Interact CardioVasc Thorac Surg 2009;9:913-915. doi:10.1510/icvts.2009.212514
© 2009 European Association of Cardio-Thoracic Surgery
Case report - Cardiac general |
Catastrophic presentation of atrial myxoma with total occlusion of abdominal aorta
Sumit Yadava,* and
John M. Alvarezb
a Department of Cardiothoracic Surgery, The Townsville Hospital, 100 Angus Smith Drive, Doulas, Townsville, QLD 4814, Australia
b Department of Cardiothoracic Surgery, Sir Charles Gairdner Hospital, Nedland, Perth, WA 6009, Australia
Received 23 May 2009;
received in revised form 8 July 2009;
accepted 13 July 2009
*Corresponding author. Tel.: +61 7 47961272; fax: +61 7 47961221.
E-mail address: sirzon{at}yahoo.com (S. Yadav).
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Abstract
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Cardiac myxoma is a rare disease with protean manifestations. Embolic phenomena are well-known entities. However, total occlusion of the descending aorta by saddle embolism along with multi-organ embolism is very unusual. We report a patient with cardiac myxoma presenting with multi-organ embolism including saddle embolism of the aorta in a previously healthy female.
Key Words: Cardiac myxoma; Peripheral embolism; Aortic occlusion
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1. Introduction
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Cardiac myxoma is a rare disease with a variety of non-specific clinical manifestations that often masquerade as many other more common diseases [1]. Systemic embolism caused by necrotic tumour fragments or thrombi from the surface of the myxoma is a well-known entity [2, 3]. Rarely, it can present as saddle aortic embolism [2–5]. We present a patient with a cardiac myxoma who presented with multi-embolism to the brain, heart, lungs and abdominal aorta.
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2. Case report
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A 62-year-old woman presented to the emergency department complaining of sudden onset of aches and pains in the arms and legs and double incontinence following ingestion of three tablets of Tricyclic antidepressant (Dothiepin, 75 mg). The patient had been previously healthy apart from a history of endogenous depression which had been successfully treated. On admission the patient was agitated, hemodynamics were normal with all peripheral pulses present. She developed acute unheralded neurological compromise requiring emergency intubation. Relevant investigations revealed a right bundle branch block (RBBB) on electrocardiogram (ECG), developing acute pulmonary edema on chest X-ray (CXR), an increasing troponin T from 0.3 µg/l on admission to 2.4 µg/l 8 h later. A computed tomography (CT) scan of brain did not show any gross abnormality. She was in low cardiac output state and was requiring ionotropic support to maintain her circulation. A transthoracic echocardiogram (TTE) revealed a 4.8x2.2 cm mass arising from the left atrium (Fig. 1) with poor left ventricle (LV) contractility and a small patent foramina ovale (PFO). At that stage, she developed mottling of the skin of her lower limbs and she developed absence of femoral pulses bilaterally.
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Fig. 1. Transthoracic echocardiogram showing a 4.8x2.2 cm mass in the left atrium which is straddling across the mitral valve.
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A CT-angiogram revealed a filling defect in left atrium, a total occlusion of the infrarenal abdominal aorta up to the femoral arteries (Fig. 2), pulmonary emboli, and likely renal and splenic infarcts. CT coronary angiogram did not reveal any significant focal coronary stenosis or thrombus in the proximal part of the coronaries.
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Fig. 2. Computed tomographic angiogram showing total occlusion of the infra renal abdominal aorta up to the femoral arteries.
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Vascular surgical opinion was emergently consulted. This patient was in extremis but unless the abdominal aorta could be cleared and, thus, flow restored to the lower body, removal of the myxoma with central cardiopulmonary bypass (CPB) was futile. The CPB circuit was primed and ready in preparation for any untoward event from sudden reperfusion to the lower body from restoration of abdominal aortic flow. The patient underwent emergent bilateral aortoiliac catheter embolectomoies and leg fasciotomies with restoration of palpable pedal pulses. Intraoperative aortography revealed a patent aorta, iliac and femoral arteries. At surgery the embolus was found to be of pale gelatinous material rather than a thrombus. During the operation transesophageal echo confirmed the presence of left atrium (LA) myxoma and severe impairment of LV function.
There were no immediate adverse hemodynamic events from restoration of aortic flow and prompt CPB was then established via a sternotomy. The myxoma and the part of interatrial septum were excised through a biatrial approach and the septum was reconstructed with a bovine pericardial patch. Histopathology of the removed mass revealed myxoma with superimposed thrombus.
Over the next two days she had gradual hemodynamic improvement. However, both legs became gangrenous and on the third day she underwent a right above-knee and a left below-knee amputation. On weaning off the sedation she woke up with left hemi-paresis and she was ventilator dependent. A CT-scan of the head done at 9 days post surgery showed extensive embolic infarction throughout the brain. Prior to this illness the patient expressed her wish not to be a dependent/nursing home occupant and this seemed unlikely in the setting of current events and so in consultation with her family, care was withdrawn and she died on the 15th day after surgery.
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3. Discussion
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Atrial myxomas have been described as a proliferation of primitive mesenchymal cells that normally differentiate into endothelial and smooth muscle cells and fibroblasts [1]. Atrial myxomas represent roughly 50% of all benign cardiac tumours. Myxomas can present with mechanical obstruction of blood flow, embolic phenomenon, and constitutional symptoms and signs. Embolic phenomena with cardiac myxoma are common, with an incidence of 30–40%. The emboli generally display a predilection for the brain, but also can affect other organs, such as the liver, spleen, kidney, retina, coronary arteries and the peripheral arteries [2–4]. Large saddle emboli in the abdominal aorta have been reported previously and may occur without preceding symptoms [5, 6]. Total detachment of cardiac myxoma causing saddle embolisation of abdominal aorta has also been rarely reported [7, 8]. Total occlusion of the aorta with myxoma has been reported to have presented as clinical features mimicking aortic dissection [9]. Early diagnosis may significantly reduce the morbidity of arterial occlusion, improve efficacy, and improve complications of reperfusion.
The diagnostic and surveillance method of choice is echocardiography [8]. CT-angiogram can provide additional information about the complications associated with myxomatous emboli as in this case it excluded obstructive coronary disease and delineated extent and size of emboli to lung and gastro intestinal (GI) tract. This patient had no previous medical conditions, constitutional symptoms, or obstructive symptoms before her embolic event, which serve as a reminder that the diagnosis of embolic myxoma should be suspected in younger, previously healthy patients presenting with suspected arterial embolism.
Our patient developed a shower of emboli. Some of it went through the patent foramina ovale and resulted in pulmonary embolism, though the possibility of total detachment of a separate right atrial myxoma cannot be ruled out. She may have had emboli to her coronaries, which may explain the raised troponin T, and severe left ventricular dysfunction. But the most catastrophic event was acute occlusion of the aorta by a large saddle embolus from the myxoma. The presenting symptoms of vague leg and arm pains and history of drug overdose made this diagnosis initially seem unlikely. The transthoracic echo and the disappearance of pulses in both the legs resulted in suspicion of myxomatous emboli.
In conclusion, this case illustrates the importance of careful clinical examination and early use of appropriate imaging tools. A diagnosis of myxoma should be considered in all cases of embolisation, particularly if more than one site might be involved. A prompt diagnosis, and therefore, a prompt combined and complete surgery procedure, could enhance clinical results avoiding late embolisation. The outcome in this case, despite prompt intervention with embolectomy and atrial myxoma removal, illustrates the critical nature of this disease.
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References
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Abstract
1. Introduction