Title: Giant Left Atrium Disguised as Right Sided Pleural Effusion

Authors: Ayushi Dixit MD, Rayhan Shariff MD, Page Yin MD, , Lauren Rosenberg MD, Victoria Vapnyar
MD

Abstract:
Rheumatic heart disease (RHD) is a complication of acute rheumatic fever which results from untreated
streptococcal pharyngitis infections. RHD related morbidity and mortality have decreased significantly in
developed countries in the last couple of decades due to advances in early recognition and management
of acute rheumatic fever. RHD is still a significant cause of cardiovascular related mortality in some parts
of the world. One of the most common complications of RHD is chronic mitral valve disease. In some
patients this leads to the dilation of the left atrium which may go undiagnosed. Patients may experience
symptoms such as cough, chest pain, and shortness of breath which may prompt investigation with
imaging. We present a case of giant left atrium disguised as a massive pleural effusion.

Case Presentation:
A 42 year old female visitor from the West Indies with past medical history of rheumatic heart disease
with mechanical mitral valve replacement two years prior, on coumadin, presented to the hospital with
symptoms of fever, productive cough, shortness of breath, and pleuritic chest pain for four days.
Physical exam revealed decreased breath sounds in right lower lung field. Pertinent labs included
hemoglobin of 10.1gm/dL, digoxin level of 0.3 and sub-therapeutic international normalized ratio (INR)
of 1.2. EKG (Image 1) showed atrial fibrillation with rapid ventricular response at 118 beats per minute
and right axis deviation with poor R wave progression around precordial leads. Chest x-ray (image 2)
suggested large right sided consolidation and effusion with lower left sided consolidation. She was
admitted with impression of community acquired pneumonia and started on antibiotics. Transthoracic
echocardiogram was performed and significant findings included massive dilatation of the left atrium,
and bi-leaflet mechanical mitral valve prosthesis which appeared to be normally functioning (Image
3a,3b). CT scan of the chest (image 4,5) with contrast showed massive dilatation of the left atrium which
measured 14 x 20 x 13 cm (craniocaudal x transverse x AP dimension) and a thrombus measuring at least
6 cm. Given normally functioning heart valve in the context of long standing mitral valve disease, no
acute intervention was not indicated. She was started on metoprolol with continuation of digoxin and
bridged with heparin until INR was therapeutic. Patient was diagnosed with rheumatic heart disease
when she was 22 years old. She reported having a normal cardiac angiogram before the surgery and
normal life after Mitral Valve replacement without any hospitalizations.

Discussion:
Giant Left Atrium is mainly associated with Rheumatic Heart Disease (RHD). It is defined as one that
touches the right lateral side of the chest wall. [1] It is a rare condition, with a reported incidence of
0.3%. [2] When the left atrium enlarges, it moves rightward and in the case of giant left atrium
syndrome, can touch the right lateral wall of the chest. There have been multiple speculations about
what causes the distortion of the left atrium. Although Hurst attributed the dilatation of the left atrium
to the rheumatic process causing pancarditis, pathological studies have failed to reveal the Aschoff
nodules (fibrinoid necrotic center found in the myocardium surrounding blood vessels, and other
regions of the body) in the atrial tissues; rather, these studies found fibrosis with chronic inflammatory
findings .[3][4] Although biopsy was not performed, we hypothesized that due to long-standing
rheumatic mitral stenosis and atrial fibrillation contributed to increased volume and pressure load and
led to left atrial enlargement in our patient. Literature review revealed that initial chest X-rays in this
disease process are often thought to show a right sided pleural effusion as was thought to be the cause
of our patients symptoms. Further imaging with echocardiogram and CT scan of the chest can provide
clarity for diagnosis.

Conclusion:
Rheumatic heart disease continues to be a common health problem in the developing world, causing
morbidity and mortality among both children and adults. Giant left atrium should be kept in mind
whenever a patient presents with rheumatic mitral disease history with right lung opacification on chest
x-ray. The echocardiography provides the evidence for diagnosis with a left atrial anterior-posterior
diameter of >8cm. As per American Society of Echocardiography, a value of 16 to 34 mL/m2 is
considered normal whereas a value of >48 mL/m2 is considered severely abnormal.[5]The correct
diagnosis of left atrial enlargement is at times not possible by routine chest x-ray alone. It may be
misdiagnosed as a mass lesion or pleural or pericardial effusion. [2] RHD is uncommon in the United
States but was reported to be the cause of 2% of cardiovascular deaths globally. [6] We must be alert to
the fact that many immigrants to the United States come from countries where rheumatic fever is still
relatively common and may have pathological findings that are rarely seen in developed countries
today.

References:

1. Hurst JW. Memories of Patients With a Giant Left Atrium. Circulation. 2001;104(22):2630-2631.
doi:10.1161/hc4701.100775.
2. El Maghraby A, Hajar R. Giant Left Atrium: A Review. Heart Views : The Official Journal of the
Gulf Heart Association. 2012;13(2):46-52. doi:10.4103/1995-705X.99227.
3. Plaschkes J, Borman JB, Merin G, Milwidsky H. Giant left atrium in rheumatic heart disease: a
report of 18 cases treated by mitral valve replacement. Annals of Surgery. 1971;174(2):194-201.
4. Sabzi F. Huge Left Atrium Accompanied by Normally Functioning Prosthetic Valve. The Journal of
Tehran University Heart Center. 2015;10(1):53-57.
5. Lang, R., Badano, L. and Mor-Avi, V. (2018). Recommendations for Cardiac Chamber
Quantification by Echocardiography in Adults: An Update from the American Society of
Echocardiography and the European Association of Cardiovascular Imaging.
6. World Health Organization. (2018). Cardiovascular diseases (CVDs). [online] Available at:
http://www.who.int/mediacentre/factsheets/fs317/en/ [Accessed 17 Feb. 2018].
Image 1: Admission EKG showing Atrial fibrillation with Rapid Ventricular Rhythm

Image 2: Chest X-ray: Reported as “There is a moderate size consolidation of the right mid lung to the
right lower lobe with silhouetting the right cardiac border and right hemidiaphragm. This may be
combination of pleural effusion and consolidation”
Image 3a: Parasternal Short view Echocardiography

Image 3b: Apical 4-chamber view Echocardiography

Image 4: CT scan with contrast showing dilated left atrium with superimposed thrombus (coronal view).
Image 5: CT scan with contrast showing dilated left atrium with superimposed thrombus (saggital view).