Nguyen Stephanie N, Chung Megan M, Vinogradsky Alice V, Richmond Marc E, Zuckerman Warren A, Goldstone Andrew B, Bacha Emile A
Section of Pediatric and Congenital Cardiac Surgery, New York Presbyterian/Morgan Stanley Children's Hospital, Columbia University Irving Medical Center, New York, NY.
Division of Pediatric Cardiology, New York Presbyterian/Morgan Stanley Children's Hospital, Columbia University Irving Medical Center, New York, NY.
JTCVS Open. 2023 Oct 4;16:726-738. doi: 10.1016/j.xjon.2023.09.032. eCollection 2023 Dec.
Septal reduction therapy via septal myectomy or a modified Konno procedure is the mainstay of therapy for drug-refractory obstructive hypertrophic cardiomyopathy (HCM), although outcomes data on septal myectomy in pediatric patients are limited. We evaluated long-term outcomes following surgery for obstructive HCM in a pediatric cohort.
We retrospectively reviewed patients age ≤18 years with obstructive HCM who underwent a left and/or right ventricular septal myectomy at our institution between 1992 and 2022. Primary endpoints were transplantation-free survival, freedom from HCM-related death, and cumulative probability of HCM-related reintervention. We further evaluated outcomes in patients with and without Noonan syndrome or other RASopathies.
Thirty-seven patients (median age, 7.4 years; interquartile range [IQR], 3.4-12.9 years) underwent transaortic septal myectomy. A combined modified Konno procedure was performed in 5 patients (13.9%). Sixteen patients (43.2%) had a RASopathy. A concomitant right ventricular outflow tract resection was performed in 9 patients (24.3%). There was 1 (2.7%) in-hospital death and 4 late deaths at a median follow-up of 10.5 years (IQR, 0.1-29.3). Twenty-year transplant-free survival and freedom from HCM-related death were 80.6% (95% confidence interval [CI], 64.2%-100%) and 87.1% (95% CI, 71.8%-100%), respectively. The 20-year cumulative probability of HCM-related reintervention was 34.2% (95% CI, 12.8%-57.1%). Seven patients required a septal reintervention. There was no difference in any primary endpoints between patients with and without a RASopathy.
Surgery for obstructive HCM, including septal myectomy with and without a modified Konno procedure, may be performed with low morbidity and good long-term outcomes in pediatric patients. Recurrent outflow tract obstruction is not uncommon.
经室间隔心肌切除术或改良的Konno手术进行的间隔减容治疗是药物难治性梗阻性肥厚型心肌病（HCM）的主要治疗方法，尽管小儿患者室间隔心肌切除术的疗效数据有限。我们评估了小儿队列中梗阻性HCM手术后的长期疗效。
我们回顾性分析了1992年至2022年间在我院接受左和/或右心室间隔心肌切除术的年龄≤18岁的梗阻性HCM患者。主要终点是无移植生存、无HCM相关死亡以及HCM相关再次干预的累积概率。我们进一步评估了有无努南综合征或其他RAS病患者的疗效。
37例患者（中位年龄7.4岁；四分位间距[IQR]为3.4 - 12.9岁）接受了经主动脉室间隔心肌切除术。5例患者（13.9%）进行了联合改良Konno手术。16例患者（43.2%）患有RAS病。9例患者（24.3%）同时进行了右心室流出道切除术。在中位随访10.5年（IQR为0.1 - 29.3）时，有1例（2.7%）住院死亡和4例晚期死亡。20年无移植生存率和无HCM相关死亡分别为80.6%（95%置信区间[CI]为64.2% - 100%）和87.1%（95% CI为71.8% - 100%）。HCM相关再次干预的20年累积概率为34.2%（95% CI为12.8% - 57.1%）。7例患者需要进行间隔再次干预。有无RAS病患者的任何主要终点均无差异。
梗阻性HCM的手术，包括有或无改良Konno手术的室间隔心肌切除术，在小儿患者中可能具有低发病率和良好的长期疗效。复发性流出道梗阻并不少见。
