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Multimodality imaging of a subtotally obstructive right ventricular myxoma in an asymptomatic 10-year-old girl.
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MedLine Citation:
PMID:  24987268     Owner:  NLM     Status:  PubMed-not-MEDLINE    
Abstract/OtherAbstract:
A 10-year-old girl presented with a supraventricular tachycardia. A heart murmur was detected during a clinical examination; therefore, echocardiography was performed. This revealed a giant right ventricular myxoma that subtotally obstructed the right ventricular outflow tract. A myxoma in the right ventricle is uncommon in children. This rare intracardiac tumor was examined using multimodality imaging, and the patient underwent surgical resection. The pathologic features confirmed our suspicion and revealed an encapsulated tumor with myxomatoid stroma containing focal hemorrhages.
Authors:
Gesa Wiegand; Ludger Sieverding; Ulrich Kramer; Susanne Haen; Michael Hofbeck
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Annals of pediatric cardiology     Volume:  7     ISSN:  0974-2069     ISO Abbreviation:  Ann Pediatr Cardiol     Publication Date:  2014 May 
Date Detail:
Created Date:  2014-07-02     Completed Date:  2014-07-02     Revised Date:  2014-07-07    
Medline Journal Info:
Nlm Unique ID:  101495459     Medline TA:  Ann Pediatr Cardiol     Country:  India    
Other Details:
Languages:  eng     Pagination:  158-9     Citation Subset:  -    
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Full Text
Journal Information
Journal ID (nlm-ta): Ann Pediatr Cardiol
Journal ID (iso-abbrev): Ann Pediatr Cardiol
Journal ID (publisher-id): APC
ISSN: 0974-2069
ISSN: 0974-5149
Publisher: Medknow Publications & Media Pvt Ltd, India
Article Information
Copyright: © Annals of Pediatric Cardiology
open-access:
Print publication date: Season: May-Aug Year: 2014
Volume: 7 Issue: 2
First Page: 158 Last Page: 159
PubMed Id: 24987268
ID: 4070211
Publisher Id: APC-7-158
DOI: 10.4103/0974-2069.132519

Multimodality imaging of a subtotally obstructive right ventricular myxoma in an asymptomatic 10-year-old girl
Gesa Wiegandaff1
Ludger Sieverdingaff1
Ulrich Kramer1
Susanne Haen2
Michael Hofbeckaff1
Department of Pediatric Cardiology, University Children's Hospital, Tuebingen, Germany
1Department of Diagnostic and Interventional Radiology, Institute of Pathology and Neuropathology, Eberhard-Karls-University, Tuebingen, Germany
2Department of General Pathology, Institute of Pathology and Neuropathology, Eberhard-Karls-University, Tuebingen, Germany
Correspondence: Address for correspondence: Dr. Gesa Wiegand, Department of Pediatric Cardiology, University Children's Hospital, Hoppe-Seyler-Strasse 1, Tuebingen - 72076, Germany. E-mail: gesa.wiegand@med.uni-tuebingen.de

CASE REPORT

A 10-year-old girl was referred for an episode of sustained tachycardia while diving in a swimming pool. The electrocardiogram registered supraventricular tachycardia at a rate of 250 beats/min, which was successfully treated with adenosine. A heart murmur was detected; therefore, transthoracic and transesophageal echocardiography was performed. This revealed a smooth tumor located below the tricuspid valve that occupied almost the entire cavity of the right ventricle and obstructed its outflow tract [Figure 1a and b]. Cardiac magnetic resonance imaging (MRI) confirmed these results; the relatively mobile tumor protruded into the right ventricular outflow tract and appeared to be pedunculated with an attachment to the right ventricular free wall [Figure 2a and b]. While the function of the tricuspid valve was normal, the septum showed paradoxical motion. Structural inhomogeneities of the tumor, which represented intratumoral hemorrhages, were obvious upon MRI [Figure 2a and b]. These hemorrhages were later examined by histopathology. Preoperative cardiac catheterization confirmed obstruction of the right ventricular outflow tract [Figure 3a and b] and identified vascular supply of the tumor by an abnormal vessel originating from the right coronary artery.

