What Should You Always Look for When You See a Congenital Abnormality?

What Should You Always Look for When You See a Congenital Abnormality?

Author's Accepted Manuscript What Should You Always Looks for When You See A Congenital Abnormality? Jelliffe Jeganathan MBBS, Mario MontealegreGalle...

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Author's Accepted Manuscript

What Should You Always Looks for When You See A Congenital Abnormality? Jelliffe Jeganathan MBBS, Mario MontealegreGallegos MD, Feroze Mahmood MD

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S1053-0770(15)00931-3 http://dx.doi.org/10.1053/j.jvca.2015.11.001 YJCAN3471

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Journal of Cardiothoracic and Vascular Anesthesia

Cite this article as: Jelliffe Jeganathan MBBS, Mario Montealegre-Gallegos MD, Feroze Mahmood MD, What Should You Always Looks for When You See A Congenital Abnormality?, Journal of Cardiothoracic and Vascular Anesthesia, http://dx.doi.org/ 10.1053/j.jvca.2015.11.001 This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting galley proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

Title Page

Title: What should you always looks for when you see a congenital abnormality? 1 1 Authors: Jelliffe Jeganathan, MBBS ; Mario Montealegre-Gallegos, MD ; 1 Feroze Mahmood, MD

Affiliations: 1

Department of Anesthesia, Critical Care and Pain Medicine, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA.

Corresponding Author: Feroze Mahmood, MD One Deaconess Road, CC 454, Boston, MA 02215 Phone: 617-754-2714 Fax: 617-754-2735 Email: [email protected] Research Support: None Acknowledgement: None Conflict of interest: None Disclosures: None

In recent decades, continuous improvements in the quality of healthcare have significantly decreased early mortality associated with congenital heart disease (CHD). Currently, more than 85% of children with CHD live on to become adults [1], and it is currently estimated that more adults are living with CHD [2]. Eventhough this is encouraging, these patients are still presenting for cardiac surgery due to either their congenital cardiac pathologies or for coexisting degenerative cardiovascular disease (e.g atherosclerosis, aortic stenosis). Certainly one of the most challenging aspects of modern cardiac anesthesia practice is managing patients with adult CHD. These patients commonly have complex cardiac pathologies that may be aggravated by coexisting diseases of adulthood, such as type 2 diabetes mellitus and hypertension. Transesophageal echocardiography (TEE) is particularly important in the evaluation and surgical planning for these patients. It is always safe to assume that cardiac congenital abnormalities are sometimes multiple and the presence of one should alert the echocardiographer to exclude other abnormalities also. The authors have presented an interesting example of this scenario, in which unexpected echocardiographic findings made the management of the case more challenging. This 47-year-old man was previously diagnosed with a symptomatic atrial septal defect (ASD), a peri-membranous ventricular septal defect (VSD) and a quadricuspid aortic valve. He was scheduled for elective surgical correction of the ASD and VSD, but intraoperative TEE demonstrated multiple unexpected findings. These consisted of an echogenic membrane inside the right atrium and another echo density in the right atrial appendage. Three-dimensional (3D) echocardiography was essential for providing a definitive diagnosis and guiding the surgical decision-making process in this

case. Based on the echocardiographic appearance of these findings they were diagnosed as a nonubstructive cor triatriatum dextrum with a bifid right atrial appendage, both of which were left alone. The case illustrates the complexity of providing anesthesia for patients with adult CHD. In these patients, one congenital cardiac abnormality frequently coexists with other congenital abnormalities. These multiple abnormalities may have significant clinical and surgical implications. 3D echocardiography is an invaluable resource for the evaluation of these patients. Due to the continuously increasing burden of adult CHD, it is imperative that cardiac anesthesia training programs increase their trainees’ exposure to these patients. Similarly, although there are training and certification guidelines for perioperative TEE, the increasing importance of 3D TEE in the management of adult CHD may mandate changing requirements for certification of imaging proficiency when caring for patients with these diseases.

References:

1. J.H. Moller, K.A. Taubert, H.D. Allen, et al: A Special Writing Group from the Task Force on Children and Youth, American Heart Association. Cardiovascular health and disease in children: current status. Circulation, 1994 Feb;89(2):923-30. 2. Marelli A; Gilboa S; Devine O; et al: Estimating the congenital heart disease population in the United States in 2010 – What are the numbers? J Am Coll Cardiol. 2012;59(13s1):E787-E787. doi:10.1016/S0735-1097(12)60788-8