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Journal of Cardiology Cases

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Case Report

Largest giant left atrium in rheumatic heart disease

Giant LA in rheumatic heart disease
Bhagya Narayan Pandit, DM, Puneet Aggarwal, DM∗, Siva Subramaniyan, DM,
Jaskaran Singh Gujral, DM, Ranjit Kumar Nath, DM, FESC, FACC, FSCAI
ABVIMS and Dr RML Hospital, New Delhi, India

a r t i c l e i n f o a b s t r a c t

Article history: In the current era of echocardiography, early diagnosis and treatment of rheumatic heart disease make
Received 30 July 2020 giant left atrium a rare condition, with a reported incidence of 0.3%, and following mainly with rheumatic
Revised 11 November 2020
mitral valve disease. We report a 50-year-old female, a known case of rheumatic heart disease who pre-
Accepted 18 November 2020
sented with breathlessness and dysphagia, and the cardiothoracic ratio on chest roentgenogram was 0.95.
Available online xxx
Echocardiography was suggestive of giant left atrium with a size of 19.4 x 18.3 cm, while magnetic res-
Keywords: onance imaging revealed a size of 22.3 x 19.2 x 20.1 cm making it the largest left atrium to be reported
Giant left atrium in the literature.
Rheumatic heart disease
Esophageal compression
© 2020 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.

Learning objectives Case report

Giant left atrium is extremely rare in the current era, and if
at all present, it is almost always secondary to rheumatic heart
disease. These patients will have long duration of rheumatic heart
disease, more chance of atrial fibrillation, compressive symptoms,
and thromboembolism. Giant atrium is an indication for anticoag-
ulation even if it is in sinus rhythm.
Introduction
Left atrial enlargement is seen in a variety of cardiac condi-
tions, including mitral valve disease, left ventricular failure, chronic
atrial fibrillation, and left-to-right shunts seen in patent ductus ar-
teriosus and ventricular septal defect, etc. [1]. Giant left atrium
is typically found in patients with rheumatic mitral valve disease
with severe mitral regurgitation (MR) or mixed stenotic with re-
gurgitation [2]. These patients are usually symptomatic along with
compressive symptoms such as dysphagia and hoarseness of voice
[3]. Because of the early detection of rheumatic heart disease and
treatment, giant atrium is rare in the current era. It is worth re-
porting this case because of the rarity of the giant left atrium, and
this is the largest left atrium reported in the literature.
∗
Corresponding author.
E-mail address: puneetaggarwal4u@gmail.com (P. Aggarwal).
A 50-year-old woman was admitted with dyspnea of New York
Heart Association class III, palpitation, and difficulty in swallow-
ing for 3 months. She had been diagnosed with rheumatic heart
disease 7 years earlier by echocardiography, which showed the mi-
tral valve area of 1.2 cm2 , moderate MR, and a left atrial diame-
ter of 4.3 cm. After that, she left the treatment and follow-up. The
physical examination revealed pulse rate - 80/min, irregular, blood
pressure 90/60 mmHg, her neck veins were distended, and grade
III parasternal heave. There was soft and variable S1, wide split S2,
mid-diastolic murmur, and grade 3/6 pansystolic murmur at apex.
Electrocardiogram showed atrial fibrillation with controlled
ventricular rate and right axis deviation (Fig. 1). A chest radiograph
(CXR) revealed massive cardiomegaly with cardio-thoracic ratio of
0.95, left ventricle type of apex, dilated left atrium reaching up to
the right lateral chest wall with widened carina (Fig. 1). On the lat-
eral view there was enlarged left atrium with posterior displace-
ment of left main bronchus with upside-down ’V’ shape of right
and left bronchus (walking man sign).
Transthoracic echocardiography (ECHO) showed a hugely di-
lated left atrium of 19.4 x 18.3 cm in apical four-chamber and
17.8 cm in parasternal long-axis view. ECHO also showed a mi-
tral valvular area of 1.32 cm2 , severe central MR with 2 jets, mild
tricuspid regurgitation with right ventricular systolic pressure of
40 mmHg, compressed right atrium, right ventricle, and left ven-
tricle (Fig. 2). Mean pressure gradient between left atrium and left
ventricle was 8 mmHg. The patient had two MR jets so vena con-
tracta was not validated however individual vena contracta of each

https://doi.org/10.1016/j.jccase.2020.11.022
1878-5409/© 2020 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved.

