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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 5  |  Issue : 2  |  Page : 96-98

Congenital left atrial appendage aneurysm: A treacherous extra chamber


Department of Cardiology, Government Medical College, Kozhikode, Kerala, India

Date of Web Publication20-Jun-2017

Correspondence Address:
K K Goyal
Department of Cardiology, Government Medical College, Super-Specialty Block, Kozhikode - 673 008, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/heartindia.heartindia_56_16

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  Abstract 

Left atrial appendage aneurysm (LAAA) without involvement of mitral valve is a rare anomaly caused due to dysplasia of atrial muscles. Patients may be asymptomatic or may present with palpitations, dyspnea, atypical chest pain, or thromboembolic complications. Treatment usually consists of surgical resection of the aneurysm, and prognosis postsurgery is very good. We herein report a case of a 4-year-old child who presented with palpitations and found to be having LAAA.

Keywords: Aneurysm, congenital, left atrial appendage


How to cite this article:
Goyal K K, Kayakkal S, Muneer K, Nair R G, Sajeev C G. Congenital left atrial appendage aneurysm: A treacherous extra chamber. Heart India 2017;5:96-8

How to cite this URL:
Goyal K K, Kayakkal S, Muneer K, Nair R G, Sajeev C G. Congenital left atrial appendage aneurysm: A treacherous extra chamber. Heart India [serial online] 2017 [cited 2021 Dec 2];5:96-8. Available from: https://www.heartindia.net/text.asp?2017/5/2/96/208558


  Introduction Top


Left atrial appendage aneurysm (LAAA) is a very rare condition with <100 cases reported worldwide.[1] The association of LAAA with an atrial septal defect (ASD) is even rarer with <10 cases reported in literature.[1],[2] Despite being rare, this is not an innocuous condition and is frequently associated with palpitations, supraventricular arrhythmias, and even thromboembolic complications. Hence, it becomes imperative to recognize and treat the case in suspected patients. We herein report a case of a 4-year-old female child with a history of surgical closure of ASD, admitted with palpitations, and found to be having LAAA.


  Case Report Top


A 4-year-old female child was admitted to our hospital with the complaint of paroxysmal palpitations for the past 1 year. These episodes were frequently associated with presyncope and chest discomfort as narrated by the mother of the patient. The patient had a history of surgical closure of an ostium secundum ASD 6 months back, which was detected while being evaluated for palpitations. On admission, electrocardiography showed a supraventricular tachycardia with a rate of about 300/min [Figure 1]a which spontaneously reverted to sinus rhythm. Chest X-ray of the patient showed increased cardiothoracic ratio with an abnormally prominent left upper cardiac boarder [Figure 1]b. Transthoracic echocardiogram revealed a large LAAA with a normal mitral valvular function [Figure 2]a. There was no residual flow through the repaired ASD. There was no spontaneous echo contrast or thrombus in the LAAA or left atrium. The patient also had grade I diastolic dysfunction. Transesophageal echocardiogram was not considered as the child was only 4 years old. A three-dimensional echocardiogram was done which confirmed the aforementioned findings [Figure 2]b. LAAA on echocardiography can sometimes be confused with pericardial cysts, mediastinal cystic tumors, or left atrial herniation through the pericardium. Hence, a cardiac computed tomography (CT) was done to confirm the diagnosis and provide further information on the structure of the aneurysm. CT showed a dilated left atrium with disproportionate dilatation of left atrial appendage [Figure 3]. Left atrial appendage measured 3 cm × 5 cm without any obvious thrombus. CT also ruled out a juxtaposed right atrial appendage.
Figure 1: (a) Electrocardiography of the patient showing supraventricular tachycardia. (b) Chest X-ray showing cardiomegaly and prominent left upper border

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Figure 2: (a) Apical 4-chamber view of a two-dimensional echocardiogram displaying left atrial appendage aneurysm. (b) Three-dimensional echocardiogram of the patient

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Figure 3: Computed tomography scan showing a large left atrial appendage aneurysm with no thrombus

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The patient was managed conservatively with beta blocker, digoxin, and amiodarone. Surgery was not considered as the parents of the patient were not willing, there was no LAAA thrombus, and there was no recurrence of palpitations. The patient is under regular follow-up on medical management and asymptomatic at present.


  Discussion Top


LAAA is a rare condition and its association with an ASD is even rarer. LAAA can be classified as congenital or acquired. Congenital LAAA is caused due to dysplasia of musculi pectinati and related muscle bands [3] whereas acquired forms are associated with left atrial enlargement due to mitral valve disease.[4],[5] Majority of LAAA are congenital and diagnosed during the second or third decade of life.[6]

LAAA can be asymptomatic and diagnosed incidentally or it can be associated with symptoms such as palpitations, breathlessness, or nonspecific chest pain. It can also present with thromboembolic complications. Palpitation is the most common presenting symptom and is frequently caused by supraventricular arrhythmias. The origin of these atrial tachyarrhythmias is often related to grossly dilated left atrial appendage which forms a substrate for triggered activity and re-entry.[7] Chest pain is frequently due to pressure on the pericardium, however it may also be due to other causes. Frambach et al. reported a case in which a 15-year-old boy presented with myocardial ischemia caused by temporary compression of left anterior descending artery by LAAA.[8]

