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Table of Contents
CASE REPORT
Year : 2022  |  Volume : 23  |  Issue : 4  |  Page : 235-239  

Cardiac computed tomography pictorial equivalent of angiographic gooseneck sign in a case of atrioventricular septal defect with cor triatriatum sinister


1 Department of Radiodiagnosis, All India Institute of Medical Sciences, Raipur, Chhattisgarh, India
2 Department of Cardiology, All India Institute of Medical Sciences, Raipur, Chhattisgarh, India

Date of Submission25-May-2022
Date of Acceptance02-Oct-2022
Date of Web Publication17-Nov-2022

Correspondence Address:
Prof. Narendra Kuber Bodhey
Department of Radiodiagnosis, All India Institute of Medical Sciences, Raipur, Chhattisgarh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/heartviews.heartviews_47_22

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   Abstract 


In the literature, multiple radiological signs of clinically important congenital cardiovascular abnormalities have been described which are named as per the object they resemble. These signs are very useful in learning. The Gooseneck sign is a sign seen in left ventricle catheter angiography in case of an atrioventricular septal defect. This sign has not been demonstrated on a cardiac computed tomography (CT) so far, to the best of our knowledge. We present a case showing cardiac CT pictorial equivalent of angiographic Gooseneck sign.

Keywords: Atrioventricular cushion defect, cardiac computed tomography angiography, cor triatriatum sinister, Gooseneck sign


How to cite this article:
Balpande N, Bodhey NK, Singh S. Cardiac computed tomography pictorial equivalent of angiographic gooseneck sign in a case of atrioventricular septal defect with cor triatriatum sinister. Heart Views 2022;23:235-9

How to cite this URL:
Balpande N, Bodhey NK, Singh S. Cardiac computed tomography pictorial equivalent of angiographic gooseneck sign in a case of atrioventricular septal defect with cor triatriatum sinister. Heart Views [serial online] 2022 [cited 2022 Dec 9];23:235-9. Available from: https://www.heartviews.org/text.asp?2022/23/4/235/361399




   Introduction Top


Cardiac imaging is a rapidly evolving field. It is important to identify the imaging features of cardiac anomalies and understand their pathophysiologic origin to accurately identify cardiac abnormalities. In the literature, multiple radiological signs of clinically important congenital cardiovascular abnormalities have been described and named according to the familiar object they resemble. These signs are very useful in learning and memorizing the diagnosis.

In left ventricle angiography, the “Gooseneck sign” is a characteristic finding seen in atrioventricular septal defect (AVSD), also known as endocardial cushion defect.[1] This sign has been described in conventional catheter angiography in literature in multiple articles.[2],[3] However, so far, to our knowledge, in cardiac computed tomography (CT), this sign has never been mentioned. In this article, we present a cardiac CT pictorial equivalent of an angiographic Gooseneck sign [Figure 1].
Figure 1: Coronal CT images of the heart showing “Gooseneck” sign in AVSD. AVSD: Atrioventricular septal defect, CT: Computed tomography

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   Case Presentation Top


A 30-year lady presented with dyspnea on exertion (NYHA class II). Her posteroanterior chest radiograph showed cardiomegaly in the form of an increased cardiothoracic ratio (76%) and an enlarged main pulmonary arterial trunk. Echocardiography revealed an AVSD with cor triatriatum sinister and an ejection fraction of 71%. She was advised cardiac CT to confirm the findings. Cardiac CT was done on a 128-detector 256-slice Siemens Definition Flash (Erlangen, Germany) scanner with retrospective electrocardiography gating. CT revealed a large atrial septal defect (ASD) and a small ventricular septal defect near the inlet. It also showed a membrane dividing the left atrium into two compartments consistent with cor triatriatum sinister [Figure 2]. There was a central defect (measuring approximately 1 cm × 1.1 cm) in this membrane through which the two compartments were communicating with each other. The proximal compartment was receiving blood from bilateral superior and inferior pulmonary veins. The other compartment was communicating with the left ventricle through the mitral valve and with the right atrium through the ASD. No patent ductus arteriosus was seen. The pulmonary trunk and its right and left main branches were dilated [Figure 3].
Figure 2: Axial section of contrast CT of heart shows a large atrial septal defect (black arrow) and a ventricular septal defect (yellow arrow). The LA is divided into two compartments (a proximal and a distal compartment) by a membrane (white arrows). The pulmonary veins (*) are draining into the proximal compartment. The two compartments are communicating with each other through an opening in the membrane. RA: Right atrium, RV: Right ventricle, LA: Left atrium, LV: Left ventricle, CT: Computed tomography

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Figure 3: Axial section of contrast CT at the level of pulmonary artery shows dilated MPA, RPA and LPA. MPA: Main pulmonary artery, RPA: Right pulmonary artery, LPA: Left pulmonary artery, CT: Computed tomography

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There was elongation and narrowing of the left ventricular outflow tract. The medial margin of the left ventricle showed concavity below the mitral valve on oblique coronal images. These findings were similar to the Gooseneck sign seen on the left ventricle angiogram in cases of AVSD.


