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Year : 2020  |  Volume : 4  |  Issue : 2  |  Page : 206-208

Right Juxtaposition of the Atrial Appendages

1 Department of Pediatric Cardiology, Rainbow Children Heart Institute, Hyderabad, Telangana, India
2 Department of Pediatric Cardiology, Care Hospitals, Hyderabad, Telangana, India

Date of Submission21-Oct-2019
Date of Decision24-Nov-2019
Date of Acceptance08-Apr-2020
Date of Web Publication19-Aug-2020

Correspondence Address:
Dr. Nageswara Rao Koneti
Department of Pediatric Cardiology, Rainbow Children Heart Institute, Road No 10 Banjara Hills, Hyderabad - 500 034, Telangana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiae.jiae_49_19

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Right juxtaposition of atrial appendages are rare and associated with congenital heart defects. We report 3 cases of right juxtaposition of atrial appendages (RJAA) diagnosed by transthoracic echocardiography. The sub-xiphoid sagittal view, parasternal short axis and apical four chambered views demonstrated RJAA. All 3 cases with RJAA were associated with congenital heart disease. Diagnosis was confirmed by CT angiogram and intra-operative findings in two children who underwent surgical correction. Right juxtaposition of atrial appendages is a rare association with congenital heart defects. Transthoracic echocardiographic assessment may give clues to the diagnosis.

Keywords: Juxtaposition, right atrial appendage, transthoracic echocardiography

How to cite this article:
Bakhru S, Gupta P, Patil S, Koneti NR. Right Juxtaposition of the Atrial Appendages. J Indian Acad Echocardiogr Cardiovasc Imaging 2020;4:206-8

How to cite this URL:
Bakhru S, Gupta P, Patil S, Koneti NR. Right Juxtaposition of the Atrial Appendages. J Indian Acad Echocardiogr Cardiovasc Imaging [serial online] 2020 [cited 2021 Oct 24];4:206-8. Available from: https://www.jiaecho.org/text.asp?2020/4/2/206/292630

  Introduction Top

The term “Juxtaposition of the atrial appendages (JAA)” was first described by Dixon AS in 1954 in autopsy specimens of unusual appendages. This anomaly is characterized by both atrial appendages adjacent to each other and placed either to the left or to the right side (right juxtraposition ) of the arterial pedicle (aorta and pulmonary artery).[1] Left-sided JAA (LJAA) occurs more frequently than right-sided JAA (RJAA), with an incidence of 6:1.[2],[3] There are several case reports of LJAA associated with complex cardiac anomalies diagnosed either at autopsy or imaged by echocardiography.[4] There are very few reports of RJAA in the literature.[5] We report three cases of RJAA in children associated with congenital cardiac anomalies. The role of transthoracic echocardiogram in the identification of RJAA is emphasized.

  Clinical Presentation Top

Case 1

A 2-year-old asymptomatic girl was referred for cardiac evaluation after an incidental detection of cardiac murmur. Cardiac examination revealed a 3/6 systolic murmur at the left parasternal border. Chest X-ray showed no abnormality. Electrocardiogram revealed normal sinus rhythm, right axis deviation, and right ventricular hypertrophy. Transthoracic echocardiogram in subxiphoid sagittal and parasternal short-axis views revealed the left atrial (LA) appendage on the right side of the vascular pedicle [Figure 1]a and [Figure 1]b. A small membranous ventricular septal defect (VSD) and right ventricular outflow tract obstruction with a peak gradient of 60 mmHg were noted due to abnormal muscle bundle in the right ventricular mid-cavity. The child underwent surgical correction for the intracardiac defects. The operative findings confirmed RJAA.
Figure 1: Subxiphoid short-axis views (a and b) of case 1 showing left atrial appendage coursing perpendicular to the atrial septum. LAA: Left atrial appendage, LA: Left atrium, RA: Right atrium

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Case 2

A 1-year-old boy presented with congestive cardiac failure with saturation of 84% at room air. Cardiac examination revealed a loud pulmonary component, 2/6 systolic murmur on the left parasternal border, and a mid-diastolic murmur at the apex. Chest X-ray revealed a cardiothoracic ratio of 0.6 with dilated pulmonary arteries. Electrocardiogram revealed biatrial enlargement and right ventricular hypertrophy. Transthoracic echocardiogram showed a large membranous VSD, patent ductus arteriosus with bidirectional shunt, and supramitral ring with severe mitral stenosis. The LA appendage was dilated and noted on the right side of the arterial pedicle (RJAA) in all views [Figure 2]a, [Figure 2]b, [Figure 2]c. Parents refused further evaluation and management.
Figure 2: Transthoracic echocardiography modified parasternal short axis views (a and b) and apical 4-chamber view (c) showing finger like left atrial appendage on the right side. The thick arrow shows supramitral ring and broken arrow shows ventricular septal defect. LAA: Left atrial appendage, LA: Left atrium, RV: Right ventricle, LV: left ventricle

