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Asian Cardiovasc Thorac Ann 2001;9:223-225
© 2001 Asia Publishing EXchange Pte Ltd

CASE STUDY

Congenital Intrapericardial Left Atrial Aneurysm

Rajinder S Dhaliwal, MCh, Deepak Puri, MCh, Kuldip S Sidhu, MCh1

Department of Cardiovascular and Thoracic Surgery Postgraduate Institute of Medical Education and Research Chandigarh, Punjab, India
1 Department of Cardiovascular and Thoracic Surgery Government Medical College Amritsar, Punjab, India
For reprint information contact: Rajinder S Dhaliwal, MCh Tel: 91 172 71 1070 Fax: 91 172 74 4401 email: rsdhaliwal{at}glide.net.in Department of Cardiovascular and Thoracic Surgery, Postgraduate Institute of Medical Education and Research, P.O. Box 1515, PGI Campus, Chandigarh, Punjab 160012, India.

   Abstract
TOP
 Abstract
INTRODUCTION
 CASE REPORT
 DISCUSSION
 References
 
A 14-year-old boy with sudden onset of palpitations and dyspnea was found to have an intrapericardial left atrial aneurysm (15 x 12 cm). Left atrial aneurysmectomy was carried out under cardiopulmonary bypass. The patient has been symptom-free during a 1-year follow-up.


   INTRODUCTION
TOP
 Abstract
 INTRODUCTION
CASE REPORT
 DISCUSSION
 References
 
Congenital left atrial (LA) intrapericardial aneurysm is a very rare anomaly; only 50 cases have been reported.1 Patients usually remain asymptomatic until the 2nd to 4th decades when supraventricular arrhythmias or systemic embolization may develop.2 The diagnosis is suggested by chest radiography and can be confirmed by echo-cardiography.3 Once recognized, aneurysmectomy should be performed to prevent life-threatening complications.4


   CASE REPORT
TOP
 Abstract
 INTRODUCTION
 CASE REPORT
DISCUSSION
 References
 
A 14-year-old boy presented in New York Heart Asso-ciation functional class II with palpitations and dyspnea on exertion of 1 month's duration. There was no history suggestive of congestive heart failure, thromboembolic episodes, or rheumatic heart disease, but he had been treated for tubercular arthritis of the knee 2 years previously. The heart rate was 130 beats•min–1 and regular. There were visible cardiac pulsations in the epigastrium. An apical beat was localized to the 5th intercostal space in the midclavicular line. The first and second heart sounds were normal, and a pansystolic murmur (grade 3/4) was audible at the mitral area, radiating to the left axilla. Chest radiography showed a globular radiopaque shadow at the left heart border below the pulmonary artery. Electrocardiography revealed supraventricular tachycardia with tall bifid P waves in leads II and III, and large S waves in the precordial leads, V1 and V2. Pulmonary function tests indicated a mildly restrictive physiology. Echocardiography in apical 2- and 4-chamber views demonstrated LA herniation (7 cm in diameter with a 2.7-cm pedicle) through a pericardial defect on the lateral aspect. It was compressing the lateral left ventricular wall, causing diastolic collapse with paradoxical motion of the interventricular septum and mild mitral regurgi-tation. Magnetic resonance imaging substantiated the diagnosis of a large LA aneurysm compressing the left ventricular wall (Figure 1Go). A left anterolateral thoracotomy was performed but no pericardial defect was found. On opening the pericardium, a large (15 x 12 cm) intra-pericardial LA aneurysm was seen filled with blood and compressing the left ventricle, but contrary to the echo findings, no narrow pedicle was present. The incision was extended transsternally to the right side, converting it into a clamshell incision, and aortic and 2-stage right atrial cannulation was instituted for cardiopulmonary bypass (CPB). Under moderate hypothermia (28°C) with intermittent topical cold saline, the aneurysm was excised, and the left atrium was repaired with a 3/0 polypropylene continuous suture during 15 minutes of fibrillatory arrest. No clot was seen within the aneurysm. The total CPB time was 51 minutes. The heart rate reverted to normal immediately after surgery but the patient had hypertension in the postoperative period, which was controlled with nitroglycerin infusion. He was discharged after 10 days. On his latest follow-up 1 year after surgery, he was in normal sinus rhythm with a heart rate of 80 beats•min–1. The cardiac silhouette was normal on chest radiography, and echocardiography showed normal LA size with no evidence of mitral regurgitation.



