Mitral and tricuspid annuli in hemophilia-A detailed analysis from the three-dimensional speckle-tracking echocardiographic MAGYAR-Path Study

Abstract

Aim: Hypocoagulability and associated alterations in myocardial mechanics seen in hemophilia could theoretically impact valvular geometry. Therefore, the purpose of the present study was to characterize mitral and tricuspid annular dimensions and functions in patients with hemophilia via 3D speckle-tracking echocardiography.

Methods: Of the 17 hemophilia patients originally assessed, four were excluded due to inadequate image quality. The remaining cohort consisted of 13 males with hemophilia (mean age: 40.8 ± 16.3 years; 11 with hemophilia A and 2 with hemophilia B). Thirty age- and gender-matched healthy males (mean age: 40.4 ± 9.2 years) served as the control group.

Results: Hemophilia patients presented with significant dilation of end-diastolic and end-systolic mitral annulus (MA) dimensions (diameter, area, and perimeter), leading to a reduction in MA fractional area change (MAFAC) compared with control subjects. In contrast, among the tricuspid annulus (TA) parameters, dilation was confined to end-diastolic values, while end-systolic dimensions were preserved.

Conclusions: Dilated MA, along with its functional deterioration, is present in patients with hemophilia compared to matched controls. TA dilation seems less pronounced.

Keywords

three-dimensional / speckle-tracking / echocardiography / hemophilia / mitral / tricuspid / annulus

References

[1] Zimmerman B, Valentino LA. Hemophilia: In Review. Pediatr Rev. 2013; 34: 289-95.

[2] Iorio A, Stonebraker JS, Chambost H, Makris M, Coffin D, Herr C, et al.; Data and Demographics Committee of the World Federation of Hemophilia. Establishing the Prevalence and Prevalence at Birth of Hemophilia in Males: A Meta-analytic Approach Using National Registries. Ann Intern Med. 2019; 171: 540-6.

[3] Böhmert S, Schubert R, Fichtlscherer S, Alesci S, Miesbach W. Endothelial Function in Patients with Severe and Moderate Haemophilia A and B. Hämostaseologie. 2019; 39: 195-202.

[4] Nemes A, Kormányos Á, Domsik P, Ambrus N, Gyenes N, Vezendi K, et al. Left ventricular rotational abnormalities in hemophilia-insights from the three-dimensional speckle-tracking echocardiographic MAGYAR-Path Study. Quant Imaging Med Surg. 2022; 12: 886-93.

[5] Nemes A, Kormányos Á, Vezendi K, Marton I, Borbényi Z. Left ventricular deformation in hemophilia (from the MAGYAR-Path Study). Rev Port Cardiol. 2022; 41: 441-43.

[6] Nemes A, Kormányos Á, Vezendi K, Marton I, Borbényi Z. Left Atrial Volumetric and Functional Properties in Hemophilia - Insights from a Three-Dimensional Speckle-Tracking Echocardiographic MAGYAR-Path Study. J Cardiovasc Echogr. 2022; 32: 148-53.

[7] Lang RM, Badano LP, Mor-Avi V, Afilalo J, Armstrong A, Ernande L, et al. Recommendations for Cardiac Chamber Quantification by Echocardiography in Adults: An Update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. J Am Soc Echocardiogr. 2015; 28: 1-39.e14.

[8] Ammar KA, Paterick TE, Khandheria BK, Jan MF, Kramer C, Umland MM, et al. Myocardial Mechanics: Understanding and Applying Three-Dimensional Speckle Tracking Echocardiography in Clinical Practice. Echocardiography. 2012; 29: 861-72.

[9] Urbano-Moral JA, Patel AR, Maron MS, Arias-Godinez JA, Pandian NG. Three-Dimensional Speckle-Tracking Echocardiography: Methodological Aspects and Clinical Potential. Echocardiography. 2012; 29: 997-1010.

[10] Muraru D, Niero A, Rodriguez-Zanella H, Cherata D, Badano L. Three-dimensional speckle-tracking echocardiography: benefits and limitations of integrating myocardial mechanics with three-dimensional imaging. Cardiovasc Diagn Ther. 2018; 8: 101-17.

