• Users Online: 155
  • Home
  • Print this page
  • Email this page
Home About us Editorial board Ahead of print Current issue Search Archives Submit article Instructions Subscribe Contacts Login 


 
 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 4  |  Issue : 3  |  Page : 240-243

Left atrial calcification: Case report and review of literature


Department of Cardiology, AIIMS, New Delhi, India

Date of Web Publication11-Jan-2019

Correspondence Address:
Dr. M Aseem Basha
Department of Cardiology, AIIMS, New Delhi
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jpcs.jpcs_48_18

Rights and Permissions
  Abstract 

Calcification of left atrium is often associated with rheumatic valvular heart disease. It has been described mostly in cases with long standing mitral stenosis and also in patients with mitral valve replacement. It should be suspected in patients with rheumatic heart disease with severe mitral stenosis or post mitral valve replacement who present with features of severe PAH and right heart failure in the presence of a normal functioning prosthetic mitral valve. Here we report a 57-year-old man who had underwent closed mitral commissurotomy about 30 years ago and mitral valve replacement about 18 years ago and now presenting with left atrial calcification. This literature also discusses the management options available.

Keywords: Calcification, rheumatic, valvular


How to cite this article:
Basha M A, Ahmed A S, Kidambi B, Teja P, Naik N. Left atrial calcification: Case report and review of literature. J Pract Cardiovasc Sci 2018;4:240-3

How to cite this URL:
Basha M A, Ahmed A S, Kidambi B, Teja P, Naik N. Left atrial calcification: Case report and review of literature. J Pract Cardiovasc Sci [serial online] 2018 [cited 2019 May 19];4:240-3. Available from: http://www.j-pcs.org/text.asp?2018/4/3/240/249934


  Introduction Top


Calcification of the left atrium is often associated with rheumatic valvular heart disease. It has been described mostly in cases with long-standing mitral stenosis and also in patients with mitral valve replacement.[1] Massive calcification of the left atrium is very rare and is generally an incidental finding on chest radiographs.


  Case Report Top


A 57-year-old man, presented with progressive shortness of breath and bilateral lower limb swelling. He had previously been treated for rheumatic mitral stenosis with mitral commissurotomy 30 years back. Eighteen years later, he underwent mechanical prosthetic valve replacement with #29 CH valve for restenosis. Thereafter, he was doing well. However, about 2 years before presentation to our institute, he developed progressive shortness of breath and features of right heart failure for which he was evaluated and was found to have severe tricuspid regurgitation (TR) (right ventricular systolic pressure [RVSP] = 88 mmHg). He underwent tricuspid valve repair using a #28 mm three-dimensional (3D) annuloplasty ring a year later. Postoperative echo revealed mild TR. Despite tricuspid valve repair, his symptoms progressed, and at the time of presentation to our institute, he was in the New York Heart Association Class IV.

At admission, he was put on decongestive measures on which he showed signs of clinical improvement. He was also evaluated for worsening right heart failure. Examination revealed bilateral lower limb edema, ascites, pallor, mild icterus, C-V wave on jugular venous pulse inspection, and pansystolic TR murmur. He was in atrial fibrillation, which was also evident on electrocardiogram. Chest radiographs posteroanterior and lateral view revealed cardiomegaly with a near complete curvilinear density, shadow outlining the left atrium suggestive of mural calcification [Figure 1] and [Figure 2]. Fluoroscopy and computed tomography (CT) also revealed circumferential calcification confirming the findings of chest radiograph [Figure 2], [Figure 3], [Figure 4]. Interatrial septum was spared suggesting “porcelain atrium.” Echocardiography done revealed a normally functioning prosthetic mitral valve, dilated right atrium and right ventricle, severe TR with RVSP = right atrial pressure + 100 mmHg, and thick left atrial (LA) wall with normal left ventricle function. Calcification of left atrium was also evident on transesophageal echocardiography which obscured the visibility of intracardiac structures [Figure 5], [Figure 6], [Figure 7], [Figure 8]. He had undergone three surgeries in the past and was also diabetic and was not willing for surgical intervention. He was put on medical management with decongestive measures, following which he showed clinical improvement. He was discharged with the plan of surgical intervention if his symptoms progress.
Figure 1: Near complete curvilinear density shadow outlining the left atrium.

Click here to view
Figure 2: Curvilinear density shadow outlining the left atrium (better visualized in lateral view than postero-anterior projection).

Click here to view
Figure 3: Fluoroscopy: RAO view showing circumferential calcification.

Click here to view
Figure 4: CT showing circumferential calcification of left atrium with sparing of interatrial septum.

Click here to view
Figure 5: Sub costal Long Axis view showing calcification around LA and LV.

Click here to view
Figure 6: TEE at Mid oesophageal level showing calcification in left atrium.

