|Ahead of print publication
Wall-to-wall heart – The largest left atrium ever reported
Saikat Bhattacharjee1, Shikha Awasthi2, Prashant Jambunathan3, Preema Sinha4, Prateek5
1 Department of Radiology, Military Hospital (Cardiothoracic Centre), Pune, Maharashtra, India
2 Department of Radiology, INHS Kalyani, Vishakapatnam, Andhra Pradesh, India
3 Department of Medicine, INHS Kalyani, Vishakapatnam, Andhra Pradesh, India
4 Department of Dermatology, Armed Forces Medical College, Pune, Maharashtra, India
5 Department of Radiology, Armed Forces Medical College, Pune, Maharashtra, India
|Date of Submission||22-Sep-2019|
|Date of Acceptance||20-Jan-2020|
|Date of Web Publication||12-Sep-2020|
Department of Radiodiagnosis and Imaging, Military Hospital (Cardiothoracic Centre), Pune - 411 040, Maharashtra
Source of Support: None, Conflict of Interest: None
A 59-year-old male patient presented with a history of dyspnea on exertion for the past 10 years, which was gradually progressive in nature. The patient had orthopnea and paroxysmal nocturnal dyspnea for the past few months. On evaluation, he was found to have cardiomegaly and a giant left atrium. Contrast-enhanced computed tomography of the heart showed the left atrium to be enlarged to a dimension of 20.7 cm which, as of the time of writing, is the largest ever reported.
Keywords: Cardiomegaly, giant left atrium, left atrial thrombi, mitral valve disease, wall-to-wall heart
| Introduction|| |
Long-standing mitral valve disease leads to enlargement of the left atrium due to compensatory mechanisms of intra-atrial pressure or volume overload. Initially, this enlargement is helpful as it leads to a reduction in pulmonary venous pressure and protects the lung against pulmonary edema or congestion. However, long-standing left atrial pressure or volume overload eventually leads to pulmonary venous congestion and pulmonary edema. The grossly enlarged left atrium is known as giant left atrium. Giant left atrium is an uncommon presentation of rheumatic heart disease seen in approximately 0.3% of cases. It has variably been described as an enlarged heart with a cardiothoracic ratio > 0.7 on chest X-ray with an anteroposterior diameter of left atrium >8 cm on echocardiography or enlargement of left atrium >6.5 cm.
| Case Report|| |
A 59-year-old male presented with a 10-year history of insidious-onset, progressive breathlessness. He had been evaluated 10 years prior to presentation and was found to have rheumatic mitral valve disease. He was also advised surgery and prophylaxis for the same, but he did not return for follow-up and was not compliant with medication. During the present evaluation, he was found to have a pulse of 116/min, a blood pressure of 88/68 mmHg, raised jugular vein pulse, pedal edema, and rales on auscultation. On radiological evaluation, he was found to have significant cardiomegaly (cardiothoracic ratio = 0.875) with an enlarged wall-to-wall heart [Figure 1]. The radiograph also showed an enlarged main pulmonary artery (MPA) and bilateral pleural effusion. The patient was taken up for a bedside two-dimensional transthoracic echocardiogram. This study revealed cardiomegaly with enlarged left atrium and multiple thrombi. Severe mitral regurgitation and mitral stenosis were also noted. A contrast-enhanced computed tomography showed grossly enlarged left atrium which was reaching up to the right lateral chest wall [Figure 2]a and [Figure 2]b. The maximum transverse dimension of the giant left atrium in its widest part was 20.7 cm [Figure 3]. Few large thrombi were noted in the enlarged left atrium. Calcification was seen in the mitral valve leaflets. The right atrium and left and right ventricles were also dilated. They were compressed against the anterior chest wall by the grossly dilated left atrium. MPA was dilated measuring 33.4 mm in diameter. Features of congestive cardiac failure were seen in the form of bilateral pleural effusion (right > left) and interstitial thickening in the visualized lung.
|Figure 1: Chest radiograph showing cardiomegaly with a cardiothoracic ratio of 0.875|
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|Figure 2: (a) Contrast-enhanced computed tomography angiography of the heart shows an enlarged left atrium with large thrombi (arrowhead) within. The left atrium is seen reaching up to the right lateral chest wall. Mitral valve calcification can also be seen (arrow). The enlarged left atrium is seen to push the left ventricle, the right atrium, and the right ventricle. (b) Large thrombi in the left atrium were seen (arrowhead). Also seen is an enlarged main pulmonary artery as compared to the aorta|
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|Figure 3: Maximum transverse diameter of the enlarged left atrium measured as 20.7 cm|
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| Discussion|| |
Rheumatic heart disease is still an important cause of acquired heart disease in the developing world. The most common valve involved is the mitral valve. Long-standing mitral valve disease leads to left atrial enlargement. The reason for left atrial enlargement is the compensatory mechanism of pressure and volume overload. An associated rheumatic pancarditis may also contribute to left atrial enlargement. Atrial fibrillation due to enlarged left atrium further increases left atrial volume overload and contributes to the enlargement. A grossly dilated left atrium is known as a giant left atrium. Several definitions of giant left atrium abound in literature., Isomura et al. initially defined and described the giant left atrium as measuring >6 cm. The exact etiology and pathogenesis of giant atrial enlargement are still not understood. However, it is mostly associated with rheumatic heart disease. A few nonrheumatic causes of giant left atrium have also been described such as mitral valve prolapse, left-to-right shunt, chronic heart failure, and chronic atrial fibrillation.,
Dilatation of the left atrium is associated with atrial fibrillation and thromboembolic complication. The reason for these is blood stasis in the enlarged left atrium. The risk of fibrillation and thrombosis increases with increasing left atrial size despite anticoagulation. The largest reported left atrial size prior to our case measured 19.3 cm × 14.7 cm and was because of chronic rheumatic heart disease. The dimensions of the left atrium in our case were 20.7 cm × 13.7 cm at its widest. As of the time of this writing, this is the largest left atrium ever reported in literature. In addition, large thrombi were seen in this enlarged left atrium.
| Conclusion|| |
Giant left atrium is an uncommon presentation of long-standing rheumatic heart disease. It is associated with an increased risk of atrial fibrillation and thromboembolic complications. Hence, a high index of suspicion should be kept in mind when dealing with a grossly enlarged heart in a patient with chronic rheumatic heart disease.
Our patient experienced worsening of symptoms during his hospitalization. He was managed for heart failure according to the existing standard of care. However, the patient succumbed to his illness.
A comprehensive written consent was obtained from the patient's family for the purposes of this publication.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's family has given consent for the patient's images and other clinical information to be reported in the journal. The patient's family understand that the patient's name and initial will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]