Ann Thorac Surg 2009;87:636-638. doi:10.1016/j.athoracsur.2008.06.019
© 2009 The Society of Thoracic Surgeons
Case Reports
Emergency Surgery for Acute Mitral Valve Obstruction Resulting From Hemorrhage Within a Left Atrial Myxoma
Uday Sonker, FRCS(I), FRCS(Ed)a,*,
Geoffrey T.L. Kloppenburg, MDa,
Esther A. Knoop, MDb,
Cornelis A. Seldenrijk, MD, PhDb,
Wim J. Morshuis, MD, PhDa
a Department of Cardiothoracic Surgery, St. Antonius Hospital, Nieuwegein, the Netherlands
b Department of Pathology, St. Antonius Hospital, Nieuwegein, the Netherlands
Accepted for publication June 2, 2008.
* Address correspondence to Dr Sonker, Department of Cardiothoracic Surgery, St. Antonius Hospital, Koekoekslaan 1, Nieuwegein, 3435 CM, the Netherlands (Email: usonker{at}hotmail.com).
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Abstract
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Left atrial myxomas are benign, slow-growing primary cardiac tumors. They present with gradual onset of one or more of a triad of obstructive, embolic, or constitutional symptoms. Transesophageal echocardiography aids in the detailed preoperative and intraoperative evaluation of the myxoma for surgical strategy planning. We describe a previously unreported case of interstitial hemorrhage in a left atrial myxoma leading to rapid expansion of the tumor with features of acute, mitral valve obstruction. Transesophageal echocardiography showed a cystic area in the left atrial tumor that corresponded to an area of recent hemorrhage confirmed on surgical removal.
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Introduction
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Cardiac myxomas are the most common benign primary tumors of the heart. The onset of obstructive symptoms depends on the size and mobility of the tumor. We present a case of left atrial myxoma complicated by hemorrhage within the tumor, which led to rapid development of severe obstructive symptoms. Transesophageal echocardiography showed a tumor mass with a hypo-echogenic area that was obstructing the mitral valve orifice. Intraoperative and histologic examination confirmed a diagnosis of acute hemorrhage within a myxoma.
A 40-year-old, previously healthy, man was referred to us with a 4-day history of sudden onset dyspnea. On examination, central venous pressure was elevated and bilateral basal rales were present. No cardiac murmurs were heard. Systemic examination was otherwise unremarkable.
Congested lung fields were present on chest roentgenogram. Transthoracic and transesophageal echocardiography showed a mobile, pedunculated mass attached to the interatrial septum in the left atrium, measuring approximately 4 x 5 cm. A hypo-echogenic area that resembled a cyst was seen within this mass (Fig 1). The mass moved into the mitral orifice during atrial systole, thus partially obstructing it.
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Fig 1. Transesophageal echocardiogram showing tumor with cystic area attached to the interatrial septum and occluding the mitral valve. (B = area of bleeding; IAS = interatrial septum; IVS = interventricular septum; LA = left atrium; LV = left ventricle; MV = mitral valve; My = myxoma; P = pedicle.)
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The patient underwent emergency surgery for tumor resection. The right ventricle was distended with decreased contractility. The right atrium was not enlarged. Cardiopulmonary bypass with cold crystalloid cardioplegic arrest and topical cooling was instituted. Atrial incision (Guiraudon) was made beginning at the roof of the left atrium. The interatrial septum was incised under guidance of the palpating, left index finger of the surgeon. The tumor pedicle was attached to the foramen ovale. It was completely excised along with a rim of surrounding atrial septum. The defect thus created was primarily closed. Intraoperative transesophageal echocardiography showed good mitral valve function and no other abnormality. The mass measured 6 x 5 x 4 cm, with mucoid, smooth surface showing an area of bluish discoloration. When sectioned, approximately 15 mL of bloody fluid was found in that area of the tumor (Fig 2).
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Fig 2. Pedunculated, smooth-surfaced tumor with areas of hemorrhage. (b = areas of blood collection; p = pedicle.)