Click here to view
Figure 7: Apical 4 chamber View showing severe Tricuspid regurgitation.

Click here to view
Figure 8: Continuous wave Doppler across tricuspid valve showing TR gradient of 100 mmHg.

Click here to view



  Discussion Top


Calcification of the left atrium is often associated with rheumatic valvular heart disease. It has been described mostly in cases with long-standing mitral stenosis and also in patients with mitral valve replacement.[1] Massive calcification of the left atrium is very rare and is generally an incidental finding on chest radiographs.

Calcification may vary from less severe forms that involve the free wall of the left atrium, LA appendage, or the mitral valve apparatus to more severe forms described as “coconut atrium” or “porcelain atrium.”[2] Calcification involving LA appendage, the free wall of LA, and the mitral valve apparatus with sparing of the interatrial septum is termed as porcelain atrium, while coconut atrium involves all areas of the left atrium.[3] Thefirst description of LA calcification was by Oppenheimer in 1912, where he described the postmortem findings of a 44-year-old male who died of congestive heart failure due to mitral stenosis with entire LA wall except interatrial septum being extensively calcified.[4] Since then, numerous case reports have been published on LA calcification.

Pathophysiology

Massive LA calcification is presumed to be the result of extensive rheumatic pancarditis. Focal calcification in areas of endocardium affected by rheumatic process with subsequent progression to chronic fibrosis and deposition of calcium in the subendocardium in varying degree of severity, results in massive calcification with time and may lead to subsequent hemodynamic changes. Strain due to LA overload may also be a stimulus of calcification. Patients who are predisposed to LA calcification include those with long-standing mitral stenosis, patients with mechanical valve replacement, postradiotherapy, and rarely patients with chronic renal failure.[4],[5],[6] Infection, trauma, tumors, and ischemic damage with pathologic calcification are also implicated in the pathogenesis of LA calcification.[7]

The decreased LA compliance consequent to calcification causes impaired LA filling and increases LA pressure. Massive LA calcification is usually associated with atrial fibrillation and can be associated with recurrent thrombosis despite optimal anticoagulant therapy. Transmission of elevated LA pressure to the pulmonary venous system, pulmonary arteries, and right ventricle may result in TR.

A classification scheme has also been proposed for LA calcification as shown in [Table 1].[8]
Table 1: Classification scheme for describing extent of calcification

Click here to view


Clinical features

Mean age at diagnosis of LA calcification has been reported to be 54 years by Harthorne et al.[4] and 60 ± 13.3 years by Hosseini et al.[7] in their review. Both the reviews have reported a striking female preponderance to the tune of 74%.[4] Symptoms of underlying disease may be present for decades before recognition of LA calcification. LA calcification is usually an incidental roentgenographic finding without immediate clinical implications. Mitral valve involvement in these patients is usually severe and is present in all patients with LA calcification. Patients usually have multivalvular involvement. Harthorne et al. had reported a striking female preponderance which might be consequent to higher incidence of the involvement of mitral valve by rheumatic process in females.[4] Atrial fibrillation is usually long standing and almost universally found in these patients probably due to decreased LA compliance.

Diagnostic modalities

Chest Radiograph: Posteroanterior and lateral views

High-voltage and well-penetrated radiographs are essential for visualization of LA calcification. Partial or complete curvilinear density shadows outlining the left atrium are suggestive of mural calcification. Round or oval shell of calcium on frontal projection and C-shaped curvilinear density with the opening of lying in front of the mitral annulus on lateral or oblique radiographic views suggests complete calcification of the left atrium.[4]

Cinefluoroscopy

Fluoroscopy, by the virtue of movement of the calcified deposits, often reveals calcification that is not apparent on routine radiographs. Fluoroscopy is also more sensitive than plain radiographs for detection of LA calcification.

Echocardiography

Transesophageal echocardiography (TEE) allows for the better visualization of left atrium compared to transthoracic echocardiography. However, TEE may obscure the visibility of intracardiac structure because of the posterior location of left atrium. Transthoracic echocardiography helps in delineating underlying valve disease, LA thrombus, and pulmonary arterial hypertension.

Computer tomography

CT chest is superior to fluoroscopy and plain chest radiographs for the detection of LA calcification. Calcification of interatrial septum cannot be confirmed with certainty on CT.[2] Multidetector CT may provide detailed 3D visualization of the LA calcification with high resolution.

Therapeutic modalities

Selection of therapeutic modality for the management of LA calcification in patients with disabling symptoms includes medical therapy or surgical approach. In the absence of long-term prospective studies on the outcome of patients with LA calcification, the decision should be based on patient's general condition, patient preference, interatrial septal calcification, and caseous necrosis of mitral valve.[9],[10],[11]

Medical management

Medical management mainly revolves mainly around the treatment of predominant right heart failure. Patients with previous surgeries for valve replacement and for tricuspid valve repair for right heart failure and who are not willing for surgical intervention or those with interatrial septum calcification or caseous necrosis of mitral valve in addition to patient preference should be taken into consideration before the decision on surgical intervention.

