Key result
Ivabradine manages challenging heart rate control in a neonate with severe HCM and LVOTO.
Why the study?
Hypertrophic cardiomyopathy affects roughly 20% of Noonan syndrome patients and drives mortality, especially in neonates with severe disease and left ventricular outflow tract obstruction where heart rate control and systemic vascular resistance maintenance are challenging.
Does ivabradine improve heart rate control without compromising hemodynamics in a neonate with severe hypertrophic cardiomyopathy?
Case Report (n=1)
Does ivabradine improve heart rate control without compromising hemodynamics in a neonate with severe hypertrophic cardiomyopathy?
Ivabradine may provide effective heart rate control without negative inotropy or hypotension in neonates with severe symptomatic hypertrophic cardiomyopathy.
Answers are grounded in this paper and cite their sources.
Should not change neonatal HCM management; leaves open ivabradine’s role pending larger trials.
BACKGROUND: Hypertrophic cardiomyopathy (HCM) affects approximately 20% of patients with Noonan syndrome and contributes significantly to mortality, particularly in neonates with severe HCM and left ventricular outflow tract obstruction, where controlling heart rate (HR) and maintaining systemic vascular resistance can be challenging. CASE SUMMARY: A full-term neonate with an Raf-1 proto-oncogene, serine/threonine kinase mutation and severe HCM developed diastolic heart failure requiring high-dose esmolol for rate control and phenylephrine for systemic vascular resistance support. After inadequate HR response and associated vasodilation due to esmolol, off-label ivabradine was started at 20 days of life, achieving HR control within 48 hours and allowing weaning of vasoactive agents and respiratory support. DISCUSSION: Standard therapies-β-blockers, nondihydropyridine calcium channel blockers, and sodium-channel blockers-can worsen left ventricular outflow tract obstruction. Ivabradine selectively inhibits the I(f) current via hyperpolarization-activated cyclic nucleotide-gated channel 4 in sinoatrial tissue, providing HR reduction without compromising contractility or hemodynamics. TAKE-HOME MESSAGE: Ivabradine provides durable HR control without negative inotropy or hypotension, supporting its use in symptomatic pediatric HCM.
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Karikari et al. (2026) conducted a case report in Severe Hypertrophic Cardiomyopathy (n=1). Ivabradine was evaluated on Heart rate control. Off-label ivabradine achieved heart rate control within 48 hours in a neonate with severe hypertrophic cardiomyopathy, allowing weaning of vasoactive agents and respiratory support.
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