While the patient was on cardiopulmonary bypass, surgical resection was performed through a right atrial approach and revealed a 5.5 × 3.7 × 3.1 cm smooth elastic tumor with gelatinous content and distinct hemorrhages. Histopathology confirmed the diagnosis of a capsulated myxoma [Figure 4]. Postoperative recovery was uneventful, and there was no recurrence of the tumor during 3.5-year follow-up. Supraventricular tachycardias recurred; therefore, an electrophysiological examination was performed, which revealed atrioventricular node reentry tachycardia that was unrelated to the cardiac myxoma. This was treated by cryomodulation of the slow pathway. During 2 years of follow-up, the patient did not exhibit symptoms of tachycardia, and she resumed competitive biathlon sports.


DISCUSSION

Primary cardiac tumors are rare in children; the most common are rhabdomyomas and teratomas, whereas myxomas and fibromas are less common.[1] Myxomas occur in all age groups, most frequently between the third and sixth decades of life, and there is a female predominance.[2] About 75% of myxomas develop in the left atrium in both children and adults; 15-20% of myxomas develop in the right atrium and myxomas rarely arise in the ventricles.[2, 3] Myxomas that arise in the right ventricle and obstruct the right ventricular outflow tract are extremely rare in children, and there are only isolated reports of such cases.[4, 5, 6]

The macroscopic appearance of myxomas is most often polypoid; however, in rare cases, such as that presented here, they have a smooth surface. Many large blood vessels derived from the subendocardium are often located at the base of a myxoma. As in the patient presented here, myxomas often contain hemorrhages and cysts, as well as foci of extramedular hematopoiesis, which may cause hemorrhages. In rare cases, such tumors contain regions of calcification, making them detectable by chest radiography[2] and MRI.

Depending on the size and location of the myxoma, patients can be asymptomatic or exhibit various degrees and types of cardiac insufficiency.[2] Typically, atrial myxomas protrude into and obstruct the atrioventricular valves and can cause syncopal episodes and sudden death. Transthoracic echocardiography is useful to describe the size and location of the tumor, whereas transesophageal echocardiography is helpful to determine where the tumor is attached.

Our patient presented owing to a first episode of supraventricular tachycardia. However, in this case, the supraventricular tachycardia was probably unrelated to the tumor and its right ventricular outflow tract obstruction. Therefore, it underlines the requirement to perform echocardiography to exclude structural heart disease in all children with cardiac arrhythmias.


Notes

Source of Support: Nil

Conflict of Interest: None declared

REFERENCES
1. Bielefeld KJ,Moller JH. Cardiac tumors in infants and children: Study of 120 operated patientsPediatr CardiolYear: 201334125822735896
2. Reynen K. Cardiac myxomasN Engl J MedYear: 1995333161077477198
3. Reddy SN,Sunil GS,Kumar RK. Surgical removal of a left ventricular myxoma in an infantAnn Pediatr CardiolYear: 201361798124688241
4. Buheitel G,Hofbeck M,Singer H,Lindemann Y,von der Emde J. Kardiale Myxome bei Kindern und JugendlichenMonatsschr KinderheilkdYear: 1996144108791
5. Kern JH,Aguilera FA,Carlson DL,Galantowicz M. Right ventricular myxoma obstructing the right ventricular outflow tractCirculationYear: 2000102E14510889142
6. Kumagai K,Sai S,Endo M,Tabayashi K. Right ventricular myxoma obstructing the pulmonary artery during early childhoodGen Thorac Cardiovasc SurgYear: 200856351318607684

Figures

[Figure ID: F1]
Figure 1 

The right ventricular outflow tract in a transthoracic echocardiographic subcostal view (a) and a transesophageal echocardiographic view at 49° (b)



[Figure ID: F2]
Figure 2 

Cardiac MRI of the axial (a) and sagittal (b) view of the right ventricle. MRI = Magnetic resonance imaging



[Figure ID: F3]
Figure 3 

Angiography of the right ventricle in the lateral (a) and anteroposterior (b) projection



[Figure ID: F4]
Figure 4 

Macroscopic presentation of the encapsulated myxoma



Article Categories:
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Keywords: Pediatric cardiology, cardiac tumor, myxoma, right ventricle.

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