Diagnosis and management of papillary fibroelastomas – a case series

    Authors

    Keywords

    cardiac tumors, papillary fibroelastoma, cardiac imaging

    DOI

    https://doi.org/10.15836/ccar2025.145

    Full Text

    **Introduction**: Papillary fibroelastomas (PFEs) are benign primary cardiac tumors, typically found downstream of the valves (1). They currently account for 10% of all cardiac tumors, with the incidence rising as the spatial resolution of imaging modalities improves. The pathogenesis is unclear, though it is suggested that they originate as microthrombi at sites of endothelial damage (2). While definitive diagnosis requires pathological identification, echocardiography offers high sensitivity and specificity for their detection (3). **Case series**: The first patient is a 62-year-old woman with a history of atrial fibrillation and hypertrophic cardiomyopathy, previously treated with alcohol septal ablation. During routine follow-up, echocardiography revealed a subvalvular mass connected to the anterior mitral cusp, most likely a PFE (**Figure 1**). Computed tomography (CT) confirmed a similar finding (**Figure 2**). The patient was not compliant with the recommended surgery; therefore anticoagulant therapy was continued along with regular follow-up. Magnetic resonance imaging (MRI) performed one year later showed no change in the tumor’s size (**Figure 3**). FIGURE 1. Transesophageal (left) and transthoracic echocardiogram (right) of the mitral valve papillary fibroelastoma (marked with white arrows). FIGURE 2. A CT image of a mitral valve papillary fibroelastoma (marked with a black arrow). FIGURE 3. A cine-MRI image of a mitral valve papillary fibroelastoma (marked with a white arrow). The second patient is a 70-year-old man who was referred to the cardiology department for further evaluation due to peripheral edema. Echocardiography revealed a mobile tumor on the tricuspid valve, most likely a PFE, along with moderate tricuspid regurgitation (**Figure 4**). The diagnosis was confirmed by CT and MRI (**Figure 5**), which also showed partial anomalous pulmonary venous connection with a significant left to right shunt. Given the significant clinical improvement with medications, conservative treatment was continued. FIGURE 4. Tricuspid valve papillary fibroelastoma (marked with a white arrow) on transthoracic echocardiography (left). Transthoracic Color Doppler showing moderate tricuspid regurgitation (right). FIGURE 5. Tricuspid valve papillary fibroelastoma (marked with white arrows) on CT (left) and cine-MRI images (right). The third patient is a 75-year-old woman who was hospitalized following an ischemic stroke. Electrocardiogram showed atrial fibrillation, whereas echocardiography showed a PFE on the aortic valve (**Figure 6**). Warfarin was initiated, and the patient was referred for elective PFE excision. Unfortunately, a month later, the patient experienced another stroke, which had a fatal outcome. FIGURE 6. Aortic valve papillary fibroelastoma (marked with white arrows) on transthoracic echocardiography. **Conclusion**: While PFEs are often asymptomatic and found incidentally, they can also present with embolic manifestations like stroke, myocardial infarction, or sudden death (2). Surgical excision is recommended for larger left-sided PFEs in eligible patients due to the risk of embolization, or during cardiac surgery for another condition. Otherwise, antiplatelet agents may be considered (3).

    Literature

    1. Tyebally S, Chen D, Bhattacharyya S, Mughrabi A, Hussain Z, Manisty C, et al. Cardiac Tumors: JACC CardioOncology State-of-the-Art Review. JACC Cardiooncol. 2020 June 16;2(2):293–311. https://doi.org/10.1016/j.jaccao.2020.05.009
    2. Gopaldas RR, Atluri PV, Blaustein AS, Bakaeen FG, Huh J, Chu D. Papillary fibroelastoma of the aortic valve: operative approaches upon incidental discovery. Tex Heart Inst J. 2009;36(2):160–3. https://pubmed.ncbi.nlm.nih.gov/19436815/
    3. Devanabanda AR, Lee LS. Papillary Fibroelastoma. 2023 Apr 26. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan–.
    Cardiologia Croatica
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    Diagnosis and management of papillary fibroelastomas – a case series

    Extended Abstract
    Issue5-6
    Published
    Pages145-147
    PDF via DOIhttps://doi.org/10.15836/ccar2025.145
    cardiac tumors
    papillary fibroelastoma
    cardiac imaging

