Key result
Percutaneous septal ablation reduces postextrasystolic gradient ~72% in a child with HOCM and Noonan syndrome.
Why the study?
Management of hypertrophic obstructive cardiomyopathy in children with Noonan syndrome and severe symptoms requires evaluation of treatment options such as septal myocardial ablation.
Does percutaneous transluminal septal myocardial ablation reduce postextrasystolic gradient in a child with hypertrophic obstructive cardiomyopathy and Noonan syndrome?
Case Report (n=1)
Does percutaneous transluminal septal myocardial ablation reduce postextrasystolic gradient in a child with hypertrophic obstructive cardiomyopathy and Noonan syndrome?
Percutaneous transluminal septal myocardial ablation can be successfully performed in a child with hypertrophic obstructive cardiomyopathy and Noonan syndrome, significantly reducing the postextrasystolic gradient.
PTSMA may be feasible in pediatric HOCM with Noonan syndrome; leaves open need for prospective validation of efficacy and safety.
We report an 8-year-old child presenting with classical features of hypertrophic obstructive cardiomyopathy and Noonan syndrome with New York Heart Association (NYHA) class III symptoms. Due to progressively worsening symptoms, the child was taken up for percutaneous transluminal septal myocardial ablation, which was successfully performed. Postextrasystolic gradient fell down from 125 to 35 mm Hg. Other than postprocedure bifascicular block, no complications were encountered till 1 year of follow-up.
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Singh et al. (2008) conducted a case report in Hypertrophic obstructive cardiomyopathy and Noonan syndrome (n=1). Percutaneous transluminal septal myocardial ablation was evaluated on Postextrasystolic gradient. Percutaneous transluminal septal myocardial ablation successfully reduced the postextrasystolic gradient from 125 to 35 mm Hg in an 8-year-old child with HOCM and Noonan syndrome.
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