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�:Left atrial myxoma associated with advanced atrioventricular block in a very elderly woman
Introduction
Primary cardiac tumours are rare and more than 90% of them are benign. Nearly 80% of benign forms are myxomas that usually develop in the atria, 75% originating in the left atrium and 15-20% in the right atrium. They occur in all age groups, most frequently between third and sixth decade, while it is rare to find them beyond the ninth decade. Women are more commonly affected. We describe the case of a 92-year-old woman with a left atrial myxoma extending toward the region of atrioventricular (AV) node, leading to advanced AV block/dissociation with narrow QRS complexes, likely due to the mechanical compression/infiltration on the AV node. AV block associated with left atrial myxoma is a rare finding ad has never been described in a so old patient up to now. We discuss our report in the context of available literature and underlie the peculiarities of the case.
Case description
A 92-year-old woman was admitted to our Unit because of advanced AV block with narrow QRS complexes symptomatic for fatigue and dyspnea. Her past medical history was characterized by arterial hypertension, type 2 diabetes, high cholesterol levels, Chronic Obstructive Pulmonary Disease (COPD), renal failure (estimated glomerular filtration rate about 30 ml/min � stage III kidney damage-). Her drug therapy was: Aspirin 100 mg daily, Furosemide 50 mg daily, Amlodipine 5 mg daily, Glibenclamide 10 mg daily. About 1 year before a cardiac tumour had been found in another hospital, with a final diagnosis of left atrial myxoma. Metastatic disease from malignancies, vegetations and thrombi had been ruled out; the transesophageal echocardiographic features of the mass strongly suggested a myxoma rather than other types of primary cardiac tumours. Because of age and comorbidities the patient did not underwent surgical excision of the tumour. Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) were not performed because of patient�s refusal (she was claustrophobic) but also for history of allergy to contrast medium and concern for Nephrogenic Systemic Fibrosis linked to exposure to gadolinium-based magnetic resonance contrast media. 
On admission in our Unit patient was in NYHA (New York Heart Association) class II; physical examination showed a loud split S1, diastolic murmur and an audible tumor �plop�. At blood exams creatinine levels were stable (1.9 mg/dl), and serum electrolytes levels were normal. The ECG showed phases of Mobitz I AV block, 2:1 block and AV dissociation with narrow QRS complexes and escape ventricular rhythm 40 beats per minute (Figure 1); sometimes 1:1 AV conduction spontaneously resumed with evidence of first degree AV block. Transthoracic echocardiogram showed a large, non pedunculated mass in the left atrium; a new transesophageal echocardiogram was performed to better delineate its nature and extension. The mass was ovoid (40 x 21 mm), echodense and non mobile, located in the left atrium, attached to interatrial septum in the lower portion of the septum primum; it extended posteriorly, to the right side of the interatrial septum and toward the atrioventricular ring compressing the region of the atrioventricular node. The left side location and its consistency were highly suggestive of left atrial myxoma (figures 2A-2B). Again, a decision was made to avoid surgical excision of the tumour. A dual chamber pacemaker was implanted without complications; both leads were inserted in the left cephalic vein. During the procedure AH interval (from atrial to His bundle electrical activation) and HV interval (from His bundle to ventricular activation) were measured during a phase of 1:1 AV conduction: AH was significantly prolonged (>350 msec), while HV was normal (45 msec); these findings supported the hypothesis of a direct compressive and/or local infiltrative effect of the tumour on the AV node. At six month follow up the patient was in satisfactory clinical condition, without symptoms at rest.
Discussion
When a mass inside the heart is diagnosed it usually represents a thrombus or a vegetation. If a cardiac mass is indeed a tumour the most likely cause is a metastatic process from remote malignancy (usually cancers from breast, lung or malignant melanoma). Primary cardiac tumours are rare: autopsy frequency ranges between 0.001% and 0.03%. Only 10% of primary cardiac tumours are malignant (expecially sarcomas), while 90% of them are benign, with nearly 80% of these benign forms representing myxomas [1]. Cardiac myxomas usually develop in the atria, 75% originating in the left atrium and 15-20% in the right atrium. They occur in all age groups, most frequently between third and sixth decade, while it is more rare to find them in the ninth decade. Women are more commonly affected [1, 2]. Myxomas characteristically arise from or near the interatrial septum at the border of the fossa ovalis membrane. Clinical manifestations depend on the size and chamber in which they are located, and include heart failure, embolization, rhythm disturbances, syncope and even sudden death [1, 2, 3]. Atrioventricular block has been already described in patients with metastatic tumours of the heart, �cor adiposum�, hematoma of interatrial septum, tubercoloma, intracardiac bronchogenic cyst [4, 5, 6, 7, 8]. In the setting of atrial myxoma first degree AV block is relatively common; a case of  AV dissociation has recently been reported in 18-year-old boy with a bi-auricular myxoma [9], but advanced AV block or dissociation has never been described in a so old patient up to now, according to our knowledge. The most commonly accepted mechanism linking myxoma and AV block is a compressive effect on the AV node, but a local infiltration cannot be ruled out even if the tumour is �benign�, due to �infiltrative�\properties of the tumour mimicking a malignant form [1]. In our report we observed a prolonged AH interval (a marker of AV node conduction properties) while HV interval was normal (so hisian and infra-hisian conduction was preserved): these findings supported the hypothesis of a compressive/locally infiltrative effect of the tumour on the AV node. A peculiarity of our case was the occurrence of AV block with a left atrial location of the tumour: actually, the AV node is usually a right-sided structure and direct compression can be more easily explained in the event of a cardiac mass located in the right atrium, as already reported in literature [7, 8, 9]. A myxoma located in the left side of the interatrial septum can compress the His bundle or the slow AV nodal pathway (that may have left-sided extension near the postero-septal mitral annulus); in our case echocardiographic examination showed that the mass had a right side and posterior extension toward the region of the compact atrioventricular node, thus explaining this apparent discrepancy.
The main limitation of our work is the absence of histological confirmation of the diagnosis because the patient did not undergo surgical intervention; moreover we could not perform neither CT nor MRI for a more precise definition of location and extension. However, when clinical and echocardiographic features strongly support the diagnosis of myxoma (in term of location and echocardiographic appearance) additional imaging with CT and MRI may be unnecessary and a reasonable diagnosis can be made even in the absence of histology based findings [1]. 














