Staged treatment of aortic dissection associated with Marfan syndrome

Congress:

ECR 2020

Poster Number:

C-11964

Type:

Educational Exhibit

Keywords:

Performed at one institution, Not applicable, Prospective, Genetic defects, Dissection, Acute, Diagnostic procedure, Contrast agent-intravenous, CT-Angiography, CT, Vascular, Cardiovascular system, Arteries / Aorta

Authors:

U. Pidvalna, D. Beshley; Lviv/UA

DOI:

10.26044/ecr2020/C-11964

DOI-Link:

https://dx.doi.org/10.26044/ecr2020/C-11964

Fig. 1: Staged treatment of aortic dissecting aneurysm in patient with Marfan syndrome

Fig. 2: De Bakey and Stanford Classification of Aortic Dissection

Fig. 3: Svenson Classification of Aortic Dissection

Fig. 4: Penn Classification of Aortic Dissection

Learning objectives

To review the most life-threatening manifestations of Marfan’s syndrome (MS) in the cardiovascular system. To demonstrate the importance of images for choosing the appropriate surgical approach. To present computed tomography (CT)-signs, which are indicative to the next stage surgical aortic repair in case of type A acute aortic dissection (AAD) in patients with MS. To describe and illustrate imaging after surgical procedure.

Background

Keynotes: •The incidence of Acute Aortic Dissection (AAD) is estimated to be 3 cases per 100.000 people per year with mortality of 1-2 % per hour without surgical treatment. •Aortic Dissection (AD) with a type A prevalence is linked in 65%+ to patients with Marfan Syndrome (MS). •Contrast-enhanced computed tomography (CT) is the gold standard in the diagnosis of AD. •According to the American Heart Association (AHA) guidelines, every fifth patient with type A AD needs the next stage surgical treatment within 5 years after...

Findings and procedure details

CT scans have been collected on patients with MS and Acute Aortic Syndrome who underwent aortic repair during the past 7 years in the Department of Cardiac Surgery. The appropriate surgical approach was based on the localization of tear, extend of the flap, patency of true and false lumens, perfusion. In all cases, an initial intimal tear was available in ascending aorta. The majority of patients had second or third surgeries for a reason of dissections at other sites or aneurysms. Proximal part of the...

Conclusion

This analysis shows that patients after the first stage surgery of AAD should be examined with CT scan at least once per year. Patients must be scheduled for surgery in case of verification that normal dimensions of the aorta are exceeded or dilatation of true or false lumens. The most common pattern of the repair was proximal ascending aortic repair, followed by descending thoracic aneurysm surgery.

Personal information and conflict of interest

Uliana Pidvalna, MD, PhD Assistant Professor. Department of Normal Anatomy, Danylo Halytsky Lviv National Medical University Resident in training. Department of Radiology, Danylo Halytsky Lviv National Medical University Pekarska, 69 79010 Lviv, Ukraine Email: [email protected] Dmytro Beshley, MD, MMSc Cardiac Surgeon. Department of Cardiac Surgery, Lviv Regional Clinical Hospital Cardiovascular Imaging Consultant. Ukrainian-Polish Heart Center “Lviv” Chernihivska, 7 79010 Lviv, Ukraine Email: [email protected] The authors have no conflicts of interest to declare.

References

1.Elsayed RS, Cohen RG, Fleischman F, Bowdish ME. Acute type A aortic dissection.Cardiology clinics. 2017;35(3),331-345 2.Gawinecka J, Schönrath F, von Eckardstein A. Acute aortic dissection: pathogenesis, risk factors and diagnosis.Swiss medical weekly. 2017;147(3334) 3.Gionis MN, Kaimasidis G, Tavlas E, et al. Medical management of acute type a aortic dissection in association with early open repair of acute limb ischemia may prevent aortic surgery.Am J Case Rep. 2013;14:52–57. doi:10.12659/AJCR.883793 4.Freeman LA, Young PM, Foley TA, Williamson EE, Bruce CJ, Greason KL. CT and MRI Assessment of...