Editorial comment
Cardiac papillary fibroelastoma: So small and yet so dangerous
Fibroelastoma papilar cardíaco: tão pequeno e no entanto tão perigoso
a Cardiac Surgery Department, Hospital Santa Cruz, Carnaxide, Portugal
b Cardiovascular Research Centre, Department of Surgery and Physiology, Faculty of Medicine, University of Porto, Porto, Portugal
c Serviço de Cardiologia, Departamento de Imagiologia Cardíaca Hospital da Luz, Faculdade de Ciências Médicas da Universidade Nova de Lisboa, Lisboa, Portugal
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The first reported pre-mortem case was an incidental finding during surgical ventricular septal defect repair in 1979. However, while histologically benign, PFE is a dangerous condition due to the potential for cerebral and coronary embolization. While in around two thirds of cases diagnosis is made fortuitously by echocardiogram, one third are diagnosed following an embolic event that triggers further exploration.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">2</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">PFE are single in 90% of cases and more than 95% are located in the left heart. Once diagnosed, surgical resection is recommended to avoid embolic complications; this constitutes definitive treatment as recurrence is very rare.</p><p id="par0015" class="elsevierStylePara elsevierViewall">PFE consists of an endocardial layer covering a dense mesh of fibroelastic bundles and a loose avascular connective tissue matrix. Its etiology is unknown and its histopathogenesis is the subject of debate, some theories favoring a hamartomatous origin while others suggest a virus-induced tumor mechanism. PFE are soft, white to tan, and friable, often with adherent thrombus, which explains their propensity to embolize.</p><p id="par0020" class="elsevierStylePara elsevierViewall">In this issue of the <span class="elsevierStyleItalic">Journal</span>, Rodrigues et al.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">3</span></a> report a series of 26 patients with cardiac PFE in whom the tumor was the primary indication for surgery. The authors are to be congratulated for drawing our attention to this peculiar benign cardiac tumor, because of its potentially severe complications. The imaging characteristics of this tumor should be well known as surgical resection is simple, effective and the only method that prevents the dreadful risk of embolization.</p><p id="par0025" class="elsevierStylePara elsevierViewall">With the increasing use of transthoracic echocardiography (TTE) for screening purposes and for a multitude of other reasons, the detection of anomalous intracardiac masses has increased. It is therefore important to know the imaging features that can help establish the differential diagnosis with other intracardiac tumors and masses.</p><p id="par0030" class="elsevierStylePara elsevierViewall">Multimodality imaging plays a key role in the assessment of cardiac masses in general and of PFE in particular, allowing its diagnosis and often establishing the differential diagnosis, based on location, attachment, size, borders, mobility, enhancement, vascularity and metabolism, among other factors.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">4</span></a> Because of their availability, low cost, and absence of radiation or contrast, together with their good match between spatial and temporal resolution, TTE, and especially transesophageal echocardiography (TEE), are the most important imaging techniques in the assessment of PFE.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">5</span></a> The typical morphological appearance of the mass (like the fronds of a sea anemone, with a shimmering border, a diameter of 5-40 mm, with a short stalk, usually attached to a valve in the left heart valve, and independently mobile), is usually well depicted by two-dimensional images. Additionally, the use of three-dimensional technology often adds further anatomical information, such as correct spatial orientation and the attachment point, that is useful in surgical planning.<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">6</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">Despite the better spatial resolution of cardiac magnetic resonance (CMR) and cardiac computed tomography (CCT), the potential advantages of these techniques in the assessment of valvular PFE are reduced by their low temporal resolution, an important limitation when assessing rapidly moving structures like PFE. However, tissue characterization by CMR (with late gadolinium enhancement in T1- and T2-weighted sequences) and CCT may be useful to differentiate PFE from other cardiac masses, helping to provide a more accurate differential diagnosis. In rare cases, when differential diagnosis is needed between a PFE and a malignant valve tumor, positron emission tomography (PET)/CMR or PET/CCT often provides the correct diagnosis, differentiating benign from malignant tumors based on different metabolic and anatomical data.<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">7</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">In the series by Rodrigues et al., patients were identified from their center's cardiovascular surgery and pathology databases, and so false positive and false negative results of TTE are unavailable.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">3</span></a> We therefore do not know how many patients had masses that were undetected, which would have been of interest. Healed vegetations of infective endocarditis, myxomas, Lambl excrescences, and even mobile thrombi, can mimic the echocardiographic findings of a cardiac PFE. It is also important to rule out a history of bacterial or non-bacterial endocarditis, antiphospholipid syndrome and lupus erythematosus, particularly in older patients with associated multiple morbidities, including atrial fibrillation, and with a higher baseline stroke risk. In a large Mayo Clinic series, a quarter of patients had cardiac PFE detected by TEE but not by TTE, reinforcing the crucial role of TEE when exploring the cause of embolic phenomena.