Case Report
A Case of Giant Right Atrial Aneurysm in a Child
World Journal for Pediatric and Congenital Heart Surgery 1-4 ª The Author(s) 2016 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/2150135115607189 pch.sagepub.com
Ravindra S. Pawar, DCH, DNB1, Ashish Tiwari, MD1, P. V. Suresh, DM1, Vimal Raj, MBBS, FRCR2, and Pradeepkumar Kaushik, MS, DNB1
Abstract Giant right atrial aneurysm is a rare entity in infants and children. It needs to be distinguished from an atrial diverticulum, which can have similar presentation. Generally, an incidental finding in children, it can present with varied symptoms. We report a case of a giant right atrial aneurysm in an asymptomatic child with a large clot in the dilated right atrium, who underwent successful resection of the atrial aneurysm. Keywords aneurysm, atrial, congenital heart disease, right atrial thrombus Submitted June 28, 2015; Accepted August 26, 2015.
Case Report A 14-month-old boy with suspected congenital heart disease (CHD) was brought to us for evaluation and management. He was completely asymptomatic and growing well, according to the parents. There was a history of admission in the neonatal period for respiratory distress, during which he was suspected to have a CHD but not evaluated further by any means. There was no history of cyanosis. On examination, he was slightly tachypneic and peripheral saturation measured by pulse oximetry in all four limbs was 93%. There were no dysmorphic features. Systemic examination revealed normal heart sounds, a III/VI systolic murmur at the lower left parasternal area, and decreased air entry over the anterior lung fields bilaterally. Electrocardiogram showed normal sinus rhythm and no evidence of any chamber dilatation. Chest radiograph (Figure 1A) showed a markedly enlarged cardiac silhouette (cardiothoracic ratio in excess of 0.8), especially involving the atria. There was no splaying of the carina. There were congestive changes in the left upper lobe. No pleural effusion was present. Echo (Figure 1B) confirmed the X-ray findings, showing a hugely dilated (99 68 mm) thin-walled, noncontractile right atrium (RA), with at least two large clots. There was a moderate-sized ostium secundum (OS) atrial septal defect (ASD) with bidirectional shunting. The tricuspid annulus was dilated, and there was severe tricuspid regurgitation (TR) with peak TR gradient of 30 mm Hg. Other cardiac chambers and great arteries appeared normal. The biventricular function was normal, and there was moderate pulmonary artery hypertension. Since there were large clots in the noncontractile portion
of the RA, and there was bidirectional shunting through the ASD, it was decided to start the child on intravenous heparin, and later oral anticoagulants were started. Meanwhile, a cardiac magnetic resonance imaging was planned for detailed evaluation of the extent and nature of the aneurysm and planning for further management strategy. Cardiac magnetic resonance (Figure 2) showed a grossly dilated RA (130 80 mm in maximum dimension). The RA appendage was not seen in isolation. The left atrium, right pulmonary artery, and the tracheobronchial tree showed significant compressive effects. There was a 5 7 mm filling defect within the RA cavity close to the right lateral wall, suggestive of thrombus. Right atrial volume was 340 mL with swirling of blood within it. The right ventricle was dilated in comparison to the left ventricle, and the biventricular function was normal. The tricuspid annulus was dilated with severe TR. Repeat echocardiography after a week of anticoagulation showed decrease in the size of the thrombus. In view of the enormous size of the RA with bidirectional shunting through
1 Department of Pediatric Cardiology, Narayana Institute of Cardiac Sciences, Bengaluru, Karnataka, India 2 Mazumdar Shaw Medical Centre, Bengaluru, Karnataka, India
Corresponding Author: Ashish Tiwari, Department of Pediatric Cardiology, Narayana Institute of Cardiac Sciences, Narayana Hridayalaya Pvt Ltd, NH Health City, 258/A, Bommasandra Industrial Area, Hosur road, Bangalore, Karnataka 560099, India. Email: [email protected]
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World Journal for Pediatric and Congenital Heart Surgery
Abbreviations and Acronyms ASD CHD OS RA TR
atrial septal defect congenital heart disease ostium secundum right atrium tricuspid regurgitation
the ASD, and multiple thrombi, a decision to perform surgical right atrial reduction plasty was taken after discussion with the parents. Operative findings were a giant, thin-walled aneurysmal RA without any clots, a moderate-sized ostium secundum ASD, and a tethered septal leaflet of the tricuspid valve with dilated
Figure 1. A, Preoperative chest radiograph showing gross cardiomegaly, especially involving the atria. B, Preoperative echocardiogram showing hugely dilated thin-walled right atrium with at least two clots and the presence of an ostium secundum atrial septal defect.