    Authors

    Anica Milinković*ORCIDUniversity Hospital Centre Zagreb, Zagreb, Croatia
    Ana Marija SliškovićORCIDUniversity Hospital Centre Zagreb, Zagreb, Croatia
    Petra Angebrandt BeloševićORCIDUniversity Hospital Centre Zagreb, Zagreb, Croatia
    Davor RadićORCIDUniversity Hospital Centre Zagreb, Zagreb, Croatia
    Richard MatasićORCIDUniversity Hospital Centre Zagreb, Zagreb, Croatia
    Eduard MargetićORCIDUniversity Hospital Centre Zagreb, Zagreb, Croatia
    Dejan DošenORCIDUniversity Hospital Centre Zagreb, Zagreb, Croatia

    *Correspondence email: anica.milinkovic@outlook.com

    Full Text

    Introduction: Papillary fibroelastomas (PFEs) are benign primary cardiac tumors, typically found downstream of the valves (1). They currently account for 10% of all cardiac tumors, with the incidence rising as the spatial resolution of imaging modalities improves. The pathogenesis is unclear, though it is suggested that they originate as microthrombi at sites of endothelial damage (2). While definitive diagnosis requires pathological identification, echocardiography offers high sensitivity and specificity for their detection (3).

    Case series: The first patient is a 62-year-old woman with a history of atrial fibrillation and hypertrophic cardiomyopathy, previously treated with alcohol septal ablation. During routine follow-up, echocardiography revealed a subvalvular mass connected to the anterior mitral cusp, most likely a PFE (Figure 1). Computed tomography (CT) confirmed a similar finding (Figure 2). The patient was not compliant with the recommended surgery; therefore anticoagulant therapy was continued along with regular follow-up. Magnetic resonance imaging (MRI) performed one year later showed no change in the tumor’s size (Figure 3).

    FIGURE 1. Transesophageal (left) and transthoracic echocardiogram (right) of the mitral valve papillary fibroelastoma (marked with white arrows).

    FIGURE 2. A CT image of a mitral valve papillary fibroelastoma (marked with a black arrow).

    FIGURE 3. A cine-MRI image of a mitral valve papillary fibroelastoma (marked with a white arrow).

    The second patient is a 70-year-old man who was referred to the cardiology department for further evaluation due to peripheral edema. Echocardiography revealed a mobile tumor on the tricuspid valve, most likely a PFE, along with moderate tricuspid regurgitation (Figure 4). The diagnosis was confirmed by CT and MRI (Figure 5), which also showed partial anomalous pulmonary venous connection with a significant left to right shunt. Given the significant clinical improvement with medications, conservative treatment was continued.

    FIGURE 4. Tricuspid valve papillary fibroelastoma (marked with a white arrow) on transthoracic echocardiography (left). Transthoracic Color Doppler showing moderate tricuspid regurgitation (right).

    FIGURE 5. Tricuspid valve papillary fibroelastoma (marked with white arrows) on CT (left) and cine-MRI images (right).

    The third patient is a 75-year-old woman who was hospitalized following an ischemic stroke. Electrocardiogram showed atrial fibrillation, whereas echocardiography showed a PFE on the aortic valve (Figure 6). Warfarin was initiated, and the patient was referred for elective PFE excision. Unfortunately, a month later, the patient experienced another stroke, which had a fatal outcome.

    FIGURE 6. Aortic valve papillary fibroelastoma (marked with white arrows) on transthoracic echocardiography.

    Conclusion: While PFEs are often asymptomatic and found incidentally, they can also present with embolic manifestations like stroke, myocardial infarction, or sudden death (2). Surgical excision is recommended for larger left-sided PFEs in eligible patients due to the risk of embolization, or during cardiac surgery for another condition. Otherwise, antiplatelet agents may be considered (3).

    Literature

    1. 1.
      Tyebally S, Chen D, Bhattacharyya S, Mughrabi A, Hussain Z, Manisty C, et al. Cardiac Tumors: JACC CardioOncology State-of-the-Art Review. JACC Cardiooncol. 2020 June 16;2(2):293–311.DOI
    2. 2.
      Gopaldas RR, Atluri PV, Blaustein AS, Bakaeen FG, Huh J, Chu D. Papillary fibroelastoma of the aortic valve: operative approaches upon incidental discovery. Tex Heart Inst J. 2009;36(2):160–3.PubMed
    3. 3.
      Devanabanda AR, Lee LS. Papillary Fibroelastoma. 2023 Apr 26. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025 Jan–.