AUTHOR=Carrel Thierry , Sharipov Islamjan , Jalilov Adham , Sromicki Juri , Vogt Paul Robert 

TITLE=A case series of rare pathologies of the aorta and the aortic arch in adolescents and younger adults: Transfer of experience for an individualized approach

JOURNAL=Frontiers in Cardiovascular Medicine

VOLUME=Volume 9 - 2022

YEAR=2022

URL=https://www.frontiersin.org/journals/cardiovascular-medicine/articles/10.3389/fcvm.2022.920614

DOI=10.3389/fcvm.2022.920614

ISSN=2297-055X

ABSTRACT=OBJECTIVE. While aneurysm of the aortic root, interrupted aortic arch and aortic coarctation are the most frequent aortic diseases in adolescents and younger adults, there is a number of rare pathologies of the thoracic aorta that need individualized treatment. 

PATIENTS. We present a small case series of unusual aortic pathologies in patients presenting with a broad spectrum of symptoms: tiredness, dysphagia, dyspnea, arterial hypertension, renal failure and claudication:
1. Segmental agenesia of the descending aorta
2. Balanced double aortic arch with complete vascular ring
3. Right descending aortic arch, left lusorian artery with Kommerell diverticulum
4. Large patent ductus (2.5 cm) and ventricular septal defect
5. Aneurysm of the aortic arch in presence of tuberous sclerosis
6. Pseudo-aneurysm of the proximal descending aorta following coarctation patch plasty
7. Supravalvular aortic stenosis combined to severe aortic valve stenosis
8. Pseudo-aneurysm following ascendens-to-descendens bypass because of recurrent coarctation
9. Takayasu arteriitis with multiple stenoses in the thoraco-abdominal aorta.

RESULTS. The following procedures were performed, using individualized cardiopulmonary bypass and canulation and cerebral protection strategies:
1. Ascending to supraceliac extra-anatomic bypass
2. Division of the ductus ligament and of the anterior aortic arch distally to the left subclavian artery 
3. Excision of the Kommerell diverticulum and translocation of the left subclavian artery
4. Patch closure of the aorto-pulmonary window and VSD-closure
5. Complete aortic arch replacement combined to debranching of the supra-aortic vessels
6. Graft interposition of the proximal descending aorta
7. Enlargement of the ascending aorta and aortic valve replacement
8. Exclusion of the pseudo-aneurysm, end-to-side graft interposition 
9. Ascending aorta to bi-iliac artery bypass (one planned). 

All patients were operated without operative mortality. One patient died in-hospital from pulmonary complications. One patient is still awaiting surgery. All other patients recovered very well from the operation and did not show any residual symptoms.

CONCLUSION. Rare pathologies of the thoracic aorta in younger patients may cause a broad spectrum of unusual symptoms. Surgical treatment can be performed with promising results. The prognosis of these patients is very favorable following surgery; imaging follow-up is simple but mandatory.