Immediate results of transaortic extended septal myectomy in children with obstructive hypertrophic cardiomyopathy

• Kozhanov R.S.
• Egunov O.A.
• Yanulevich O.S.
• Sokolov A.A.
• Krivoshekov E.V.

Abstract

Background. Hypertrophic cardiomyopathy (HCM) is inherited disease characterized by pronounced clinical and genetic heterogeneity, with progredient course. Transaortic extended septal myectomy is the gold standard for the treatment of this pathology in children.

Aim. To assess the immediate results of transaortic extended septal myectomy in children with obstructive hypertrophic cardiomyopathy (HCM).

Material and methods. A retrospective analysis of patients with a diagnosis of obstructive HCM after transaortic extended septal myectomy, performed at the Cardiosurgery Department # 2 of the Research Institute of Cardiology of the Tomsk National Research Medical Center for the period from 2010 to 2022.

Results. The study included 40 patients diagnosed with obstructive HCM. Median age was 7 [2.9; 12.3] years. All patients underwent transaortal extended septal myectomy. In 11 (27,5%) cases, combined elimination of biventricular obstruction was required. After myectomy, median left ventricle outflow tract peak gradient decreased from 88 [74.5; 109] to 14.0 [8.5; 20.0] mm Hg. Mitral valve (MV) regurgitation decreased from 1,88±0,93 degree up to 0,77±0,58 degree (p<0,001). SAM-syndrome (p=0.001) was effectively eliminated after surgery. There was no mortality in the early postoperative period. Two (5%) patients required MV replacement and three (7.5%) received a permanent pacemaker. Before surgery, 38 (95%) patients had FC III heart failure (HF). At the time of discharge, 37 (92.5%) patients the clinical status improved to NYHA/Ross HF functional class I.

Conclusion. Properly performed transaortic extended septal myectomy safely and effectively eliminates LV obstruction, MV insufficiency, and the main symptoms of HF in the early postoperative period.

Keywords:obstructive hypertrophic cardiomyopathy; transaortic extended septal myectomy; obstruction of the outflow tract of the left ventricle

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