scholarly journals Papillary Fibroelastoma of the Ascending Aorta

2019 ◽  
Vol 7 ◽  
pp. 232470961984037
Author(s):  
Karim M. Al-Azizi ◽  
Mohanad Hamandi ◽  
Ronald Baxter ◽  
Anita Krueger ◽  
Alexander W. Crawford ◽  
...  

Papillary fibroelastomas are rare benign primary cardiac tumors. They are typically found on valvular surfaces, most commonly, the aortic valve. In this article, we report a case of papillary fibroelastoma arising from the sinotubular junction of the ascending aorta, a rare and unusual site causing an embolic stroke.

Author(s):  
Mustafa Zakkar ◽  
Vito D Bruno ◽  
Pavel Zacek ◽  
Isabelle Di Centa ◽  
Christophe Acar ◽  
...  

Abstract OBJECTIVES Aortic valve (AV) insufficiency with normal root and ascending aorta is most frequently treated by valve replacement with significant prosthetic-related complications and reduced life expectancy. We compared the outcomes of a new standardized isolated AV repair approach using an external aortic annuloplasty ring at the subvalvular level (single ring annuloplasty) and the role of an additional supravalvular ring at the sinotubular junction (double ring annuloplasty). METHODS Single centre data were collected from the Aortic Valve Insufficiency and ascending aorta Aneurysm InternATiOnal Registry (AVIATOR) between 2003 and 2017. A total of 93 patients (56 single ring and 37 double ring) underwent isolated AV repair. RESULTS The overall 30-day mortality rate and the need for a permanent pacemaker were 1% and 2%, respectively. The overall survival rate at 6 years was similar for sex- and age-matched members of the general population (89% vs 95%; P = 0.1) and did not differ between the double and single ring groups (82% vs 93%; P = 0.4) at 6 years. There were no thromboembolic or bleeding events in the entire cohort. However, at 6 years, the cumulative incidence of valve-related reintervention was 26% in the single ring annuloplasty group compared to 3% in the double ring annuloplasty (P = 0.02) group. Similarly, at 6 years, the cumulative incidence of moderate-to-severe (>2) aortic insufficiency was 30% in the single ring annuloplasty group compared to 0% in the double ring annuloplasty group (P = 0.007). CONCLUSIONS Standardized AV repair with external ring annuloplasty has a survival rate similar to that of the general population. The additional stabilization of the sinotubular junction with a second supravalvular ring (double ring annuloplasty) is associated with better outcomes compared to single subvalvular annuloplasty. It can be considered as a first line intervention for patients with isolated aortic insufficiency and pliable leaflets.


2017 ◽  
Vol 20 (2) ◽  
pp. 052
Author(s):  
Hongfei Xu ◽  
Liangwei Chen ◽  
Chengmeng Ye ◽  
Kwabena Gyabaah Owusu-Ansah ◽  
Yiming Ni ◽  
...  

Primary cardiac tumors are extremely rare with occurrence range of about 0.0017-0.28%. Papillary fibroelastoma is a benign cardiac neoplasms. Improvement in the early diagnosis has been made possible with the help of high-resolution imaging technology, such as transesophageal echocardiography, combined with ischemic or embolic complications which patients typically present with clinically. We herein present a 51-year-old female patient with a papillary fibroelastoma on the aortic cusp, with its origin from the left coronary sinus. Her only clinical manifestation was angina-like chest pain and syncope. Surgical resection with aortic valve replacement due to the defect as a result of the resected tumor was performed with the patient on cardiopulmonary bypass. The patient recovered uneventfully and was discharged.  A 2-year follow-up showed an intact valve without tumor recurrence.


2014 ◽  
Vol 71 (6) ◽  
pp. 600-602 ◽  
Author(s):  
Filip Vukmirovic ◽  
Mihailo Vukmirovic ◽  
Irena Tomasevic-Vukmirovic

Introduction. Primary tumors of the heart are rare, usually benign and occur mostly in adults, and usually originate from the endocardium, followed by the myocardium and rarest of the pericardium. Papillary fibroelastoma accounts for less than 10% of all cardiac tumors, but they are most common valvular tumors. The clinical presentation of papillary fibroelastoma varies from asymptomatic cases to cases which have severe clinical presentation that is most likely due to embolic complications. Tumor can usually be discovered by echocardiography or during autopsy. Case report. We reported a case of 53- year-old man submitted to routine echocardiographic examination. The patient had the history of hypertension for five years, without any other symptoms. Echocardiography found a round tumor attached to the noncoronary cuspis of the aortic valve. The tumor was surgically removed and pathohistological examination confirmed diagnosis of papillary fibroelastoma. After surgery the patient fully recovered without tumor recurrence or aortic regurgitation. Conclusion. Histologically, papilary fibroelastoma is benign tumor of the heart. As demonstrated in this case, a papillary fibroelastoma can be an incidental finding discovered during echocardiography in patients with the history of hypertension.