Figure 2. Cardiac magnetic resonance (CMR) images showing grossly enlarged right atrium (RA) in four-chamber view (A & B) with significant tricuspid regurgitation. D, Three-dimensional Downloaded volumen-rendered images showing grossly right atrium (RA). from pch.sagepub.com at University of Wollongong on Aprilenlarged 15, 2016
Pawar et al
3
Figure 3. A, Poststernotomy image showing grossly enlarged right atrium before right atrium (RA) reduction plasty. B, Post-right atrium reduction plasty image showing reduced right atrial size. C, Image of histopathology specimen showing involvement of all three layers of heart with thinning of myocardial wall and lack of inflammation and with significant fibrosis. D, Postsurgical chest radiograph showing median sternotomy sutures and a significnat reduction in the cardiac size in comparison to previous study.
annulus (Figure 3A). Additionally, a small patent ductus arteriosus was found. He underwent RA reduction plasty, tricuspid valve repair, ASD closure, and PDA ligation (Figure 3B). The septal leaflet of the tricuspid valve was resuspended using polypropylene neochordae, and an anteroseptal–anteroposterior commisuroplasty was done. Postoperative recovery was uneventful and postoperative X-ray showed near normalization of the cardiac silhouette (Figure 3D). Postoperative echocardiogram showed RA size slightly greater than normal, and mild tricuspid regurgitation with peak TR gradient of 18 mm Hg. Histopathology of the excised tissue showed involvement of all three layers of the heart with thinned out myocardial wall with lack of inflammation and significant fibrosis (Figure 3C) suggestive of aneurysm.
Discussion Although incidental as well as symptomatic presence has been described from fetal life to old age, congenital giant right atrial aneurysm (sometimes referred to as idiopathic dilatation of the right atrium) is a rare entity.1 In our case, we could not establish it as a congenital aneurysm, as the patient had not been fully
evaluated in neonatal period, but we consider it to be a strong possibility. From its first description by Bailey et al2 in 1953, there have been several case reports and a relatively recent review by Binder et al,1 describing 60 cases with right atrial aneurysm, along with other abnormalities of the right atrium and coronary sinus. An aneurysm must be differentiated from a diverticulum. An aneurysm is defined as dilatation of the atrium involving all layers of the atrial wall. While in a diverticulum, an entry across the atrial wall into the enlarged cavity is present with fibromuscular strands stretched between its walls.3 As can be expected, the presentation varies from incidental detection in asymptomatic individuals, to symptomatic presentation including rhythm disturbances, thromboembolic events, and even sudden death.1 Most right atrial aneurysms in children are asymptomatic, as in our case. Various conditions can mimic this entity on a radiograph, including Ebstein’s anomaly, pericardial effusion, pericardial cysts, and tumors. Careful differentiation needs to be done by echocardiography or with additional imaging modalities. Symptomatic individuals are managed based on the predominant symptom (arrhythmia or thromboembolic episode), and many of them benefit from surgical reduction plasty.1 Surgical correction has been done at all
Downloaded from pch.sagepub.com at University of Wollongong on April 15, 2016
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World Journal for Pediatric and Congenital Heart Surgery
ages including neonates.4 The appropriate management strategy for asymptomatic individuals has been controversial, with some advocating surgery and others suggesting conservative treatment. However, given the propensity for arrhythmias, the risks of thromboembolic complications, and even sudden death, we advocate elective surgical reduction plasty of the RA. Acknowledgments The authors acknowledge the contribution of Dr Akhila L, pathologist, for providing the pictures of the histopathogical slides.
Authors’ Note This study is undertaken and reported with parental consent.
Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
References 1. Binder TM, Rosenhek R, Frank H, Gwechenberger M, Maurer G, Baumgartner H. Congenital malformations of the rightatrium and the coronary sinus. Chest. 2000;117(6): 1740-1748. 2. Bailey CP. Surgery of the Heart. Philadelphia, PA: Lea &Febiger; 1955:413. 3. Kobza R, Oechslin E, Pretre R, Kurz DJ, Jenni R. Enlargement of the Right Atrium—Diverticulum or Aneurysm? Eur J Echocardiography. 2003;4(3): 223-225. 4. Lee Y, Cho J, Kim G, Lee S, Hyun M, Kim Y. Surgical repair of giant right atrial aneurysm in a neonate. Korean CircJ. 2011; 41(6): 331-333.
