Congenital Heart Surgeons' Society

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Transatrial-transmitral Septal Myectomy In Children: How I Do It?
Sertac Cicek, MD1, Ahmet H. Arslan, MD2, Sibel AY, MD3, FATMA BAHCECI, MD4, RESMIYE BESIKCI, MD4.
1Mayo Clinic, ROCHESTER, MN, USA, 2Anadolu Medical Center ISTANBUL, TURKEY, ISTANBUL,, Turkey, 3Anadolu Medical Center, ISTANBUL, Turkey, 4ANADOLU MEDICAL CENTER, ISTANBUL, Turkey.

Objective: Transaortic septal myectomy provides excellent relief of obstruction and is the gold standard surgical treatment for hypertrophic obstructive cardiomyopathy. In small children this might be a suboptimal approach due to limited exposure with potential for damage to aortic valve and insufficient muscle resection resulting in residual obstruction. Transatrial-transmitral approach has been attempted as an alternative. Operative technique and steps has been presented on this video.
Methods: A 7-year-old, 24 kg child with angina referred for evaluation. Transthoracic echocardiogram demonstrated hypertrophied ventricular septum with thickest segment measured 26 mm extending to the base of papillary muscles, left ventricular outflow maximal instantaneous Doppler gradient 170 mmHg and systolic anterior motion with 2+ mitral regurgitation. A decision for septal myectomy was made. Pre-CPB direct intracardiac pressures were LV: 202/11/88 mmHg and Ao: 74/47/54 mmHg. A transatrial-transmitral myectomy was performed with detachment of anterior mitral leaflet. Post-repair direct pressure measurements were LV: 91/8/43 mmHg and Ao; 85/39/54 mmHg. TEE showed widely patent LVOT with laminar flow and no residual gradients, no SAM and trivial mitral regurgitation.
Results: Patient stays asymptomatic currently with most recent echo at 4 years showing no residual LVOT gradient or mitral regurgitation.
Conclusions: This technique is a valuable addition to our armamentarium especially for children with small aortas. Benefits include a panoramic exposure extending from the basal septum to the base of papillary muscles and LV apex, no risk of injury to the aortic valve cusps, addressing the concomitant mitral abnormalities and an enhanced ability to teach trainees.