Primary cardiac tumors are very rare, and PFEs are probably the most common type of primary cardiac tumor [40],Approximately 6%-15% of the PFEs are located on the tricuspid valve[19]. Most tricuspid valve papillary fibroelastoma are asymptomatic and are almost detected by examination.However, its clinical manifestations can show severe symptoms of pulmonary embolism[26], characterized by sudden dyspnea and chest discomfort. Those with tricuspid valve obstructive symptoms may cause lower limb edema and hepatic stasis. There are also arrhythmias that cause palpitations, vertigo or syncope [41].The case we reported also presented with nonspecific symptoms of dyspnea as the main complaint, but no pulmonary embolism was found.
The size of cardiac papillary fibroelastoma varies from 2 to 70 mm, but most tumors are about 10 mm in diameter [42], which is also consistent with the case we reported.
Transthoracic echocardiography is routinely performed for tricuspid valve papillary fibroelastoma. However, when the tumor is smaller than 2 mm the sensitivity and specificity of the diagnosis of transthoracic echocardiography is low, and further transesophageal echocardiography should be performed to evaluate the tumor, transesophageal echocardiography is more sensitive and advantageous, the relationship between the tumor and the valve is seen more clearly, and it is more meaningful for differential diagnosis of the tumor [43].The echocardiographic feature of tricuspid valve papillary fibroelastoma is a well-defined, small, mobile, pedicled mass on the tricuspid valve or tendon cords that can prolapse into the cardiac chambers with the cardiac cycle.In addition, another characteristic feature is the presence of a "speckled or stippled" appearance of peripheral echolucencies in the blood-tumor interface[41].Cardiac computed tomography (CCT) and cardiac magnetic resonance (CMR) are used as secondary imaging modalities for the diagnosis of tricuspid valve papillary fibroelastoma [25].CCT has low resolution, and CMR may provide better resolution and be helpful for diagnosis [44].Tricuspid valve papillary fibroelastoma need to be differentiated from thrombi and valvular vegetations. Generally thrombi rarely occur on valves and are relatively fixed with little mobility.The case we report lacked risk factors for infectious disease, and had a normal white blood cell count, no fever, and blood cultures did not show bacterial growth, so we did not consider the diagnosis of Infective endocarditis.It also needs to be differentiated from myxoma, which generally have larger diameters, significant obstructive symptoms, markedly affect valve function, and a distinctly different appearance [45]. Therefore, we also did not consider myxoma in our case.
A review of the literature, including our case, retrieved a total of 69 reported cases of tricuspid valve papillary fibroelastoma. By analyzing the time of publication of the articles, 33 cases were reported in the last five years, which accounted for approximately half of all patients, suggesting that the incidence of tricuspid valve papillary fibroelastoma may be on the rise in recent years.However, the detail datas could not be accessed in 37 cases (10cases [4], 10cases [9], 6cases [46], 5cases [47], 2cases [48], 1case [49], 2cases [20], 1case [50]).The remaining 32 cases are shown in Table 1.The oldest was 81 years old and the youngest just 1 year old, and the M(Q3-Q1) of age was 63 (71.75–50.25). Male/Female (17/15).In terms of clinical symptoms, 10 cases had no clinical symptoms and were found during examination; 15 cases showed dyspnea, 7 cases had chest pain, 3 cases had fever, 2 cases had syncope, 1 case had dizziness, 1 case had palpitations, and all these symptoms were non-specific, which were not considered to be related to tricuspid valve papillary fibroelastoma.The minimum tumor size was 5X6mm and maximum was 40X35mm, there were no cases exceeding 40mm and one case had a more significant enlargement in a short period of time [37].Tricuspid valve insufficiency was the main comorbidity, and five cases were complicated by pulmonary embolism. Surgical resection plus tricuspid valvuloplasty is the main treatment methods, two cases were resected via cardiac catheterization, which may be a treatment worth recommended, and one patient did not undergo surgery, and the mass did not show any significant change in three years of follow-up observation [21].The number of days of hospitalization for surgical patients was 6.38 ± 3.09 days.There was one recurrence at follow-up, but this patient died of stroke 15 days after surgery [21],but I suspect that this tumor may have been incompletely removed.