Chest X-ray usually shows a prominent upper left heart border which is a nonspecific finding. Diagnosis can be confirmed by noninvasive imaging techniques such as echocardiography, CT, and magnetic resonance imaging. Angiocardiography used frequently in the past is rarely needed nowadays for diagnosis or evaluation of a LAAA. There is no clear definition of a LAAA. Diagnostic criteria for a congenital LAAA as given by Foale et al. include (i) origin from an otherwise normal atrial chamber, (ii) well-defined communication with the atrium, (iii) intrapericardial location, and (iv) distortion of the left ventricle.[9] These criteria are, however, not absolute and were given to differentiate LAAA from other conditions such as acquired left atrial aneurysm, extrapericardial herniations, and pericardial or mediastinal cysts. Further, there is no consensus on what size should be called as aneurysmal. Veinot et al. stated the size of normal left atrial appendage in different age groups and sexes on the basis of their study on 500 normal autopsy hearts.[10] The mean diameter, width, and length of left atrial appendage in women <20 years were 0.86 cm, 1.38 cm, and 2.29 cm, respectively. Left atrial appendage in our case measured 3 cm × 5 cm which was well above the normal range.

Surgery (aneurysmectomy) is the recommended treatment for LAAA even in asymptomatic patients as it could be the source of life-threatening thromboembolic complications. Aneurysmectomy alone is enough to eliminate the risk of atrial tachyarrhythmias in most of the patients; however in patients with large dysmorphic left atrium, concurrent Cox-Maze type or other ablative procedures may be performed along with resection of the LAAA. Although a number of approaches have been described, a median sternotomy with cardiopulmonary bypass is usually preferred. Prognosis after surgery is generally good with most of the patients not experiencing any recurrence of symptoms. In patients not undergoing surgery, medical management can be useful for treating and preventing tachyarrhythmias and thromboembolic complications as in our patient. However, a close follow-up is required, and there is always a risk of potential catastrophic thromboembolic complications in such patients.

Our patient had undergone surgical closure of ASD 6 months back and the operative notes mentioned that a prominent left atrial appendage was noted. The authors believe that LAAA was present during the surgery and was the cause of palpitations in our patient. The patient could have undergone aneurysmectomy along with surgical closure of ASD which was probably missed during the preoperative evaluation of ASD. This further highlights the need for a proper preoperative evaluation of any patient undergoing open-heart surgery and LAAA despite being a rare condition should be ruled out in all such patients.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Aryal MR, Hakim FA, Ghimire S, Ghimire S, Giri S, Pandit A, et al. Left atrial appendage aneurysm: A systematic review of 82 cases. Echocardiography 2014;31:1312-8.  Back to cited text no. 1
    
2.
Sevimli S, Gundogdu F, Aksakal E, Arslan S, Gurlertop Y, Senocak H. A rare congenital anomaly: Biatrial appendage aneurysm with atrial and ventricular septal defect. Echocardiography 2007;24:987-90.  Back to cited text no. 2
    
3.
Victor S, Nayak VM. Aneurysm of the left atrial appendage. Tex Heart Inst J 2001;28:111-8.  Back to cited text no. 3
    
4.
Culver DL, Bezante GP, Schwarz KQ, Meltzer RS. Transesophageal echocardiography in the diagnosis of acquired aneurysms of the left atrial appendage. Clin Cardiol 1993;16:149-51.  Back to cited text no. 4
    
5.
Wagshal AB, Applebaum A, Crystal P, Goldfarb B, Erez A, Tager S, et al. Atrial tachycardia as the presenting sign of a left atrial appendage aneurysm. Pacing Clin Electrophysiol 2000;23:283-5.  Back to cited text no. 5
    
6.
Chowdhury UK, Seth S, Govindappa R, Jagia P, Malhotra P. Congenital left atrial appendage aneurysm: A case report and brief review of literature. Heart Lung Circ 2009;18:412-6.  Back to cited text no. 6
    
7.
Cujec B, Bharadwaj B, Orchard RC, Lopez JF. Transesophageal echocardiography in the diagnosis of left atrial appendage aneurysm. J Am Soc Echocardiogr 1990;3:408-11.  Back to cited text no. 7
    
8.
Frambach PJ, Geskes GG, Cheriex EC, Wellens HJ, Penn OC. Giant intrapericardial aneurysm of the left atrial appendage. Eur Heart J 1990;11:848-53.  Back to cited text no. 8
    
9.
Foale RA, Gibson TC, Guyer DE, Gillam L, King ME, Weyman AE. Congenital aneurysms of the left atrium: Recognition by cross-sectional echocardiography. Circulation 1982;66:1065-9.  Back to cited text no. 9
    
10.
Veinot JP, Harrity PJ, Gentile F, Khandheria BK, Bailey KR, Eickholt JT, et al. Anatomy of the normal left atrial appendage: A quantitative study of age-related changes in 500 autopsy hearts: Implications for echocardiographic examination. Circulation 1997;96:3112-5.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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