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Published on: August 30, 2024
[Non-drug treatment for hypertrophic obstructive cardiomyopathy in children]
1Department of Pediatric Cardiology, Guangdong Academy of Medical Sciences, Guangzhou 510100, China.
Insights
Percutaneous transluminal septal myocardial ablation (PTSMA) and septal myectomy (SM) offer safe and effective relief for pediatric hypertrophic obstructive cardiomyopathy (HOCM). These non-medical therapies show promising short-term outcomes in improving symptoms and reducing obstruction.
Area of Science:
- Cardiology
- Pediatric Medicine
- Interventional Cardiology
Background:
- Hypertrophic obstructive cardiomyopathy (HOCM) in children can be drug-refractory, necessitating alternative treatments.
- Non-medical therapies are explored for symptomatic pediatric HOCM patients.
Observation:
- Four pediatric patients with drug-refractory HOCM underwent either percutaneous transluminal septal myocardial ablation (PTSMA) or septal myectomy (SM).
- Initial follow-up demonstrated symptom relief and significant reduction in left ventricular outflow tract gradients (LVOTG) and mitral regurgitation (MR).
Findings:
- PTSMA and SM were associated with significant short-term improvements in LVOTG and MR in pediatric HOCM patients.
- While most patients maintained relief, one experienced recurrence of LVOTO, though MR remained reduced.
- No severe complications like heart block or arrhythmias were observed; transient or persistent bundle branch blocks were noted.
Implications:
- PTSMA and SM appear to be safe and effective initial treatment options for symptomatic, drug-refractory pediatric HOCM.
- Further long-term studies are warranted to fully assess the durability and potential complications of these interventions.
Objective:
To retrospectively summarize the effect of non-medical therapies for pediatric patients with hypertrophic obstructive cardiomyopathy (HOCM).
Methods:
From Nov. 2008 to Jun. 2010, 4 children with drug-refractory HOCM were admitted to our hospital. Their ages were 14, 7, 9 and 6 years old, respectively. Their body weights were 38, 17, 21.5 and 17 kg, respectively. Before operation, the pressure gradients over left ventricular outflow tract (LVOTG) were 60, 147, 58 and 114 mm Hg (1 mm Hg = 0.133 kPa), respectively. And mitral regurgitation (MR) areas were 2.2, 7.3 cm(2) and 2.9 cm(2), respectively, except that it was trivial in one case. Percutaneous transluminal septal myocardial ablation (PTSMA) was performed in case 1 and 2. Septal myectomy (SM) was performed in case 3 and 4. Follow-up was first performed right after operation or before discharge, then 1 month, 3 months, 6 months, and 12 months after operation, and then once a year. The follow-up period was 1 - 18 (9.3 ± 8.1) months.
Results:
All patients experienced relieved symptoms. Three of them had their NYHA functional class improved except case 2. Echocardiography revealed that LVOTGs right after operations were 38, 79, 20 and 0 mm Hg, respectively, suggesting significant improvement of left ventricular outflow tract obstruction (LVOTO) in all patients. During follow-up, case 2 suffered from recurrence of LVOTO, while the other 3 cases showed sustained relief. In the last follow-up, the LVOTGs of the four patients were 19, 168, 16 and 0 mm Hg, respectively. Echocardiography also revealed that MRs of all patients were significantly reduced, even in case 2 whose LVOTG rebounded, with no recurrence during follow-up. Severe complications were absent, such as ventricular septum perforation, cardiac tamponade, ventricular tachycardia or ventricular fibrillation. No one suffered from complete heart block. Transient complete right bundle branch block (CRBBB) was observed in case 1 after PTSMA and converted to intraventricular block after 1 month. Complete left bundle branch block (CLBBB) was present in both case 3 and 4, who received SM. In case 4, it converted to intraventricular block after 1 month while in case 3 CLBBB persisted.
Conclusions:
The initial experience showed that PTSMA and SM were safe and effective for drug-refractory symptomatic HOCM children, with satisfactory short-term results. Further studies are needed to evaluate the long-term results and complications.
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