Congenital aneurysm of the left atrial appendage
1 Department of Cardiology, Hospital Reina Sofía, Carretera Tarazona sn, Tudela, Navarra, España
2 Department of Cardiology, Hospital de Navarra, C/Irunlarrea 3, 31008 Pamplona, España
3 Department of Cardiac Surgery, Hospital de Navarra, C/Irunlarrea 3, Pamplona, España
Received 20 March 2007; accepted after revision 4 May 2007; online publish-ahead-of-print 16 July 2007.
* Corresponding author. Tel: +34 848 422 148; fax: +34 848 422 303. E-mail addresses: amunarra{at}cfnavarra.es, aitzibermu{at}mixmail.com (A. Munárriz), folazpre{at}cfnavarra.es
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Abstract |
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Congenital aneurysm of the left atrial appendage is quite infrequent. Most instances are asymptomatic. Patients can report a variety of symptoms, one of the most frequent being onset of auricular tachyarrhythmia. Various imaging techniques are useful in diagnosis and allow the differential diagnosis with other pathologies.
We describe the case of a 24-year-old male with congenital aneurysm of the left atrial appendage. The patient presented with auricular fibrillation. Diagnosis was based on transthoracic and trans-esophageal echocardiography, and the patient was treated by surgical resection of the aneurysm under extracorporeal circulation.
Keywords: Aneurysm; Left atrial appendage; Arrhythmia; Echocardiogram
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Introduction |
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Aneurysm of the left atrial appendage (LAA) is a very infrequent pathology. The majority of cases are congenital and become evident during the second or third decades of life. Such aneurysms most frequently present as auricular tachyarrhythmias or thromboembolic events. Resection of the aneurysm has proved to be an effective treatment which stops both auricular tachyarrhythmia and thromboembolisms.
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Case report |
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A 24-year-old man was referred to our cardiology department (Departments of Cardiology and Cardiac Surgery, Hospital de Navarra, C/Irunlarrea 3, Pamplona, España) after detection of auricular fibrillation by electrocardiography (ECG). From a cardiological perspective the patient was asymptomatic and in functional class I. He had no relevant antecedents and had undergone regular pediatric check-ups throughout childhood without anything untoward being detected. The results of physical examination were normal. ECG showed auricular fibrillation at 70 bpm with a vertical axis. A thoracic X-ray indicated a cardiothoracic index of 0.5 and, apart from a very prominent left auricular arch, was normal (Figure 1). The results of standard laboratory tests, including those for thyroid function, were normal.
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Transthoracic echocardiography showed a giant aneurysm of the LAA. The aneurysm measured 123 mm in the longitudinal axis in the subcostal window, with a transverse diameter of 58 mm in the apical window. The neck of the appendage was 25 mm in diameter. There was severe, spontaneous echo-contrast inside the appendage, although no clear image of thrombus.
Flow study of the LAA indicated that mechanical activity was conserved (filling wave was 63 cm/s and emptying wave was 46 cm/s). The left atrium (LA) was not dilated. The left ventricle (LV) was of normal size, systolic function was conserved, and there was paradoxical motion of the posterior and lateral faces relative to the LAA. The visceral layer of the pericardium appeared to be intact. The remaining aspects of the study were unremarkable (Figure 2).
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To complete the study we did transesophageal echocardiography with an omniplane transducer; the aneurysm of the LAA measured 117 mm x 53 mm (in the long, transgastric, axis) with a neck of 23 mm. There was severe spontaneous echo-contrast in LAA and LA, and pseudothrombus at the level of the base of the LAA. The mechanical activity of the LAA was conserved. There was a slight mitral insufficiency secondary to dilation of the mitral ring (39 mm), which was probably distorted by the aneurysm of the LAA (Figure 3).
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The patient was given anticoagulation treatment with Sintrom, and the aneurysm of the LAA was surgically resected after median thoractomy and under extracorporeal circulation (Figure 4).
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Anatomical pathology confirmed the diagnosis of aneurysm of the LAA. The myocardium had almost disappeared resulting in direct contact between the endocardium and the pericardium. There were foci of inflammation and fibrosis in the pericardium. The muscle fibres did not have morphological anomalies.
The postoperative evolution of the patient was satisfactory; the patient being in sinus rhythm after surgery. Postoperative transthoracic echocardiography found no anomalies. At a check-up two months after surgery, the patient was asymptomatic and in sinus rhythm.
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Discussion |
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Aneurysm of the LAA is a very rare pathology; there are about 50 cases described in the literature.1 The majority of these cases are congenital, although there are also reports of aneurysms acquired in relation to mitral valve pathology. The origin of congenital aneurysms has been attributed to dysplasia of the pectinate muscles and of the related bundles of muscles of the LA.2
Congenital aneurysms of the LAA are rarely diagnosed during childhood; generally they become manifest during the second or third decades of life. However, most cases are asymptomatic. Presentation symptoms include palpitations related to auricular arrhythmias, dyspnea, thoracic pain related to compression of the left coronary artery, and cerebrovascular incidents or sudden death related to systemic embolisms.3
Congenital aneurysms of the LAA are not linked with any other pathologies and so there are established criteria for the differential diagnosis with other pathologies such as congenital deficit of pericardium, mitral valve pathology or juxtaposition of appendages. These criteria are the presence of an LA of normal characteristics, a direct continuity of blood flow between the LA and the appendage, the absence of pericardial defects, and the presence of characteristics of a normal LAA in terms of anatomical pathology.4 The basis of diagnosis is imaging techniques, in particular, trans-thoracic and transesophageal echocardiography. Cardiac magnetic resonance can also play an important role in diagnosis.
Once the diagnosis has been established, aneurysms of the LAA must be treated because of the morbidity and mortality associated with the pathology.5 For congenital aneurysms the recommended treatment is resection, generally with extracorporeal circulation, after thoracotomy. Good surgical results have also been reported for resection after left thoracotomy and without extracorporeal circulation.6,7 Surgery, which has a level of risk, has proved effective in the prevention of both new auricular arrythmias and of systemic embolisms. There are reports in the literature of cases free of auricular arrythmias eight years after aneurysmectomy.8 Although resection alone is usually adequate, surgery is sometimes combined with the Cox-Maze procedure for ablation of auricular fibrillation; especially if there is dilation of the LA or if a previous electrophysiological study has shown induction of auricular fibrillation by auricular foci.8
In the case we report here, we opted for resection alone because there was no dilation of the LA. Surgery was by median sternotomy, under extracorporeal circulation. After the operation, the patient was asymptomatic and with a stable sinus rhythm.
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Tel: +34 848 434 000. 
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References |
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[Abstract/Free Full Text] - Gold JP, Afifi HY, Ko W, Horner N, Hahn R. Congenital giant aneurysm of the left atrial appendage: diagnosis and management. J Cardiac Surg (1996) 11:147–50.[CrossRef][Web of Science][Medline]
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[Abstract/Free Full Text] - Kiaii B, Doll N, Kueh M, Mohr FW. Minimal invasive endoscopic resection of a giant left atrial appendage aneurysm. Ann Thorac Surg (2004) 77:1437–8.
[Abstract/Free Full Text] - Tanoue Y, Kado H, Shiokawa Y, Sagawa K. Left atrial appendage aneurysm in a child. Ann Thorac Surg (2004) 77:721–3.
[Abstract/Free Full Text] - Mathur A, Zehr KJ, Sinak LJ, Rea. RF. Left atrial appendage aneurysm. Ann Thorac Surg (2005) 79:1392–3.
[Abstract/Free Full Text]
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