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Ann Thorac Surg 2001;72:2132-2134
© 2001 The Society of Thoracic Surgeons

Case report

Spontaneous intramural left atrial hematoma associated with systemic amyloidosis

Ko Watanabe, MD*a, Bruno Miguel, MDa, Jean L. Kemeny, MDa, Bernard Citron, MDa, Lionel F. Camilleri, MDa

a Department of Cardiovascular Surgery, Pr Charles de Riberolles, Gabriel Montpied University Hospital, Clermont-Ferrand, France

Accepted for publication February 3, 2001.

* Address reprint requests to Dr Watanabe, Chirurgie Cardio-Vasculaire, Hôpital Gabriel Montpied, Place Henri Dunant, BP 69, F-63003 Clermont-Ferrand Cedex 1, France
e-mail: bmiguel{at}chu-clermontferrand.fr


   Abstract
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 Abstract
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Spontaneous intramural left atrial hematoma is very rare. We describe a case of spontaneous intramural left atrial hematoma that had to be semiurgently resected. Postoperatively, the patient was diagnosed as having systemic immunocyte-derived (AL) amyloidosis, because of rare manifestations of fatal bleeding. Though spontaneous intramural left atrial hematoma is one of the severe complications of systemic AL amyloidosis, we believe that amyloid deposits caused fragility of the left atrial wall.


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Fatal hemorrhage is a rare clinical problem in patients with systemic amyloidosis although focal or generalized hemorrhage is common. We encountered a spontaneous intramural left atrial hematoma as a manifestation of severe bleeding.

A 70-year-old man was admitted to our hospital for acute pericarditis, complaining of dyspnea and chest pain. The patient had received an oral anticoagulant for suspected pulmonary embolism after an orthopedic operation. At admission a prothrombin test was performed with a result of 20% (International Normalized Ratio, 5.6).

Two days after admission, the patient suffered a sudden heart failure. A transesophageal echocardiogram revealed a 5.7 x 7.0 cm left intraatrial mass that adhered to the atrial septum and occupied the left atrium. A pericardial effusion of some importance induced compression of the right ventricle. Magnetic resonance imaging revealed an intracardiac mass in the left atrium, more likely to be a thrombus than a tumor (Fig 1).



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  		Fig 1. Preoperative magnetic resonance imaging demonstrating the mass resulting in a decrease of the left atrial cavity (arrow).

 
The patient underwent a semiurgent operation. Fibrins were diffusely attached to the surface of the pericardium and the pericardial effusion contained 300 mL of blood. We approached the left atrium using transseptal superior exposure. The mass was located in the intramural space, not in the intracardiac cavity. The left atrial cavity was filled almost entirely by the mass, which was attached to the septal wall, and extended through the mitral annulus to the pulmonary veins within the intramural space. Though the pulmonary veins were not involved, and no tear of the left atrial wall was observed, the infiltrated tissue was very fragile. It seemed that the hematoma had enlarged and delacerated the intramural space. After a pathologic diagnosis of the mass as a thrombus, we enucleated it. We resected fragile tissues from the left pulmonary veins to the right pulmonary veins, down to the mitral annulus, and reconstructed the left atrial wall with a pericardial patch. Postoperative convalescence was uneventful except for atrial fibrillation.

Pathologically, the mass was an encysted hematoma without proliferative cells. The atrial wall was infiltrated to the epicardium by amyloid, which was stained with Congo red and presented the characteristic apple-green birefringence when viewed under polarized light (Fig 2A). These deposits remained visible after they were treated with permanganate. Likewise, amyloid deposits were present in the thickened walls of some pericardial vessels (Fig 2B). Monoclonal immunoglobulin of IgG kappa was detected postoperatively in the serum by means of protein electrophoresis, though rectal biopsy proved negative.



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  		Fig 2. Amyloid deposits presenting characteristic birefringence in Congo red stain viewed under polarized light in the left atrial wall (x 140) (A), as well as in the thickened wall of epicardial vessels (arrow) (x 350) (B).

 
The patient had no family history of amyloidosis. An echocardiogram did not reveal ventricular wall thickening usually observed in patients suffering from systemic immunocyte-derived (AL) amyloidosis with heart involvement [1, 2]. Isolated factor X deficiency, which is one cause of the hemorrhage associated with amyloidosis, was not present [1, 3]. The patient was diagnosed as having systemic AL amyloidosis.

We have concluded that the intramural left atrial hematoma occurred spontaneously as a severe bleeding manifestation of systemic AL amyloidosis, though the patient was under anticoagulation therapy, suggesting that amyloid deposits caused fragility of the left atrial walls.

The patient has shown no signs of recurrence and remains well 9 months after the operation.


   Comment
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Spontaneous intramural left atrial hematoma is very rare. Intramural blood collection is thought to be caused by chest trauma, infectious endocarditis, mitral annular calcification, pericardial puncture, placement of a pacemaker, or complications after an operation, where anticoagulation therapy might be an additional factor [4, 5].

In spite of this, focal or generalized hemorrhage is a commonly encountered clinical problem in patients with amyloidosis [1, 2]. Yood and colleagues [3] reported that the frequency of bleeding problems in patients with amyloidosis was approximately 40%, with most cases being only mild hemorrhages. However, some cases were far more severe, to the point of being fatal, such as upper gastrointestinal bleeding and retroperitoneal bleeding.

Furthermore, Yood and colleagues [3] stated that bleeding occurred frequently, even when patients had no abnormalities of coagulation tests, and they suggested that amyloid infiltration of blood vessels with consequent increased fragility contributed to hemorrhaging in amyloidosis.

Our patient did not show an evident cause of preoperative hemorrhaging aside from the anticoagulation therapy. Postoperative diagnosis was systemic immunocyte-derived (AL) amyloidosis, without ventricular wall thickening, and electrocardiogram abnormality, although the rectal biopsy was negative.

We concluded that amyloid deposition, which contributed to left atrial wall fragility, in addition to anticoagulation therapy, was responsible for the spontaneous intramural left atrial hematoma. Throughout our investigation, there has been no other reports of spontaneous intramural left atrial hematoma associated with systemic amyloidosis.

We have experienced a very rare case of spontaneous intramural left atrial hematoma as one of the severe bleeding manifestations of systemic immunocyte-derived (AL) amyloidosis, implying that amyloid deposits induced the left atrial wall fragility. Nonetheless, hematoma resection, in the presence of a very fragile left atrial wall, followed by pericardial patch reconstruction, has proven to be successful.


   References
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 Abstract
 Introduction
 Comment
 References
 

  1. Pepys M.B. Amyloidosis, 4th ed, Vol 1. In: Samter M., ed. Immunological diseases. Boston/Toronto: Little, Brown and Company, 1988:631-674.
  2. Dubrey S.W., Cha K., Anderson J., et al. The clinical features of immunoglobulin light–chain (AL) amyloidosis with heart involvement. QJM 1998;91:141-157.[Abstract/Free Full Text]
  3. Yood R.A., Skinner M., Rubinow A., Talarico L., Cohen A.S. Bleeding manifestation in 100 patients with amyloidosis. JAMA 1983;249:1322-1324.[Abstract/Free Full Text]
  4. Jimenez J.F.D., Rufilanchas J.J., Pajuelo C.G. Spontaneous left atrial haematoma. Int J Cardiol 1991;31:353-356.[Medline]
  5. Schecter S.O., Fyfe B., Pou R., Goldman M.E. Intramural left atrial haematoma complicating mitral annular calcification. Am Heart J 1996;132:455-457.[Medline]



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