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Aorto-left ventricular tunnel: case series of a rare disease

Published online by Cambridge University Press:  19 October 2020

Obayda M. Diraneyya Open the ORCID record for Obayda M. Diraneyya [Opens in a new window] ,
Fahad Alhabshan ,
Abdullah Alghamdi ,
Hussein Moafa ,
Mohammed Alnasef  and
Mohamed S. Kabbani
Obayda M. Diraneyya
Affiliation:
Department of Cardiac Sciences, Ministry of National Guard Health Affairs, Riyadh, Saudi Arabia King Abdullah International Medical Research Center, Riyadh, Saudi Arabia
Fahad Alhabshan
Affiliation:
Department of Cardiac Sciences, Ministry of National Guard Health Affairs, Riyadh, Saudi Arabia King Abdullah International Medical Research Center, Riyadh, Saudi Arabia King Saud bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia
Abdullah Alghamdi
Affiliation:
Department of Cardiac Sciences, Ministry of National Guard Health Affairs, Riyadh, Saudi Arabia King Abdullah International Medical Research Center, Riyadh, Saudi Arabia King Saud bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia
Hussein Moafa
Affiliation:
Department of Cardiac Sciences, Ministry of National Guard Health Affairs, Riyadh, Saudi Arabia
Mohammed Alnasef
Affiliation:
Pediatric Cardiology Department, Our Lady’s Children Hospital, Dublin, Ireland
Mohamed S. Kabbani*
Affiliation:
Department of Cardiac Sciences, Ministry of National Guard Health Affairs, Riyadh, Saudi Arabia King Abdullah International Medical Research Center, Riyadh, Saudi Arabia King Saud bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia
*
Author for correspondence: Dr Mohamed S. Kabbani, Pediatric Cardiac ICU Division, Department of Cardiac Sciences, Mail code 1423, King Abdulaziz Medical City, Ministry of National Guard – Health Affairs, P.O. Box 22490, Riyadh11426, Saudi Arabia. Tel: +966 11 801 1111: extension 13621; Fax: +966118011111: extension 16773. E-mail: Kabbanim@ngha.med.sa
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Abstract

Introduction:

Aorto-left ventricular tunnel is a rare disease that can cause significant morbidity early in life due to volume overload and left ventricular failure. Surgical intervention is usually curative with minimal early complications. However, long-term effects and outcome are not fully determined.

Objective:

We are reporting a case series of this rare CHD with its long-term outcome.

Methods:

We conducted a retrospective analysis of all children from birth to 14 years of age who were admitted between 2001 and 2020 with the diagnosis of aorto-left ventricular tunnel. Demographic, echocardiographic, and perioperative data were collected and reviewed. The pre-operative data were compared with data reviewed on the last outpatient follow-up.

Results:

Total of five patients fulfilled our inclusion criteria. Three patients were diagnosed after auscultating an incidental murmur, one had symptoms of congestive heart failure, and one had an abnormal fetal echocardiogram. Echocardiography demonstrated stenotic and regurgitant aortic valve with severely depressed left ventricle function in two patients, one of them with also single left coronary artery. The other three patients had normal aortic valve structure and normal ventricular function. All five patients had surgical repair, two by patch closure at aortic end of aorto-left ventricular tunnel, two by patch closure at both aortic and left ventricular ends, and one by aortic root replacement using a homograft. During follow-up, there was no residual aorto-left ventricular tunnel in any of our five cases, two had moderate aortic regurgitation and one had moderate residual aortic stenosis.

Conclusions:

Spectrum of presentation for aorto-left ventricular tunnel varies from an occult lesion to frank left heart failure due to volume or less commonly, pressure overload. Early surgical repair is recommended and is usually associated with complete resolution. Long-term follow-up is recommended for aortic root dilatation and aortic valve competency, as valve function need to be addressed in a timely manner to avoid further complications.

Keywords

CHDaorto-left ventricular tunnelcardiac surgerypaediatric cardiology
Type
Original Article
Information
Cardiology in the Young , Volume 31 , Issue 1 , January 2021 , pp. 47 - 51
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Copyright
© The Author(s), 2020. Published by Cambridge University Press

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