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Membranous septal aneurysm causing severe right ventricular outflow obstruction in an adult with trisomy 18

Published online by Cambridge University Press:  14 May 2021

Christopher Herron Open the ORCID record for Christopher Herron [Opens in a new window] ,
Thomas J Forbes  and
Daisuke Kobayashi
Christopher Herron
Affiliation:
Division of Cardiology, Children’s Hospital of Michigan, Detroit, MI, USA Department of Pediatrics, Central Michigan University College of Medicine, Mount Pleasant, MI, USA
Thomas J Forbes
Affiliation:
Division of Cardiology, Children’s Hospital of Michigan, Detroit, MI, USA Department of Pediatrics, Central Michigan University College of Medicine, Mount Pleasant, MI, USA
Daisuke Kobayashi*
Affiliation:
Division of Cardiology, Children’s Hospital of Michigan, Detroit, MI, USA Department of Pediatrics, Central Michigan University College of Medicine, Mount Pleasant, MI, USA
*
Author for correspondence: Daisuke Kobayashi, MD, MPH, Division of Cardiology, Children’s Hospital of Michigan, Detroit, MI, USA; 3901 Beaubien Blvd, Mount Pleasant, MI 48201-2119, USA. Tel: +1 (313) 745-5481; Fax: +1 (313) 993-0894. E-mail: dkobayas@dmc.org
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Abstract

Membranous ventricular septal aneurysm is a known entity but rarely causes severe right ventricular outflow obstruction. We report a 40-year-old female with trisomy 18 who developed severe right ventricular outflow obstruction caused by an enormous membranous septal aneurysm associated with unrepaired inlet ventricular septal defect with perimembranous extension.

Keywords

Membranous septal aneurysmventricular septal defectright ventricular outflow tract obstruction

Information

Type
Images in Congenital Cardiac Disease
Information
Cardiology in the Young , Volume 31 , Issue 9 , September 2021 , pp. 1498 - 1499
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Copyright
© The Author(s), 2021. Published by Cambridge University Press

Membranous ventricular septal aneurysms are a rare condition with most being asymptomatic in nature though can have symptoms of arrhythmia, thrombosis or more rarely outflow tract obstruction.Reference Carcano, Kanne and Kirsch1 A 40-year-old female with trisomy 18 and unrepaired ventricular septal defect was referred to our centre because transthoracic echocardiography showed severe right ventricular outflow obstruction. Her large inlet ventricular septal defect extended into the perimembranous septum. The enormous aneurysmal tricuspid valve tissue not only closed off the ventricular septal defect with no residual shunt but also extended into the right ventricular outflow tract causing severe obstruction. Clinically she remained asymptomatic. She had past medical history of developmental delay, brain cyst and hypertension. Cardiac catheterisation showed severely elevated right ventricular systolic pressure (98 mmHg) with a gradient of 70 mmHg to the pulmonary artery. Pulmonary vascular resistance was 3 Woods Units. Transthoracic and intracardiac echocardiography showed to and fro flow through the ventricular septal defect from left ventricle to the blind pouch created by the membranous ventricular septal aneurysm (Fig 1, Video). This aneurysm crossed the pulmonary valve in systole and caused severe right ventricular outflow obstruction. Angiography in the left and right ventricles showed a large membranous ventricular septal aneurysm which caused dynamic obstruction in the right ventricular outflow obstruction (Fig 2). She was then referred for surgical repair.

Figure 1.

(a ) Transesophageal echocardiography showing large inlet ventricular septal defect (VSD) and enormous membranous septal aneurysm (arrow) extending into the right ventricular outflow tract (RVOT). (b ) Intracardiac echocardiography showing the membranous septal aneurysm (arrow) crossing the pulmonary valve leaflets in systole and causing severe RVOT obstruction. RA = right atrium; RV = right ventricle; LA = left atrium; LV = left ventricle; Ao = aorta; pulmonary artery.

Figure 2.

(a ) Left ventricular (LV) angiography shows the large membranous septal aneurysm (*) protruding into the right ventricular (RV) outflow tract. (b ) RV angiography showing the membranous septal aneurysm (*). Pulmonary artery (PA) is dilated.

Supplementary material

To view supplementary material for this article, please visit https://doi.org/10.1017/S1047951121001931

Acknowledgements

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 procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. Informed consent was obtained from all individual participants included in this case report.

References

Carcano, C, Kanne, JP, Kirsch, J. Interventricular membranous septal aneurysm: CT and MR manifestations. Insights Imaging. 2016; 7: 111–117.CrossRefGoogle ScholarPubMed
Figure 0

Figure 1. (a) Transesophageal echocardiography showing large inlet ventricular septal defect (VSD) and enormous membranous septal aneurysm (arrow) extending into the right ventricular outflow tract (RVOT). (b) Intracardiac echocardiography showing the membranous septal aneurysm (arrow) crossing the pulmonary valve leaflets in systole and causing severe RVOT obstruction. RA = right atrium; RV = right ventricle; LA = left atrium; LV = left ventricle; Ao = aorta; pulmonary artery.

Figure 1

Figure 2. (a) Left ventricular (LV) angiography shows the large membranous septal aneurysm (*) protruding into the right ventricular (RV) outflow tract. (b) RV angiography showing the membranous septal aneurysm (*). Pulmonary artery (PA) is dilated.

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