Key result
Medical therapy fails in neonatal idiopathic HCM with severe RVOT stenosis, prompting surgical consideration.
Why the study?
Idiopathic hypertrophic cardiomyopathy in neonates can rapidly progress to severe right ventricular outflow tract stenosis with limited effective medical treatments.
Case Report (n=1)
In neonates with hypertrophic cardiomyopathy and severe right ventricular outflow tract stenosis mimicking pulmonary atresia, balloon atrial septostomy combined with a Blalock-Taussig shunt may be a potential therapy when medical treatment fails.
We discuss the clinical course of aneonate with idiopathic hypertrophic cardiomyopathy who showed rapid progression of stenosis of the right ventricular outflow tract and reduction in size of the right ventricular cavity due to thicken ing of the ventricular septum. Medical treatment proved unsuccessful. We suggest that balloon atrial septostomy combined with construction of a Blalock-Taussig shunt may be effective in view of the hemodynamics, which mimic pulmonary atresia with intact interventricular septum.
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Orino et al. (1999) conducted a case report in Idiopathic hypertrophic cardiomyopathy with severe stenosis of the right ventricular outflow tract (n=1). Medical treatment was evaluated on Clinical course.
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