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Microscopic examination showed a smooth surface with endothelial lining and abundant myxoid matrix (Fig 3). Extensive hemosiderin deposition, degenerative elastic tissue, and calcification, and so-called gamma bodies were present. Vascular structures and adjoining areas of bleeding with signs of active inflammatory reaction and no signs of organization were seen. No mitotic figures were identified. These histologic features are consistent with recent bleeding within a myxoma.
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Fig 3. Hematoxylin & eosin stained sections (original magnification x200) through the tumor mass showing (A) myxoid matrix with polygonal cells deposition of hemosiderin pigment (arrow) as a sign of chronic bleeding and presence of erythrocytes as a sign of recent bleeding. (B) Myxoid matrix in which some bleeding (a), and degenerative elastin fibers with calcification (gamma bodies) (b).
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Postoperatively the patient required diuretic therapy to optimize arterial oxygen saturations. Further postoperative recovery was uneventful. There was no recurrence of the myxoma on follow-up.
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Comment
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We present a case of mitral valve obstruction due to an acute bleeding in a myxoma requiring emergency surgery. Acute bleeding into the tumor caused a sudden increase in tumor size, now large enough to partially occlude the mitral valve annulus, which gave rise to the patient's acute and rapid deterioration of symptoms.
Primary tumors of the heart are rare, nearly 75% of them are benign and almost half of these are myxomas [1]. Since the availability of two-dimensional echocardiography from 1977 onward, the median delay before surgery after development of symptoms was 3 months [2]. Most patients present with at least one or more symptoms of the classic triad of obstructive cardiac signs, embolic signs, or constitutional and systemic manifestations. Locally the tumor may cause hemodynamic derangement due to either obstruction to flow within the cardiac chambers or the deformation of valves. Myocardial ischemia may be the presenting symptom. Constitutional symptoms are believed to be secondary to the production of interleukin-1, interleukin-6, and interleukin-8. Distant complications are due to emboli that may occur in any vascular bed, leading to a variety of signs and symptoms [2–4].
Clusters of small and tortuous vessels arising from the coronary arteries (tumor vascularity) have been visualized within myxomas [5]. In more than half of the tumors, extensive scarring that replaces myxoid areas, calcification, and metaplastic bone may be present. Hemorrhage and organizing thrombus result in abundant hemosiderin laden macrophages. In 10% of tumors, elastic tissue and calcification surround the hemosiderin, resulting in gamma bodies [4]. Multifocal and recurrent diseases are believed to have familial and genetic predisposition [6, 7]. Local recurrence usually occurs due to inadequate surgical resection.
Transesophageal echocardiography shows finer details and is considered superior to transthoracic echocardiography in the diagnosis of intracardial and pericardial tumors [8]. Postoperative management should include periodic echocardiography for several years, even though recurrence after adequate surgical resection is rare.
We report an unusual case of a left atrial myxoma complicated by acute hemorrhage within the tumor, successfully treated by emergency surgery. We believe that this presentation has not been reported previously. This report confirms the value of transesophageal echocardiography in the detailed preoperative and intraoperative evaluation of atrial myxoma for planning the surgical strategy.
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References
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- Silver MD, Gotlieb AI, Schoen FJ. Cardiovascular pathology3rd edition. Philadelphia, PA: Churchill Livingstone; 2001. pp. 591-595.
- Chow WH, Chow TC, Tai YT, Yip AS, Cheung KL. Angiographic visualization of "tumor vascularity" in atrial myxoma Eur Heart J 1991;12:79-82.[Abstract/Free Full Text]
- Shinfeld A, Katsumata T, Westaby S. Recurrent cardiac myxoma: seeding or multifocal disease? Ann Thorac Surg 1998;66:285-288.[Abstract/Free Full Text]
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- Engberding R, Daniel WG, Erbel R, et al. Diagnosis of heart tumors by transesophageal echocardiography: a multicentre study in 154 patients Eur Heart J 1993;14:1223-1228.[Abstract/Free Full Text]
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March 15, 2011;
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Abstract
Introduction
