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Ann Thorac Surg 2002;74:1691-1694
© 2002 The Society of Thoracic Surgeons


Case report

Paroxysmal postural dyspnea related to a left atrial ball thrombus

Daniel Grandmougin, MDa*, Thierry Letourneau, MDb, Jean-Pierre Favre, MDa, Xavier Barral, MDa

a Department of Cardiovascular Surgery, Hôpital Nord-CHU, Saint-Etienne, France
b Department of Echocardiography, Hôpital Cardiologique-CHU, Lille, France

Accepted for publication May 30, 2002.

* Address reprint requests to Dr Grandmougin, Service de Chirurgie Cardiovasculaire, Hôpital Nord-CHU, Pr X. Barral, 42055 Saint-Etienne, Cedex 2, France.
e-mail: daniel-grandmougin{at}wanadoo.fr


    Abstract
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 Abstract
 Introduction
 Comment
 References
 
We report herein an uncommon clinical observation of a 82-year-old woman with paroxysmal postural dyspnea related to a giant ball-thrombus located in the left atrium and partly protruding through the mitral orifice. No mitral stenosis was otherwise disclosed. The patient had a previous medical history of chronic atrial fibrillation without any anticoagulant therapy. The atrial mass was easily removed and the postoperative course was uneventful. Disclosure of such a free-floating ball-thrombus in the left atrial cavity requires prompt surgical treatment because of high risks of acute hemodynamic decompensation due to obstruction of the left ventricular inflow or, more rarely, systemic embolic events.


    Introduction
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 Abstract
 Introduction
 Comment
 References
 
Thrombus in the left atrium is a well-known pathology [1] and commonly disclosed by echography in mitral valve disease, particularly in left atrial appendage, in patients with atrial fibrillation. On the other hand, ball-thrombus is a rare and specific entity, generally associated with mitral stenosis [2]. This particular disorder may produce severe complications such as sudden death due to mitral valve orifice occlusion. Even if previously described in cases of atrial thrombus [3], systemic embolization specifically related to a ball-thrombus is rare [4] because of an endothelialized surface and enclosure by the restricted mitral orifice. We report an uncommon case of a patient with a paroxysmal and postural dyspnea related to a giant ball-thrombus located in the left atrium, requiring urgent surgical management because of a high propensity to protrude through the mitral orifice.

A 82-year-old woman with a medical history of chronic atrial fibrillation, was urgently referred for surgical management of an atrial myxoma. The patient was otherwise healthy, except for obesity (96 kg/160 cm). Despite chronic atrial fibrillation (at least 12 years), no oral anticoagulant therapy had been administered. The patient related a recent and unusual paroxysmal postural dyspnea, worsened by minimal exertion and significantly decreased by decubitus.

On admission, the patient was apyretic, and physical examination was normal with muffled heart sounds because of obesity and irregular pulses. A discrete systolic murmur was focused in the median axillary line in the fourth left intercostal space. The electrocardiogram assessed atrial fibrillation with no low-voltage QRS complexes. Chest roentgenogram showed a cardiomegaly (cardiothoracic ratio: 0.56) with a moderate left atrial enlargement and without any increased pulmonary vasculature. Echocardiography (transthoracic and transesophageal) disclosed a giant spherical and free-floating mass approximately 4 cm in diameter (Fig 1A), filling the left atrium, bouncing and rebounding off the mitral valve, with a partial and intermittent protrusion through the annulus (Fig 1B). No evidence of septal or wall attachment was found. The mitral valve was thin without any stenotic mechanisms, however, with a trivial leak. The left atrium was enlarged (50 mm). The mass was smooth, homogenous, without any calcifications, and well demarcated. Neither tissular invasion nor infiltration were disclosed. The aortic valve was competent without any gradient, and the left ventricle was free of thrombus.



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Fig 1. Transesophageal echocardiogram showing a free-floating ball-thrombus (BT, dotted line) approximately 4 cm in diameter (A) filling the left atrium (LA) and (B) rebounding off the mitral valve (MV) with a partial and intermittent protrusion through the annulus. (LV= left ventricle.)

