Papillary Fibroelastoma

Introduction

Papillary fibroelastoma (PFE) is a rare, benign cardiac tumor that primarily affects the heart valves. Despite its non-cancerous nature, it can have serious clinical consequences due to its potential to cause embolic events.

Causes and Risk Factors

The exact cause of PFE is unknown. However, it is thought to result from: - Endothelial damage leading to an overgrowth of fibroelastic tissue. - Aging – it is more common in older adults. - Previous cardiac surgery or inflammatory processes may also play a role.

Symptoms

PFE is often asymptomatic and discovered incidentally. However, when symptoms occur, they are usually due to embolization and may include: - Stroke or transient ischemic attack (TIA). - Coronary embolism, which can mimic a heart attack. - Valvular dysfunction, leading to heart murmurs or heart failure symptoms. - Sudden cardiac death (rare).

Diagnosis

PFE is typically diagnosed through imaging studies such as: - Echocardiography (Transthoracic or Transesophageal) – the gold standard for visualizing the tumor. - Cardiac MRI or CT scan – used for further structural assessment.

Treatment

Treatment depends on the size, location, and symptoms: - Asymptomatic small PFE: Regular monitoring with echocardiography. - Symptomatic or large PFE: Surgical removal (valve-sparing resection if possible) to prevent embolic complications. - Anticoagulation therapy may be considered in patients at high risk of embolization who cannot undergo surgery.

Prognosis

After surgical excision, the prognosis is excellent, with a very low recurrence rate. Regular follow-up is advised to monitor for possible complications.

Conclusion

PFE, though benign, should not be ignored due to its potential for serious complications. Early diagnosis and appropriate management can prevent life-threatening outcomes.

Source recommendations

1. American Heart Association Guidelines on Management of Cardiac Tumors

  1. https://www.ahajournals.org/doi/10.1161/JAHA.120.016032
  2. https://www.heart.org/
  3. https://www.ahajournals.org/doi/10.1161/CIR.0000000000000679
  4. https://www.heart.org/en/news/2019/02/25/the-heart-isnt-particularly-vulnerable-to-cancer-and-heres-why
  5. https://professional.heart.org/en/

2. European Society of Cardiology Guidelines on Cardiac Masses

  1. https://www.escardio.org/Guidelines/Clinical-Practice-Guidelines/Cardio-oncology-guidelines
  2. https://www.jacc.org/doi/abs/10.1016/j.jaccao.2020.05.009
  3. https://www.escardio.org/static-file/Escardio/Education-Subspecialty/Courses/EACVI/EACVI%20preparatory%20course%20to%20cerfication%20in%20CMR/Cardiac%20masses%20and%20tumors_C.%20Bucciarelli-Ducci_on_behalf_of_L.Natale%20.pdf
  4. https://academic.oup.com/ehjcimaging/article/24/12/1605/7256773
  5. https://www.escardio.org/Journals/E-Journal-of-Cardiology-Practice/Volume-4/vol4n17-Title-MRI-for-the-diagnosis-of-cardiac-tumors

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