Table 1
Clinical Presentations of the Reported Cases and the Current Case
| Reference | Age (years) | Sex | Clinical Finding | Size(mm) or M(Q1-Q3) | Surgery | Comorbidity | Days in hospital | Follow-up(months) |
|---|
| Ali Ahmad et al,[10]2024 | 43 | M | None | 6X5 | TVP,MV repair,PVI,app ligation, Resection | AF | 3 | NR in 1 |
| Kamel I et al,[11]2024 | 60 | M | Syncope | 16X17 | Resection | HTN;head injury | None | NA |
| Ku L et al,[12]2024 | 31 | M | None | 14X12 | Resection | None | 7 | NA |
| Chen D et al,[5]2024 | 55 | M | Chest pain | 19X14 | Resection | None | None | NA |
| Bashir H et al,[13]024 | 81 | M | Chest pain; afebrile | 13X12 | CABG,AVR; Resection | CHD,HTN | None | NA |
| Wang Y et al,[14]2023 | 73 | M | None | 8.4X6 | TVP Resection | None | 8 | NR in 15 |
| Actis Dato GM et al,[15]2023 | 69 | F | None | 20X10 | Resection | None | 6 | NA |
| Phan TQ et al, [16]2023 | 17 | F | Dyspnea | 8.7X9.6 | Resection | None | 3 | NR in 6 |
| Zhang RS et al, [17]2023 | 60 | F | None | 15X11 | Resection by cardiac catheterization | DM; HTN;TR,ischemic stroke | NA | NA |
| Fang L et al,[18]2022 | 51 | M | Dyspnea | NA | TVR, Resection, | TR | NA | NA |
| Rana Y et al, [19]2021 | 66 | M | Dyspnea | 17X13 | Resection, PFO closure | HIV,DM,HTN,hyperlipidemia | 3 | NR |
| Kashiwagi Y et al, [20]2021 | 62 | F | Palpitation | 23X17 | Resection | RBBB,PE | NA | NA |
| Kavalerchyk V et al,[21]2018 | 74 | M | Chest pain, dyspnea | 10X8 | Conservative treatment | CHD | - | No change in 36 |
| Artunduaga M et al,[22]2017 | 1 | F | None | 15X15 | TVP; Resection | None | 6 | NA |
| Rohani A et al,[23]2017 | 64 | f | Chest pain | 9X6 | Resection | TR | NA | NR in 6 |
| Gollol-Raju NS et al,[24]2016 | 71 | M | Dyspnea | 11X9.4 | Resection by cardiac catheterization | Non-ischemic cardiomyopathy;HTN; | NA | NR in 1 |
| Li W et al,[25]2016 | 75 | M | None | 20X15 | TVP, Resection | CHD,HTN,ischemic stroke | 7 | NR in 6 |
| Choi KB t al,[26]2016 | 72 | M | Dyspnea, afebrile | 13X16 | TVP; Resection | None | NA | NR |
| Shah RA et al,[27]2015 | 65 | M | Dyspnea | 10X10X5 | Resection | TR,PE,PH | NA | NR in 12 |
| Srivatsa SV et al, [28]2013 | 61 | F | Dyspnea | 13X9X11 | Resection | TR,PH | NA | NR in 2 |
| Karimi M et al,[29]2013 | 8 | M | Chest pain dyspnea | 25X15 | TVR; Resection | TR,PE | NA | NR |
| Karapanagiotidis GT et al,[30]2012 | 81 | F | Dyspnea, Dizzy spells | 20X25 | TVP; Resection | TR | 7 | NR in 3 |
| Haron H et al,[31]2012 | 68 | M | Afebrile | 10X10 | MVR;TVP; Resection | DM; HTN;MI;TR | 6 | NR in 12 |
| Massarenti L et al,[32]2009 | 50 | F | None | 16X17 | TVP; Resection | None | 7 | NA |
| Boodhwani M et al,[33]2007 | 38 | M | Dyspnea | 15X20 | TVP; Resection | NA | NA | NA |
| Mastroroberto P et al,[34]2006 | 53 | M | Chest pain; dyspnea | 20X30 | CABG; Resection | CHD,PE | NA | NA |
| Georghiou GP al,[35]2003 | 67 | F | Dyspnea | 25X16 | Resection | None | NA | NR in 4 |
| Nishimura Y et al,[36]1998 | 42 | F | None | 40X35 | TVP; Resection | TR | NA | NR in 12 |
| Neerukonda SK et al,[37]1991 | 75 | F | Dyspnea | 1.5X2.0/ 33X2.0X18 | TVP; Resection | PE | NA | NA |
| Wolfe JT 3rd et al,[38]1991 | 75 | F | None | 27X27 | CABG; Resection | Cerebral ischemic | 15 | Recurrence in 0.5 |
| Frumin H et al,[39]1983 | 64 | F | Chest pain syncope | 15X15 | CABG; Resection | CHD | NA | NA |
| Current study | 53 | F | Dyspnea | 10X9 | Resection | None | 5 | NR in 12 |
| M,male;F,female;TVP,tricuspid valvuloplasty;MV, mitral valve;PVI, pulmonary vein isolation;AF,atrial fibrillation;NR, non-tumor recurrence; NA,not available; HTN, hypertension;CABG, coronary artery bypass grafting; CHD,coronary artery disease;AVR, aortic valve replacement;DM, diabetes mellitus;TR,tricuspid regurgitation;TVR,tricuspid valve replacement;HIV,human immunodeficiency virus;PFO,patent foramen ovale;PE,pulmonary embolism;RBBB,right bundle-branch block;PH:pulmonary arterial hypertension;MI,mitral insufficiency;MVR, mitral valve replacement |
There is a lack of standardized treatment protocols for the treatment of tricuspid valve papillary fibroelastoma, and the treatment experience remains limited [20].Surgical treatment is also controversial. Some authors suggest that surgical resection remains the primary treatment method unless there are contraindications to surgery[51],Some authors have also considered the possibility of embolic complications and recommended surgical resection regardless of the size of the mass [15].However, some authors have suggested that conservative treatment is usually performed unless there are significant obstructive symptoms or a greater risk of embolization [11].Antiplatelet therapy may be considered if due to high surgical risk. However, the relevant evidence is limited[43].In our case we gave tumor resection considering its high activity and risk of embolization.If the valve is damaged during removal of the mass due to the large pedicle of the mass, or if the tumor involves the valve, the affected valve needs to be removed to prevent recurrence of the tumor, and tricuspid valvuloplasty should be performed to correct the blood flow abnormality.In our case, the pedicle of the mass was small and the mass was easily resected without damaging the tricuspid valve leaflets, thus the tricuspid valve did not require valvuloplasty.
Since tricuspid valve elastofibroma is a benign tumor and has a good prognosis after resection, postoperative recurrence is rare [42].Another case of papillary fibroelastoma of the mitral valve was reported in the literature as a recurrence 9 years after resection of a tricuspid valve papillary fibroelastoma [52]. However, no recent recurrence cases have been reported in the literature.Our case was followed up for a year and there was no evidence of recurrence either.