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[A case of successfully operated hypertrophic obstructive cardiomyopathy in a child]
1Second Division of Surgery, Children's Research Hospital, Kyoto Prefectural University of Medicine, Japan.
Insights
Surgical treatment for hypertrophic obstructive cardiomyopathy (HOCM) in a 5-year-old boy, the youngest in Japan, successfully reduced pressure gradients. Myectomy and myotomy provided a satisfactory outcome without mitral valve replacement.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Surgery
- Medical Imaging
Background:
- Hypertrophic obstructive cardiomyopathy (HOCM) is a significant cause of pediatric heart disease.
- Conservative management with beta-blockers and calcium antagonists may fail to alleviate symptoms in severe pediatric HOCM cases.
- Surgical intervention is considered when medical therapy is insufficient.
Observation:
- A 5-year-old boy, the youngest reported in Japan, presented with worsening dyspnea and chest oppression due to HOCM.
- Cardiac catheterization revealed a significant left ventricular-aortic pressure gradient (110 mmHg).
- Echocardiography demonstrated systolic anterior motion (SAM) of the mitral valve and mitral regurgitation (MR).
Findings:
- Transaortic septal myectomy and myotomy were performed based on preoperative echocardiogram and MRI findings.
- The surgical procedure resulted in a marked reduction of the systolic pressure gradient.
- Postoperative assessment showed a significant decrease in SAM and MR.
Implications:
- Septal myectomy and myotomy can be an effective surgical strategy for pediatric HOCM.
- Preoperative imaging analysis (echocardiography, MRI) is crucial for guiding surgical planning in pediatric HOCM.
- This approach offers a satisfactory outcome without the need for mitral valve replacement in select pediatric HOCM patients.
Abstract:
A case of surgical treatment of hypertrophic obstructive cardiomyopathy (HOCM) in a 5-year-old boy is reported. This is the youngest case in Japan. He had been diagnosed as HOCM and treated conservatively with beta-blocker and Ca-antagonist, but his complaints of dyspnea and chest oppression during efforts had increased. Cardiac catheterization showed a peak systolic pressure gradient of 110 mmHg between the left ventricle and systemic artery, and echocardiogram disclosed a systolic anterior motion (SAM) of the mitral valve and mitral regurgitation (MR). At operation transaortic septal myectomy and myotomy were performed according to preoperative findings by echocardiogram and MRI, and a marked reduction in pressure gradient and a decrease in SAM and MR were obtained. Thus, a satisfactory result can be obtained by myectomy and myotomy without mitral valve replacement in a pediatric patient with HOCM, based on the preoperative image analysis.