VSRR in Loeys-Dietz - JST - CTSNet.m4v (788.8 MB)

Valve-Sparing Aortic Root Replacement in Loeys-Dietz Patient With Anomalous Aortic Origin of the Right Coronary Artery

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posted on 2020-05-20, 21:46 authored by James Tweddell, Alan ODonnell

The patient was a 22-year-old 72.7 kg man with a diagnosis of Loeys-Dietz syndrome with a mutation in TGRBR1 (Loeys-Dietz Type 1 variant). The patient was initially diagnosed as a teenager due to an incidental finding of basilar artery dolichoectasia on a head CT. The patient was subsequently referred to Cincinnati Children’s Hospital Medical Center Cardiovascular Genetics program and diagnosed with Loeys-Dietz syndrome.

The patient was managed medically with Losartan upon original diagnosis. Atenolol was then added to his regimen during a subsequent visit two years later. The patient was also followed with serial transthoracic echocardiograms and MRIs to assess for aortic root dilatation.

The patient continued to have progressive aortic root enlargement and the latest MRI showed aortic root dilatation with a sinus dimension of 44 mm (z-score = 6.8). The patient’s aortic valve annulus was 25 mm (z-score of 1.8) by transthoracic echocardiography. The aortic valve functioned well without any stenosis or regurgitation.

Valve-sparing aortic root replacement indicated to prevent aortic complications associated with Loeys-Dietz syndrome, such as aortic dissection.

Of note, coronary artery origins and proximal courses had previously been evaluated and read as normal. Intraoperatively, the left coronary origin was found to be slightly higher above the left sinus of Valsalva. The right coronary artery origin, however, was found to arise on the anterior surface of the ascending aorta. The right origin was approximately 15 mm from the left coronary origin. The intraoperative decision was made to leave the right coronary artery button in-situ, leaving the proximal right coronary to course over anterior aspect of reconstructed aortic root until it resumed the normal course in the atrioventricular groove.

The patient had an uncomplicated postoperative course and was discharged on postoperative day four.


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