Alexander E , Masoor , and Daniel: Pulmonic Papillary Fibroelastoma: Cause of Recurrent Pulmonary Emboli and Treatment.

Cardiac papillary fibroelastomas are the third most common primary cardiac tumor after atrial myxomas and lipomas. The overall incidence of primary cardiac tumors is approximately 0.02%1. The incidence of pulmonary valve papillary fibroelastoma is estimated to be less than 0.03% by autopsy and 0.019% by clinical series2. These tumors typically involve the left-sided heart valves; aortic valve (44.5%) and mitral valve (36.4%). The pulmonary valve is the least common location (8%)3. To date, only one other case report has documented pulmonary embolism from a pulmonary valve papillary fibroelastoma3. We present an unusual case of a papillary fibroelastoma serving as the source of multiple pulmonary emboli presenting as severe acute respiratory distress syndrome.

Figure 1:

Transesophageal echocardiography reveals a mass with a long stalk in the right ventricular outflow tract crossing the pulmonary valve.

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A 74 year old white male presented with increasing shortness of breath of four days. He denied chest pain, orthopnea, paroxysmal nocturnal dyspnea, palpitations, fever, chills, change in weight, and edema. He quit smoking 20 years ago. He was diagnosed with severe acute respiratory distress syndrome, acute on chronic kidney injury, acute liver injury, and non-ST elevation myocardial infarction (NSTEMI).

Physical exam revealed an afebrile patient with heart rate 112, blood pressure 144/82 mmHg, respiratory rate 22, oxygen saturation 60% on room air, improving to 70% on 100% FiO2 via CPAP mask. Arterial blood gas revealed pH 7.35, pCO2 34 mmHg, pO2 21 mmHg, and a bicarbonate level of 18.8 mmol/L. Lactic acid was 9.5 mmol/L. He continued to deteriorate and was intubated. Cardiac exam had a regular rhythm with a gallop noted but no murmurs or jugular venous distention. Peripheral pulses were 1+ bilaterally and extremities were warm. Scattered wheezes were heard throughout lung fields.

Leukocytosis of 16,420 cells/µL with elevated neutrophil count with 20% bandemia was seen on laboratory testing. Blood and sputum cultures were negative. BUN and creatinine were 19 and 2.1, respectively. Liver enzymes were also elevated with AST 463 and ALT 346. Troponin at time of admission was 0.98 ng/ mL but trended up to 5.68 ng/mL. BNP was 405 pg/mL. D-dimer was elevated at 574 ng/mL. EKG showed lateral lead ST segment depression. Chest x-ray findings included multilobar interstitial opacities concerning for pneumonia.

Two days after admission, the patient was stable enough to travel to radiology for a computed tomography scan of the chest with contrast, which revealed diffuse ground glass opacities and bilateral subsegmental pulmonary emboli involving the right and left upper lobes. The right ventricular outlet at the area of the pulmonic valve showed a filling defect. Lower extremity venous ultrasound did not reveal evidence of deep vein or superficial thrombus. Transesophageal echocardiography found a single, mobile, filamentous, echogenic mass in the right ventricular outflow tract crossing the pulmonic valve, measuring about 5 cm with a stalk of approximately 10 mm (Figure 1).

The patient was started on heparin to prevent further thrombus formation on the surface of the tumor and embolization. However, he was diagnosed with heparin-induced thrombocytopenia when his platelet count decreased, so he was switched to argatroban. He remained asymptomatic during the remainder of his admission. He was discharged home on lifetime anticoagulation with warfarin and follow up was arranged to re-evaluate potential surgical needs when his functional status improved. At the two month follow up appointment, he remained asymptomatic without need for further intervention.

The most common presentation from papillary fibroelastoma of all locations are stroke or transient ischemic attack (19.6%), angina (8.0%), myocardial infarction (4.6%), heart failure (4.0%), sudden death (3.4%), presyncope or syncope (2.0%), blindness (1.1%), pulmonary embolism (0.5%), peripheral emboli (0.5%), mesenteric ischemia (0.3%), and renal infarction (0.2%)1. Of those involving the pulmonary valve, patients commonly present with dyspnea on exertion (34.6%), atypical chest pain (11.5%), and intermittent palpitations (7.6%). Other symptoms include cough, fever, transient blindness, syncope, weakness, anorexia, and tingling and numbness of the hands3.

Characteristic findings on echocardiography for papillary fibroelastomas include: a) the tumor is round, oval, or irregular in appearance, with well-demarcated borders and a homogenous texture; b) most cardiac papillary fibroelastomas are small (99% were <20 mm in the largest dimension); c) nearly half had small stalks, and those with stalks were mobile; and d) they are usually single (91%) but may be multiple and are most often associated with cardiac valvular disease6. Hakim et al. found that transesophageal echocardiography detected 100% of cases involving the pulmonary valve while transthoracic echocardiography only detected 90%. The average size of pulmonary valve papillary fibroelastoma was 1.36 x 1.30 cm (range 0.1-2.8 cm)3. Most have a stalk 1-3 mm in length6.

This tumor is larger with longer stalk than average. Bacterial and Libman Sack’s endocarditis are less likely given negative blood cultures and anti-nuclear antibody. The presence of a round, mobile, pedunculated lesion attached to the leaflet of the pulmonary valve confirms the presence of tumor-like lesion instead of infection or debris9. Libman Sack’s endocarditis rarely presents on the right heart and has no stalk. Lambl’s excrescences are multiple in greater than 90% of cases6. Echocardiography findings and lack of support for other diagnoses make papillary fibroelastoma the most likely diagnosis.

Consensus from literature review recommends surgical excision1,3,6,8, but anticoagulation was used given the patient’s poor functional status. Since papillary fibroelastomas have the potential to cause thrombus formation8, anticoagulation will reduce clot formation and embolization. To our knowledge, this is the first case in which a patient with pulmonary valve papillary fibroelastoma has been managed with anticoagulation alone. Two other case reports note successful management with anticoagulation over a four month interval for a left atrial appendage papillary fibroelastoma9 and a four year period for a left ventricular papillary fibroelastoma10.

Statement of ethical publishing

The authors agree to abide by the requirements of the “Statement of publishing ethics of the International Cardiovascular Forum Journal.”11

Conflict of interest:

The authors declare there is no conflict of interest.

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Natarajan A, Tan S, Patel HN et al Mass in the left atrial appendage: A therapeutic dilemma. Canadian Journal of Cardiology 2013; 29: 1329.e13–1329.e15 10.1016/j.cjca.2012.05.019

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Mutlu H, Demir IE, Leppo J et al Nonsurgical management of a left ventricular pedunculated papillary fibroelastoma: A case report. Journal of the American Society of Echocardiography 2013; 21: 877.e4-877.e7 10.1016/j.echo.2007.10.001

11. 

Shewan LG, Coats AJS, Henein M Requirements for ethical publishing in biomedical journals. International Cardiovascular Forum Journal 2015; 2: 2 10.17987/icfj.v2i1.4



Copyright (c) 2015 Alexander Eugene Urban, Masoor Kamalesh, Daniel Gutteridge

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