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Paediatric ischaemic stroke as the first manifestation of a giant left atrium: a case report

Published online by Cambridge University Press:  08 August 2025

María Cecilia Martínez MoralesOpen the ORCID record for María Cecilia Martínez Morales[Opens in a new window],
Miguel Angel Martínez CárdenasOpen the ORCID record for Miguel Angel Martínez Cárdenas[Opens in a new window],
Marco Antonio Ruiz OntiverosOpen the ORCID record for Marco Antonio Ruiz Ontiveros[Opens in a new window] and
Iván Guillermo Jiménez Delgado
María Cecilia Martínez Morales*
Affiliation:
Instituto Mexicano del Seguro Social, UMAE 48, León, Guanajuato, México
Miguel Angel Martínez Cárdenas
Affiliation:
Instituto Mexicano del Seguro Social, UMAE 48, León, Guanajuato, México
Marco Antonio Ruiz Ontiveros
Affiliation:
Instituto Mexicano del Seguro Social, UMAE 48, León, Guanajuato, México
Iván Guillermo Jiménez Delgado
Affiliation:
Instituto Mexicano del Seguro Social, HGR 58, León, Guanajuato, México
*
Corresponding author: María Cecilia Martínez Morales; Email: m.ceciliamtz.m@gmail.com
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Abstract

Giant left atrium is extremely rare in the paediatric population, especially in asymptomatic cases. We report a 5-year-old child with a giant left atrium due to “non-rheumatic” mitral valve regurgitation that presented as a stroke secondary to atrial tachycardia. This case is noteworthy for the unusual “silent” manifestation of a giant left atrium.

Keywords

giant left atriumpaediatric ischaemic strokethrombus

Information

Type
Case Report
Information
Cardiology in the Young , Volume 35 , Issue 9 , September 2025, pp. 1953 - 1955
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Copyright
© The Author(s), 2025. Published by Cambridge University Press

Introduction

The left atrium is an asymmetrical cavity oriented leftward and posterior to the right atrium in the mediastinum. It functions as a reservoir, conduit, and pump to facilitate left ventricular filling. Reference Muthiah1 Typically, it is an ovoid-elliptical shaped chamber and tends to become more rounded when enlarged. Left atrial enlargement is usually induced by increased pressure and/or volume overload, mainly caused by rheumatic mitral valve disease. Reference Hurst2 In children, however, it is more commonly linked to congenital mitral valve disease, left-to-right shunting, or coronary artery fistulae. Reference Hurst2

This enlargement can result in a giant left atrium, defined as a diameter ≥65 mm on two-dimensional parasternal long-axis echocardiography Reference Oh3 , or >80 mm according to other authors. It is also characterised by a cardio-thoracic ratio >0.7 on chest X-ray. Reference Hurst2,Reference Piccoli, Massini and Di Eusanio5

A giant left atrium is a rare condition in the paediatric population and can cause cardiac complications such as displacement of the heart to the right side of the chest, palpitations, chest pain, fatigue, orthopnea, atrial fibrillation, or heart failure. Extracardiac complications may arise due to airway compression (such as cough, shortness of breath, dyspnoea, or stridor), oesophageal compression, or thromboembolic events. Reference Hurst2,Reference Kar, Joshi, Joshi, Aggarwal and Mridul4 Despite these risks, a minority of cases may remain asymptomatic. Reference Badui, Delgado and Enciso6

We present a case of a child with an acute ischaemic stroke as the first clinical manifestation of a giant left atrium.

Case report

A 5-year-old female presented to the emergency department with syncope, focal neurological deficits, speech alteration, and right hemiparesis. On physical examination, the patient was noted to have low height and weight for her age. She presented with a systolic murmur at the left lower sternal border and an arrhythmia.

The electrocardiogram revealed left atrial appendage focal atrial tachycardia due to micro-reentry. A chest X-ray demonstrated cardiomegaly with a cardiothoracic ratio >0.78, splaying of the carina with increased tracheal bifurcation angle greater than 90 degrees, and double density sign, as well as the third mogul sign (Figure 1).

Figure 1.

A chest X-ray with significant cardiomegaly secondary to left atrial enlargement showing double density and the third mogul sign. B and C four-chamber views with a giant left atrium causing displacement of the heart to the right side of the chest in 2D and colour Doppler images. D Parasternal modified long-axis view shows severe mitral regurgitation. E Left ventricular outflow tract without obstructions. A chest X-ray with significant cardiomegaly secondary to left atrial enlargement showing double density and the third mogul sign. B and C four-chamber views with a giant left atrium causing displacement of the heart to the right side of the chest in 2D and colour Doppler images. D Parasternal modified long axis view shows severe mitral regurgitation. E Left ventricular outflow tract without obstructions.

Figure 2.

( a ) Hyperechoic image in the dilated left atrial appendage suggesting intracardiac thrombus. ( b ) Decrease in thrombus size after treatment with enoxaparin. ( c ) Five-chamber view, without obstruction to the left ventricular outflow tract. ( d ) Parastenal short-axis view with unaltered.