Please cite this article as: B.N. Pandit, P. Aggarwal, S. Subramaniyan et al., Largest giant left atrium in rheumatic heart disease, Journal
of Cardiology Cases, https://doi.org/10.1016/j.jccase.2020.11.022
JID: JCCASE
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B.N. Pandit, P. Aggarwal, S. Subramaniyan et al.
Fig. 1. (A) Electrocardiogram showing right axis deviation with atrial fibrillation, (B)
chest X-ray posteroanterior view showing huge cardiomegaly with cardiothoracic
ratio of 0.95 and also left atrium touches the right lateral wall with widened carinal
angle, (C) lateral view showing enlarged left atrium with walking man sign.
Fig. 2. Echocardiogram. (A) Apical four-chamber view showing left atrial size of 19.4 x 18.3 cm. (B) Short-axis view of the level of mitral valve with mitral valve area (MVA)
of 1.32 cm2 . (C) Apical four-chamber view showing severe mitral regurgitation with regurgitant area of 42.9 cm2 . (D) Continuous wave Doppler at aortic valve showing no
aortic stenosis.
LA, left atrium; RA, right atrium; RV, right ventricle.
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jet was 0.5 cm and 0.7 cm with cumulative regurgitation jet frac-
tion of 65%. The left ventricular ejection fraction was 60%.
Since the patient had dysphagia, cardiac magnetic resonance
imaging (MRI) was done, which showed giant left atrium measur-
ing 22.3 x 19.2 x 20.1 cm with volume 2896 ml. MRI also con-
firmed the findings of elevation of left bronchus and compression
of the esophagus. Left atrium was anteriorly rotated and occupying
the anterior of heart in position (Fig. 3).
The patient was treated with diuretics, oral anticoagulation,
and β -blocker. After stabilization, she was discharged, and she is
waiting for a mitral valve replacement with left atrium reduction
surgery.
Discussion
Left atrial anterior-posterior diameter of greater than 80 mm
on transthoracic echocardiography is considered diagnostic of giant
left atrium [4].
Although rheumatic heart disease with MR or mixed lesion rep-
resents the main cause of the giant left atrium, other etiologies
such as mitral valve prolapse [5], hypertrophic cardiomyopathy [6],
and cardiac amyloidosis [7] have also been reported.
Giant atrium was initially thought to occur because of the
rheumatic process causing pancarditis [3]. However, pathological
studies have found fibrosis with chronic inflammatory findings
rather than Aschoff nodules [3] supporting long-standing chronic
volume and pressure overload as etiology more than being a part
of the rheumatic process.
Giant left atrium can cause intracardiac or extracardiac com-
pression manifestations such as shortness of breath, dysphagia,
palpitations, chest pain, swelling of the body, and thromboembolic
events [3].
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B.N. Pandit, P. Aggarwal, S. Subramaniyan et al.
Fig. 3. Magnetic resonance imaging showing giant left atrium and compression of esophagus.
Patients with mitral valve disease with giant left atrium will
have a long history of rheumatic heart disease, more risk of atrial
fibrillation, more chance of compressive symptoms such as dys-
phagia, hoarseness of voice, and more chance of thromboembolism
which is the major complication of giant left atrium. Therefore, gi-
3
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ant left atrium is an indication for the initiation of anticoagulant
therapy [3].
Hurst [3] defined a giant left atrium on CXR as “one that
touches the right lateral side of the chest wall.” He considers the
CXR diagnostic of a giant left atrium, when the left atrium touches
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B.N. Pandit, P. Aggarwal, S. Subramaniyan et al.
the right lateral side of the chest wall. Giant left atrium should al-
ways be suspected in a patient with rheumatic mitral disease who
develops right lung opacification on chest X-ray [3]. Echocardio-
gram will give the cause of giant atrium and diameter of the left
atrium [1].
Additional imaging modalities such as MRI or computed tomog-
raphy should be considered to precisely assess the size and its re-
lationship with surrounding structures. The largest left atrial diam-
eter measured in the literature was 20 x 22 cm in a patient with
a prosthetic mitral valve [8]. To the best of our knowledge, this is
the largest left atrium of size 22.3 x 19.2 x 20.1 cm to be reported
in the literature to date.
Management of giant atrium is surgery, and the aim of surgery
is to correct the mitral valve abnormalities, to treat compression
manifestations, to prevent thromboembolism, and to revert atrial
fibrillation to normal sinus rhythm. Mitral valve surgery with or
without left atrium volume reduction is indicated in giant left
atrium [9]. The main indication for volume reduction is the pres-
ence of intracardiac or extracardiac compressive symptoms. But
some surgeons believe that successful mitral valve surgery alone
will result in the eventual reduction of left atrial size as the vol-
ume and mean atrial pressure decline [9].
Declaration of Competing Interest
The authors declare that there is no conflict of interest.
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Acknowledgments
Nil.
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