Cardiac Papillary Fibroelastoma: An Echocardiographic Surprise

AFFILIATIONS:
¹Department of Internal Medicine, Saint Peter’s University Hospital, New Brunswick, NJ
²Cardiometabolic Institute, Somerset, NJ

CITATION:
Shah M, Alarmanazi F, Shah C. Cardias papillary fibroelastoma: an echocardiographic surprise. Consultant. 2023;63(3):e7. doi:10.25270/con.2023.01.000005

Received April 13, 2022. Accepted October 28, 2022. Published online January 18, 2023.

DISCLOSURES:
The authors report no relevant financial relationships.

ACKNOWLEDGEMENTS
None.

CORRESPONDENCE:
Monarch Shah, Department of Internal Medicine, Saint Peter’s University Hospital, 1050 George Street, Apartment 4i, New Brunswick, NJ (monarch.shah08@gmail.com)

Introduction. Cardiac papillary fibroelastoma is a rare benign endocardial papilloma which may have a myriad of clinical presentations including chest pain, palpitations, and cerebrovascular accidents. Surgical excision is an effective treatment option, with a high likelihood of valve preservation and a low recurrence rate. Patients not undergoing surgery are at risk of systemic thromboembolism, particularly cerebral and retinal thromboembolisms.

Case presentation. A 74-year-old woman with a history of hypertension was seen in an outpatient office with complaints of intermittent palpitations with associated headaches and dizziness for 1 week duration. Vital signs were within normal limits except for a heart rate of 110 beats/min. The rest of the physical examination and biochemical investigations were within normal limits. An electrocardiogram showed sinus tachycardia. A transthoracic echocardiogram showed an ejection fraction of 65%, moderate mitral regurgitation, and mild aortic regurgitation with an echo density over the aortic valve. Transesophageal echocardiography was done for evaluation of the aortic valve. A papillary fibroelastoma was seen on the non-coronary cusp of the aortic valve (Figures 1 & 2). It was strand-like measuring approximately 1.5 cm in the largest dimension. Surgical excision was deferred by the patient and she was prescribed acetylsalicylic acid 81 mg once daily.

Figure 1. Transesophageal echocardiography mid esophageal long axis view showing a papillary fibroelastoma on the non-coronary cusp of the aortic valve. (LA- Left Atrium, LV- Left Ventricle, AO- Aorta, RVOT-Right Ventricle Outflow Tract, PFE- Papillary Fibroelastoma)

Figure 2. Transesophageal echocardiography mid esophageal aortic valve short axis view showing a papillary fibroelastoma on the non-coronary cusp of the aortic valve. (LA- Left Atrium, RA- Right Atrium, AO- Aorta, RVOT-Right Ventricle Outflow Tract, PFE- Papillary Fibroelastoma)
 

Patient outcome. The patient is being followed up at the cardiology outpatient office every 6 months and serial EKGs during the follow-up visits have shown no new arrhythmias. Holter monitoring has also shown no arrhythmias. The patient has not had any cerebrovascular events to date since the diagnosis was made. She continues to remain asymptomatic and is on acetylsalicylic acid 81 mg once daily.

Discussion. Cerebrovascular accidents (CVAs) are one of the leading causes of mortality worldwide, according to the World Health Organization.¹ One of the causes of CVAs is papillary fibroelastoma (PFE). PFEs are considered the second most common type of benign primary cardiac tumor following myxoma and the most common valvular tumors which mostly affects people in their 6th or 8th decade of life. When left untreated, PFEs account for a 6% risk of CVA at 1 year and 13% at 5 years.²

When examined grossly, PFEs resemble sea anemones with a frond-like appearance. Histologically, the tumor appears to be covered by endothelium, followed by a layer of acid mucopolysaccharide, with an inner vascular core of connective tissue matrix that contains variable amounts of smooth muscles, collagen, cells, and elastic fibers. On echocardiography, these tumors appear to be homogenous, well-demarcated oval, round, or irregular masses. The size of these tumors varies from 2 mm to 28 mm, with 43.6% of tumors having a stalk that ranges from 1 mm to 3 mm in length.³

While they can affect any valve, the most commonly affected valves are the aortic valve with a prevalence of 35% to 63%, the mitral valve with a prevalence of 9% to 35%, and the tricuspid and pulmonary valves with a prevalence of 6% to 15% and 0.5% to 8%, respectively.⁴

Many patients affected by PFEs are asymptomatic and they are usually diagnosed incidentally during echocardiography for other reasons, cardiac surgery, or autopsies. However, with the increased information available on PFEs, improved technology with higher resolution, and increased use of echocardiography, there has been an increase in the diagnosis of PFEs and more indications for testing and treatment.⁴

Multiple case reports have been published attributing severe cardiac and neurological manifestations to PFEs such as; syncope, transient ischemic attack, blindness, angina, myocardial infarction, strokes, valvular dysfunction, ventricular fibrillation, heart failure, and sudden deaths.⁴ Some of these symptoms are attributed to the possibility of thrombus formation on the tumor surface or embolization of a tumor fragment to distant sites. This can cause cerebral, pulmonary, and retinal artery emboli. When present on the aortic valve specifically, PEFs can cause sporadic obstruction of the right coronary ostium, which may lead to recurrent myocardial infarctions resulting in heart failure.⁵

Even though PFEs are considered histologically benign, due to the potentially life-threatening complications they should be considered in elderly patients and appropriate testing should be done. The preferred diagnosis method for PFEs is transesophageal echocardiography due to its high resolution. Depending on the location PFEs may also be visualized using transthoracic echocardiography. Recently, 3-D echocardiography, magnetic resonance imaging, and multi-slice spiral computed tomography were also used for diagnosis.⁶

Treatment options depend on the symptoms and tumor mobility. They include either administration of anticoagulation and antiplatelet agents or surgical resection. The most effective treatment is surgical resection to prevent cardiovascular or embolic events. However, there’s some controversy regarding the treatment of asymptomatic patients and whether medical management alone can be effective without surgical intervention. Some studies showed increased incidents of cerebrovascular accidents and mortality rates in patients who had an echocardiographically diagnosed PFEs but refused surgical removal. There is limited data on the effectiveness of anticoagulation and antiplatelet agents alone.⁷

PFEs cannot be stratified into high-risk or low-risk using echocardiographic findings. Current data supports safe excision and the possibility to preserve native valves, thus PFE removal surgery is highly recommended. There are very low incidents of recurrence of PFEs at the same site, with the exception of if there were some tumor cells left behind, with other recurrences being new at different sites. The total recurrence rate was found to be 1.6%.²

Recommendations for surgery must consider the risks to benefits ratio, especially with elderly patients. This can be influenced by comorbid conditions and bleeding or embolic risks. Patients that are at be at low-risk for surgery (those with a less than 1% score using the Society of Thoracic Surgeons scale) are highly recommended to undergo surgical excision of PFEs. This recommendation is supported by decreased PFE complications risks after excision and the possibility to preserve valve function in 98% of patients. If patients are not good surgical candidates or refuse to undergo surgery, we recommend treatment with long-term antiplatelet agents and following up regularly with a cardiologist.²