References
1. Bruce CJ (2011) Cardiac tumours: diagnosis and management. Heart 97: 151-160.
2. Burke A, Jeudy J Jr, Virmani R (2008) Cardiac tumours: an update. Heart 94: 117-123.
3. Auger D, Pressacco J, Marcotte F, Tremblay A, Dore A et al. (2011) Cardiac masses: an integrative approach using echocardiography and other imaging modalities. Heart 97: 1101-1109.
4. Almange C, Lebrestec T, Louvet M, Courgeon P, Guerin D et al. (1978)  HYPERLINK "/pubmed/219501"Complete atrio-ventricular block caused by a metastatic tumor of the heart. Apropos of a case. Sem Hop 54: 1419-24.
5. Spain DM, Cathcart RT (1946)  HYPERLINK "/pubmed/20274725"Heart block caused by fat infiltration of the interventricular septum (cor adiposum). Am Heart J 32: 659-64. 
6. Miyazaki T, Shiotani H, Kado T, Kazumi T, Matsunaga K et al. (1990)  HYPERLINK "/pubmed/2232118"Hematoma of the interatrial septum associated with complete atrioventricular block. Jpn Circ J 54: 636-43.
7. Ngow HA, Wan Khairina WM (2010)  HYPERLINK "/pubmed/20971714"Isolated right atrium tuberculoma causing complete heart block. Postgrad Med J 86: 624-6. 
8. Mart�nez-Mateo V, Arias MA, Ju�rez-Tosina R, Rodr�guez-Padial L (2008)  HYPERLINK "/pubmed/18326510"Permanent third-degree atrioventricular block as clinical presentation of an intracardiac bronchogenic cyst. Europace 10: 638-40. 
9. Tantchou Tchoumi JC, Giamberti A, Cirri S, Butera G (2012)  HYPERLINK "/pubmed/22059857"Bi-auricular myxoma associated with atrioventricular dissociation in an 18-year-old boy: a case report. Cardiol Young 22: 341-3.






Figure legends
Figure 1. ECG showing advanced atrioventricular block/dissociation with narrow QRS complexes and phases of first degree atrioventricular block. 

Figure 2. Transesophageal echocardiogram view showing ovoid mass (40 x 21 mm), echodense and non mobile, located in the left atrium, attached to interatrial septum in the lower portion of the septum primum and extended posteriorly toward the atrioventricular ring. The left side location and its consistency were highly suggestive of left atrial myxoma.

























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