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Vital Importance of Delineation of Coronary Artery Anatomy in Atypical Congenital Giant Right Atrial Aneurysm


1 Children’s Medical Center (Pediatrics Center of Excellence), Tehran University of Medical Sciences, Tehran, IR Iran
*Corresponding author: Elaheh Malakan Rad, Children’s Medical Center (Pediatrics Center of Excellence), Tehran University of Medical Sciences, Tehran, IR Iran. Tel: +98-2188274759, E-mail: erad@tums.ac.ir.
Journal of Comprehensive Pediatrics. 7(3): e37586 , DOI: 10.17795/compreped-37586
Article Type: Case Report; Received: Mar 9, 2016; Revised: May 30, 2016; Accepted: Jun 20, 2016; epub: Jul 31, 2016; collection: Aug 2016

Abstract


Introduction: Giant congenital right atrial aneurysm is a very rare congenital heart lesion, which may be asymptomatic or present a variety of symptoms, particularly supraventricular arrhythmias and intracardiac thrombosis formation.

Case Presentation: This is a report on a 3.5-month-old male infant with imperforated anus and an unusual-shaped congenital giant right atrial aneurysm with retro-ventricular extension. This unusual shape prevented appearance of cardiomegaly on the chest X-Ray. Surgical resection of the aneurysm was attempted. However, posterior descending coronary artery, which was embedded in the wall of the aneurysm, was irreversibly damaged during the operation. The patient died in the operation room. We concluded that pre-operative delineation of coronary arteries in cases with congenital giant right atrial aneurysm (CGRAA) with extension to the posterior left ventricle is mandatory. Despite the current data that surgical excision of the aneurysm is the treatment of choice, our case required simple closure of the aneurysmal neck from inside the right atrium to be an easier and safer surgical approach for treatment of CGRAA with a tricky anatomy.

Conclusions: This case indicates that delineation of coronary artery anatomy in atypical congenital giant right atrial aneurysm is of vital importance. Closure of the aneurysmal sac, instead of aneurysmal resection, is a safer and more simple approach in atypical cases.

Keywords: Congenital Giant Right Atrial Aneurysm; Coronary Artery Anatomy

1. Introduction


Congenital giant right atrial aneurysm (CGRAA) is a very rare disease with diverse clinical manifestations. Surgical excision of the aneurysm is the treatment of choice (1, 2). To the best of our knowledge, to date, no CGRAA with retro-left ventricular extension involving coronary artery has been reported. We report a case with an unusually huge congenital right atrial aneurysm with extension to the posterior aspect of the left ventricle with subtle adherence of the posterior descending coronary artery to its wall.

2. Case Presentation


A 3.5-month-old infant with the diagnosis of imperforated anus and hypospadias was referred to our clinic with a history of colostomy in the neonatal period. On physical examination, he was acyanotic with normal cardiac auscultation. Electrocardiogram and chest X-Ray were normal. On echocardiography, an echo-free space was seen behind the left ventricular posterior wall, both in the parasternal long-axis and modified four-chamber views (Figure 1 echo videos 1 and 2 in the supplementary file). There were tissue strands within this space, mimicking the presence of purulent pericardial effusion. Lowering the Nyquist frequency revealed blood flow in this huge effusion-like, echo-free space (Figure 2). Thorough examination showed an unusually shaped congenital giant right atrial aneurysm (CGRAA), with an oblique lie, extending from the right side and anterior of the heart to the left and posterior of the left ventricle (Videos 3 - 5 in the supplementary file). The infant experienced episodes of witnessed transient supraventricular tachycardia during echocardiographic examination and afterwards during admission. He underwent surgical operation for resection of aneurysm (Video 6 in the supplementary file). Immediately, after weaning from cardiopulmonary bypass (CPB), he developed hypotension. The CPB was re-established. Posterior descending coronary artery (PDA) was damaged and the patient died in the operation room.

Figure 1.
M-Mode of Parasternal Long-Axis View
Figure 2.
Subcostal two-dimensional echocardiogram on the left shows the huge CGRAA, with an oblique lie from right and anterior to left and posterior of the heart. The right image shows the appearance of blood flow within the aneurysm by lowering the Nyquist frequency to about 10 cycles/second.

3. Discussion


We performed a literature review on congenital right atrial aneurysm from 2001 to 2014 (Table 1). Fifteen cases, with age ranging from 20 weeks of gestation to 75 years, are reported from eight countries. However, none of them had the unusual shape and geometry of CGRAA seen in our case. Furthermore, in none of these cases, the coronary artery was attached to the aneurysm.

