From Diagnosis to Intervention: The Role of Echocardiography in Guiding Management of a Giant Left Atrium in Rheumatic Mitral Stenosis
DOI:
https://doi.org/10.46799/adv.v4i9.665Keywords:
Giant Left Atrium, Rheumatic Heart Disease, Mitral Stenosis, Pulmonary Hypertension, Left Atrial RemodelingAbstract
Giant left atrium (GLA) is an uncommon but notable manifestation of advanced rheumatic heart disease (RHD), particularly in patients with longstanding rheumatic mitral stenosis. Extreme atrial remodeling may be associated with intracavitary blood stasis, pulmonary hypertension, and progressive right-sided cardiac involvement. A 50-year-old woman with congestive heart failure secondary to RHD presented with intermittent dyspnea, easy fatigability, and palpitations. Electrocardiography revealed atrial fibrillation (AF), and chest radiography demonstrated cardiomegaly with pulmonary vascular congestion. Echocardiography showed severe rheumatic mitral stenosis (MS) with mild mitral regurgitation and a Wilkins score of 9. The left atrium was markedly enlarged, measuring 7.0 cm, with a left atrial volume index (LAVI) of 239 mL/m² and spontaneous echo contrast without identifiable thrombus. Additional findings included mild aortic regurgitation, moderate tricuspid regurgitation, mild pulmonary regurgitation, a D-shaped left ventricle during systole and diastole, isolated post-capillary pulmonary hypertension, and reduced right ventricular systolic function despite preserved left ventricular systolic function. This case demonstrated the progression of chronic rheumatic mitral obstruction from sustained left atrial pressure overload to extreme atrial remodeling, blood stasis, pulmonary hypertension, and right ventricular involvement. Preserved left ventricular systolic function may coexist with substantial advanced hemodynamic impairment. Mitral valve replacement with left atrial reduction and left atrial appendage occlusion was recommended. This case highlights the central role of comprehensive echocardiography in identifying the structural, hemodynamic, and thromboembolic consequences of severe rheumatic MS with GLA. Echocardiography provided essential information for individualized therapeutic decision-making.
References
Adalti, S., Patel, K. G., Doshi, C. P., Ananthnarayanan, C., Mehta, C. N., & Wadhawa, V. A. (2018). Concomitant Left Atrial Reduction in Rheumatic Mitral Valve Disease with Giant Left Atrium: Our Technique with Midterm Results. Innovations, 13(5), 349–355. https://doi.org/10.1097/IMI.0000000000000559
Baumgartner, H., Hung, J., Bermejo, J., Chambers, J. B., Evangelista, A., & Griffin, B. P. (2009). Echocardiographic Assessment of Valve Stenosis: EAE/ASE Recommendations for Clinical Practice. European Journal of Echocardiography, 10(1), 1–25. https://doi.org/10.1093/ejechocard/jen303
Bhasin, D., Sharma, Y. P., Yadav, M., & Sharma, A. (2024). Giant left atrium in rheumatic heart disease. Acta Cardiologica, 79(2), 235–237. https://doi.org/10.1080/00015385.2023.2246193
Figueiredo, F. D. A., Esteves, W. A. M., Hung, J., & Gomes, N. F. A. (2024). Left atrial function in patients with rheumatic mitral stenosis: addressing prognostic insights beyond atrial fibrillation prediction. European Heart Journal - Imaging Methods and Practice, 2(2), qyae067. https://doi.org/10.1093/ehjimp/qyae067
Gewitz, M. H., Baltimore, R. S., Tani, L. Y., Sable, C. A., Shulman, S. T., & Carapetis, J. (2015). Revision of the Jones Criteria for the Diagnosis of Acute Rheumatic Fever in the Era of Doppler Echocardiography. Circulation, 131(20), 1806–1818. https://doi.org/10.1161/CIR.0000000000000205
Ghamari, S., Abbasi-Kangevari, M., Saeedi Moghaddam, S., Aminorroaya, A., Rezaei, N., & Shobeiri, P. (2022). Rheumatic Heart Disease Is a Neglected Disease Relative to Its Burden Worldwide: Findings From Global Burden of Disease 2019. Journal of the American Heart Association, 11(13). https://doi.org/10.1161/JAHA.122.025284
Kim, J. S. (2023). Effect of Psychological Meaningfulness on Job Involvement, Proactive Behavior, and Performance: Focusing on the Mediating Effect of Self-Efficacy.