Due to clinical signs of acute ischaemic stroke, a CT scan was performed, revealing a hypodense lesion in the parietal region (intimal capsule and basal ganglia). Transthoracic echocardiography showed left atrial enlargement (measuring 66.7 mm × 93.7 mm), severe mitral valve regurgitation, and a left atrial volume index (LAVI) of 60 ml/m2. A thrombus measuring 15.5 × 14.4 mm was visualised in the left atrial appendage—non-mobile, non-pedunculated, and adherent to the anterior wall.

The mitral valve was dilated, with shortened tendinous chords intersecting at the papillary muscles, resulting in a closure defect and three severe regurgitant jets directed toward the atrial roof. The arrhythmia was initially managed with intravenous amiodarone, later transitioned to oral administration, along with propranolol, although the arrhythmia recurred.

The thrombus was managed with anticoagulation therapy (enoxaparin), with subsequent echocardiography demonstrating a reduction in thrombus size. Magnetic resonance imaging was not feasible due to persistent atrial tachycardia. Surgical management, including mitral valve repair and left atrial plication, was planned. However, intraoperative complications—including low cardiac output and ventricular fibrillation—culminated in the patient’s death.

Discussion

A giant left atrium is a rare condition in the paediatric population. Patients often have a long-standing history of mitral valve disease with predominant mitral regurgitation and atrial fibrillation (the most common sustained cardiac arrhythmia in these cases). Reference El Maghraby and Hajar7 This is due to the massive dilation of the left atrium in all directions, increasing the risk of arrhythmias.

Various symptoms have been described, often associated with the displacement of surrounding thoracic structures. These patients may present with haemodynamic and/or respiratory complications, as well as atrial thrombus formation. However, a minority of cases may remain asymptomatic.

The manifestations of chronic heart disease should be more thoroughly investigated. Even mild symptoms, such as dyspnoea, are noteworthy. In our paediatric patient, left atrial volume overload was attributed to congenital mitral regurgitation. The enlargement of the atrium provided a substrate for atrial tachycardia. The atrial walls may become fibrotic, contributing to atrial remodelling and an increased risk of complications such as thromboembolic events. Reference Kar, Joshi, Joshi, Aggarwal and Mridul4

This case is particularly notable because the parents denied any associated symptoms. The only manifestations were low height and weight and NYHA functional class II symptoms, none of which prompted emergency care.

In our case, the initial presentation that led to medical evaluation was a syncopal episode secondary to a cerebrovascular event, itself caused by left atrial enlargement, atrial tachycardia, and thrombus formation. Signs such as growth retardation, cardiomegaly, and arrhythmia were only confirmed retrospectively and were not evident to caregivers.

Cardioembolic ischaemic stroke is more commonly associated with rheumatic heart disease and atrial fibrillation and rarely occurs in non-rheumatic cases. This case is unique in that the first evident manifestation was a cerebrovascular event, which is extremely rare in paediatric patients with this condition.

To our knowledge, no cases have been reported in the literature associating focal left atrial tachycardia with a giant left atrium in which the first manifestation was an acute ischaemic stroke, particularly in the paediatric population.

Cardiac MRI is useful for anatomical delineation, functional assessment, and flow analysis. Reference Singh, Mukherjee, Kumar and Gupta8 However, it was not possible in this case due to persistent atrial tachycardia. The definitive treatment for this condition is surgical intervention (mitral valve repair and left atrial plication), Reference Apostolakis and Shuhaiber9 but early diagnosis is crucial due to the imminent risk of severe pulmonary hypertension, left ventricular dysfunction, and persistent heart failure.

Conclusion

A giant left atrium is a rare condition in children. In many cases, subtle clinical signs may go unnoticed by caregivers, which can negatively impact life expectancy. This case is unique in that it describes an acute ischaemic stroke caused by thromboembolism secondary to atrial tachycardia, as the first clinical manifestation in a child.

Consistent monitoring during well-child visits is essential to detect early clinical signs, such as arrhythmia, murmurs, and low weight or height for age. Early diagnosis allows for timely management before complications develop, ultimately improving life expectancy.

Financial support

This research received no specific grant from any funding agency, commercial, or not-for-profit sectors.

Competing interests

None.

References

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Figure 0

Figure 1.A chest X-ray with significant cardiomegaly secondary to left atrial enlargement showing double density and the third mogul sign. B and C four-chamber views with a giant left atrium causing displacement of the heart to the right side of the chest in 2D and colour Doppler images. D Parasternal modified long-axis view shows severe mitral regurgitation. E Left ventricular outflow tract without obstructions. A chest X-ray with significant cardiomegaly secondary to left atrial enlargement showing double density and the third mogul sign. B and C four-chamber views with a giant left atrium causing displacement of the heart to the right side of the chest in 2D and colour Doppler images. D Parasternal modified long axis view shows severe mitral regurgitation. E Left ventricular outflow tract without obstructions.

Figure 1

Figure 2.(a) Hyperechoic image in the dilated left atrial appendage suggesting intracardiac thrombus. (b) Decrease in thrombus size after treatment with enoxaparin. (c) Five-chamber view, without obstruction to the left ventricular outflow tract. (d) Parastenal short-axis view with unaltered.