Pediatr Cardiol 11:82-85, 1990

Pediatric Cardiology 9 Springer-Verlag N e w York Inc. 1990

A t r i a l S e p t a l A n e u r y s m in I n f a n c y Isao Shiraishi, 1 Kenji Hamaoka, l Shosei Hayashi, 1 Eikan Koh, 1 Zenshiro Onouchi, 1 and Tadashi Sawada 2 1Division of Pediatric Medicine, Children's Research Hospital; and -'Department of Pediatrics, Kyoto Prefectural University of Medicine, Kyoto, Japan

SUMMARY. Thirteen infants were found to have atrial septal aneurysms (ASAs) on twodimensional (211) echocardiography and their characteristics and natural course have been studied by 21) Doppler echocardiography. Atrial septal aneurysm was accompanied by atrial septal defect (AS11) or other cardiac malformations in 12 of 13 patients, and as an isolated anomaly in one patient. Spontaneous closure of AS11 by aneurysmal formation of the interatrial septum was observed in 10 patients. The aneurysms of all 13 patients showed a moderate reduction in size, 14 to 25 months (mean 18 months), after these aneurysms had been first diagnosed and 10 subsequently disappeared. It is likely that ASA occurring in infancy will develop in patients with spontaneous closure of an AS11, and that such an aneurysm has a tendency to regress as the patient grows up. KEY WORDS: Atrial septal aneurysm - - Atrial septal defect

Atrial septal aneurysm (ASA) has been considered to be a rare cardiac malformation that could be detected only by autospy or cardiac angiography [8]. Recently, the diagnosis of ASA has been made in adults by two-dimensional (2D) echocardiography [1-5, 7, 9, 10]. However, few infants with ASA have been diagnosed and followed serially. In this report, we describe the clinical course and alterations in echocardiographic findings of ASA in 13 infants, and observe the process of formation and regression of these aneurysms.

Patients and Methods Echocardiographic examinations (including 265 infants) were performed in 1700 consecutive patients suspected of having cardiac abnormalities at the Children's Research Hospital and the Department of Pediatrics, Kyoto Prefectural University of Medicine between June 1986 and May 1987. Among these, 15 patients

Address offprint requests to: Isao Shiraishi, Division of Pediatric Medicine, Children's Research Hospital, Kyoto Prefectural University of Medicine, Kawaramachi Hirokoji, Kamikyo-ku, Kyoto City, 602 Japan.

(0.9%) were found to have ASA. The age at diagnosis of these 15 ranged from 2 days after birth to 3 years. To limit the investigation of the pathogenesis and natural history of ASA to early infancy, two patients aged more than 1 years (a 2-year-old boy with Kawasaki's disease and a 3-year-old boy with tricuspid atresia) were excluded from this study. The remaining 13 of the 265 infants (4.9%) with ASA received regular check-ups and were examined by chest x-ray, ECG, 2D echocardiography, and 2D Doppler echocardiography. Because a 3- to 6-mm long small pocket in the fossa ovalis has been described in normal subjects [4], we defined the lesion as an ASA when there was a igrotrusion of more than 0.6 cm beyond the plane of the atrial septum as measured on the subcostal or apical four-chamber view (Fig. 1). Evaluation of interatrial shunts or diagnosis of accompanying cardiac anomalies was performed by 2D Dopper echocardiography (Fig. 2). No infant was diagnosed as having an ASA by cardiac angiography, surgical operation, or autopsy. All 13 patients were girls and the age at diagnosis ranged from 2 days after birth to 12 months (mean 2.2 months). Atrial septal aneurysm occurred as an isolated anomaly in one patient (case 1), and in association with ventricular septal defect (VSD) in another (case 2). The remaining 11 patients (cases 3-13) had an atrial septal defect (ASD) with or without other cardiac malformations, such as VSD, persistent ductus arteriosus (PDA), pulmonary stenosis (PS), or pulmonary regurgitation (PR). Sixteen of the 265 infants were cyanotic, but none had ASA. All 13 patients were referred to our hospital after being diagnosed as having heart murmurs. Case 7 had congestive heart failure from a large PDA shunt, and it was surgically ligated. The other 12 patients were asymptomatic.

