Septal Myectomy for Severe Neonatal Hypertrophic Cardiomyopathy Caused by PTPN11 Gene Mutation: A Case Report

Ningning Zhang, Liang Chen and Jing Zhang

Department of Pediatric Cardiac Surgery, Peking Union Medical College, People's Republic of China

^*Correspondance to: Jing Zhang 

 PDF  Full Text DOI: 10.25107/2474-1647.2566

Abstract

Noonan syndrome is rare disease with autosomal dominant genetic pattern. Hypertrophic Obstructive Cardiomyopathy (HOCM) is frequently observed in patients with Noonan syndrome and also represents the major determinant of the patient’s outcome. The reported experience of septal myectomy for infant Noonan syndrome patient with HOCM is very limited worldwide. In present study, we report a Chinese Noonan syndrome patient with severe neonatal hypertrophic obstructive cardiomyopathy, who underwent septal myectomy procedure at 8 months old. This patient received favorable perioperative result and good clinical outcome after 2 years follow-up. This is the earliest report of septal myectomy surgery for Noonan syndrome patient under 1 year of age, which supports that septal myectomy surgery for infant Noonan syndrome patients with HOCM is scalable with substantial benefit for patients.

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Cite the article

Zhang N, Chen L, Zhang J. Septal Myectomy for Severe Neonatal Hypertrophic Cardiomyopathy Caused by PTPN11 Gene Mutation: A Case Report. Clin Surg. 2019; 4: 2566..