Background. Young patients with connective tissue disorders (CTDs) create a unique challenge for valve selection due to abnormal tissue biology, long life expectancy, re-intervention risk, anticoagulation considerations, and patient priorities such as lifestyle and pregnancy. [1, 2] Current ACC/AHA and ESC/EACTS guidelines emphasize patient-centered care but provide little guidance for young CTD patients or a framework comparing valve-sparing procedures, the Ross procedure, and prosthetic replacements. [1, 2]. Recent studies have investigated the Ross, bioprosthetic structural valve deterioration (SVD), and durability of valve-sparing surgery in CTD. On the engineering front, finite element analysis (FEA) has been used to better understand valve mechanics. [3, 4, 5, 6, 7] This review integrates these findings into a clinical and biomechanical framework for valve selection and durability in young CTD patients.
Methods. A literature review was performed using PubMed and guideline repositories. Search terms included “connective tissue disorder,” “Marfan,” “aortic valve replacement,” “Ross procedure,” “valve-sparing root replacement,” “bioprosthetic structural valve deterioration,” and “finite element analysis.” Recent guidelines, clinical series, studies of SVD, valve-sparing outcomes in CTD, and engineering analyses of valve and aortic root biomechanics were prioritized. [1, 2, 3, 4, 5, 6, 7] Five anchor articles were reviewed and used alongside guideline recommendations. [3, 5, 6, 7]
Results. Noten boom et al. and Mokhles et al. demonstrate the Ross procedure, which, when performed in appropriately selected patients, shows long-term survival and low reintervention rates, supporting it as a physiologically compatible option. [3, 4] Kostyunin et al. show the primary mechanism of bioprosthetic failure is cyclic mechanical fatigue rather than immune rejection, emphasizing the importance of hemodynamic load and leaflet stress in patients. [5] Svensson et al. demonstrate excellent durability of valve-sparing root replacement in CTD patients, with low operative mortality and high freedom from reoperation. [6] Mutlu et al. show that aortic geometry and valve sizing influence wall stress and stent deformation, highlighting the role of computational modeling in identifying mechanical risk. [7] Together, these data support a framework integrating anatomy, durability, anticoagulation, reintervention, and patient preferences. [1, 2, 3, 4, 5, 6, 7]
Conclusions. Valve selection in young CTD patients cannot be reduced to a simple mechanical versus bioprosthetic choice. Integrating Ross outcomes, SVD mechanisms, valve-sparing durability, and biomechanical modeling supports a structured clinical–biomechanical framework for decision-making. [3, 4, 5, 6, 7] This approach may help standardize multidisciplinary discussions, clarify lifetime tradeoffs, and guide future clinical and modeling studies to improve durability-focused decision-making. [1, 2]
Work Cited
[1] Writing Committee Members, Otto, C. M., Nishimura, R. A., Bonow, R. O., Carabello, B. A., Erwin, J. P., 3rd, Gentile, F., Jneid, H., Krieger, E. V., Mack, M., McLeod, C., O’Gara, P. T., Rigolin, V. H., Sundt, T. M., 3rd, Thompson, A., & Toly, C. (2021). 2020 ACC/AHA Guideline for the Management of Patients With Valvular Heart Disease: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Journal of the American College of Cardiology, 77(4), e25–e197. https://doi.org/10.1016/j.jacc.2020.11.018
[2] Vahanian, A., Beyersdorf, F., Praz, F., Milojevic, M., Baldus, S., Bauersachs, J., Capodanno, D., Conradi, L., De Bonis, M., De Paulis, R., Delgado, V., Freemantle, N., Gilard, M., Haugaa, K. H., Jeppsson, A., Jüni, P., Pierard, L., Prendergast, B. D., Sádaba, J. R., Tribouilloy, C., … ESC/EACTS Scientific Document Group (2022). 2021 ESC/EACTS Guidelines for the management of valvular heart disease. European heart journal, 43(7), 561–632. https://doi.org/10.1093/eurheartj/ehab395
[3] Notenboom, M. L., Melina, G., Veen, K. M., De Robertis, F., Coppola, G., De Siena, P., Navarra, E. M., Gaer, J., Ibrahim, M. E. K., El-Hamamsy, I., Takkenberg, J. J. M., & Yacoub, M. H. (2024). Long-Term Clinical and Echocardiographic Outcomes Following the Ross Procedure: A Post Hoc Analysis of a Randomized Clinical Trial. JAMA cardiology, 9(1), 6–14. https://doi.org/10.1001/jamacardio.2023.4090
[4] Romeo, J. L. R., Papageorgiou, G., da Costa, F. F. D., Sievers, H. H., Bogers, A. J. J. C., El-Hamamsy, I., Skillington, P. D., Wynne, R., Mastrobuoni, S., El Khoury, G., Takkenberg, J. J. M., & Mokhles, M. M. (2021). Long-term Clinical and Echocardiographic Outcomes in Young and Middle-aged Adults Undergoing the Ross Procedure. JAMA cardiology, 6(5), 539–548. https://doi.org/10.1001/jamacardio.2020.7434
[5] Kostyunin, A. E., Glushkova, T. V., Ovcharenko, E. A., Onischenko, P. S., Klyshnikov, K. Y., Akentieva, T. N., Bogdanov, L. A., Koshelev, V. A., Stasev, A. N., Khromov, A. A., & Kutikhin, A. G. (2025). Structural valve deterioration is primarily caused by cyclic fatigue but not immune rejection. Frontiers in immunology, 16, 1652294. https://doi.org/10.3389/fimmu.2025.1652294
[6] Svensson, L. G., Vargo, P. R., Desai, M. Y., Kalahasti, V., Griffin, B., Roselli, E. E., Rosinski, B. F., Rajeswaran, J., & Blackstone, E. H. (2023). Aortic valve reimplantation in patients with connective tissue disorders: Are the leaflets durable?. The Journal of thoracic and cardiovascular surgery, 166(6), 1617–1626.e6. https://doi.org/10.1016/j.jtcvs.2022.11.019
[7] Mutlu, O., Mazhar, N., Saribay, M., Yavuz, M. M., Ozturk, D., Ghareeb, A. N., Alnabti, A., & Yalcin, H. C. (2025). Finite Element Analysis of Evolut Transcatheter Heart Valves: Effects of Aortic Geometries and Valve Sizes on Post-TAVI Wall Stresses and Deformations. Journal of Clinical Medicine, 14(3), 850. https://doi.org/10.3390/jcm14030850