1. Shiraishi et al.: Atrial Septal Aneurysm in Infancy

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Table 1. Echocardiographic features of 13 patients

Case

Age first diagnosed as ASA

Complicated anomalies

Echocardiography ASA direction

LA ~ RA shunt

1 2 3 4 5 6 7 8 9 10 11 12

1 month 1 week 1 week 1 month 4 months 1 week 2 weeks 3 weeks 7 months 2 days 1 week t month 1 month

RA ---, ( - ) RA ~ ( - ) ( - ) ~ RA ~ ( - ) RA ~ ( - ) ( - ) ~ RA ~ ( - ) RA ~ ( - ) RA --~ ( - ) RA ~ ( - ) ( - ) --~ RA RA ~ ( - ) RA ~ ( - ) Bidirectional --o RA ( - ) ~ RA

(-) (-) (+) --~ ( - ) (+) ~ ( - ) (+) --~ ( - ) (+) --~ ( - ) (+) --~ ( - ) (+) ~ ( - ) (+) ~ ( - ) (+) ~ ( - ) (+) --* ( - ) (+) ~ ( - )

13

(-) VSD ASD# ASD# ASD# ASD#, PDA# ASD#, PDA## ASD#, PDA# ASD#, VSD# ASD#, VSD ASD#, VSD ASD#, PDA VSD# ASD, PSI

(+)

ASA, atrial septal aneurysm; RA, right atrium; LA, left atrium; ASD, atrial septal defect; PDA, persistent ductus arteriosus; VSD, ventricular septal defect; PSI, pulmonary stenosis and regurgitation; #, spontaneous closure of additional cardiac anomaly; # # , ligation of PDA. Results

Fig. 1. Case 9. Two-dimensional echocardiogram in subxiphoid view. An atrial septal aneurysm projecting into the right atrium is noted. LA, left atrium; RA, right atrium.

The follow-up period of these 13 patients ranged from 14 to 25 months (mean 18 months). Follow-up echocardiography was performed every 2 or 3 months once an aneurysm had been detected.

The clinical and echocardiographic features of these 13 p a t i e n t s a r e s u m m a r i z e d in T a b l e 1. T h e E C G s r e v e a l e d right v e n t r i c u l a r o v e r l o a d (Rvl > 2.0 m V ) in five p a t i e n t s ( c a s e s 6 - 1 0 ) w i t h o t h e r c a r d i a c a n o m a l i e s . T h e E C G s w e r e n o r m a l in the other eight patients. I n 12 o f t h e s e 13 p a t i e n t s t h e A S A s p r o j e c t e d i n t o t h e r i g h t a t r i u m d u r i n g all p h a s e s o f c a r d i a c a n d r e s p i r a t o r y c y c l e s . I n o n e p a t i e n t ( c a s e 12); h o w ever, the aneurysm moved bidirectionally, projecting i n t o t h e r i g h t a t r i u m d u r i n g v e n t r i c u l a r s y s t o l e to e a r l y d i a s t o l e a n d m o v i n g b a c k to t h e left a t r i u m d u r i n g m i d - t o l a t e d i a s t o l e . D i l a t a t i o n o f t h e left a t r i u m w a s o b s e r v e d in o n e p a t i e n t w i t h s y m p t o m a t i c P D A ( c a s e 7). A l e f t - t o - r i g h t a t r i a l s h u n t w a s d e t e c t e d in 11 p a t i e n t s w i t h A S D ( c a s e s 3 - 1 3 ) b y 2D Doppler echocardiography. The characteristic changes of these ASAs are a l s o s u m m a r i z e d in T a b l e I. I n f o u r o f 11 p a t i e n t s w i t h A S D ( c a s e s 3, 5, 9, a n d 13), a n A S A w a s n o t d e t e c t e d at t h e first e x a m i nation. However, a downward growth of the interatrial septum was observed and aneurysms developed gradually. D u r i n g a 14- to 2 5 - m o n t h ( m e a n 18 m o n t h s ) follow-up period, spontaneous closure of the ASD by aneurysmal formation of the interatrial septum was o b s e r v e d in 10 o f 11 p a t i e n t s ( c a s e s 3 - 1 2 ; F i g . 2). Other cardiac anomalies (VSD or PDA) spontaneo u s l y c l o s e d in t h r e e p a t i e n t s ( c a s e s 6, 8, a n d 9) a n d a P D A w a s l i g a t e d in o n e ( c a s e 7, P D A ) . In t h e s e four patients, spontaneous ASD closure was observed before the closure of other cardiac anomalies.

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Pediatric CardiologyVol. 11, No. 2, 1990

Changes in two-dimensional Doppler echocardiogram.A Left-toright atrial shunt is recognized as an orange-coloredjet during ventricularsystole to early diastole (12 months old). B ASD closed spontaneouslyand interatrial shunt cannot be detected (18 monthsold). LA, left atrium;RA, right atrium.

