Cor Vasa 2026, 68(3):374-383

Aneurysm of ascending aorta and its surgical management via implantation of personalized external aortic support (PEARS), guidelines for the experts in the Czech Republic

Alice Krebsováa, Daniela Žákováb, c, Petr Filab, c, Ondrej Szárszoid, Radka Kočkováe, f, Dominik Gardáša, Josef Kautznera, Ivan Netukad, Petr Němecb, c, Jan Pirkd
a Centrum dědičných kardiovaskulárních onemocnění, Klinika kardiologie, Institut klinické a experimentální medicíny, Praha,
Česká republika
b Centrum kardiovaskulární a transplantační chirurgie Brno, Brno, Česká republika
c Klinika kardiovaskulární a transplantační chirurgie, Lékařská fakulta Masarykovy univerzity, Brno, Česká republika
d Klinika kardiovaskulární chirurgie, Institut klinické a experimentální medicíny, Praha, Česká republika
e Oddělení kardiochirurgie, Fakultní nemocnice Motol a Homolka, Praha, Česká republika
f I. interní kardioangiologická klinika, Fakultní nemocnice Hradec Králové, Hradec Králové, Česká republika

Aneurysm of ascending aorta is a potentially lethal disease due to the risk of acute dissection. Medical care for patients is a complex process including accurate imaging of aorta, identification of the aetiology inclusive of genetic consultation, and family cascade screening. These may lead to individualized treatment, where cardiac surgery represents the most effective prevention of aortic dissection. Surgical implantation of personalized external aortic support (PEARS) is being promoted in the Czech Republic, but it is not yet anchored in international recommendations due to the limited experience and data on patient survival. A team of cardiologists, cardiac surgeons and geneticists decided to formulate recommendations for care for patients with aneurysm of ascending aorta, indications for PEARS implantation, perioperative care, and postoperative monitoring based on their previous experience and available literature. PEARS in the hands of experienced specialists seems so far to be a considerate surgical procedure bringing many advantages especially for younger patients.

Keywords: Ascending aortic aneurysm, National registry, PEARS, Preventive surgery

Received: April 30, 2026; Revised: April 30, 2026; Accepted: May 26, 2026; Prepublished online: June 2, 2012; Published: July 1, 2026  Show citation

ACS AIP APA ASA Harvard Chicago Chicago Notes IEEE ISO690 MLA NLM Turabian Vancouver
Krebsová A, Žáková D, Fila P, Szárszoi O, Kočková R, Gardáš D, et al.. Aneurysm of ascending aorta and its surgical management via implantation of personalized external aortic support (PEARS), guidelines for the experts in the Czech Republic. Cor Vasa. 2026;68(3):374-383.
Share...
Download citation
  • BibTeX (.bib)
  • Bookends (.ris)
  • EasyBib (.ris)
  • EndNote (.enw)
  • EndNote 8 (.xml)
  • ISI WoS (.isi)
  • Medlars (.medlars)
  • Mendeley (.ris)
  • MODS (.xml)
  • Papers (.ris)
  • RefWorks (.txt)
  • RefManager (.ris)
  • RIS (.ris)
  • MS Word (.xml)
  • Zotero (.ris)
    • Open full article

