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American Journal of Public Health Research
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The Implementation of a Tele-Homecare System with Service Oriented Architecture and HL7 Message Transmission Standard
Ching-Sung Wang

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The Influence of Ethanol on Pyruvate Kinases Activity in Vivo, in Vitro, in Silico
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American Journal of Medical Sciences and Medicine
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Clinical and Histological Features in Wegener Granulomatosis
Li Cavoli Gioacchino, Passantino Rita, Bono Luisa, Tortorici Calogera, Ferrantelli Angelo, Giammarresi Carlo, Rotolo Ugo

American Journal of Infectious Diseases and Microbiology
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The Curse of Lymphatic Filariasis: Would the Continual Use of Diethylcarbamazine Eliminate this Scourge in Papua New Guinea?
Anne Lanham, Lillian Mwanri

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Mystery of the Right Sided Heart Failure in a Young Patient

Gaurav Khandelwal*, Manish Ruhela, Rajeev Bagarhatta

Senior Resident, Department of Cardiology, SMS Medical College and Hospital, Jaipur, Rajasthan, India

Abstract: A 45 yr old female presented with clinical features of right side heart failure. 2D Echocardiography examination showed a homogenous, irregular, fimbriated mass attached to pulmonary valve, causing right ventricular outflow obstruction, with severe tricuspid regurgitation. To the best of our knowledge, this is the first case report of pulmonary valve mass, possible papillary fibroelastoma, which is large enough to cause right ventricular outflow obstruction, leading to severe tricuspid regurgitation and right sided heart failure.

Keywords: pulmonary valve, mass, right sided heart failure

Received April 14, 2014; Revised May 16, 2014; Accepted May 19, 2014

Citation: Gaurav Khandelwal, Manish Ruhela, Rajeev Bagarhatta, Mystery of the Right Sided Heart Failure in a Young Patient. American Journal of Medical Case Reports, 2 (5). 94-96. January 2014. doi:10.12691/ajmcr-2-5-1

1. Introduction

Pulmonary valve mass (fibroelastoma) is a rare cardiac tumour and it may cause right ventricular outflow obstruction, when sufficiently large in size. Many case reports in literature describe various masses on pulmonary valve, but this case report is unique because here we describe a large mass on pulmonary valve causing right sided heart failure due to right ventricular outflow obstruction, which was never described in the past.

2. Case Report

Figure 1. 2D Echocardiogram modified parasternal long axis view, showing mass attached to pulmonary valve causing right ventricular outflow obstruction

Figure 2. 2D Echocardiogram apical view with color Doppler showing severe tricuspid regurgitation, with continuous wave Doppler showing pressure gradient of 54 mm Hg across tricuspid valve

A 45 year old female presented with complaints of gradually progressive dyspnea on exertion for 6 months, abdominal distention and decreased appetite for 1 month. On examination, she had JVP up to 8 cm of blood column above angle of Louis, hepatomegaly up to 8 cm below sub costal margin, and free fluid in abdomen, single S2 (A2), ejection systolic murmur in left 2nd parasternal area and pansystolic murmur in left lower parasternal area. No other cardiac risk factors or significant past medical history were present. Haemogram and blood biochemistry were in normal limit. Ultrasound examination of abdomen showed congestive hepatomegaly. 2D Echocardiography showed a homogenous, irregular, fimbriated mass measuring 33 x 20 mm in RV outflow, attached to pulmonary valve, causing RV outflow obstruction (Figure 1). There was severe tricuspid regurgitation with RVSP = RAP + 54 mm Hg (Figure 2). RA and RV size were dilated and inferior vena cava (IVC) was dilated and noncollapsible. Patient was treated with diuretics and stabilized and was advised for further evaluation, but she refused. Patient was discharged on medical management.

3. Discussion

Differentials for the mass may be papillary fibroelastoma, cardiac myxoma, vegetation, nonbacterial thrombotic endocarditis (NBTE) or organized thrombus.

Three out of four valvular tumors are papillary fibroelastomas. Papillary fibroelastomas are the second most common form of cardiac tumor, and more common in elderly. A valvular location is seen in 86% of cases – about 8 % are found on pulmonary valve [1,2]. The lesions are single in 91% of the cases, the average size is about 1 cm in diameter, and it can range from 0.2 to 4.6 cm. [1] The irregular,well demarcated, homogenous and fimbriated appearance of the mass on echocardiography in this case favours papillary fibroelastoma.

