Left Ventricular Papillary Fibroelastoma Presenting with Dyspnea on Exertion.
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ABSTRACT:
SUBMITTER: de Klerk S
PROVIDER: S-EPMC7451855 | biostudies-literature | 2020 Aug
REPOSITORIES: biostudies-literature
Publications
Left Ventricular Papillary Fibroelastoma Presenting with Dyspnea on Exertion.
de Klerk Storm S Dogon Calli C Grossman Moshe M Adler Jeremy E JE McFalls Jeanne J Furukawa Satoshi S Goldberg Sheldon S
CASE (Philadelphia, Pa.) 20200229 4
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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.
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Project description:Papillary fibroelastoma (PFE) is a rare, slow-growing cardiac tumor. We encountered an 80-year-old man with PFE accidentally revealed by transthoracic echocardiography (TTE) to evaluate cardiac function before a non-cardiac operation. A 10-mm mass lesion adhered to the anterior papillary muscle of the left ventricle, which had not been detected with TTE performed nine months before. Emergency cardiac surgery to remove the mass was performed, and the mass was diagnosed as a PFE. The PFE grew to 10 mm in a maximum of 9 months; to our knowledge, this is the fastest growth of PFE in the left ventricle reported to date.Learning objectivePapillary fibroelastoma (PFE) is a rare, slow-growing cardiac tumor. The surgical indication of PFE is sometimes controversial. The rapid growth of PFE might be considered as a criterion for surgery because this might result in the rapid progression of symptoms and complications.
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Project description:A papillary fibroelastoma is a rare, avascular, cardiac tumour that is often found incidentally using transthoracic echocardiography (TTE). Peripheral i.v. injection of a microbubble contrast agent is often used to characterize abnormal masses within the heart allowing further delineation of physical features, the area of attachment, and vascularity of the mass in order to differentiate the growth from a tumour or a thrombus. This case highlights a potential pitfall when assessing a cardiac tumour's vascularity using contrast TTE. A cardiac mass was identified on a TTE of a 53-year-old man and was further investigated with microbubble contrast-enhanced TTE. Contrast TTE imaging suggested a vascularized structure in the left ventricle. However, after histological examination the tumour was found to be entirely avascular.Differentiation of cardiac tumour is usually best performed with contrast echocardiography.Contrast echocardiography may not be best tool to determine if cardiac mass is vascularized.A papillary fibroelastoma can appear vascularized with contrast echocardiography due to it's frond-like structures.Physicians should be aware of this potential confusion when assessing a cardiac tumour in patients.
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Project description:We hypothesized that among patients presenting with dyspnea on exertion (DOE), those who were found to have hyperdynamic left ventricle (i.e. LVEF ≥ 70%) on stress radionuclide myocardial perfusion imaging (RNMPI), are more likely to have features of diastolic dysfunction on transthoracic echocardiography.Medical records of 1892 consecutive patients who presented between February 2011 and September 2012 with the chief complaint of DOE and were referred to stress RNMPI were reviewed. Among these, patients who had no evidence of reversible ischemia and had hyperdynamic left ventricle on perfusion imaging, were selected and their recent echocardiograms were reviewed for evidence of diastolic dysfunction. Logistic regression analysis was used to develop an equation to predict diastolic dysfunction with the ejection fraction as the predictor. A two-way analysis of variance model was used to detect differential patterns of ejection fraction across diastolic dysfunction and gender.A hyperdynamic left ventricle identified on stress RNMPI was found to be a significant predictor of diastolic dysfunction on echocardiography in logistic regression analysis (odds ratio = 1.24, 95% CI = 1.13-1.35, p < 0.0001). A hyperdynamic left ventricle on stress RNMPI has a specificity of 96.77% (CI 83.24-99.46%) and a positive predictor value of 97.83% (CI 88.43-99.64%) in identifying diastolic dysfunction.In patients presenting with DOE who have no evidence of reversible ischemia on radionuclide stress testing but have hyperdynamic left ventricle, a search should be made for alternate cardiac etiology for this complaint such as diastolic dysfunction and heart failure with preserved ejection fraction.
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Project description:We present the case of a 69-year-old female patient with a history of endometrial carcinoma in 1996, who underwent a total hysterectomy and bilateral adnexectomy. The patient also received chemotherapy and mediastinal radiotherapy followed by cancer remission. Ten years later she presented with heart failure and her Doppler-echocardiogram showed severe mitral regurgitation with pulmonary hypertension and a papillary fibroelastoma in the left ventricle. In 2011, she underwent a mitral valve replacement with a biological prosthesis and the pathology exam revealed valve damage consistent with radiotherapy- induced changes and confirmed the presence of a papillary fibroelastoma. This unusual mechanism of papillary fibroelastoma should be disseminated among cardiology physicians and in patients who have survived for long periods after radiotherapy. It is important to remember that cardiac complications may indeed occur, and the treating physician is responsible for detecting them.
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