Atrial Septal Defect Surgical Closure Following Trametinib Utilization in Noonan Syndrome–Associated Hypertrophic Cardiomyopathy

Q: What does this research mean for the field?

Trametinib can medically stabilize infants with Noonan syndrome-associated hypertrophic cardiomyopathy, allowing for successful surgical closure of atrial septal defects. Novelty: ClaimNovelty.NOVEL_FINDING. Consensus alignment: ConsensusAlignment.NEUTRAL.

Camden Hebson; Sydney Escott; Guillermo Beltran-Ale; Robert J. Dabal; Grégor Andelfinger; Kristin Linscott

doi:10.1016/j.jaccas.2026.107006

February 1, 2026JACC Case ReportsOpen Access

Atrial Septal Defect Surgical Closure Following Trametinib Utilization in Noonan Syndrome–Associated Hypertrophic Cardiomyopathy

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Key Points

To evaluate the safety and effectiveness of surgical closure of atrial septal defect in infants receiving trametinib for Noonan syndrome-associated hypertrophic cardiomyopathy.
Infants diagnosed with Noonan syndrome and hypertrophic cardiomyopathy were treated with trametinib.
Patients underwent surgical closure of the atrial septal defect after stabilization with trametinib.
Outcomes of surgical intervention were assessed post-operatively.
Surgical closure of the atrial septal defect was successful in the majority of cases.
Trametinib effectively stabilized infants for subsequent heart surgery.
Improved cardiac function was observed following the surgical procedure.

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Implication

Infants with Noonan syndrome and heart defects show successful surgery after trametinib treatment, indicating improved outcomes.

Abstract

Infants with severe presentations of NS-HCM and congenital heart defects can be medically stabilized with trametinib and then undergo successful structural heart surgery.

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Cite This Study

Hebson et al. (2026) studied this question.

synapsesocial.com/papers/699bee1c1c6c6bad5397fd1f https://doi.org/10.1016/j.jaccas.2026.107006

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Atrial Septal Defect Surgical Closure Following Trametinib Utilization in Noonan Syndrome–Associated Hypertrophic Cardiomyopathy

Key Points

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Implication

Abstract

Discussion

Cite This Study

Atrial Septal Defect Surgical Closure Following Trametinib Utilization in Noonan Syndrome–Associated Hypertrophic Cardiomyopathy

Key Points

Ask about this paper

Implication

Abstract

Discussion

Cite This Study

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