P-065 - TOTAL PERCUTANEOUS ENDOVASCULAR REPAIR OF A THORACIC AORTIC ANEURYSM IN A RIGHT-SIDED AORTIC ARCH: A CASE REPORT AND REVIEW OF LITERATURE

TOPIC:
Thoraco-abdominal Aortic Disease
AUTHORS:
Spalla F. (Ospedale Cardinal Massaia ~ Asti ~ Italy) , Trevisan A. (Ospedale Cardinal Massaia ~ Asti ~ Italy) , Pitzalis B. (Ospedale Cardinal Massaia ~ Asti ~ Italy) , Pennica D. (Ospedale Cardinal Massaia ~ Asti ~ Italy) , Zandrino F. (Ospedale Cardinal Massaia ~ Asti ~ Italy) , Tavolini V. (Ospedale Cardinal Massaia ~ Asti ~ Italy) , Cumino A. (Ospedale Cardinal Massaia ~ Asti ~ Italy) , Monti A. (Ospedale Cardinal Massaia ~ Asti ~ Italy) , Pecchio A. (Ospedale Cardinal Massaia ~ Asti ~ Italy)
Introduction:
Aortic arch anomalies are extremely rare, among these a right-sided aortic arch (RAA) occurs in approximately 0,1% of the population. (1) Edwards classified the following types of RAA: 1) mirror image branching (RAMI) of the aortic vessels; 2) RAA with an aberrant left subclavian artery; 3) isolation of the left subclavian artery with collateralization. (2) Often they are diagnosed during childhood, in association with concomitant congenital heart disease. (3) Usually RAAs are asymptomatic, but symptoms might be dyspnoea, dysphagia and thoracic pain. (4) Due to the rarity of the pathology, there is still no consensus on the treatment. We herein present the first case of a total percutaneous endovascular repair of a symptomatic aortic diverticulum in an adult patient with RAA and RAMI. We also reviewed all the cases of aortic aneurysms in RAA - mirror image branching described in literature.
Methods:
In April 2021 a 57-year-old woman was brought to our attention with a computed tomography angiography (CTA) showing a congenital RAA with a mirror-image branching and a 20mm diameter descending aortic diverticulum (total aortic diameter 43mm) with a 20mm proximal and angulated neck, causing compression of oesophagus and trachea. The patient suffered from dysphagia for solid foods, persistent cough and few episodes of dyspnoea. The past medical history was mute. After thorough preoperative exams were performed, along with an accurate planning, the patient underwent a total endovascular repair of the descending aorta by placement of a single body conformable thoracic endograft under general anesthesia. Bilateral percutaneous ultrasound-guided femoral access were performed. After systemic heparinization, under controlled hypotension, a conformable thoracic endograft was placed distally to the right subclavian artery. The patient was discharged in 3rd post-operative day without neither local nor systemic complications and in single anti-platelet therapy and referring the complete remission of all symptoms. The 30-day post-operative CTA showed the complete exclusion of the aortic diverticulum with a good patency of the graft and supra-aortic trunks.
Results:
Patients with RAMI and RAA are in most cases asymptomatic (60%) and occasionally diagnosed during radiological examinations. Rarely they may present with dysphagia or respiratory symptoms. Patients affected by RAA are usually males, but women are more likely to present symptoms. (4) In the reviewed literature 78% of cases presented with a small aneurysm or aortic diverticulum (AD) and only 2 cases required emergency surgery for a dissection. (5) RAA with RAMI might have a higher potential risk of acquired aortic disease such as dissection or aneurysm due to the more acute curvature of the aortic arch with increased stress on the proximal descending aortic wall. In fact, almost all aortic dissections in RAMI cases occur in the descending aorta. (3,6) Due to the rarity of this pathology, there has been no accepted gold standard for the treatment so far. Some authors suggest surgery only above 3cm AD diameter, however rupture and dissections have been described also in smaller diameters. (4) Usually the treatment of choice is surgical, either open, endovascular or hybrid. We present the first case of a total percutaneous approach in the treatment of an aortic aneurysm in RAA and RAMI. TEVAR is nowadays the leading treatment in thoracic aorta disease, however one must keep in mind that not all the anatomies are suitable for this approach since the conventional devices require: valid vascular access, limited tortuosity of the distal aortic arch and adequate proximal neck morphology. (7) In literature the most common option is endovascular treatment, with variability in the choice of the graft but overall good results. In our case we preferred a Gore Endovascular Conformable because of its feature to conform to severely angulated or tortuous arches.
Conclusion:
Based on our experience and review of the literature we can conclude that RAA with RAMI is probably an underreported pathology, due to its lack of symptoms. However, it must be kept in mind in differential diagnosis, especially in women who suffer from prolonged obstructive pulmonary disease. Treatment is mandatory, because of its rupture risk or dissection even in small aneurysms. Even though TEVAR could be technically challenging in patients with a RAA- RAMI, compared to conventional open surgical repair, it can be a mini-invasive, feasible and safe therapeutic option in suitable anatomies.
References:
(1) Nagata T, Yamada T, Johno H. Modified total arch replacement with the frozen elephant trunk technique for a right-sided aortic arch and aortic diverticulum in a teenager. J Card Surg. 2021 Jan;36(1):323-325. doi: 10.1111/jocs.15114. Epub 2020 Oct 8. PMID: 33032384. (2) Kanne JP, Godwin JD. Right aortic arch and its variants. J Cardiovasc Comput Tomogr. 2010 Sep-Oct;4(5):293-300. doi: 10.1016/j.jcct.2010.07.002. Epub 2010 Jul 21. PMID: 20829147. (3) Hayashi T, Ichikawa T, Yamamuro H, Ono S, Kobayashi M, Nomura T, Hashida K, Yashiro H, Okochi T, Koizumi J, Shimura S, Hasebe T, Imai Y. Right Aortic Arch with Mirror-image Branching in Adults: Evaluation Using CT. Tokai J Exp Clin Med. 2018 Apr 20;43(1):30-37. PMID: 29637537. (4) Barr JG, Sepehripour AH, Jarral OA, Tsipas P, Kokotsakis J, Kourliouros A, Athanasiou T. A review of the surgical management of right-sided aortic arch aneurysms. Interact Cardiovasc Thorac Surg. 2016 Jul;23(1):156-62. doi: 10.1093/icvts/ivw058. Epub 2016 Mar 21. PMID: 27001673; PMCID: PMC4986736. (5) Kaneda T, Lemura J, Zhang Z, Inoue T, Onoe M, Kitayama H, Nakamoto S, Oka H, Otaki M, Oku H. A case of Standford type B aortic dissection involving a right-sided aortic arch with mirror-image branching and right-sided descending aorta. Thorac Cardiovasc Surg. 2001 Feb;49(1):51-3. doi: 10.1055/s-2001-9910. PMID: 11246740. (6) Kim JB, Yang DH, Kang JW. Right aortic arch and an aberrant left subclavian artery arising from a Kommerell diverticulum complicated by acute aortic dissection. J Thorac Cardiovasc Surg. 2012 Oct;144(4):978-9. doi: 10.1016/j.jtcvs.2012.06.055. Epub 2012 Jul 24. PMID: 22835226. (7) Nomura Y, Yuri K, Kimura N, Okamura H, Itoh S, Matsumoto H, Yamaguchi A, Adachi H. Endovascular repair of thoracic aortic aneurysm associated with right-sided aortic arch: report of two cases. Gen Thorac Cardiovasc Surg. 2016 Sep;64(9):552-7. doi: 10.1007/s11748-014-0514-7. Epub 2014 Dec 30. PMID: 25547542.
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