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Congenital Aneurysm of the Left Atrial Appendage

2008; Elsevier BV; Volume: 85; Issue: 6 Linguagem: Inglês

10.1016/j.athoracsur.2007.12.064

1552-6259

A. de la Fuente, Arancha Urchaga, Ramón Sánchez, J Urman Fernández, Ignacio Moriones,

Cardiac Arrhythmias and Treatments

Aneurysm of the left atrial appendage (LAA) is very infrequent. We present the case of 24-year-old man in functional class I with atrial fibrillation but no other symptoms. Radiology showed an abnormality in the outline of the heart. Echocardiography revealed left appendage aneurysm. After median sternotomy, aneurysmectomy was performed under cardiopulmonary bypass without cross clamping. Atrial fibrillation ceased as soon as the aneurysm had been removed. There were no postoperative complications. Three months later the patient remained asymptomatic and in sinus rhythm. Aneurysm of the left atrial appendage (LAA) is very infrequent. We present the case of 24-year-old man in functional class I with atrial fibrillation but no other symptoms. Radiology showed an abnormality in the outline of the heart. Echocardiography revealed left appendage aneurysm. After median sternotomy, aneurysmectomy was performed under cardiopulmonary bypass without cross clamping. Atrial fibrillation ceased as soon as the aneurysm had been removed. There were no postoperative complications. Three months later the patient remained asymptomatic and in sinus rhythm. Congenital aneurysm of the left atrial appendage is extremely rare because since the first case, described by Diamond in 1960 [1Diamond E.G. Kittle C.F. Voth D.W. Extreme hypertrophy of the left artrial appendage: the case of the giant dog ear.Am J Cardiol. 1960; 5: 122-125Abstract Full Text PDF PubMed Scopus (30) Google Scholar], only some 50 cases have been reported in the literature. Most cases are recognized by a supraventricular arrhythmia that results in cardiac insufficiency and systemic embolism and their grave sequelae. A 24-year-old man presented with palpitations but no other symptoms. An electrocardiogram revealed an atrial fibrillation arrhythmia. Chest roentgenogram showed that the left border of the heart was greatly enlarged. Transthoracic and transesophageal echocardiography revealed a large cystic mass connected with the left atrium in the place of left atrial appendage. The mass, which was 12.30 cm long and 5.80 cm wide, with a neck of diameter 2.5 cm, was located in such a manner that it displaced and compressed the left ventricle (Fig 1). There was no evidence of thrombus inside. We gained surgical access to the aneurysm by medial sternotomy. After opening the pericardium, the aneurysm was found to be on the left myocardial border and pressing against the left ventricle, which was consequently deformed (Fig 2a). We established standard cardiopulmonary bypass to decompress the heart and facilitate its manipulation. An XposeTM 4 access device (Guidant Corp, Indianapolis, IN) was used to elevate and displace the heart apex so that the neck of the aneurysm was visible and accessible. Two a-traumatic clamps were applied to the aneurysmal neck, and the aneurysm was excised between these clamps (Fig 2, Fig 3). On resection, sinus rhythm was immediately recovered. We closed the left atrium with two rows of running sutures. There were no postoperative complications. Three months later the patient remained asymptomatic and in sinus rhythm.Fig 3The excised aneurysm.View Large Image Figure ViewerDownload (PPT) Aneurysms of the left atrium are very rare and may be congenital or acquired. Acquired aneurysms are more frequent, tend to affect the auricle as a whole, and result from pathologies that increase the intra-auricular pressure, or pathologies such as mitral valve disease or myocarditis that weaken the auricular wall in chronic manner. Congenital aneurysms are extremely rare, the auricle is not usually enlarged, and such aneurysms are not associated with other pathologies [2Zhao J. Ge Y. Yan H. Pan Y. Liao Y. Treatment of congenital aneurysms of the left atrium and left atrial appendage.Tex Heart Inst J. 1999; 26: 136-139PubMed Google Scholar]. The most frequent location is the left atrial appendage, although they have also been found in the wall of the left atrium and exceptionally in the right atrium. Generally, the aneurysm is intrapericardial, but there have been a few cases with partial defects of the pericardium [3Yaliniz H. Salih O.K. Alhan C. Gocen U. Tokcan A. Congenital intrapericardial left atrial appendage aneurysm.J Cardiovasc Surg. 2007; 48: 109-111PubMed Google Scholar]. The origin of congenital aneurysms is unknown; some authors have attributed it to dysplasia of the musculi pectinati [4Victor S. Nayak V.M. Aneurysm of the left atrial appendage.Tex Heart Inst J. 2001; 28: 111-118PubMed Google Scholar]. In the majority of cases (75%), the main symptom leading to diagnosis of congenital left atrial appendage is recurrent