Congenital giant right atrial aneurysm is a very rare cardiac anomaly and right atrial aneurysms are rarer than the left (3, 4). Yildirim et al. reported 18 cases up to 2006, whereas Chatrath et al. reported 60 cases up to 1998 (4, 5). Klisiewicz et al. reported only seven cases up to 2004 (6). About half-of the reported cases were asymptomatic (7-9). Clinical presentation included arrhythmia, thrombosis, heart failure and incidental cardiomegaly on chest X-ray (10-13). In a recent review by Harder et al., all cases underwent resection of aneurysm (13).

Table 1.
Literature Review on Congenital Right Atrial Aneurysm From 2001 to 2014

Normal P wave on Electrocardiography (ECG), despite huge enlargement of right atrium (RA) (1), can be explained by the absence of myocardium in the aneurysmal wall.

We report on an infant with an unusual–shaped CGRAA and imperforated anus, leading to death because of injury to the posterior descending coronary artery (PDA) that was attached to the aneurysmal wall. This case indicates the importance of careful preoperative delineation of coronary arteries in patients with CGRAA with a retro-left ventricular extension. Resection of aneurysm is not the optimal treatment for all cases of CGRAA. Closure of the neck of the aneurysm from the inside of the right atrium is recommended in cases with this special anatomy, as in our case.

3.1. Conclusion

This case was the first reported case of CGRAA associated with imperforated anus and retro-left ventricular extension of the CGRAA with posterior descending coronary artery embedded in the wall of the aneurysm. Our patient died due to damage to PDA. Careful delineation of coronary artery anatomy by either coronary computerized tomography (CT) angiography or conventional coronary angiography, is mandatory in these cases. Simple closure of the neck from inside RA seems to be a safer surgical approach.

Supplements


Supplementary material(s) is available |http://comprped.com/?page=download&file_id=64147|.

Acknowledgments

The authors appreciate the assistance of Dr. Nasir Hematian for recording the video clips of the surgical operation.

Footnotes

Authors’ Contribution: Both authors contributed to this manuscript during all phases.
Financial Disclosure: There was no financial disclosure to declare.

References


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Table 1.

Literature Review on Congenital Right Atrial Aneurysm From 2001 to 2014

AuthorsYearCountryNumber of CasesType of ArticleAge at DiagnosisGenderAge at OperationTreatmentOutcomePresentationDuration of Follow-Up
1Harder et al. (13)2014USA5Case series and review26 monthsFemale26 monthsResectiongoodSupraventricular tachycardia4 m
20 weeks (prenatal)Not defined4 monthsResectiongood-3 years of age
PrenatalNot defined15 monthsResectiongood-4 years
18 weeks (prenatal)Not defined6 weeksResectiongood-2 years
5Male5 yearsResectiongoodIncidental finding of cardiomegaly10 years
2Uppu et al. (12)2013USA1Case report2Male2 yearsResection (surgical reduction)goodIncidental finding of massive cardiomegalyNot defined
3Narain et al. (11)2012India1Case report18Male18 yearsMedical treatmentgoodRight heart failureNot defined
4Zaqout et al. (14)2011Belgium1Case report7 daysMaleNot definedResectiongoodCardiac murmurNot defined
5Lee et al. (15)2011Korea1Case report2 weeksNot defined2 weeksResectiongoodReferral caseNot defined
6Zhao-xia et al. (16)2010China1Case report38Male38 yearsResectiongoodParoxysmal chest painNot defined
7Agematsu et al. (17)2009Japan1Case report31Male31 yearsResectiongoodCardiomegaly on chest X-Ray(CXR)Not defined
8Yoon et al. (8)20091Case report69Female--goodIncidental finding of cardiomegaly on CXRNot defined
9Yildirim et al. (4)2006Turkey1 (authors state 18 cases were reported to 2006)Case report42FemaleMedical treatment (anticoagulation therapy)goodPalpitation, dyspnea and precordial painNot defined
10Klisiewicz et al. (6)2004Poland1 (authors state 7 cases were reported to 2004)Case report30Female30 yearsResectiongoodAbnormal cardiac silhouetteNot defined
11Chatrath et al. (5)2001USA1 (authors state 60 cases were reported to 1998)Case report5Male5 yearsResectiongoodIncidental finding of cardiomegaly1 year

Figure 1.

M-Mode of Parasternal Long-Axis View
An echo-free space mimicking a pericardial effusion is seen posterior to the left ventricular free wall. This is in fact, the retro-left ventricular segment of the Congenital Giant Right Atrial Aneurysm (CGRAA).

Figure 2.

Subcostal two-dimensional echocardiogram on the left shows the huge CGRAA, with an oblique lie from right and anterior to left and posterior of the heart. The right image shows the appearance of blood flow within the aneurysm by lowering the Nyquist frequency to about 10 cycles/second.