    References

    1. Mazzolai L, Teixido-Tura G, Lanzi S, et al. 2024 ESC Guidelines for the management of peripheral arterial and aortic diseases. Eur Heart J 2024;45: 3538-3700.
    2. Isselbacher EM, Preventza O, Hamilton Black J, et al. 2022 ACC/AHA Guideline for the Diagnosis and Management of Aortic Disease: A Report of the American Heart Association/American College of Cardiology Joint Committee on Clinical Practice Guidelines. Circulation 2022;146:e334-e482. Go to original source...
    3. Nemec P, Pepper J, Fila P. Personalized external aortic root support. Interact Cardiovasc Thorac Surg 2020;31:342-345.
    4. Treasure T, Austin C, Kenny LA, Pepper J. Personalized external aortic root support in aneurysm disease. Curr Opin Cardiol 2022;37:454-458. Go to original source...
    5. Lopez L, Colan S, Stylianou M, et al.; Pediatric Heart Network Investigators. Relationship of Echocardiographic Z Scores Adjusted for Body Surface Area to Age, Sex, Race, and Ethnicity: The Pediatric Heart Network Normal Echocardiogram Database. Circ Cardiovasc Imaging 2017;10:e006979.
    6. Quezada E, Lapidus J, Shaughnessy R, et al. Aortic dimensions in Turner syndrome. Am J Med Genet A 2015;167A:2527-2532. Go to original source...
    7. Paruchuri V, Salhab KF, Kuzmik G, et al. Aortic Size Distribution in the General Population: Explaining the Size Paradox in Aortic Dissection. Cardiology 2015;131:265-272. Erratum in: Cardiology 2015;132:44.
    8. Weinsaft JW, Devereux RB, Preiss LR, et al. GENTAC Registry Investigators. Aortic Dissection in Patients With Genetically Mediated Aneurysms: Incidence and Predictors in the GenTAC Registry. J Am Coll Cardiol 2016;67:2744-2754. Go to original source...
    9. Krebsová A, Kutílková E, Kubánek M, et al. Genetické vyšetření v kardiologii: Aktualizované souhrnné vyjádření a doporučení odborníků pracovní skupiny kardiogenetiky při ČAPK/ČKS, SLG a ČSSL a ST při ČLS JEP. Cor Vasa 2025;67:511-534.
    10. Loeys BL, Dietz HC, Braverman AC, et al. The revised Ghent nosology for the Marfan syndrome. J Med Genet 2010;47:476-485.
    11. MacCarrick G, Black JH, Bowdin S, et al. Loeys-Dietz syndrome: a primer for diagnosis and management. Genet Med 2014;16:576-587.
    12. Evangelista A, Sitges M, Jondeau G, et al. Multimodality imaging in thoracic aortic diseases: a clinical consensus statement from the European Association of Cardiovascular Imaging and the European Society of Cardiology working group on aorta and peripheral vascular diseases. Eur Heart J Cardiovasc Imaging 2023;24:e65-e85. Erratum in: Eur Heart J Cardiovasc Imaging 2023;24:e208.
    13. Wu J, Zafar MA, Li Y, et al. Ascending Aortic Length and Risk of Aortic Adverse Events: The Neglected Dimension. J Am Coll Cardiol 2019;74:1883-1894. Go to original source...
    14. Votýpka P, Krebsová A, Norambuena-Poustková P, et al. Post-mortem genetic testing in sudden cardiac death and genetic screening of relatives at risk: lessons learned from a Czech pilot multidisciplinary study. Int J Legal Med 2023;137:1787-1801.
    15. Zeman M, Kučerová Š, Vojtíšek T, Hejna P. Standard autopsy and diagnostic procedure in forensic departments in cases of sudden cardiac death (SCD) in individuals under 40 years of age. Soud Lek 2023;68:2-10.
    16. Basso C, Burke M, Fornes P, et al. Guidelines for autopsy investigation of sudden cardiac death. Virchows Arch 2008;452:11-18.
    17. Verhagen JMA, Kempers M, Cozijnsen L, et al. IMBH; National Working Group on BAV & TAA. Expert consensus recommendations on the cardiogenetic care for patients with thoracic aortic disease and their first-degree relatives. Int J Cardiol 2018;258:243-248.
    18. Franken R, den Hartog AW, Radonic T, et al. Beneficial Outcome of Losartan Therapy Depends on Type of FBN1 Mutation in Marfan Syndrome. Circ Cardiovasc Genet. 2015;8:383-388. Go to original source...
    19. Mullen MJ, Flather MD, Jin XY, et al. A prospective, randomized, placebo-controlled, double-blind, multicenter study of the effects of irbesartan on aortic dilatation in Marfan syndrome (AIMS trial): study protocol. Trials 2013;14:408.
    20. Al-Abcha A, Saleh Y, Mujer M, et al. Meta-analysis Examining the Usefulness of Angiotensin Receptor blockers for the Prevention of Aortic Root Dilation in Patients With the Marfan Syndrome. Am J Cardiol 2020;128:101-106.
    21. Doyle JJ, Doyle AJ, Wilson NK, et al. GenTAC Registry Consortium; MIBAVA Leducq Consortium. A deleterious gene-by-environment interaction imposed by calcium channel blockers in Marfan syndrome. Elife 2015;4:e08648. Go to original source...
    22. Pelliccia A, Sharma S, Gati S, et al. ESC Scientific Document Group. 2020 ESC Guidelines on sports cardiology and exercise in patients with cardiovascular disease. Eur Heart J 2021;42:17-96. Erratum in: Eur Heart J 2021;42:548-549. Go to original source...
    23. Němec P, Pirk J, Skalský I, et al. Výsledky léčby externí podpory aortálního kořene a ascendentní aorty u prvních 100 pacientů v České republice. Cor Vasa 2022;64:579-583. Go to original source...
    24. Kočková R, Malý J, Krebsová A, et al. Inflammatory response after ExoVasc® personalized external aortic root support (PEARS) procedure in patients with Marfan syndrome or non-Marfan genetic aortopathy. Cor Vasa 2021;63:435-440.
    25. Van Hoof L, Rega F, Golesworthy T, et al. Personalised external aortic root support for elective treatment of aortic root dilation in 200 patients. Heart. 2021;107:1790-1795.
    26. Conci L, Laufer G, Zimpfer D, et al. Ross procedure with personalized external aortic root support. Multimed Man Cardiothorac Surg 2023;2023. doi: 10.1510/mmcts.2023.077. Go to original source... Go to PubMed...

    This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC 4.0), which permits non-comercial use, distribution, and reproduction in any medium, provided the original publication is properly cited. No use, distribution or reproduction is permitted which does not comply with these terms.


    Return to the content