Fig. 2. Case 9.

The ASAs of all 13 patients showed reduction in size, and subsequently disappeared in ten.

Discussion

The prevalence of ASA detected by consecutive echocardiographic examination in adults is 0.20.5% [4, 5]. However, the prevalence reported here is 0.9% in children and 4.9% in infants. This discrepancy implies that in infancy an ASA is found more often by careful examination than is generally believed and, furthermore, that some of the aneurysms in infancy may disappear spontaneously as the patient grows up.

Various mechanisms of the formation of ASAs have been postulated [1-3, 5-7, 9, 10]. Aneurysmal formation of the septum primum has usually been attributed to a hemodynamic abnormality, the presence of an elevated right or left atrial pressure resulting from an associated cardiac disorder [4, 5]. Moreover, associations of ASA with mitral valve prolapse [1, 2] or Marfan's syndrome [6] implies that congenital connective tissue laxity, generally accepted as the underlying condition in these malformations, is also responsible for ASA formation. In this study, however, 12 of 13 patients were asymptomatic and did not show any significant left or right atrial dilatation. Furthermore, none of the 13 patients had Marfan's syndrome or mitral valve

I. Shiraishi et al.: Atrial Septal Aneurysm in Infancy

prolapse. These findings suggest that such an aneurysm develops from other causes. Atrial septal aneurysm was not detected at the initial examination in four of 11 patients; however, it gradually developed. Aneurysmal formation of interatrial septum was observed in the course of spontaneous closure of ASD in 10 of 11 patients (cases 3-12) with ASD. In all 13 patients with ASA it was noted to decrease in size, and in 10 disappeared during the follow-up period. There are four stages to this process: (1) left-to-right atrial shunt without ASA; (2) continued with developing ASA; (3) no shunt but ASA; and (4) no shunt with ASA regressing. Cases 1 and 2 showed the shift from stages 3-4. Cases 3 and 5 were in stages I-4, cases 4, 6-8, 10, and 11 were in stages 2-4, case 9 was in stages 1-3, case 12 was in stages 2-3 and case 13 was in stages I-2. This process is the main mechanism in the formation and regression of these aneurysms. During follow-up most aneurysms regress and disappear as the patients grow up. This is the reason why the incidence of ASAs in infants is higher than that reported in adults [4, 5]. Many aspects regarding the development and regression of these aneurysms are still unclear. Observing how these aneurysms alter as the patients grow up should be the subject of further research.

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2. Arvan S (1986) Incidental interatrial septal aneurysm associated with mitral valve prolapse. A m Heart J 111:603-605 3. Awan IH, Rice R, Moodie DS (1982) Spontaneous closure of atrial septal defect with interatrial aneurysm formation. Pediatr Cardiol 3:143-145

4. Gallet B, Malergue MC, Adams C, Saudemont JP, Collot AM, Druon MC, Hiltgen M (1985) Atrial septal a n e u r y s m - - a potential cause of systemic embolism. Br Heart J 53:292297 5. Hanley PC, Tajik AJ, Hynes JK, Edward WD, Reeder GS, Hagler DJ, Seward LB (1985) Diagnosis and classification of atrial septal aneurysm by two-dimensional echocardiography: report of 80 consecutive cases. J A C C 6:1370-1382 6. Magherini A, Margiotta C, Bandini F, Simonetti L, Bartolozzi G (1986) Atrial septal aneurysm, ectasia of valsalva and mitral valve prolapse in Marfan's syndrome. A m J Cardiol 58:172-173

7. Sapire DW, Casta D (1982) Aneurysmal bulging of the internal septum in a newborn infant with arteriovenous fistula and congestive heart failure. Chest 82:629-651 8. Silver ND, Dorsy JS (1978) Aneurysms of the septum primum in adults. Arch Pathol Lab Med 102:62-65 9. Wolf WJ, Casta A, Sapire DW (1987) Atrial septal aneurysms in infants and children. Am Heart J 113:1149-1153 10. Yiannikas J, Moodie DS, Sterba R, Gill CC (1984) Intravenous digital subtraction angiography to assess aneurysms of ventricular and atrial septum pre- and postoperatively. Am J Cardiol 53:383-385

Atrial septal aneurysm in infancy.

Thirteen infants were found to have atrial septal aneurysms (ASAs) on two-dimensional (2D) echocardiography and their characteristics and natural cour...
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