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Successful treatment of severe subaortic stenosis with left ventricular dysfunction in children

  • Enny Karyani ,
  • Mahrus Ahmad Rahman ,
  • I Ketut Alit Utamayasa ,
  • Taufiq Hidayat ,
  • Teddy Ontoseno ,

Abstract

Link of Video Abstract: https://youtu.be/tHcMqNHfLyY

Background:
Subaortic stenosis (SAS) is the second most common form of left ventricular outflow obstruction, accounting for 14% of LVOT obstruction in the general population. This obstruction is in the area of the heart under the aortic valve. While only a few cases have been reported in pediatrics, SAS is a gradually progressive disorder rarely seen at birth and infancy. This case study aims to evaluate the successful treatment of severe SAS with left ventricular dysfunction in children

Case Presentation: We present the case of an 8-year-old boy who presented with shortness of breath on exertion and diaphoresis 3 months ago. Transthoracic echocardiography revealed severe aortic stenosis (PG 69.86 mmHg) with discrete tissue in the anterior LVOT and left ventricular systolic function decreased (EF Teich 29.44%), with global hypokinetic. Transesophageal echocardiography (TEE) results in severe subvalvular AS with a fixed subaortic membrane (PG 51.16 mmHg; Stenotic area 0.1 cm2), anterior to the aortic valve 1 cm long. There was mild mitral regurgitation and moderate tricuspid regurgitation. The patient then undergoes successful medical treatment and surgical subaortic membrane resection with tricuspid repair. After one year's operation, the patient remained stable, in good condition and free of symptoms.

Conclusion: Subaortic stenosis is a gradually progressive disorder and could develop into heart failure with significant left ventricular dysfunction. Monitoring is an important aspect, given the nature of disease progression. Successful management of a patient with subaortic stenosis involves a multidisciplinary approach, including an early diagnosis, a comprehensive understanding of cardiac pathophysiology, risk factors of recurrence, optimal medical management and surgical strategy.

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How to Cite

Karyani, E., Rahman, M. A., Utamayasa, I. K. A., Hidayat, T., & Ontoseno, T. (2023). Successful treatment of severe subaortic stenosis with left ventricular dysfunction in children. Bali Medical Journal, 12(2), 2278–2283. https://doi.org/10.15562/bmj.v12i2.4234

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