Abstract
Thoracic aortic dilation (AD) has commonly been described in conotruncal defects (CTDs), such as tetralogy of Fallot, double outlet right ventricle and transposition of the great arteries, and truncus arteriosus. Several theories for this have been devised, but fairly recent data indicate that there is likely an underlying histologic abnormality, similar to that seen in Marfan and other connective tissue disease. The majority of aortic dissection in the general population occurs after the age of 45 years, and there have been very few case reports of aortic dissection in CTD. Given advances in cardiac surgery and increasing survival over the past several decades, there has been rising concern that, as patients who have survived surgical correction of these defects age, there may be increased morbidity and mortality due to aortic dissection and aortic regurgitation. This review discusses the most recent developments in research into AD in CTD, including associated genetic mutations.
Highlights
Conotruncal cardiac defects (CTDs) include a variety of congenital heart defects, such as tetralogy of Fallot (TOF), truncus arteriosus (TA), double outlet right ventricle (DORV), and transposition of the great arteries (TGA)
We may discover advantages to elective aortic root replacement to improve LV–aorta coupling, even in patients not thought to be at acute risk of aortic dissection, as surgical techniques improve
Newer discoveries will lead to new therapies that prevent, or even reverse, aortopathies
Summary
Conotruncal cardiac defects (CTDs) include a variety of congenital heart defects, such as tetralogy of Fallot (TOF), truncus arteriosus (TA), double outlet right ventricle (DORV), and transposition of the great arteries (TGA) These defects represent 5–10% of congenital heart disease and, generally, lead to severe cyanosis, necessitating repair in the newborn period or early in infancy. There has only been one case report of an aortic dissection in TGA after Mustard repair (14) In this case, the patient had been lost to cardiology follow-up for over two decades, had several pregnancies, and smoked cigarettes, and it was unknown what size the aorta was prior to dissection. There has been only one case report of elective aortic root replacement in a TGA patient after Mustard procedure – which was performed in a 30-year-old man, for an aortic aneurysm, measuring 4.5 cm (16). In a study by Marino and others, severe neo-aortic valve regurgitation was present in 3.7% and trivial to mild regurgitation in 81% of patients at mid-term follow-up (22)
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