2012 ◽  
Vol 2012 ◽  
pp. 1-5 ◽  
Author(s):  
Jaroslav Benedik ◽  
Kevin Pilarczyk ◽  
Daniel Wendt ◽  
Jiri Indruch ◽  
Radek Flek ◽  
...  

Objectives. Bicuspid aortic valve (AV) represents the most common form of congenital AV malformation, which is frequently associated with pathologies of the ascending aorta. We compared the mechanical properties of the aortic wall between patients with bicuspid and tricuspid AV using a new custom-made device mimicking transversal aortic wall shear stress.Methods. Between 03/2010 and 07/2011, 190 consecutive patients undergoing open aortic valve replacement at our institution were prospectively enrolled, presenting either with a bicuspid (group 1,n=44) or a tricuspid (group 2,n=146) AV. Aortic wall specimen were examined with the “dissectometer” resulting in nine specific aortic-wall parameters derived from tensile strength curves (TSC).Results. Patients with a bicuspid AV showed significantly more calcified valves (43.2% versus 15.8%,P<0.001), and a significantly thinner aortic wall (2.04±0.42 mm versus2.24±0.41 mm,P=0.008). Transesophageal echocardiography diameters (annulus, aortic sinuses, and sinotubular junction) were significantly larger in the bicuspid group (P=0.003,P=0.02,P=0.01). We found no difference in the aortic wall cohesion between both groups as revealed by shear stress testing (P=0.72,P=0.40,P=0.41).Conclusion. We observed no differences of TSC in patients presenting with tricuspid or bicuspid AVs. These results may allow us to assume that the morphology of the AV and the pathology of the ascending aorta are independent.


Author(s):  
Paul P Urbanski ◽  
Vadim Irimie ◽  
Anno Diegeler ◽  
Aleksandra Morka ◽  
Tarvo Thamm ◽  
...  

Abstract OBJECTIVES The study objective was to describe the technique and outcomes of original coronary ostial slide plasty in patients with anomalous coronary artery origin (ACAO) localized in the aneurysmal ascending aorta (AA) being replaced because of its isolated pathology and otherwise non-pathological aortic root. METHODS A total of 23 patients (median age 52 years) with the ascending phenotype of proximal aorta aneurysm and ACAO of at least 1 coronary artery localized in the AA being replaced underwent ostial slide plasty to transpose the ACAO to the respective sinus of Valsalva and, consequently, to allow an AA replacement with placement of the proximal anastomosis at the level of the sinotubular junction (STJ). In 15 patients, the aortic valve was bicuspid, and all but 3 patients presented with a relevant valve defect. In addition to remodelling the STJs (all patients), valve-sparing repair or replacement was performed in 12 and 8 patients, respectively. RESULTS No patient died during the entire follow-up (median 72, range 3–183 months). One patient required replacement of a recurrently insufficient valve that was repaired primarily using cusp patch plasty, but there were no further cardiac reoperations nor any re-interventions on the proximal aorta, aortic valve and/or coronary artery ostia. Two patients received peripheral coronary stents (8 and 7 years after surgery, respectively) due to coronary heart disease. CONCLUSIONS Transposition of the ACAO from the replaced AA into the normal sinus of Valsalva using the ostial slide plasty offers a simple and safe surgical option enabling a recreation of a durable STJ at the level of the anastomosis between the root and the aortic graft.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
A Castelo ◽  
M Silva ◽  
A Goncalves ◽  
L Branco ◽  
P Coelho ◽  
...  

Abstract Introduction Papillary fibroelastomas are rare benign primary cardiac tumors that more frequently involve cardiac valves. They are frequently incidentally discovered by echocardiography but may also cause symptoms. Purpose The aim of this study was to characterize several features of histologically confirmed fibroelastomas. Methods Retrospective analysis of patients with echocardiographic suspicion of fibroelastoma between 2009 and 2019 in a single tertiary center. Echocardiography was compared with histology, and echocardiographic, surgical and pathological information about confirmed fibroelastomas was collected. Results 37 patients (P) (54.1% men) with an echocardiographic suspicion and/or histologically confirmed fibroelastoma were included, with a mean age of 58 +- 3 years (min 22, max 82). Echocardiographic report was analyzed in 34P (91.9%), with 32P (94.1%) reporting a likely fibroelastoma and 2P (5.9%) reporting a non-specified mass. 21P (56.8%) had surgery, with 12P (57.1%) having a surgical suspicion of a fibroelastoma, 2P (9.5%) of a mixoma, 1P (4.8%) of a non-specified mass and 6P (28.6%) with undefined suspicion. Of the 21P who had surgery, 66.7% (14P) had a histologically confirmed fibroelastoma, 1P (4.8%) had a mixoma, and 6P (28.6%) had other diagnoses. From the 14P with histologically confirmed fibroelastoma 64.3% had this suspicion by echocardiography and 35.7% had an echocardiogram reporting a non-specified. There was a global concordance between echocardiography and histology in 52.9%. The mean age of confirmed fibroelastoma P was 54 +-5years, and 50% were men. 7P (50%) were asymptomatic, 2 (14.3%) had a stroke, 2 (14.3%) had syncope, 1 (7.1%) had fatigue, 1 (7.1%) had palpitations and 1P had consciousness alteration. In echocardiography most P (71.4%) had only one mass but 1P had 4 different masses. The tumors had a longer axis between 6 and 25mm, with the majority (57.1%) measuring more than 10mm. 12P (85.7%) had valvular fibroelastomas, 50% of these in the aortic valve (3 in non-coronary cusp, 1 in right coronary cusp and 2 non-specified) and 50% in the mitral valve (all in sub-valvular apparatus, involving anterior leaflet, tendinous chord or papillary muscle). 1P had a left ventricular fibroelastoma (apical) and 1P had four masses in the left atrium. Macroscopically 4 lesions had a gelatinous consistency, 2 of them were membranous, 2 were elastic, 2 were friable, 1 was villainous and in 3 of them consistency was not described. The majority (57%) was white, 14% was translucent and in the rest the color was not specified. There was no described recurrence after surgery and there were no deaths registered. Conclusion In this population there was a reasonable concordance between echocardiography and histology, but in some cases the diagnosis was undefined or wrong. 50% of the patients were asymptomatic and the majority had valvular fibroelastomas, but a few had a different location.