Downloaded from pch.sagepub.com at University of Wollongong on April 15, 2016
A Case of Giant Right Atrial Aneurysm in a Child
World Journal for Pediatric and Congenital Heart Surgery 1-4 ª The Author(s) 2016 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/2150135115607189 pch.sagepub.com
Ravindra S. Pawar, DCH, DNB1, Ashish Tiwari, MD1, P. V. Suresh, DM1, Vimal Raj, MBBS, FRCR2, and Pradeepkumar Kaushik, MS, DNB1
Abstract Giant right atrial aneurysm is a rare entity in infants and children. It needs to be distinguished from an atrial diverticulum, which can have similar presentation. Generally, an incidental finding in children, it can present with varied symptoms. We report a case of a giant right atrial aneurysm in an asymptomatic child with a large clot in the dilated right atrium, who underwent successful resection of the atrial aneurysm. Keywords aneurysm, atrial, congenital heart disease, right atrial thrombus Submitted June 28, 2015; Accepted August 26, 2015.
Case Report A 14-month-old boy with suspected congenital heart disease (CHD) was brought to us for evaluation and management. He was completely asymptomatic and growing well, according to the parents. There was a history of admission in the neonatal period for respiratory distress, during which he was suspected to have a CHD but not evaluated further by any means. There was no history of cyanosis. On examination, he was slightly tachypneic and peripheral saturation measured by pulse oximetry in all four limbs was 93%. There were no dysmorphic features. Systemic examination revealed normal heart sounds, a III/VI systolic murmur at the lower left parasternal area, and decreased air entry over the anterior lung fields bilaterally. Electrocardiogram showed normal sinus rhythm and no evidence of any chamber dilatation. Chest radiograph (Figure 1A) showed a markedly enlarged cardiac silhouette (cardiothoracic ratio in excess of 0.8), especially involving the atria. There was no splaying of the carina. There were congestive changes in the left upper lobe. No pleural effusion was present. Echo (Figure 1B) confirmed the X-ray findings, showing a hugely dilated (99 68 mm) thin-walled, noncontractile right atrium (RA), with at least two large clots. There was a moderate-sized ostium secundum (OS) atrial septal defect (ASD) with bidirectional shunting. The tricuspid annulus was dilated, and there was severe tricuspid regurgitation (TR) with peak TR gradient of 30 mm Hg. Other cardiac chambers and great arteries appeared normal. The biventricular function was normal, and there was moderate pulmonary artery hypertension. Since there were large clots in the noncontractile portion
of the RA, and there was bidirectional shunting through the ASD, it was decided to start the child on intravenous heparin, and later oral anticoagulants were started. Meanwhile, a cardiac magnetic resonance imaging was planned for detailed evaluation of the extent and nature of the aneurysm and planning for further management strategy. Cardiac magnetic resonance (Figure 2) showed a grossly dilated RA (130 80 mm in maximum dimension). The RA appendage was not seen in isolation. The left atrium, right pulmonary artery, and the tracheobronchial tree showed significant compressive effects. There was a 5 7 mm filling defect within the RA cavity close to the right lateral wall, suggestive of thrombus. Right atrial volume was 340 mL with swirling of blood within it. The right ventricle was dilated in comparison to the left ventricle, and the biventricular function was normal. The tricuspid annulus was dilated with severe TR. Repeat echocardiography after a week of anticoagulation showed decrease in the size of the thrombus. In view of the enormous size of the RA with bidirectional shunting through
1 Department of Pediatric Cardiology, Narayana Institute of Cardiac Sciences, Bengaluru, Karnataka, India 2 Mazumdar Shaw Medical Centre, Bengaluru, Karnataka, India
Corresponding Author: Ashish Tiwari, Department of Pediatric Cardiology, Narayana Institute of Cardiac Sciences, Narayana Hridayalaya Pvt Ltd, NH Health City, 258/A, Bommasandra Industrial Area, Hosur road, Bangalore, Karnataka 560099, India. Email: [email protected]
Downloaded from pch.sagepub.com at University of Wollongong on April 15, 2016
2
World Journal for Pediatric and Congenital Heart Surgery
Abbreviations and Acronyms ASD CHD OS RA TR
atrial septal defect congenital heart disease ostium secundum right atrium tricuspid regurgitation
the ASD, and multiple thrombi, a decision to perform surgical right atrial reduction plasty was taken after discussion with the parents. Operative findings were a giant, thin-walled aneurysmal RA without any clots, a moderate-sized ostium secundum ASD, and a tethered septal leaflet of the tricuspid valve with dilated
Figure 1. A, Preoperative chest radiograph showing gross cardiomegaly, especially involving the atria. B, Preoperative echocardiogram showing hugely dilated thin-walled right atrium with at least two clots and the presence of an ostium secundum atrial septal defect.
