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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| Ano/Mês | Html | Total | |
|---|---|---|---|
| 2024 Novembro | 14 | 3 | 17 |
| 2024 Outubro | 81 | 39 | 120 |
| 2024 Setembro | 111 | 34 | 145 |
| 2024 Agosto | 95 | 26 | 121 |
| 2024 Julho | 83 | 31 | 114 |
| 2024 Junho | 76 | 32 | 108 |
| 2024 Maio | 68 | 24 | 92 |
| 2024 Abril | 65 | 33 | 98 |
| 2024 Maro | 50 | 28 | 78 |
| 2024 Fevereiro | 66 | 39 | 105 |
| 2024 Janeiro | 68 | 35 | 103 |
| 2023 Dezembro | 56 | 31 | 87 |
| 2023 Novembro | 84 | 34 | 118 |
| 2023 Outubro | 69 | 13 | 82 |
| 2023 Setembro | 84 | 24 | 108 |
| 2023 Agosto | 64 | 26 | 90 |
| 2023 Julho | 60 | 18 | 78 |
| 2023 Junho | 51 | 17 | 68 |
| 2023 Maio | 70 | 34 | 104 |
| 2023 Abril | 47 | 5 | 52 |
| 2023 Maro | 95 | 27 | 122 |
| 2023 Fevereiro | 85 | 23 | 108 |
| 2023 Janeiro | 69 | 23 | 92 |
| 2022 Dezembro | 57 | 23 | 80 |
| 2022 Novembro | 69 | 48 | 117 |
| 2022 Outubro | 69 | 32 | 101 |
| 2022 Setembro | 83 | 43 | 126 |
| 2022 Agosto | 106 | 44 | 150 |
| 2022 Julho | 116 | 49 | 165 |
| 2022 Junho | 92 | 34 | 126 |
| 2022 Maio | 67 | 40 | 107 |
| 2022 Abril | 80 | 39 | 119 |
| 2022 Maro | 64 | 45 | 109 |
| 2022 Fevereiro | 79 | 42 | 121 |
| 2022 Janeiro | 98 | 33 | 131 |
| 2021 Dezembro | 79 | 42 | 121 |
| 2021 Novembro | 113 | 51 | 164 |
| 2021 Outubro | 108 | 62 | 170 |
| 2021 Setembro | 99 | 37 | 136 |
| 2021 Agosto | 101 | 31 | 132 |
| 2021 Julho | 63 | 30 | 93 |
| 2021 Junho | 55 | 19 | 74 |
| 2021 Maio | 74 | 32 | 106 |
| 2021 Abril | 148 | 37 | 185 |
| 2021 Maro | 99 | 20 | 119 |
| 2021 Fevereiro | 84 | 30 | 114 |
| 2021 Janeiro | 82 | 16 | 98 |
| 2020 Dezembro | 75 | 14 | 89 |
| 2020 Novembro | 70 | 19 | 89 |
| 2020 Outubro | 73 | 15 | 88 |
| 2020 Setembro | 50 | 22 | 72 |
| 2020 Agosto | 55 | 13 | 68 |
| 2020 Julho | 36 | 13 | 49 |
| 2020 Junho | 39 | 13 | 52 |
| 2020 Maio | 60 | 21 | 81 |
| 2020 Abril | 66 | 19 | 85 |
| 2020 Maro | 58 | 17 | 75 |
| 2020 Fevereiro | 141 | 29 | 170 |
| 2020 Janeiro | 45 | 16 | 61 |
| 2019 Dezembro | 36 | 18 | 54 |
| 2019 Novembro | 23 | 8 | 31 |
| 2019 Outubro | 71 | 13 | 84 |
| 2019 Setembro | 68 | 12 | 80 |
| 2019 Agosto | 38 | 11 | 49 |
| 2019 Julho | 26 | 13 | 39 |
| 2019 Junho | 31 | 20 | 51 |
| 2019 Maio | 27 | 13 | 40 |
| 2019 Abril | 24 | 15 | 39 |
| 2019 Maro | 22 | 17 | 39 |
| 2019 Fevereiro | 31 | 23 | 54 |
| 2019 Janeiro | 92 | 38 | 130 |
| 2018 Dezembro | 33 | 30 | 63 |
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