The role of transaortic extended septal myectomy in children with Noonan syndrome and obstructive hypertrophic cardiomyopathy
Abstract
Background. Approximately 20-30% of patients with Noonan syndrome have asymmetric
hypertrophic cardiomyopathy (HCM) with left ventricular outflow tract (LVOT)
obstruction and mitral regurgitation. There is relatively little data on the
role of transaortic extended septal myectomy in children with this syndrome.
Aim. To evaluate the immediate and mid-term results of transaortic extended
septal myectomy in children with obstructive HCM and Noonan syndrome.
Material and
methods. A retrospective analysis of patients was carried out
after transaortic extended septal myectomy performed in the cardiac surgery
department No. 2 of the Research Institute of Cardiology of the Tomsk National
Research Medical Center from 2010 to 2023. The patients were divided into two
groups. Group I included 14 patients with HCM and Noonan syndrome, group II
included 26 non-syndromic patients with HCM.
Results. 40 patients were included in the study. Median age was 5.2 [2.3; 10.5]
years and 7.5 [3.4; 13.2] years for groups I and II (p=0.281). At the same
time, the age of initial diagnosis in group I was 1 [1; 3] months, and in group
II – 12 [6; 51] months (р<0.001). The rate of intraoperative complications for group I was
7.1% (n=1), for group II – 23.1% (n=6). All patients in group I were diagnosed
with an anomaly of the subvalvular apparatus of the mitral valve; in 14.3% of
cases, its replacement with a mechanical bicuspid prosthesis was required to
eliminate the obstruction. There was no mortality in the early and mid-term
postoperative follow-up period. The median follow-up time for patients in both
groups was 3.0 [2.9; 3.2] years. Significant differences according to mid-term
observations in patients of group I, compared with group II, were observed in
the residual gradient on the LVOT and the z-index of the posterior wall of the
left ventricle (p=0.001; p=0.011).
Conclusion. Transaortic extended septal myectomy can effectively relieve LVOT
obstruction in children with Noonan syndrome and concomitant obstructive HCM
with satisfactory early and mid-term postoperative results.
Keywords:Noonan syndrome; hypertrophic cardiomyopathy; transaortic extended septal myectomy; left ventricular outflow tract obstruction
Funding. The study had no sponsor support.
Conflict of
interest. The authors declare no conflict of interest.
For citation: Kozhanov R.S., Egunov О.A., Naumov S.S., Vtorushin S.V., Krivoshchekov
E.V. The role of transaortic extended septal myectomy in children with Noonan
syndrome and obstructive hypertrophic cardiomyopathy. Clinical and Experimental
Surgery. Petrovsky Journal. 2024; 12 (1): 30–9. DOI: https://doi.org/10.33029/2308-1198-2024-12-1-30-39
(in Russian)
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