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Case 3

A 2½-year-old boy was found to have a 3/6 ejection systolic murmur on the left parasternal border. The echocardiographic evaluation showed a RJAA confused as fossa ovalis atrial septal defect. There were moderate perimembranous VSD and severe right ventricular outflow tract obstruction. Computed tomography angiogram of axial and volume-rendered images confirmed the RJAA [Figure 3]a and [Figure 3]b. The child underwent intracardiac repair for the defects. The external morphological anatomy showed RJAA [Figure 4]. The LA appendage was excised in view of difficulty in surgical field.
Figure 3: Computed tomography angiogram (a) volume-rendered imaging (b) showing both the atrial appendages on the right side of the arterial pedicle (Ao and PA). LAA: Left atrial appendage, RAA: Right atrial appendage, Ao: Aorta, PA: Pulmonary artery

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Figure 4: Intraoperative picture of case 3 showing both atrial appendages on the right side of the arterial pedicle (Ao). The bright red color finger-like left atrial appendage is superiorly wrapping the dark color right atrial appendage

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  Discussion Top

Echocardiographic imaging of JAA is reported by Rice et al.[4] We encountered RJAA in three cases of congenital cardiac anomaly identified by transthoracic echocardiography. The right atrial appendage is short, fat, and broad based and best seen in subxiphoid sagittal view. The LA appendage is long thin and narrow based like finger, best seen in parasternal short-axis and apical four-chamber views. Left JAA of the atrial appendage is usually evident in the parasternal and subxiphoid views. We noticed RJAA of the atrial appendage can be better profiled in subxiphoid sagittal view where the wall of the LA appendage can be seen coursing perpendicular to the atrial septum to the right of the arterial pedicle.

In cases 1 and 3, RJAA is associated with VSD and right ventricular outflow tract obstruction, whereas in case 2, it is associated with VSD and supramitral ring. Two of our cases underwent successful surgical correction.

Mathew et al. in their series reported that RJAA is commonly associated with complex congenital heart disease (CHD) in which VSD is seen in 9 out of 12 cases. LJAA, on the other hand, is associated with CHDs such as tricuspid atresia, complex double-outlet right ventricle, and single ventricle. The diagnosis of JAA is important in the management of congenital cardiac disease.[6] JAA might pose a hindrance in balloon septostomy for critically ill neonates with CHDs, septal puncture for left atrioventricular valve procedures, and transcatheter closure of the LA appendage. Surgeries such as Senning, Mustard, and lateral tunnel Fontan for complex CHDs might not be possible due to anatomical considerations in the presence of JAA. Sometimes, atrial appendage excision allows for better visualization of surgical field as in two of our cases.

During cardiac embryogenesis, the relative positions of the sinus, atrium, ventricle, and bulbus are determined by the folding of the primitive cardiac tube. Later, the primitive cardiac tube undergoes a left–right asymmetrical looping on the right side (d-loop). The overtorsion of the primitive cardiac tube will cause the left-sided atrial appendage to come and lie on the right side of the vascular pedicle to produce RJAA, whereas undertorsion leads to LJAA as proposed by Werner in 1907 and later by Dixon [Figure 5].
Figure 5: Modified picture of embryological hypothesis showing the development of juxtaposed atrial appendages

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  Conclusion Top

Right JAA is rare and often associated with intracardiac structural heart defects. Careful assessment by transthoracic echocardiography will give clues to the diagnosis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Dixon AS. Juxtaposition of the atrial appendages; two cases of an unusual congenital cardiac deformity. Br Heart J 1954;16:153-64.  Back to cited text no. 1
Charuzi Y, Spanos PK, Amplatz K, Edwards JE. Juxtaposition of the atrial appendages. Circulation 1973;47:620-7.  Back to cited text no. 2
Becker AE, Becker MJ. Juxtaposition of atrial appendages associated with normally oriented ventricles and great arteries. Circulation 1970;41:685-8.  Back to cited text no. 3
Rice MJ, Seward JB, Hagler DJ, Edwards WD, Julsrud PR, Tajik AJ. Left juxtaposed atrial appendages: Diagnostic two-dimensional echocardiographic features. J Am Coll Cardiol 1983;1:1330-6.  Back to cited text no. 4
Mathew R, Replogle R, Thilenius OG, Arcilla RA. Right juxtaposition of the atrial appendages. Chest 1975;67:483-6.  Back to cited text no. 5
Anjos RT, Ho SY, Anderson RH. Surgical implications of juxtaposition of the atrial appendages. A review of forty-nine autopsied hearts. J Thorac Cardiovasc Surg 1990;99:897-904.  Back to cited text no. 6


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]


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