Abstract

SUMMARY
 A 5-day old female child with complaints of tachypnea, had TTE which revealed an echogenic mass on septal leaflet of Tricuspid valve. Based on echocardiographic features, it was diagnosed as Papillary fibroelastoma.
 CASE DESCRIPTION
 A 5-day old female child was referred for transthoracic echocardiographic study due to respiratory difficulty. The child was afebrile and had a respiratory rate of 60/min, heart rate 130 bpm, and blood pressure 80/50 mmHg. No murmur or added sound heard on CVS examination. TTE revealed a dense mass (Figure 1) attached to right atrial side of septal leaflet of Tricuspid valve by a small pedicle. The mass was mobile, prolapsing into right ventricle during diastole (Clips) without causing obstruction with size of 0.8 X 0.74 cm. Intracardiac masses attached to valves are usually Papillary fibroelastomas. Other possible differential diagnoses could be myxoma, vegetation, Lambl’s excrescence or secondary tumors etc. Based on the location and echo features of the mass (mobility, attachment to downstream side of valve, and frond like speckled appearance) the diagnosis of Papillary fibroelastoma was made.
 LEARNING POINTS
 
 Cardiac tumors are rare and mostly benign.
 The three most common primary tumors of heart, in order of frequency, are myxomas, lipomas and papillary fibroelastomas respectively. PFE constitute 7% - 9% of all.1
 PFE usually arise from valves of the heart and are mostly left-sided.2
 They are mobile, range from 0.5 – 2 cm in size and have a frond-like appearance. On echo, they are dense with a speckled interior and shimmering surface.3
 Being prone to embolization and thrombosis, their clinical course may not be benign, especially left sided.
 
 Clips
 
 Subcostal Clip: https://youtu.be/_xTZmx--WcA
 PS SAX View: https://youtu.be/lLh6D9Vn4Fw
 A4C View: https://youtu.be/_xZZvTIh0S8
 A4C RV Directed View: https://youtu.be/vJRyHrevftA
 
 QUESTIONS WITH ANSWERS
 
 Question 1: What is the most common complication of papillary fibroelastoma?
 Question 2: The behavior of right and left sided papillary fibroelastomas could be markedly different, Y/N?
 Question 3: What is the most important feature of papillary fibroelastoma for embolization?
 Question 4: PFE preferentially affects females, Y/N?
 
 Answers
 
 Question 1: Cerebral embolism
 Question 2: Yes
 Question 3: Mobility
 Question 4: No
 
 References
 
 Bouhzam N, Kurtz B, Doguet F, Eltchaninoff H, Bauer F. Incidental papillary fibroelastoma multimodal: imaging and surgical decisions in 2 patients. Tex Heart Inst J. 2012;39(5):731-5.
 Eidem BW, Cetta F, O’ Leary PW. Echocardiography in pediatric and adult congenital heart disease. Lippincott, Williams and Wilkins, Wolters Kluwer. 2010 p 355-361.
 Klarich KW, Enriquez-Sarano M, Gura GM, Edwards WD, Tajik AJ, Seward JB. Papillary fibroelastoma: echocardiographic characteristics for diagnosis and pathologic correlation. J Am Coll Cardiol. 1997;30(3):784-90.

Highlights

  • A 5-day old female child was referred for transthoracic echocardiographic study due to respiratory difficulty

  • PFE usually arise from valves of the heart and are mostly left-sided.[2 4]

  • They are mobile, range from 0.5 – 2 cm in size and have a frond-like appearance. They are dense with a speckled interior and shimmering surface.[3 5]

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Summary

CASE DESCRIPTION

A 5-day old female child was referred for transthoracic echocardiographic study due to respiratory difficulty. The child was afebrile and had a respiratory rate of 60/min, heart rate 130 bpm, and blood pressure 80/50 mmHg. No murmur or added sound heard on CVS examination. TTE revealed a dense mass (Figure 1) attached to right atrial side of septal leaflet of Tricuspid valve by a small pedicle. The mass was mobile, prolapsing into right ventricle during diastole (Clips) without causing obstruction with size of 0.8 X 0.74 cm. Intracardiac masses attached to valves are usually Papillary fibroelastomas. Other possible differential diagnoses could be myxoma, vegetation, Lambl’s excrescence or secondary tumors etc. Based on the location and echo features of the mass (mobility, attachment to downstream side of valve, and frond like speckled appearance) the diagnosis of Papillary fibroelastoma was made

LEARNING POINTS
QUESTIONS WITH ANSWERS
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