Papillary Fibroelastoma

Introduction

Papillary fibroelastoma (PFE) is a rare, benign cardiac tumor that arises from the endocardium, the inner lining of the heart. It is most commonly found on heart valves and is often discovered incidentally during echocardiography or cardiac surgery.

Causes and Risk Factors

The exact cause of papillary fibroelastoma is unknown. However, some factors may contribute to its development: - Congenital origin – Some cases are thought to be congenital (present at birth). - Degenerative or inflammatory changes – Chronic inflammation or repetitive trauma to the endocardium may lead to tumor formation. - History of cardiac surgery or radiotherapy – Some cases are associated with prior surgical interventions or radiation exposure.

Symptoms

Many patients with PFE are asymptomatic. However, in symptomatic individuals, the tumor can cause: - Embolic events (stroke, transient ischemic attack, myocardial infarction) – Tumor fragments or thrombi can dislodge and block blood vessels. - Valve dysfunction – Large PFEs can interfere with valve motion and cause regurgitation (backflow of blood) or obstruction. - Chest pain or shortness of breath – Rarely, PFEs can mimic symptoms of other cardiovascular conditions.

Diagnosis

The primary diagnostic tool for detecting PFE is echocardiography, particularly transesophageal echocardiography (TEE), which provides a more detailed view of cardiac structures. Other imaging modalities may include: - Cardiac MRI – Helps differentiate PFE from other cardiac masses. - CT Angiography – Useful in assessing embolic complications.

Treatment

The approach to treatment depends on symptoms and the risk of complications: - Surgical removal (valve-sparing excision) is recommended for symptomatic PFEs or tumors with high embolic risk. - Anticoagulation therapy may be considered in patients at risk of embolization but who are not surgical candidates. - Close monitoring with serial echocardiography is an option for small, asymptomatic tumors.

Prognosis

The prognosis after surgical resection is excellent, with a low recurrence rate. However, untreated PFEs pose a risk of thromboembolism, emphasizing the importance of appropriate management.

Conclusion

Papillary fibroelastoma is a rare but potentially serious cardiac tumor. While often asymptomatic, it can present with embolic events or valve dysfunction. Echocardiography plays a central role in diagnosis, and surgical excision is the treatment of choice for symptomatic cases. Regular follow-up is essential for monitoring small, asymptomatic PFEs.

Source recommendations

1. American College of Cardiology/American Heart Association (ACC/AHA) Guidelines on the Management of Cardiac Tumors

1. https://www.acc.org/
2. https://www.ahajournals.org/doi/10.1161/CIR.0000000000001193
3. https://www.acc.org/Guidelines
4. https://www.ahajournals.org/doi/10.1161/CIR.0000000000001168
5. https://tools.acc.org/ascvd-risk-estimator-plus/

2. European Society of Cardiology (ESC) Guidelines on Cardiovascular Imaging

1. https://www.escardio.org/Sub-specialty-communities/European-Association-of-Cardiovascular-Imaging-(EACVI)
2. https://academic.oup.com/ehjcimaging
3. https://www.escardio.org/Guidelines/Scientific-Documents/Recommendations-and-position-papers
4. https://www.jacc.org/doi/10.1016/j.jcmg.2022.06.018
5. https://www.escardio.org/Guidelines/Recommended-Reading/Cardiovascular-Imaging