 
Because of a high risk of obstruction of the mitral orifice, the patient was urgently scheduled for surgery. The intervention was carried out through a median sternotomy with a normothermic cardiopulmonary bypass. Cardiac arrest and myocardial protection were obtained with antegrade cold blood cardioplegia. The left atrium was opened in the Sondergaard plane. Surgical findings confirmed echocardiographic data showing a perfectly spherical and totally free mass, rolling inside the atrial cavity, that was very easily removed (Fig 2). No endothelial wall injury was detected. The mitral valve was within the limits of normality, considering the age of the patient. No stenotic aspect was disclosed, and valvular competence was tested with saline solution. Weaning off cardiopulmonary bypass with a total cross-clamping time of 30 minutes was uneventful. The patient was discharged on the 10th postoperative day, with oral anticoagulant therapy because of the persistent chronic atrial fibrillation.



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Fig 2. Operative view showing a complete, free, spherical, smooth mass located in the left atrium. Ball-thrombus after removal (inset).

 
Histologic study eliminated a myxoma, and confirmed a thrombus with a superficial endothelial layer without any disruption of its integrity.


    Comment
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 Abstract
 Introduction
 Comment
 References
 
Left atrial thrombi are not infrequent in patients with mitral valve disease [5]. Incidence may dramatically increase when mitral valve disorder is associated with atrial fibrillation or an enlarged left atrium. Specific conditions are also well known to further the occurrence of left atrial thrombi [6]. A ball-thrombus has to be considered a specific subgroup of thrombi with specific behavior (mitral orifice protrusion) and clinical consequences, but also with the main macroscopic characteristics of spherical shape and smooth structure. As a well-demarcated mass, it might sometimes mimic a myxoma, with an absence of attachment to the septum or, more rarely, to the mitral valve itself, known as the usual macroscopic criteria for diagnosis of this latter tumor.

In our case, this echographic feature was an important step to invalidate the suspicion of isolated left atrial myxoma. The potential for complications has been established recently by Abe and colleagues [7], who correlated systemic embolism in atrial fibrillation with the shape, site, mobility, number, and maximum dimension of left atrial thrombi. Thus, independently of other criteria, mobility of the thrombus remains the most important risk factor for cerebral or arterial embolic events. Transthoracic echocardiography optimized by a transesophageal analysis appears to be the main, primary, noninvasive tool for diagnosis of this particular thrombus; it is also used to assess the potential of complications, although incidental diagnosis has been established by routine echographic examination as a prelude to surgery. However, as previously related [4], when considering macroscopic characteristics of a ball-thrombus, embolism usually is not associated with this specific subgroup of thrombi. This paradoxical specificity is probably related to an endothelial-like superficial layer, coating the surface of ball-thrombi, whose integrity may reduce the propensity to aggregate platelets. Furthermore, the spherical shape of the ball-thrombus may prevent mechanical injuries, as it rolls like a pebble in the left atrial cavity.

If low pressure in the left atrium, emphasized by decreased atrial transport due to fibrillation, induces a pejorative blood stagnation leading to thrombus formation, it may also decrease the propensity of mechanical collision between the thrombus and the atrial wall. Thus, the formation of a ball-thrombus may result from an initial free-floating or mural microthrombus, with an accretion phenomenon gradually enlarging the mass like a growing snowball. Thus, if a rolling spherical shape resulting from chronic blood stagnation appears to be partly a parameter preventing an embolism, it also should be considered as the growth factor of the ball-thrombus.

