Eur J Cardiothorac Surg 2001;19:943-944
© 2001 Elsevier Science NL
Congenital aneurysm of the right atrium
Fernando Moraes,
Cleusa Lapa Santos,
Vital Lira,
Carlos R. Moraes
Heart Institute of Pernambuco, Real Hospital Portugues, Av. Portugal 163, Recife 52010-010, Brazil
Received 23 October 2000;
received in revised form 2 March 2001;
accepted 4 April 2001.
Corresponding author. Tel.: +55-81-2315968; fax: +55-81-2315968
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Abstract
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Congenital aneurysm of the right atrium is described in a 1-year-old girl who presented with cardiomegaly and symptoms of paroxysmal supraventricular tachycardia. Echocardiography and cineangiography both established the definitive diagnosis and surgical resection was successful. The rarity of this condition is pointed out and its main features outlined.
Key Words: Congenital right atrial aneurysm • Atrial aneurysm • Aneurysm of the heart
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1. Introduction
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Aneurysm of the right atrium is considered to be congenital in origin, in the absence of predisposing conditions such as tricuspid valve disease, congenital heart disease, arterial pulmonary hypertension or acquired inflammatory changes in the myocardium. It is a rare condition and the scarcity of published cases [1–8] prompted us to contribute this report.
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2. Case report
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The patient, a 1-year-old girl, was first seen by a physician at the age of 15 days because, according to her mother, a diagnosis of congenital heart disease had been suspected by ultrasonography at 8 months of gestation. She had been born by normal delivery, at term. At this time she was considered normal. At the age of 1 year she was admitted to the emergency room of our institution presenting a paroxysmal supraventricular tachycardia which subsided with digoxin. Physical examination revealed a well-developed girl weighing 10 kg. The blood pressure was 80/50 mmHg, and the pulse was regular at 112/min. The peripheral pulses were all equal and synchronous. The heart was not enlarged on clinical examination, the heart sounds were normal, and no murmurs were heard. There was no hepatomegaly and the chest was clear to auscultation. Chest X-rays (Fig. 1) showed cardiomegaly and normal pulmonary vascular markings. The electrocardiogram was normal. Echocardiogram demonstrated a massively dilated right atrium without abnormalities of the tricuspid valve. Subsequent cardiac catheterization and cineangiography confirmed the presence of a large aneurysm on the right atrium, but no other cardiac anomalies were noted (Fig. 2). The child was kept on digoxin but subsequently presented several episodes of tachyarrhythmia and surgery was indicated. The patient underwent surgical exploration on March 30, 1999, through a median sternotomy. The pericardium, which was complete and enlarged, was opened longitudinally. The entire right atrium body was aneurysmatic, but the atrial appendage was normal. Plication was not feasible because the right atrium was extremely thin-walled and tense. Normothermic cardiopulmonary bypass was instituted. The right atrium was opened. No thrombi were present, the atrial septum was normal and the tricuspid valve leaflets were normal. No demarcation between normal and abnormal tissue could be observed. The aneurysm was resected as much as necessary to approach a normal sized right atrium. The resected tissue measured 11x6 cm. The remaining right atrium was closed with a continuous 6-0 Prolene suture. The postoperative course was uneventful. Pathologic examination disclosed that the right atrial wall was formed by normal endocardium and epicardium but with few myocardial fibres. The patient is free of symptoms with no medication 1 year after operation. Chest X-ray and echocardiogram are normal.
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Fig. 2. Cineangiocardiography showing (A) huge right atrial dilatation; (B) normal right ventricle and pulmonary arteries.
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3. Discussion
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Right atrial aneurysm is a rare condition. Its ætiology is unknown, but it is probably congenital in origin [3]. This is indicated, in the present case, by the suspicion of a cardiac anomaly during ultrasonography at gestation, by the absence of any predisposing cardiac cause and by the appearance of symptoms in infancy.
An overview of the reported cases shows that right atrial aneurysms can occur from neonatal to adult life. Patients may be asymptomatic [3,8] but more frequently showed supraventricular arrhytmias and/or congestive heart failure [1,2,4–8]. Our patient presented paroxysmal supraventricular tachycardia. Formation of mural thrombus in the right atrium, which is a potential risk for pulmonary embolism, has been reported [7], but was not seen in the present case.
Regarding diagnosis, cardiomegaly on the chest X-ray is a common finding and the electrocardiogram may either be normal or show supraventricular arrhythmias. Echocardiography may be the preferred method of diagnosis and for excluding associated conditions. On occasion it may be difficult to distinguish atrial aneurysms from tumors of the heart or of the contiguous structures [7]. Computed tomography (CT) and magnetic resonance imaging (MRI) of the chest may be helpful for definitive diagnosis [7,8], which can easily be made by selective cineangiocardiography, as in our patient.
The analysis of the literature does not allow one to establish the prognosis of this abnormality without surgical intervention or the risk of recurrence of the atrial dilation after surgery. As in our case, patients who presented symptoms responded well to surgical treatment [1,6,7]. So, due to the potential risk of life-threatening complications (tachyarrhthmia, congestive heart failure, pulmonary embolism), we think that aneurysm resection is indicated even for asymptomatic patients. Principles of operative management include the use of extracorporeal circulation, resection of the aneurysm and reduction plasty of the atrium. This procedure involves a low operative risk and is appropriate for correcting the abnormalities resulting from right atrial aneurysm.
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References
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Abstract
1. Introduction
