diagnosis and to develop an optimal surgical approach. Methods. The surgical treatment of 331 patients with tumors of the heart was analysed, of which 45 (13.6%) were radically distant primary tumors of the heart valves including 33 (73.3%) myxoma and 12 (26.7%) non-myxoma tumors. Among considered in this study 12 non-myxoma tumors 9 cases were papillary fibroelastoma, 1 - neurolemmoma, 1 - lipoma, 1 - fibroma. Results. The leading non-invasive preoperative diagnostic procedures for non-myxoma primary tumors of the heart valves were transthoracic and transoesophageal echocardiographic studies, the sensitivity of which were 91.7 and 100% respectively. In unclear cases, a computer and magnetic resonance imaging were used. Additionally, electrocardiography, phonocardiography, radiological methods were performed, clinical and biochemical blood tests were analysed. Intraoperative revision and morphological verification ensures the establishment of tumors final clinical diagnosis. Patients with non-myxoma tumors of the heart valves had polymorphic symptomatic picture which included circulatory failure, false angina, arrhythmias, clinical signs imitating heart defects, embolic syndrome, dizziness and/or syncope, causeless fever. Surgical removal of the 12 non-myxoma tumor was performed by cardiopulmonary bypass and pharmacological cold cardioplegia, in 4 cases, the intervention combined with the replacement of the affected valves by artificial prostheses, in 1 case - coronary artery bypass grafting. Hospital mortality of patients was zero. Quality of life of operated patients was rated as good in 9 (75%) patients, satisfactory - in 2 (16.7%), unsatisfactory - in 1 (8.3%) patients, which was due to the death of the patient 13 months after removal of neurolemmoma in connection with multiple metastasis in the brain and spinal cord. Conclusion. Timely surgical intervention helped to stabilize the functional state of the patients, to create a favorable prognosis to improve the quality of life and increase its length.

Incidental finding of cardiac papillary fibroelastoma of the left ventricular outflow tract, associated with rheumatic mitral and tricuspid stenosis

Cardiac papillary fibroelastomas (CPFs) are one of the most common benign cardiac tumours. However, due to their location and pedunculated nature, they are associated with substantial complications secondary to systemic embolization or coronary ostial obstruction. Surgical resection should be offered to all patients when the mass is >1 cm in diameter. Valve-sparing excision produces good long-term results in most instances. We present the case of a young female scheduled for elective mitral and tricuspid valve replacement and maze procedure for chronic atrial fibrillation. A CPF was incidentally discovered during routine intraoperative transoesophageal echocardiography with an unusual location; at the left ventricular outflow tract.

An unusual presentation of a cardiac papillary fibroelastoma: a case report

Bu calismada atriyal septumun sol tarafindan koken alan ender gorulen kardiyak fibroelastom olgusu sunuldu. Bu kitle her atriyal sistolde mitral kapaktan sol ventrikule dogru hareketliydi. Ancak, hastada herhangi kardiyak veya serebral semptom yoktu. Ekokardiyografik bulgulara gore kitle sol atriyal miksoma olarak kabul edildi ve hastaya oncelikli olarak ameliyat planlandi. Sol atriyal septuma yapisik olan kitle, septum ile birlikte eksize edildi. Cerrahi olarak olusan septal defekt, otolog perikardiyal yama ile kapatildi. Ameliyat sirasi cekilen transozofageal ekokardiyografide artik kitle saptanmadi ve mitral kapak fonksiyonu normaldi. Kesin tani histopatolojik inceleme ile kondu. Septumdan koken alan papiller fibroelastomalar oldukca nadir gorulur, ancak yerlesim yeri nedeniyle serebrovaskuler emboli acisindan yuksek risklidir. Histopatolojik olarak benign karakterde olmasina ragmen, fatal komplikasyon risklerinden dolayi erken tani ve tedavi cok onemlidir. Anah tar soz cuk ler: Atriyal septum; kardiyak tumor, papiller fibroelastom. In this article, we report an uncommon case of cardiac fibroelastoma originating from the left atrial septum. The mass moved from the mitral valve through the left ventricle in each atrial systole. However, the patient was free of any cardiac or cerebral symptom. Based on the echocardiographic findings, the mass was considered the left atrial myxoma and the patient was primarily scheduled for surgery. The mass which adhered to the left atrial septum was excised with the septum. Surgical septal defect was closed with an autologous pericardial patch. Intraoperative transesophageal echocardiography revealed no residual mass with a normal mitral valve function. The definitive diagnosis is made by histopathological examination. Papillary fibroelastomas originating from septum are extremely rare, but have high-risk for cerebrovascular embolism due to their location. Despite histopathologically benign nature, early diagnosis and treatment is of utmost importance due to fatal complication risks.

