Images in…

Congenital large aneurysmal left atrium: a rare cause

BMJ Case Rep: first published as 10.1136/bcr-2022-250314 on 15 April 2022. Downloaded from http://casereports.bmj.com/ on June 26, 2022 by guest. Protected by copyright.
of 100% cardiothoracic ratio
M Harsha Sagar, Kanhai Lalani ‍ ‍, M Sudhakar Rao ‍ ‍,
Padmakumar Ramachandran ‍ ‍

Cardiology, Kasturba Medical DESCRIPTION

College, Manipal, Manipal The majority of congenital or acquired cardiac
Academy of Higher Education, diseases are routinely diagnosed by echocardio-
Manipal, Karnataka, India
gram; however, a chest X-­ray is frequently used as
the initial imaging investigation. The correct inter-
Correspondence to
Dr M Sudhakar Rao; pretation of these radiographs allows for the most
​msudhakar88@​gmail.​com suitable work-­up and care.1 2 Cardiothoracic ratio
(CTR) was measured as ‘the ratio of the maximal
Accepted 5 April 2022 transverse diameter of the cardiac silhouette to the
distance between the internal rib margins at the
level of the right hemidiaphragm’.2 3
A middle-­ aged male patient presented with
complaints of worsening breathlessness for the last
6 months (New York Heart Association class IV). Figure 2 Chest X-­ray, posteroanterior view showing
His chest X-­ ray (figure 1) showed gross cardio- a reduction in the cardiothoracic ratio after surgical
megaly, with a CTR of 1, frequently known as resection of the aneurysmal left atrium and itral valve
the ‘wall-­ to-­
wall’ heart. Though 100% CTR is replacement.
uncommon, Ebstein’s anomaly, massive pericar-
dial effusion, multivalvular heart diseases, dilated
resection of the aneurysmal LA. He underwent
cardiomyopathy, biatrial enlargement in restrictive
successful left atrial aneurysm resection with
cardiomyopathy and cardiac tumours are among
mitral valve replacement with a 23 mm St. Jude
the differentials for massive cardiomegaly on this
mechanical valve. Postprocedure was uneventful.
chest X-­ray.
He is presently stable and on regular follow-­up on
His transthoracic echocardiography showed situs
aspirin, diuretics and warfarin. His follow-­up chest
solitus dextrocardia, atrioventricular/ventriculoar-
X-­ray is shown in figure 2.
terial concordance, with a large aneurysmal left
Congenital left atrial aneurysm with dextrocardia
atrium (LA), severe mitral regurgitation and severe
is a very rare condition and can be worsened by
pulmonary hypertension. The massively dilated LA
atrial arrhythmias and embolic events, resulting in
forced the dextroposed heart to the right side of
significant morbidity and mortality. In most cases,
the chest and filled the whole left thoracic cavity,
resulting in a CTR of 1 in the chest X-­ray. He was
diagnosed to have atrial fibrillation, dextrocardia
with congenital left atrial aneurysm with conges- Learning points
tive heart failure. The patient was initially stabi-
lised medically with anticoagulation, diuretics, ► Though 100% cardiothoracic ratio (CTR) is rare,
beta blocker and vasodilator, followed by surgical other differentials of massive cardiomegaly on
chest X-­ray may include Ebstein’s abnormality,
gross pericardial effusion, multivalvular heart
diseases, dilated cardiomyopathy, biatrial
enlargement in restrictive cardiomyopathy and
cardiac tumours.
► Congenital left atrial aneurysm with
dextrocardia is an extremely rare anomaly that
may lead to atrial arrhythmias and systemic
embolism and should be thought of as a rare
© BMJ Publishing Group cause of a 100% CTR.
Limited 2022. No commercial ► Chest radiography is a low-­cost, frequently used
re-­use. See rights and imaging method for determining cardiac size
permissions. Published by BMJ.
and shape, as well as extracardiac structures.
To cite: Sagar MH, Lalani K, However, echocardiography is commonly used
Rao MS, et al. BMJ Case for confirmation of diagnosis.
Rep 2022;15:e250314.
► The cornerstone of treatment is surgical
doi:10.1136/bcr-2022- Figure 1 Chest X-­ray posteroanterior view showing
250314 resection.
massive cardiomegaly with a cardiothoracic ratio of 1.
Sagar MH, et al. BMJ Case Rep 2022;15:e250314. doi:10.1136/bcr-2022-250314 1
 Images in…
the aneurysm affects the LA appendage rather than the entire left ORCID iDs

BMJ Case Rep: first published as 10.1136/bcr-2022-250314 on 15 April 2022. Downloaded from http://casereports.bmj.com/ on June 26, 2022 by guest. Protected by copyright.
atrial body.4 5 Patient life depends on timely diagnosis and treat- Kanhai Lalani http://orcid.org/0000-0001-7399-4630
M Sudhakar Rao http://orcid.org/0000-0001-9439-3703
ment. Non-­invasive imaging procedures such as echocardiog- Padmakumar Ramachandran http://orcid.org/0000-0001-8121-3304
raphy, CT scan and cardiac MRI are used to make the diagnosis.
These individuals should then be treated with the appropriate
REFERENCES
medical and surgical procedures.1 1 Shams KA, Sinning C, Van De Sande D. When the left atrium becomes a monster: a case
report. Eur Heart J Case Rep 2020;4:1–4.
Contributors MHS and KL wrote the manuscript. PR and MSR verified the 2 Hubbell FA, Greenfield S, Tyler JL, et al. The impact of routine admission chest x-­ray
manuscript and modified it. The patient was under care of PR. films on patient care. N Engl J Med 1985;312:209–13.
Funding The authors have not declared a specific grant for this research from any 3 Dimopoulos K, Giannakoulas G, Bendayan I, et al. Cardiothoracic ratio from postero-­
funding agency in the public, commercial or not-­for-­profit sectors. anterior chest radiographs: a simple, reproducible and independent marker of
disease severity and outcome in adults with congenital heart disease. Int J Cardiol
Competing interests None declared. 2013;166:453–7.
Patient consent for publication Consent obtained directly from patient(s). 4 de la Fuente A, Urchaga A, Sánchez R, et al. Congenital aneurysm of the left atrial
appendage. Ann Thorac Surg 2008;85:2139–40.
Provenance and peer review Not commissioned; externally peer reviewed. 5 Zhao J, Ge Y, Yan H. Treatment of congenital aneurysms of the left atrium and left atrial
Case reports provide a valuable learning resource for the scientific community and appendage. Texas Hear Inst J 1999;26:136.
can indicate areas of interest for future research. They should not be used in isolation
to guide treatment choices or public health policy.

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2 Sagar MH, et al. BMJ Case Rep 2022;15:e250314. doi:10.1136/bcr-2022-250314