Ann Thorac Surg 2000;69:1569-1571
© 2000 The Society of Thoracic Surgeons
Case reports
Congenital intrapericardial aneurysm of the left atrial appendage
Giuseppe Pome, MDa,
Stefano Pelenghi, MDa,
Modestina Grassi, MDa,
Gabriele Vignati, MDa,
Alessandro Pellegrini, MD, PhDa
a "A. De Gasperis" Cardio-Thoracic Department, Cá Granda-Niguarda Hospital, Milan, Italy
Address reprint requests to Dr Pome, Divisione Cardio-Toracica "A. De Gasperis", Ospedale Niguarda Cà Granda, Unità di Cardiochirurgia, Piazza Ospedale Maggiore 3, 20162 Milan, Italy;
e-mail: speleng{at}tin.it
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Abstract
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Congenital aneurysmal dilatation of the left atrial appendage is a rare but correctable lesion. It represents a diagnostic dilemma in patients with cardiomegaly and is commonly associated with supraventricular arrhythmias and life-threatening systemic embolization. We describe the diagnostic evaluation and surgical treatment during 1997 of a patient with congenital aneurysmal dilatation of the left atrial appendage. The patient was discharged previously from our hospital in 1967 with the diagnosis of congenital dilatation of the pulmonary artery.
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Introduction
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Congenital aneurysmal dilatation of the left atrial appendage is a rare, but correctable, lesion. It represents a diagnostic dilemma in patients with cardiomegaly and is commonly associated with supraventricular arrhythmias and life-threatening systemic embolization. Patients affected by aneurysm of the left atrial appendage are at risk of significant morbidity and mortality, but with prompt and low-risk surgical resection, prognosis is excellent.
An 8-year-old girl was referred to our institution in 1967 for cardiac evaluation due to an unusual cardiac silhouette in a chest radiograph in the presence of cardiac thrill. She was evaluated and discharged from the hospital with the diagnosis of congenital dilatation of the pulmonary artery.
Thirty years later the patient became symptomatic for paroxysmal palpitation and presyncope. Physical examination and routine biochemical screening results were normal, including the first and second heart sounds, jugular and carotid pulsations, lung fields, and neurologic examinations. Electrocardiogram (ECG) showed sinus rhythm and no evidence of atrial enlargement, abnormal axis, or arrhythmia. Chest roentgenogram (Fig 1) demonstrated a significant prominence of the entire left heart border. ECG study revealed a large narrow-necked saccular dilatation (6.9 x 5.6 cm) of the left atrial appendage. Cardiac catheterization demonstrated a large aneurysm of the left atrial appendage (LAAA) (Fig 2) which displaced the left ventricle to the right; hemodynamics, valves, and coronary arteries were normal.
The patient was operated through a median sternotomy. The aneurysm occupied the left lateral portion of the pericardial cavity and protruded anteriorly and inferiorly and deformed the left ventricle. Extracorporeal circulation was instituted with bicaval cannulation and left atrial vent, and myocardial protection was achieved by cold cardioplegia with local cooling. The formation was thin walled and measured approximately 7.5 x 6 cm and contained no thrombus (Fig 3). The aneurysmal neck was defined clearly by a muscular ring that separated it from the normal atrial cavity. After resection of the aneurysm the left atrium was closed with two running sutures. The postoperative course was uneventful and the patient was discharged in sinus rhythm 7 days after operation. At 8-month follow-up the patient is free of symptoms.

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Fig 3. Aneurysmal dilatation of the left atrial appendage: the formation was thin walled and measured approximately 7.5 x 6 cm.
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Comment
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Dilatation of the LAAA may be congenital or acquired. If acquired it is usually associated with general left atrial enlargement secondary to mitral valve disease [1]. Congenital variety may be divided into intrapericardial type [2] and extrapericardial type [3]. In the latter case the primary pathologic condition is the congenital defect in the pericardium through which a portion of the left atrium herniates and may resemble a dog ear [4].
The LAAA arises from a weak appendage wall with no other associated pathology such as mitral valve disease, tuberculosis, syphilis, or pericardial defects, and must fulfill the following criteria; normal atrium, direct continuity with blood flow through the atrium itself, no pericardial defect, and histologically must present elements of normal atrial appendage [5].
Despite its congenital cause, symptoms usually do not arise until about the second decade with supraventricular tachyarrhythmias. Because of persistent symptoms and risk of severe neurologic sequelae surgical resection is recommended. Median sternotomy versus left lateral thoracotomy is favored for a better exposure of the mass, and cardiopulmonary bypass provides a safety excision in a motionless field and precludes embolization. Despite the report by Burke and associates [6], we consider stapling of the aneurysm a risky procedure.
Patients affected by LAAA are at risk for significant morbidity and mortality [2], but with prompt and low-risk surgical resection prognosis is excellent.
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References
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Semans J.H., Taussig H.B. Congenital "aneurysmal" dilatation of the left auricle. Bull Johns Hosp 1938;63:404.
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Coselli J.S., Beall A.C., Jr, Ziaddi G.M. Congenital intrapericardial aneurysmal dilatation of the left atrial appendage. Ann Thorac Surg 1985;39:466-468.[Abstract/Free Full Text]
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Fry W. Herniation of the left auricle. Am J Surg 1953;86:736.
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Dimond E.G., Kittle C.F., Voth D.W. Extreme hypertrophy of the left atrial appendage. Am J Cardiol 1960;5:122.
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Gold J.P., Afifi H.Y., Ko W., Horner N., Hahn R. Congenital giant aneurysm of the left atrial appendage. J Cardiac Surg 1996;11:147-150.[Medline]
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Burke R.P., Mark J.B., Collins J.J., Jr, Cohn L.H. Improved surgical approach to left atrial appendage aneurysm. J Cardiac Surg 1992;7:104-107.[Medline]
Accepted for publication September 14, 1999.
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