Surgical options

Surgical option in patients with unoperated mitral valve disease with LA calcification is a total endoatriectomy with mitral valve replacement.[9] Interatrial septum is used as a cleavage plane for entry in the left atrium, and the presence of interatrial septal calcification may be a contraindication for surgery. Patients with LA calcification occurring decades after mitral valve replacement have also been successfully operated by a transseptal superior approach with endoatrioectomy and replacement of mitral valve by prosthesis. In patients with coconut atrium that is LA calcification with calcification of interatrial septum, the calcified endothelium on the atrial septum and free wall of the left atrium can be peeled off and the rim of interatrial septum may be reconstructed with a bovine pericardium.[12] Dislodgement of thrombi, uncontrollable hemorrhage due to incision over a calcified region of the left atrium, and difficulty in suturing calcified structures are potential problems during the procedure.[9] All said, there are no prospective long-term studies done to evaluate recurrence of calcification, atrial compliance, and long-term mortality.


  Conclusion Top


LA calcification is a rare but increasingly reported complication of long-standing rheumatic valvular heart disease. It should be suspected in patients with rheumatic heart disease with severe mitral stenosis or postmitral valve replacement who present with features of right heart failure in the presence of a normal functioning prosthetic mitral valve. Surgical option should be taken into consideration in patients with features of right heart failure consequent to decreased LA compliance who worsen despite medical therapy.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Meyners W, Peters S. A coconut left atrium 23 years after mitral valve replacement for chronic mitral stenosis. Z Kardiol 2003;92:82-3.  Back to cited text no. 1
    
2.
Del Campo C, Weinstein P, Kunnelis C, DiStefano P, Ebers GM. Coconut atrium: Transmural calcification of the entire left atrium. Tex Heart Inst J 2000;27:49-51.  Back to cited text no. 2
    
3.
Lee WJ, Son CW, Yoon JC, Jo HS, Son JW, Park KH, et al. Massive left atrial calcification associated with mitral valve replacement. J Cardiovasc Ultrasound 2010;18:151-3.  Back to cited text no. 3
    
4.
Harthorne JW, Seltzer RA, Austen WG. Left atrial calcification. Review of literature and proposed management. Circulation 1966;34:198-210.  Back to cited text no. 4
    
5.
Lahey T, Horton S. Massive left atrial calcification and devastating systemic emboli in a patient with chronic renal failure. Am J Kidney Dis 2002;40:416-9.  Back to cited text no. 5
    
6.
Jenkins NP, Brooks NH, Greaves M. Coconut atrium following thoracic radiotherapy. Heart 2004;90:1376.  Back to cited text no. 6
    
7.
Hosseini S, Rezaei Y, Samiei N, Sadeghpour A, Peighambari MM, Mestres CA, et al. Massive left atrial calcification: A case report and review of the literature. Gen Thorac Cardiovasc Surg 2017;65:653-6.  Back to cited text no. 7
    
8.
Shaw DR, Chen JT, Lester RG. X-ray appearance and clinical significance of left atrial wall calcification. Invest Radiol 1976;11:501-7.  Back to cited text no. 8
    
9.
Leacock K, Duerinckx AJ, Davis B. Porcelain atrium: A case report with literature review. Case Rep Radiol 2011;2011:501396.  Back to cited text no. 9
    
10.
Yokoyama N, Konno K, Suzuki S, Isshiki T. Images in cardiovascular medicine. Serial assessment of liquefaction necrosis of mitral annular calcification by echocardiography and multislice computed tomography. Circulation 2007;115:e1-2.  Back to cited text no. 10
    
11.
Vanovermeire OM, Duerinckx AJ, Duncan DA, Russell WG. Caseous calcification of the mitral annulus imaged with 64-slice multidetector CT and magnetic resonance imaging. Int J Cardiovasc Imaging 2006;22:553-9.  Back to cited text no. 11
    
12.
Tsumaru S, Minakata K, Yamazaki K, Nakamura M, Sakaguchi H, Sakata R, et al. Redo mitral valve replacement in patient with “coconut atrium”. Ann Thorac Surg 2015;99:1454.  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]
 
 
    Tables

  [Table 1]



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
   Abstract
  Introduction
  Case Report
  Discussion
  Conclusion
   References
   Article Figures
   Article Tables

 Article Access Statistics
    Viewed293    
    Printed20    
    Emailed0    
    PDF Downloaded42    
    Comments [Add]    

Recommend this journal


[TAG2]
[TAG3]
[TAG4]