Lang, R. M., Badano, L. P., Mor-Avi, V., Afilalo, J., Armstrong, A., & Ernande, L. (2015). Recommendations for Cardiac Chamber Quantification by Echocardiography in Adults: An Update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. Journal of the American Society of Echocardiography, 28(1), 1–39. https://doi.org/10.1016/j.echo.2014.10.003
Med Sidi El Moctar, E., El Hadj Sidi, C., Abdulrazzak, M., Eldeghedi, M., Thoraya, A., & Boye, K. (2023). Giant left atrium and management modalities (surgical vs. conservative): a case report from Mauritania. Annals of Medicine & Surgery, 85(9), 4624–4628. https://doi.org/10.1097/MS9.0000000000001132
Niu, H., Li, Y., & Wang, Y. (2022). The impact of quality of work life and work motivation on job satisfaction and turnover intention among Chinese nurses. BMC Nursing, 21(1), 152.
Otto, C. M., Nishimura, R. A., Bonow, R. O., Carabello, B. A., Erwin, J. P., & Gentile, F. (2021). 2020 ACC/AHA Guideline for the Management of Patients With Valvular Heart Disease. Circulation, 143(5). https://doi.org/10.1161/CIR.0000000000000923
Pandit, B. N., Aggarwal, P., Subramaniyan, S., Gujral, J. S., & Nath, R. K. (2021). Largest giant left atrium in rheumatic heart disease. Journal of Cardiology Cases, 24(1), 10–13. https://doi.org/10.1016/j.jccase.2020.11.022
Praz, F., Borger, M. A., Lanz, J., Marin-Cuartas, M., Abreu, A., & Adamo, M. (2025). 2025 ESC/EACTS Guidelines for the management of valvular heart disease. European Heart Journal, 46(44), 4635–4736. https://doi.org/10.1093/eurheartj/ehaf194
Remenyi, B., Carapetis, J., Wyber, R., Taubert, K., & Mayosi, B. M. (2013). Position Statement of the World Heart Federation on the Prevention and Control of Rheumatic Heart Disease. Nature Reviews Cardiology, 10(5), 284–292. https://doi.org/10.1038/nrcardio.2013.34
Ríos-Ortega, J. C., Talledo-Paredes, L., Yepez-Calderón, C., & Callalli-Mattos, E. (2023). A new surgical technique for left atrial reduction in giant left atrium. JTCVS Techniques, 17, 56–64. https://doi.org/10.1016/j.xjtc.2022.10.013
Soesanto, A. M., Almazini, P., Ariani, R., Rudiktyo, E., Ardini, T. W., & Mumpuni, H. (2025). Characteristics and Problems of Rheumatic Heart Disease in Indonesia. JACC: Asia, 5(9), 1171–1183. https://doi.org/10.1016/j.jacasi.2025.03.010
Suyanto, S. A. (2016). Faktor-faktor yang berhubungan dengan kejadian neuropati perifer diabetik. Keperawatan Dan Pemikiran Ilmiah, 2(6), 1.
Vahanian, A., Beyersdorf, F., Praz, F., Milojevic, M., Baldus, S., & Bauersachs, J. (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
Watkins, D. A., Johnson, C. O., Colquhoun, S. M., Karthikeyan, G., Beaton, A., & Bukhman, G. (2017). Global, Regional, and National Burden of Rheumatic Heart Disease, 1990–2015. The Lancet, 390(10092), 2310–2320. https://doi.org/10.1016/S0140-6736(17)31812-6
Zühlke, L., Beaton, A., Engel, M., Gomanju, A., Kumar, R. K., & Marangou, J. (2026). Acute rheumatic fever and rheumatic heart disease. Nature Reviews Disease Primers, 12(1), 7. https://doi.org/10.1038/s41572-026-00685-y