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  		Figure 1. Cardiac-gated gradient-echo magnetic resonance image of a coronal section of the heart passing through the left atrium, showing massive enlargement of the chamber. LAA = left atrial aneurysm.

 

   DISCUSSION
TOP
 Abstract
 INTRODUCTION
 CASE REPORT
 DISCUSSION
References
 
Congenital LA aneurysm was first reported by Fry5 in 1953. It can be extrapericardial or intrapericardial.6 With extrapericardial aneurysms, there is a congenital defect in the pericardium, but congenital intrapericardial LA aneurysm is always associated with an intact pericardium and arises from a developmental weakness in the atrial wall. Mean age at presentation is 26 years.7 Only one-third of patients are symptomatic before 10 years old as the aneurysm becomes obvious only after years of enlargement. Symptoms include dyspnea, palpitations, and atypical or anginal chest pain. Usual modes of presentation are an abnormal chest radiograph, systemic embolization, and supraventricular arrhythmias, either alone or in combination.2 Arrhythmias are produced by increased atrial irritability, LA dilatation, and ectopic focus formation. Stasis of blood within the aneurysmal cavity accounts for thrombosis and embolization. Chest radiographs show cardiomegaly with a prominent convexity at the upper left cardiac border in the region between the pulmonary artery and the left ventricle.7 Magnetic resonance imaging confirmed the diagnosis in this case but could not differentiate whether the aneurysm was intrapericardial or extrapericardial. Electrocardio-grams show supraventricular arrhythmias (as in this case) in 70% of patients.2 Characteristic features on echocardio-graphy described by Faole and colleagues8 were also noted in this case: origin from an otherwise normal left atrium; well-defined communication with the left atrium; position within the pericardial cavity; and distortion of the left ventricular free wall because the left atrium indents the left ventricle in utero, when pressures in both chambers are low. Echocardiography also helped in excluding other rare associated cardiac abnormalities such as atrial septal defect, persistent superior vena cava, and anomalous pulmonary venous drainage.

Surgical excision of the aneurysm is recommended because of the risks of embolic complications and supra-ventricular arrythmias.4 Surgery is performed through a left thoracotomy or median sternotomy. Aneurysms of the LA appendage can usually be excised without CPB, but origin from the LA wall or presence of intraluminal thrombus necessitates the use of CPB. Echocardiography in this patient suggested an extrapericardial LA appendage aneurysm, but operative findings revealed it to be intrapericardial. Therefore, the left thoracotomy had to be extended for the institution of CPB. Fibrillatory arrest with moderate systemic hypothermia was used because the actual procedure took only 15 minutes and we have been using this technique successfully for the last 2 decades for other short open heart procedures such as closure of atrial septal defects and distal anastomosis in coronary artery bypass grafting. Aneurysmectomy should be performed in all cases of LA aneurysm, patients become asymptomatic immediately after surgery, and the risk of life-threatening complications in the future is abolished.


   References
TOP
 Abstract
 INTRODUCTION
 CASE REPORT
 DISCUSSION
 References
 

  1. Gold JP, Afifi HY, Ko W, Horner N, Hahn R. Congenital giant aneurysms of the left atrial appendage: diagnosis and management. J Card Surg 1996;11:147–50.[Medline]

  2. Bramlet DA, Edward JE. Congenital aneurysms of left atrial appendage. Br Heart J 1981;45:97–100.[Abstract/Free Full Text]

  3. Cujee B, Bharadwaj B, Orchard RC, Lopez JF. Transesophageal echocardiography in the diagnosis of left atrial appendage aneurysm. J Am Soc Echo 1990;3: 408–11.[Medline]

  4. Grinfeld R, Trainini JC, Roncoroni A, Fabrykant F, Cacheda H, Tripodi G. Congenital aneurysm of the left atrium. Ann Thorac Surg 1985;39:469–71.[Abstract]

  5. Fry W. Herniation of the left auricle. Am J Surg 1953; 86:736–8.

  6. Williams WG. Dilation of the left atrial appendage. Br Heart J 1963;25:637–43.

  7. Tanabe T, Ishizaki M, Ohta S, Sugie S. Intrapericardial aneurysm of the left atrial appendage. Thorax 1980;35: 151–3.[Abstract/Free Full Text]

  8. Foale RA, Gibson TC, Guyer DE, Gillam L, King ME, Weyman AE. Congenital aneurysms of the left atrium: recognition by cross-sectional echocardiography. Circulation 1982;66:1065–9.[Abstract/Free Full Text]





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