Figure 2. Cardiac magnetic resonance (CMR) images showing grossly enlarged right atrium (RA) in four-chamber view (A & B) with significant tricuspid regurgitation. D, Three-dimensional Downloaded volumen-rendered images showing grossly right atrium (RA). from pch.sagepub.com at University of Wollongong on Aprilenlarged 15, 2016
Pawar et al
3
Figure 3. A, Poststernotomy image showing grossly enlarged right atrium before right atrium (RA) reduction plasty. B, Post-right atrium reduction plasty image showing reduced right atrial size. C, Image of histopathology specimen showing involvement of all three layers of heart with thinning of myocardial wall and lack of inflammation and with significant fibrosis. D, Postsurgical chest radiograph showing median sternotomy sutures and a significnat reduction in the cardiac size in comparison to previous study.
annulus (Figure 3A). Additionally, a small patent ductus arteriosus was found. He underwent RA reduction plasty, tricuspid valve repair, ASD closure, and PDA ligation (Figure 3B). The septal leaflet of the tricuspid valve was resuspended using polypropylene neochordae, and an anteroseptal–anteroposterior commisuroplasty was done. Postoperative recovery was uneventful and postoperative X-ray showed near normalization of the cardiac silhouette (Figure 3D). Postoperative echocardiogram showed RA size slightly greater than normal, and mild tricuspid regurgitation with peak TR gradient of 18 mm Hg. Histopathology of the excised tissue showed involvement of all three layers of the heart with thinned out myocardial wall with lack of inflammation and significant fibrosis (Figure 3C) suggestive of aneurysm.
Discussion Although incidental as well as symptomatic presence has been described from fetal life to old age, congenital giant right atrial aneurysm (sometimes referred to as idiopathic dilatation of the right atrium) is a rare entity.1 In our case, we could not establish it as a congenital aneurysm, as the patient had not been fully
evaluated in neonatal period, but we consider it to be a strong possibility. From its first description by Bailey et al2 in 1953, there have been several case reports and a relatively recent review by Binder et al,1 describing 60 cases with right atrial aneurysm, along with other abnormalities of the right atrium and coronary sinus. An aneurysm must be differentiated from a diverticulum. An aneurysm is defined as dilatation of the atrium involving all layers of the atrial wall. While in a diverticulum, an entry across the atrial wall into the enlarged cavity is present with fibromuscular strands stretched between its walls.3 As can be expected, the presentation varies from incidental detection in asymptomatic individuals, to symptomatic presentation including rhythm disturbances, thromboembolic events, and even sudden death.1 Most right atrial aneurysms in children are asymptomatic, as in our case. Various conditions can mimic this entity on a radiograph, including Ebstein’s anomaly, pericardial effusion, pericardial cysts, and tumors. Careful differentiation needs to be done by echocardiography or with additional imaging modalities. Symptomatic individuals are managed based on the predominant symptom (arrhythmia or thromboembolic episode), and many of them benefit from surgical reduction plasty.1 Surgical correction has been done at all
Downloaded from pch.sagepub.com at University of Wollongong on April 15, 2016
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World Journal for Pediatric and Congenital Heart Surgery
ages including neonates.4 The appropriate management strategy for asymptomatic individuals has been controversial, with some advocating surgery and others suggesting conservative treatment. However, given the propensity for arrhythmias, the risks of thromboembolic complications, and even sudden death, we advocate elective surgical reduction plasty of the RA. Acknowledgments The authors acknowledge the contribution of Dr Akhila L, pathologist, for providing the pictures of the histopathogical slides.
Authors’ Note This study is undertaken and reported with parental consent.
Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
References 1. Binder TM, Rosenhek R, Frank H, Gwechenberger M, Maurer G, Baumgartner H. Congenital malformations of the rightatrium and the coronary sinus. Chest. 2000;117(6): 1740-1748. 2. Bailey CP. Surgery of the Heart. Philadelphia, PA: Lea &Febiger; 1955:413. 3. Kobza R, Oechslin E, Pretre R, Kurz DJ, Jenni R. Enlargement of the Right Atrium—Diverticulum or Aneurysm? Eur J Echocardiography. 2003;4(3): 223-225. 4. Lee Y, Cho J, Kim G, Lee S, Hyun M, Kim Y. Surgical repair of giant right atrial aneurysm in a neonate. Korean CircJ. 2011; 41(6): 331-333.
Downloaded from pch.sagepub.com at University of Wollongong on April 15, 2016