2013 ◽  
Vol 2013 ◽  
pp. 1-3 ◽  
Author(s):  
Nowell M. Fine ◽  
David A. Foley ◽  
Jerome F. Breen ◽  
Joseph J. Maleszewski

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.


2017 ◽  
Vol 19 (4) ◽  
pp. 454
Author(s):  
Alin Alexandru Ionescu ◽  
Bogdan Radulescu ◽  
Vlad Herlea ◽  
Ioan Miclea ◽  
Irinel Parepa ◽  
...  

Papillary fibroelastomas (PFEs) are one of the most frequent primary cardiac tumors and occur more often in patients with hypertrophic obstructive cardiomyopathy (HOCM). PFEs have been linked to an increased risk of neurological events. We report a case of a 59-year-old woman with HOCM in whom echocardiography (transthoracic and transesophageal, using 2D and 3D techniques) revealed multiple masses in various locations in the left cardiac chambers. Surgical excision of the cardiac tumors and aortic valve replacement was performed and the pathologic report confirmed the diagnosis of PFEs. Patient followup using ultrasonography is crucial since recurrence is a possibility. Current cardiac ultrasound techniques are essential for diagnosing and for guiding the management of these conditions.


2015 ◽  
Vol 4 ◽  
pp. 92
Author(s):  
Alexander Eugene Urban ◽  
Masoor Kamalesh ◽  
Daniel Gutteridge

<p>Cardiac papillary fibroelastomas are rare, typically benign primary cardiac tumors.  The pulmonary valve is the least common site for valvular papillary fibroelastomas.  They commonly present with dyspnea on exertion and can show right ventricular outflow tract obstruction on echocardiography.  Embolic phenomena are one of the most serious consequences.  Treatment usually consists of surgical excision.  We report the first case of pulmonary emboli from pulmonary valve papillary fibroelastoma treated with anticoagulation.  This occurred in only the second case of pulmonary emboli from pulmonary papillary fibroelastoma reported in literature to date.</p>


Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Thieu Nguyen ◽  
Andrea Z Beaton ◽  
Wyman W Lai ◽  
Prema Ramaswamy ◽  
Ira A Parness ◽  
...  

Objectives: To explore the difference in ascending aortic dilatation between subgroups of bicuspid aortic valve (BAV) patients with and without coarctation of the aorta (CoA). Methods: Our echocardiographic database (1993–2006) was searched for BAV patients with CoA (Group A) and without CoA (Group B). Measurements at the aortic annulus, root, sinotubular junction, and ascending aorta were obtained for each patient, and body surface area-adjusted Z-score values were compared. Exclusion criteria included more than mild aortic stenosis or regurgitation, previous balloon aortic valvuloplasty, or complex left heart disease; plus Turner, Noonan, Williams, and Marfan Syndromes. Results: The median age in Group A (n=53) was 11.3 yrs (range 0 to 30) with median follow-up of 7 yrs (0 to 12.7); median age in Group B (n=145) was 8.7 yrs (0 to 29) with median follow-up of 4 yrs (0 to 13.1). Group B patients had significantly greater aortic annulus, sinotubular junction, and ascending aortic dimensions (ascending aorta Z-scores shown in Figure , p<0.0001). Group A ascending aortic dimensions did not differ significantly from the normal population. The rate of growth of the ascending aorta in Group B was higher in the first 10 years of life. Conclusion: The ascending aorta in patients with bicuspid aortic valve and coarctation does not dilate to the same degree as patients with isolated bicuspid aortic valve. This may reflect an inherent difference in aortic wall properties between the two groups. Comparison of Ascending Aorta Z Scores


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