Minimally invasive robotically assisted surgical resection of left atrial endocardial papillary fibroelastomas

Minimally invasive robotically assisted surgical resection of left atrial endocardial papillary fibroelastomas

Clinical significance and prevalence of valvular strands during routine echo examinations.

Lambl's excrescences (valvular strands) have been associated with an increased embolic risk. In previous studies, valvular strands have mostly been detected by transoesophageal echo (TEE). The current high-resolution echo systems allow better detection of valvular strands often even by transthoracic echocardiography (TTE). We attempted to determine the incidence of valvular strands during routine echo exams, TTE and/or TEE, and their relation to the cardiovascular risk factor, co-morbidities, and outcome. Over 21 000 echo exams were performed at our hospital during 2008-12 and were searched for reporting of valvular strands. One hundred and fifty such studies were identified and the presence of valvular strands was confirmed. These patients were then evaluated for clinical characteristics, co-morbidities, and outcome, and compared with 150 age- and gender-matched patients without valvular strands. Incidence of valvular strands was maximal at age 61-70 (0.94%), and they were found more commonly in men than in women, 92 vs. 58, P < 0.00001. Valvular strands occurred more often on the aortic than on the mitral valve, 125 vs. 36, respectively, P < 0.00001, were more often associated with thickened or calcified aortic or mitral valve, and occupied the ventricular side of the aortic valve and the atrial side of the mitral valve, P < 0.00001. Embolic events occurred in 40 of the 150 patients with strands (27%). Valvular strands were not associated with increased mortality. Valvular strands (Lambl's excrescences) appear not to affect life expectancy, but are often associated with embolic/cerebrovascular events, are more common in older patients, in men than in women, and are more commonly located on the aortic than on the mitral valve.

Cardiac Tumors in the Neonatal Period: Clinical Features and Echocardiographic Evaluation

ABSTRACT Introduction Primary cardiac tumors are rare in the fetal and neonatal period (0.0017-0.28%), but considerably more frequent than the metastatic ones; most of them are benign. The objective of this study was to analyze the clinical and echocardiographic features of cardiac tumors during the neonatal period. Case presentations In the period of 6 years (2007-2012), we diagnosed five newborns with cardiac tumors of which two newborns with left ventricle tumors suggestive of rhabdomyomas, both diagnosed in utero; a newborn with multiple heart and brain tumors, suggestive of tuberous sclerosis, who died after 24 days; a newborn with tricuspid valve papillary fibroelastoma, hemodynamically stable during the monitoring; and a newborn of the mother with high-risk pregnancy with a single right atrial tumor. Conclusion Cardiac tumors are rare but have good prognosis when not accompanied by arrhythmias or hemodynamic changes. Echocardiographic evaluation is an effective method for the diagnosis and monitoring of patients with cardiac tumors. How to cite this article Hăşmăşanu M, Opriţa S, Kovacs T, Andreica S, Mátyás M, Decean E, Stamatian F, Zaharie G. Cardiac Tumors in the Neonatal Period: Clinical Features and Echocardiographic Evaluation. Donald School J Ultrasound Obstet Gynecol 2014;8(2):201-207.