Myxomas comprise 50% of all cardiac tumors. Myxomas mostly manifest in between the fourth and seventh decade, with female predominance. 83% of cardiac myxomas occur in the left atrium, occurrence in the ventricles is uncommon; only 0.6% of myxomas occur in the right ventricle.[3] Rarely, cardiac myxoma can involve heart valves directly[3.4]. Most of them have a smooth compact surface, and are pedunculated with a fibromuscular stalk. Cardiac myxoma is a less possibility in this case as the mass is irregular and fimbriated, it is directly attached to pulmonary valve; and pulmonary valve is very rare site for myxoma [5].

There was no predisposition for vegetation, like intravenous drug abuse or right sided catheterization in this case; there was also any history of fever. The pulmonary valve is least involved in patients with infective endocarditis and pulmonary regurgitation is often found. In NBTE, the vegetations are dense, smaller than 1.0 cm, broad based, and irregular in shape. NBTE has been reported in patients with advanced-stage malignancy, hematologic disorders, connective tissue disease, SLE, AIDS, hypercoagulable-state disorders & trauma from indwelling pulmonary or central venous catheter. No such condition was present in this patient.

There was no hypercoaguable condition or DVT in this patient, which could have lead to organized thrombus on the pulmonary valve.

The description of the mass and its location to ventricular aspect of pulmonary valve goes in favour of papillary fibroelastoma.

This is a rare case of a large mass on pulmonary valve, possibly papillary fibroelastoma causing RVOT obstruction leading to hypertensive severe tricuspid regurgitation.

To the best of our knowledge, about 50 cases of pulmonary valve papillary fibroelastoma have been described to date [1,2,6,7,8]. But most of them are small and do not disrupt the valve function, most of them have been found incidently at autopsy, and few as incidental finding in echocardiography examination [2,6].

4. Conclusion

Posiibility of pulmonary valve mass should be considered in a patient with right side heart failure without any risk factors. This is probably the first case report of pulmonary valve mass described, possibly papillary fibroelastoma which is which is large enough to cause RVOT obstruction leading to hypertensive severe tricuspid regurgitation and right sided failure.

Statement of Competing Interests

Authors have no competing interests.

References

[1]   | Sun JP, Asher CR, Yang XS, et al: Clinical and echocardiographic characteristics of papillary fibroelastomas: A retrospective and prospective study in 162 patients. Circulation 2001; 103: 2687.
[2]   | Gowda RM, Khan IA, Nair CK, Mehta NJ, Vasavada BC, Sacchi TJ. Cardiac papillary fibroelastoma: a comprehensive analysis of 725 cases. Am Heart J 2003; 146(3): 404-410.
[3]   | Burke A, Virmani R: Tumors of the Heart and Great Vessels. Atlas of Tumor Pathology. 3rd Series, Fascicle 16Washington, DC, Armed Forces Institute of Pathology, 1996.
[4]   | Kuon E, Kreplin M, Weiss W, et al: The challenge presented by right atrial myxoma. Herz 2004; 29: 702.
[5]   | Hekmat M, Rafieian S, Aval ZA, Khani M, Taherkhani M, Movahed MR. Asymptomatic pediatric pulmonic valve myxoma involving the right ventricular outflow tract: a case report and review of the literature. J Heart Valve Dis. 2012 May; 21(3): 398-400.
[6]   | Bhagwandien NS, Shah N, Costello JM Jr, Gilbert CL, Blankenship JC. Echocardiographic detection of pulmonary valve papillary fibroelastoma. J Cardiovasc Surg (Torino). 1998 Jun; 39(3): 351-4.
[7]   | Park MY, Shin JS, Park HR, Lim HE, Ahn JC, Song WH. Papillary fibroelastoma of the pulmonary valve. Heart Vessels. 2007 Jul; 22(4): 284-6.
[8]   | Costa MJ, Makaryus AN, Rosman DR. A rare case of a cardiac papillary fibroelastoma of the pulmonary valve diagnosed by echocardiography. Int J Cardiovasc Imaging. 2006 Apr; 22(2): 199-203.