Therefore, the initial microthrombus step is dominated by the risk of embolism [3], whereas the major consequence of a single free-floating ball-thrombus is acute hemodynamic decompensation, even sudden death due to the left ventricular inflow obstruction, the so-called "hole-in-one thrombus" effect [8]. In the case of concomitant ventricular location, risk of sudden death appears to be dramatically increased by a "hole-in-two thrombi" effect, as was reported [9]. However, ball-thrombus and embolic events have been reported [4] and result from either fragmentation of the ball-thrombus as it is traumatized by the valve during the ventricular systole, or the atrium itself independently from the ball-thrombus, then depending on previous mitral surgery or chronic atrial fibrillation.

Our observation is rare as it is associated with a moderate mitral leak without any stenotic disease. Therefore, atrial fibrillation remains the prime mover of the physiopathogenic mechanism. We further speculate that postural dyspnea clearly results from an increased mobility towards the mitral valve during verticalization of the patient.

Therapeutic strategy depends on several factors mainly represented by: (1) echographic characteristics; (2) clinical state and symptoms; and (3) previous medical history (mitral valve disease, recent valve surgery, atrial fibrillation, or giant left atrium).

Single anticoagulant therapy may be an interesting and successful option [10], nevertheless requiring a regular echographic follow-up to control the outcome of the thrombus and assess its disappearance. However, considering the potential complications, surgical management remains the main option. We believe that detection of a ball-thrombus in the left cavity requires urgent surgical removal, particularly when the following pejorative echographic criteria are present: (1) surgical mitral valve disease; (2) enlarged or giant left atrium; (3) atrial fibrillation; (4) complete mobility; (5) diameter greater than 1 cm; (6) multiple ball-thrombi; and (7) left ventricular location ("hole-in-two thrombi" effect).

In addition to the thrombus removal, the surgical procedure may include complementary steps depending on the preoperative findings. Thus, left atrial reduction, mitral valve replacement or repair, atrial radiofrequency, or Cox Maze procedures have to be considered.


    References
 Top
 Abstract
 Introduction
 Comment
 References
 

  1. Jordan R.A., Scheifley C.H., Edwards J.E. Mural thrombosis and arterial embolism in mitral stenosis. A clinicopathological study of 51 cases. Circulation 1951;3:363-367.[Medline]
  2. Vitale M., Agnino A., Serena D., et al. Asymptomatic large left-atrial ball-thrombus. Secondary to mitral stenosis. Tex Heart Inst J 1997;24:376-378.[Medline]
  3. Sherman W., Nozad S.E., Stoian A., Madias J.E. Free-floating left atrial thrombus and systemic embolization. Chest 1985;87:694-695.[Abstract/Free Full Text]
  4. Furui E., Hanzawa K., Hoshiyama M., Nakajima T., Fukuhara N. Cerebral embolism due to left atrial ball thrombus without mitral stenosis—usefulness of the transesophageal echocardiography for the diagnosis. Rinsho Shinkeigaku 1998;1:3813-3816.
  5. Manga P., Pocock W.A. Two-dimensional and Doppler echocardiographic features of a left atrial thrombus. Echocardiography 1997;14:383-386.[Medline]
  6. Sanae T., Kazama S., Nie M., et al. Free-floating left atrial thrombus early after mitral valve replacement. Ann Thorac Cardiovasc Surg 2000;6:408-410.[Medline]
  7. Abe Y., Asakura T., Gotou J., et al. Prediction of embolism in atrial fibrillation: classification of left atrial thrombi by transesophageal echocardiography. Jpn Circ J 2000;64:411-415.[Medline]
  8. Lie J.T., Entman M.L. "Hole-in-one" sudden death: mitral stenosis and left atrial ball-thrombus. Am Heart J 1976;91:798-804.[Medline]
  9. Ying-Siu Lee A., Chich-Kuang Chang M., Tien-Jen C., Wen-Fung C. Left atrial and ventricular ball thrombi complicating rheumatic heart disease with combined mitral and aortic stenosis. Echocardiography 2001;18:159-161.[Medline]
  10. Nagaraja Kamalakar K.V., Jaishankar S., Venkateswara Rao C., et al. Free-floating ball thrombus in left atrium. Echocardiography 1998;15:377-380.[Medline]



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