   Discussion Top


AVSDs (or endocardial cushion defects) constitute around 4% of all cases of congenital heart disease.[3] The endocardial cushion normally forms the lower portion of the atrial septum, the upper portion of the interventricular septum, and the septal leaflets of the mitral valve and the tricuspid valve.[3] AVSD occurs due to retarded growth and development of the posteroinferior and anterosuperior mesenchymal endocardial cushions of the atrioventricular (AV) canal. In the normal heart, the growth and fusion of these structures lead to the formation of the AV septum during the 5th week of gestation [Figure 4]. When this fusion does not occur, a large septal defect is formed that includes portions at the AV and ventricular levels resulting in a relatively wide range of defects involving the atrial septum, ventricular septum, and one or both of the tricuspid or mitral valve. It can occur in two forms, either with a common AV valve or two separate AV valves within the common AV junction.[4]
Figure 4: (a-d) Schematic diagram showing embryological development of atrioventricular canals. It depicts growth and development of posterosuperior and antero-superior cushions leading to the normal formation and fusion of the atrioventricular septum. RA: right atrium, RV: right ventricle, LA: left atrium, LV: left ventricle

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In AVSD, there is a shortening of the left ventricular inflow tract and elongation and narrowing of the left ventricular outflow tract due to deficiency of conus and sinus portions of the interventricular septum. Furthermore, the medial margin of the left ventricle shows concavity below the mitral valve, which along with the narrowing of the left ventricular outflow tract results in the typical Gooseneck deformity.

Cor triatriatum refers to the presence of three atria in the heart. It is a rare congenital anomaly and occurs when either the left atrium or the right atrium is divided into two compartments by a fibromuscular membrane. It is reported in 0.4% of patients with congenital heart disease detected at autopsy[5] and found in <0.1% of clinically diagnosed cardiopathies.[6] There is a defect or opening in the membrane through which the two compartments communicate.

Typical presentation includes dyspnea, heart failure, and failure to thrive. It can be a cause of unexplained pulmonary hypertension in children. However, clinical presentation depends on the degree of stenosis in the membrane, the integrity of the interatrial septum, and the presence of associated cardiovascular malformations. It is termed cor triatriatum sinister when it occurs on the left side and cor triatriatum dexter when seen on the right side. The sinister (also known as the classical) type is more common than the dexter type. In the classical type, abnormal incorporation of pulmonary veins into the left atrium with a resultant membranous partition occurs through the atrial chamber. A similar partition occurring in the right atrium leads to cor triatriatum dexter. The membrane can usually be detected on echocardiography and confirmed by a cardiac CT or magnetic resonance imaging.

The symptomatic presentation in cor triatriatum is related to the size of the opening in the membrane. If it is small, it will lead to significant back pressure changes and will present in neonates or infants requiring prompt treatment. If the size of the opening is sufficiently large, the patient may remain asymptomatic. The appearance of symptoms in adulthood is usually due to a reduction in the size of the opening caused by fibrosis or calcification. Definitive treatment in symptomatic individuals is surgical resection of the membrane.[7]


   Conclusion Top


With the advent and rapid development of CT imaging, cardiac CT is commonly being used for the noninvasive study of the cardiac structures in congenital cardiac anomalies. It allows detailed evaluation of the heart and provides a roadmap for planning surgical management. CT proves to be a good noninvasive imaging method for the evaluation of cardiac as well as associated extracardiac abnormalities. Thus, it is essential to appreciate the signs seen on CT which are equivalent to those observed on conventional catheter angiogram.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Calabrò R, Limongelli G. Complete atrioventricular canal. Orphanet J Rare Dis 2006;1:8.  Back to cited text no. 1
    
2.
Available from: https://thoracickey.com/atrioventricular-septal-defects-2/. [Last accessed on 2022 Jun 12].  Back to cited text no. 2
    
3.
Ferguson EC, Krishnamurthy R, Oldham SA. Classic imaging signs of congenital cardiovascular abnormalities. Radiographics 2007;27:1323-34.  Back to cited text no. 3
    
4.
Mahle WT, Shirali GS, Anderson RH. Echo-morphological correlates in patients with atrioventricular septal defect and common atrioventricular junction. Cardiol Young 2006;16 Suppl 3:43-51.  Back to cited text no. 4
    
5.
Jegier W, Gibbons JE, Wiglesworth FW. Cortriatriatum: Clinical, hemodynamic and pathological studies surgical correction in early life. Pediatrics 1963;31:255-67.  Back to cited text no. 5
    
6.
Talner CN. Report of the new England regional infant cardiac program, by Donald C. Fyler, MD, pediatrics, 1980;65(suppl):375-461. Pediatrics 1998;102:258-9.  Back to cited text no. 6
    
7.
Nassar PN, Hamdan RH. Cor triatriatum sinistrum: Classification and imaging modalities. Eur J Cardiovasc Med 2011;1:84-7.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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