[11] Seo Y, Ishizu T, Atsumi A, Kawamura R, Aonuma K. Three-Dimensional Speckle Tracking Echocardiography. Circ J. 2014; 78: 1290-301.

[12] Nemes A, Kovács Z, Kormányos Á, Domsik P, Kalapos A, Piros GÁ, et al. The mitral annulus in lipedema: Insights from the three-dimensional speckle-tracking echocardiographic MAGYAR-Path Study. Echocardiography. 2019; 36: 1482-91.

[13] Nemes A, Kormányos Á, Domsik P, Kalapos A, Gyenes N, Lengyel C. Normal reference values of three-dimensional speckle-tracking echocardiography-derived mitral annular dimensions and functional properties in healthy adults: Insights from the MAGYAR-Healthy Study. J Clin Ultrasound. 2021; 49: 234-9.

[14] Nemes A, Rácz G, Kormányos Á. Tricuspid Annular Abnormalities in Isolated Left Ventricular Non-compaction-Insights From the Three-dimensional Speckle-Tracking Echocardiographic MAGYAR-Path Study. Front Cardiovasc Med. 2022; 9: 694616.

[15] Nemes A, Kormányos Á, Rácz G, Ruzsa Z, Ambrus N, Lengyel C. Normal reference values of tricuspid annular dimensions and functional properties in healthy adults using three-dimensional speckle-tracking echocardiography (insights from the MAGYAR-Healthy Study). Quant Imaging Med Surg. 2023; 13: 121-32.

[16] Santagata D, Abenante A, Squizzato A, Dentali F, Donadini MP, Ageno W, et al. Rates of venous thromboembolism and use of thromboprophylaxis after major orthopedic surgery in patients with congenital hemophilia A or B: a systematic review. J Thromb Haemost. 2024; 22: 1117-31.

[17] Ghosh K, Shetty S. Anticoagulation in haemophilia patients with prosthetic valve replacement. Haemophilia. 2004; 10: 743.

[18] Badescu MC, Badulescu OV, Costache AD, Mitu O, Lupu VV, Dmour BA, et al. Atherosclerosis in Patients with Congenital Hemophilia: A Focus on Peripheral Artery Disease. Life (Basel). 2023; 13: 2221.

[19] Franchini M, Focosi D, Mannucci PM. How we manage cardiovascular disease in patients with hemophilia. Haematologica. 2023; 108: 1748-57.

[20] Amoozgar H, Fath M, Jooya P, Karimi M. Evaluation of Heart Function in Patients With Hemophilia. Clin Appl Thromb Hemost. 2017; 23: 374-8.

[21] Özdemir ZC, Köşger P, Uçar B, Bör Ö. Myocardial functions, blood pressure changes, and arterial stiffness in children with severe hemophilia A. Thromb Res. 2020; 189: 102-7.

[22] Nemes A, Kormányos Á, Rácz G, Ruzsa Z, Achim A, Ambrus N, et al. Tricuspid annular and right atrial volume changes are associated in healthy adults-insights from the three-dimensional speckle-tracking echocardiographic MAGYAR-Healthy Study. Front Cardiovasc Med. 2023; 10: 1140599.

[23] Nemes A, Kormányos Á, Ambrus N, Lengyel C. Associations between Mitral Annular and Left Atrial Volume Changes in Healthy Adults-Detailed Analysis from the Three-Dimensional Speckle-Tracking Echocardiographic MAGYAR-Healthy Study. Rev Cardiovasc Med. 2022; 23: 194.

[24] Nemes A, Kormányos Á, Ruzsa Z, Achim A, Ambrus N, Lengyel C. Three-Dimensional Speckle-Tracking Echocardiography-Derived Tricuspid Annular Dimensions and Right Atrial Strains in Healthy Adults-Is There a Relationship? (Insights from the MAGYAR-Healthy Study). J Clin Med. 2023; 12: 4240.