or continuous supraventricular arrhythmia. Another important symptom with which patients often present is systemic embolism. Other, less frequent, symptoms are cardiac insufficiency and chest pain. A routine chest roentgenogram always shows that the left border of the heart is enlarged [2Zhao J. Ge Y. Yan H. Pan Y. Liao Y. Treatment of congenital aneurysms of the left atrium and left atrial appendage.Tex Heart Inst J. 1999; 26: 136-139PubMed Google Scholar, 3Yaliniz H. Salih O.K. Alhan C. Gocen U. Tokcan A. Congenital intrapericardial left atrial appendage aneurysm.J Cardiovasc Surg. 2007; 48: 109-111PubMed Google Scholar, 4Victor S. Nayak V.M. Aneurysm of the left atrial appendage.Tex Heart Inst J. 2001; 28: 111-118PubMed Google Scholar, 5Kiaii B. Doll N. Kuehl M. Kuehl M. Mohr F.W. Minimal invasive endoscopic resection of a giant left atrial appendage aneurysm.Ann Thorac Surg. 2004; 777: 1437-1438Abstract Full Text Full Text PDF Scopus (35) Google Scholar, 6Mathur A. Zehr K.J. Sinak L.J. Rea R.F. Left atrial appendage aneurysm.Ann Thorac Surg. 2005; 79: 1392-1393Abstract Full Text Full Text PDF PubMed Scopus (31) Google Scholar, 7Vagefi P.A. Choudhry M. Hilgenberg A.D. Excision of an aneurysm of the left atrial appendage.J Thorac Cardiovasc Surg. 2007; 133: 822-823Abstract Full Text Full Text PDF PubMed Scopus (16) Google Scholar]. When confronted with a case of a young patient with recurrent or continuous palpitations, and an abnormal chest x-ray film, it is necessary to take into account the possibility of congenital left atrial appendage. To be able to confirm or rule out left atrial appendage, conventional, noninvasive imaging techniques, such as echocardiography and magnetic resonance, are sufficient [2Zhao J. Ge Y. Yan H. Pan Y. Liao Y. Treatment of congenital aneurysms of the left atrium and left atrial appendage.Tex Heart Inst J. 1999; 26: 136-139PubMed Google Scholar, 3Yaliniz H. Salih O.K. Alhan C. Gocen U. Tokcan A. Congenital intrapericardial left atrial appendage aneurysm.J Cardiovasc Surg. 2007; 48: 109-111PubMed Google Scholar, 4Victor S. Nayak V.M. Aneurysm of the left atrial appendage.Tex Heart Inst J. 2001; 28: 111-118PubMed Google Scholar, 5Kiaii B. Doll N. Kuehl M. Kuehl M. Mohr F.W. Minimal invasive endoscopic resection of a giant left atrial appendage aneurysm.Ann Thorac Surg. 2004; 777: 1437-1438Abstract Full Text Full Text PDF Scopus (35) Google Scholar, 6Mathur A. Zehr K.J. Sinak L.J. Rea R.F. Left atrial appendage aneurysm.Ann Thorac Surg. 2005; 79: 1392-1393Abstract Full Text Full Text PDF PubMed Scopus (31) Google Scholar, 7Vagefi P.A. Choudhry M. Hilgenberg A.D. Excision of an aneurysm of the left atrial appendage.J Thorac Cardiovasc Surg. 2007; 133: 822-823Abstract Full Text Full Text PDF PubMed Scopus (16) Google Scholar]. The treatment is always surgery to cut out the aneurysm. Various approaches to aneurysmectomy have been successfully used and are described in the literature. Access to the aneurysm has been achieved by median sternotomy, left thoracotomy, mini-thoracotomy [7Vagefi P.A. Choudhry M. Hilgenberg A.D. Excision of an aneurysm of the left atrial appendage.J Thorac Cardiovasc Surg. 2007; 133: 822-823Abstract Full Text Full Text PDF PubMed Scopus (16) Google Scholar]. The resection has been carried out with or without extracorporeal circulation. The range of approaches used is striking, but all have proved adequate. Resection of the aneurysm results in an immediate stop to the atrial arrhythmia and a return to sinus rhythm [2Zhao J. Ge Y. Yan H. Pan Y. Liao Y. Treatment of congenital aneurysms of the left atrium and left atrial appendage.Tex Heart Inst J. 1999; 26: 136-139PubMed Google Scholar, 3Yaliniz H. Salih O.K. Alhan C. Gocen U. Tokcan A. Congenital intrapericardial left atrial appendage aneurysm.J Cardiovasc Surg. 2007; 48: 109-111PubMed Google Scholar, 4Victor S. Nayak V.M. Aneurysm of the left atrial appendage.Tex Heart Inst J. 2001; 28: 111-118PubMed Google Scholar, 5Kiaii B. Doll N. Kuehl M. Kuehl M. Mohr F.W. Minimal invasive endoscopic resection of a giant left atrial appendage aneurysm.Ann Thorac Surg. 2004; 777: 1437-1438Abstract Full Text Full Text PDF Scopus (35) Google Scholar, 6Mathur A. Zehr K.J. Sinak L.J. Rea R.F. Left atrial appendage aneurysm.Ann Thorac Surg. 2005; 79: 1392-1393Abstract Full Text Full Text PDF PubMed Scopus (31) Google Scholar, 7Vagefi P.A. Choudhry M. Hilgenberg A.D. Excision of an aneurysm of the left atrial appendage.J Thorac Cardiovasc Surg. 2007; 133: 822-823Abstract Full Text Full Text PDF PubMed Scopus (16) Google Scholar]. In conclusion, a young patient with an abnormal electrocardiogram and an abnormal chest x-ray film may have congenital aneurysm of the left atrial appendage. This possibility can be ruled out easily by standard, noninvasive imaging techniques. A diagnosis of a congenital left atrial appendage indicates prompt aneurysmectomy, which eliminates all the symptoms and potential complications.