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Cyanosis in atrial septal defect with prominent crista terminalis

Published online by Cambridge University Press:  17 January 2023

Hiroya TakafujiOpen the ORCID record for Hiroya Takafuji[Opens in a new window],
Hidewo Amano and
Kotaro Obunai
Hiroya Takafuji*
Affiliation:
Department of Cardiology, Tokyo Bay Urayasu Ichikawa Medical Center, Urayasu, Japan
Hidewo Amano
Affiliation:
Department of Cardiology, Tokyo Bay Urayasu Ichikawa Medical Center, Urayasu, Japan
Kotaro Obunai
Affiliation:
Department of Cardiology, Tokyo Bay Urayasu Ichikawa Medical Center, Urayasu, Japan
*
Author for correspondence: Hiroya Takafuji, 3-4-32, Todaijima, Urayasu-city, Chiba 279-0001, Japan. Tel: +81 47 351 3101; Fax: +81 47 352 6237. E-mail: hiroyat@jadecom.jp
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Abstract

Right-to-left shunt in atrial septal defect without pulmonary hypertension is a rare condition and can present with complications such as cyanosis. This is a rare case of cyanosis caused by right-to-left shunt atrial septal defect related to prominent crista terminalis

Keywords

Cyanosisatrial septal defectprominent crista terminalisGORE CARDIOFORM ASD Occluder

Information

Type
Images in Congenital Cardiac Disease
Information
Cardiology in the Young , Volume 33 , Issue 2 , February 2023, pp. 306 - 308
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Copyright
© The Author(s), 2023. Published by Cambridge University Press

A 38-year-old woman with cyanosis of unknown cause resulting from hypoxaemia was referred to our hospital. She had a history of cyanosis of the finger since teenager and general fatigue; however, the cause was not clear. Her oxygen saturation on admission was 91% in room air and arterial blood gases showed significant hypoxaemia (PO2, 57.8 mmHg). Transesophageal echocardiography revealed a secundum 18 × 12 mm atrial septal defect (Fig 1). Additionally, an abnormal mass in the right atrium was diagnosed as prominent crista terminalis (Fig 1). Doppler flow through the atrial septal defect showed bidirectional interatrial shunt (Fig 2). The abnormal structure was involved in directing the flow toward the left atrium (Fig 3, Videos 1 and 2). Right-heart catheter examination showed no pulmonary hypertension. A contrast-enhanced chest CT showed no evidence of pulmonary arteriovenous fistula or thrombus. A 44-mm GORE CARDIOFORM ASD Occluder (GORE) was deployed with no residual shunt flow (Fig 4). After deployment, the patient’s oxygen saturation immediately improved to 100%, and her cyanosis resolved.

Figure 1.

Transesophageal echocardiography showing a single secundum atrial septal defect and prominent crista terminalis (red arrow). RA, right atrium; LA, left atrium; Ao, aorta; SVC, superior vena cava.

Figure 2.

Doppler flow on preprocedural transesophageal echocardiography through the atrial septal defect showing bidirectional interatrial shunt.

Figure 3.

Preprocedural transesophageal echocardiography showing that prominent crista terminalis is involved in directing the flow toward the left atrium. RA, right atrium; LA, left atrium; Ao, aorta; SVC, superior vena cava.

Figure 4.

A 44-mm GORE CARDIOFORM ASD Occluder is successfully deployed with completely no residual shunt flow. RA, right atrium; LA, left atrium.

Right-to-left shunt in atrial septal defect without pulmonary hypertension is a rare condition and can present with complications such as cyanosis. In general, cyanosis in the setting of high pulmonary vascular resistance (Eisenmenger syndrome) is well-known. However, cyanosis in some cases is not associated to high pulmonary vascular resistance. Crista terminalis is known to be normal anatomical variant; however, this structure can vary considerably in thickness and depth. Reference Hahn, Abraham and Adams1 This is a rare case of cyanosis caused by right-to-left shunt atrial septal defect related to prominent crista terminalis.

Supplementary material

To view supplementary material for this article, please visit https://doi.org/10.1017/S1047951122004255

Acknowledgement

None.

Financial support

This research received no specific grant from any funding agency, commercial, or not-for-profit sectors.

Conflicts of interest

None.

Ethical standards

All study procedures complied with the ethical standards of the Helsinki Declaration.

References

Hahn, RT, Abraham, T, Adams, MS, et al. Guidelines for performing a comprehensive transesophageal echocardiographic examination: recommendations from the American Society of Echocardiography and the Society of Cardiovascular Anesthesiologists. J Am Soc Echocardiogr 2013; 26: 921–964.10.1016/j.echo.2013.07.009CrossRefGoogle ScholarPubMed
Figure 0

Figure 1.Transesophageal echocardiography showing a single secundum atrial septal defect and prominent crista terminalis (red arrow). RA, right atrium; LA, left atrium; Ao, aorta; SVC, superior vena cava.

Figure 1

Figure 2.Doppler flow on preprocedural transesophageal echocardiography through the atrial septal defect showing bidirectional interatrial shunt.

Figure 2

Figure 3.Preprocedural transesophageal echocardiography showing that prominent crista terminalis is involved in directing the flow toward the left atrium. RA, right atrium; LA, left atrium; Ao, aorta; SVC, superior vena cava.

Figure 3

Figure 4.A 44-mm GORE CARDIOFORM ASD Occluder is successfully deployed with completely no residual shunt flow. RA, right atrium; LA, left atrium.

Takafuji et al. supplementary material

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