<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">8</span></a> Unlike most series, which report the aortic valve as the most common PFE location, Rodrigues et al. observed 14/25 cases (55%) of PFE on the mitral valve, 9/26 (35%) on the aortic valve and only two that did not involve a cardiac valve.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">3</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">Although cardiac PFE are benign, they can have a malignant behavior due to their embolic potential.<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">3,8</span></a> In the present report, PFE presented with neurological deficits in eight cases (who had undergone echocardiography to exclude a cardiac embolic source). The authors could not identify any imaging characteristic differentiating between the eight patients who presented with stroke and the 18 patients who were asymptomatic, except that the former were significantly younger (42±17 years vs. 54.3±18.4 years for the overall population), confirming that size, mobility or location of the tumor cannot be relied upon to exclude stroke risk.<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">8</span></a> This may due to their small sample size, as a review of 725 cases showed that tumor mobility was the only independent predictor of PFE-related death or non-fatal embolization.<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">9</span></a> As the tumor is usually single and still attached to the cardiac structures after the neurological event, the mechanisms of stroke or transient ischemic attack (TIA) in patients with PFE are poorly understood; they may be related to tumor fragments or thrombi attached to the tumor. It would have been interesting to know the clinical characteristics of neurologically symptomatic patients (type and severity of stroke or TIA, and the timing of surgery after stroke).</p><p id="par0050" class="elsevierStylePara elsevierViewall">The authors were able to perform valve-sparing operations in all 24 patients with a valvular tumor location. During a median follow-up of around five years, there were no deaths or recurrence of embolic events. However, as there was no systematic echocardiographic follow-up, it is uncertain whether there was tumor recurrence. Although rare, recurrence has been reported in 1.6% of cases, stressing the importance of TTE follow-up.<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">8</span></a> There is also evidence that the risk of subsequent cerebrovascular accident (CVA) is greater in patients with echocardiography-identified but unoperated PFE, compared with an age- and gender-matched population, and that excision substantially decreases CVA risk and even mortality from PFE.<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">8</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">In summary, it should be stressed that echocardiography is not 100% accurate in making a tissue diagnosis of intracardiac masses, highlighting the role of a multimodality imaging approach. Young patients without significant comorbidities and a high diagnostic suspicion of cardiac PFE should be offered surgical resection, independently of symptoms, particularly if the tumor is mobile. The surgical risk is low, valve repair can be achieved in virtually all patients and the risk of embolic stroke is reduced. For older or sicker patients, antiplatelet or anticoagulant therapy have been proposed, but supporting data are limited and therefore the final decision should be based on discussions by the heart team.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of interest</span><p id="par0060" class="elsevierStylePara elsevierViewall">The authors have no conflicts of interest to declare.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Conflicts of interest" ] 1 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:9 [ 0 => array:3 [ "identificador" => "bib0050" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Papillary fibroelastoma: increasing recognition of a surgical disease" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:3 [ 0 => "R.A. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 5 | 5 | 10 |
| 2024 October | 37 | 21 | 58 |
| 2024 September | 50 | 22 | 72 |
| 2024 August | 38 | 21 | 59 |
| 2024 July | 34 | 21 | 55 |
| 2024 June | 42 | 18 | 60 |
| 2024 May | 44 | 17 | 61 |
| 2024 April | 25 | 35 | 60 |
| 2024 March | 43 | 20 | 63 |
| 2024 February | 22 | 17 | 39 |
| 2024 January | 32 | 25 | 57 |
| 2023 December | 23 | 24 | 47 |
| 2023 November | 41 | 27 | 68 |
| 2023 October | 35 | 13 | 48 |
| 2023 September | 36 | 19 | 55 |
| 2023 August | 25 | 11 | 36 |
| 2023 July | 38 | 9 | 47 |
| 2023 June | 28 | 13 | 41 |
| 2023 May | 69 | 26 | 95 |
| 2023 April | 30 | 5 | 35 |
| 2023 March | 52 | 17 | 69 |
| 2023 February | 68 | 19 | 87 |
| 2023 January | 81 | 14 | 95 |
| 2022 December | 80 | 20 | 100 |
| 2022 November | 67 | 18 | 85 |
| 2022 October | 44 | 23 | 67 |
| 2022 September | 57 | 38 | 95 |
| 2022 August | 47 | 36 | 83 |
| 2022 July | 47 | 44 | 91 |
| 2022 June | 48 | 34 | 82 |
| 2022 May | 39 | 28 | 67 |
| 2022 April | 48 | 37 | 85 |
| 2022 March | 42 | 41 | 83 |
| 2022 February | 27 | 30 | 57 |
| 2022 January | 57 | 25 | 82 |
| 2021 December | 30 | 43 | 73 |
| 2021 November | 38 | 33 | 71 |
| 2021 October | 56 | 41 | 97 |
| 2021 September | 51 | 23 | 74 |
| 2021 August | 41 | 23 | 64 |
| 2021 July | 27 | 18 | 45 |
| 2021 June | 37 | 24 | 61 |
| 2021 May | 61 | 41 | 102 |
| 2021 April | 115 | 56 | 171 |
| 2021 March | 71 | 26 | 97 |
| 2021 February | 64 | 17 | 81 |
| 2021 January | 94 | 12 | 106 |
| 2020 December | 57 | 12 | 69 |
| 2020 November | 38 | 12 | 50 |
| 2020 October | 70 | 9 | 79 |
| 2020 September | 49 | 13 | 62 |
| 2020 August | 37 | 12 | 49 |
| 2020 July | 52 | 9 | 61 |
| 2020 June | 57 | 15 | 72 |
| 2020 May | 44 | 3 | 47 |
| 2020 April | 44 | 5 | 49 |
| 2020 March | 29 | 8 | 37 |
| 2020 February | 87 | 32 | 119 |
| 2020 January | 58 | 15 | 73 |
| 2019 December | 46 | 4 | 50 |
| 2019 November | 17 | 9 | 26 |
| 2019 October | 16 | 5 | 21 |
| 2019 September | 61 | 4 | 65 |
| 2019 August | 16 | 9 | 25 |
| 2019 July | 18 | 8 | 26 |
| 2019 June | 18 | 17 | 35 |
| 2019 May | 26 | 6 | 32 |
| 2019 April | 21 | 15 | 36 |
| 2019 March | 17 | 20 | 37 |
| 2019 February | 25 | 11 | 36 |
| 2019 January | 42 | 18 | 60 |
| 2018 December | 7 | 10 | 17 |
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