Coşkun Yücel Nazlı Melis, Aydın Ahmet, Sabuncu Timuçin, Alpat Şafak, Güvener Murat, Yılmaz Mustafa
Department of Cardiovascular Surgery, Hacettepe University Faculty of Medicine, Ankara, Türkiye.
Turk Gogus Kalp Damar Cerrahisi Derg. 2024 Dec 31;32(4 Suppl 2):095-95. doi: 10.5606/tgkdc.dergisi.2024.msb-73. eCollection 2024 Nov.
This study aimed to share our surgical experience with 10 pediatric patients with hypertrophic cardiomyopathy (HOCM).
This study included 10 HOCM patients, aged between 8 months and 18 years, who underwent surgery due to conditions such as aortic insufficiency, mitral insufficiency, significant narrowing of the left ventricular cavity, and left ventricular outflow tract (LVOT) obstruction.
Two patients underwent isolated myectomy, while the remaining eight underwent myectomy combined with mitral valve replacement (MVR). Myectomy was performed via aortotomy, left atriotomy, and apical ventriculotomy. All patients had preoperative signs of systolic anterior motion (SAM) and a gradient in the LVOT. In the two patients who underwent isolated myectomy, SAM signs persisted postoperatively. Additionally, the gradient in the LVOT was measured to be higher in isolated myectomy cases compared to cases in which MVR was performed. Despite being in the pediatric age group with narrow annuli, all patients tolerated the MVR procedure with low-profile valves.
According to our experience, simultaneous MVR in pediatric HOCM cases requiring surgery is more effective in enlarging the left ventricular cavity and reducing the gradient in the LVOT, as it allows both the removal of the mitral valvular apparatus and the prevention of SAM. It provides a greater cavity volume gain compared to the alternative manipulation of the mitral valve apparatus. In our clinic, MVR surgery combined with myectomy is safely performed in pediatric HOCM cases.
本研究旨在分享我们对10例肥厚型心肌病(HOCM)患儿的手术经验。
本研究纳入10例年龄在8个月至18岁之间的HOCM患者,这些患者因主动脉瓣关闭不全、二尖瓣关闭不全、左心室腔显著狭窄及左心室流出道(LVOT)梗阻等情况接受手术。
2例患者接受单纯室间隔心肌切除术,其余8例接受室间隔心肌切除术联合二尖瓣置换术(MVR)。室间隔心肌切除术通过主动脉切开、左心房切开及心尖心室切开进行。所有患者术前均有收缩期前向运动(SAM)体征及LVOT压力阶差。在接受单纯室间隔心肌切除术的2例患者中,术后SAM体征持续存在。此外,与行MVR的病例相比,单纯室间隔心肌切除术病例的LVOT压力阶差测量值更高。尽管处于瓣环狭窄的儿童年龄组,但所有患者均耐受使用小号瓣膜的MVR手术。
根据我们的经验,在需要手术的小儿HOCM病例中同时进行MVR在扩大左心室腔及降低LVOT压力阶差方面更有效,因为它既能切除二尖瓣装置又能预防SAM。与二尖瓣装置的其他操作相比,它能使心室容积获得更大增加。在我们的诊所,小儿HOCM病例中安全地进行了MVR手术联合室间隔心肌切除术。