Van den Eynde Jef, van der Palen Roel L F, Bökenkamp Regina, Hazekamp Mark G
Department of Cardiothoracic Surgery, Leiden University Medical Center, Leiden, Netherlands.
Department of Cardiovascular Sciences, KU Leuven, Leuven, Belgium.
J Surg Case Rep. 2022 Nov 3;2022(11):rjac495. doi: 10.1093/jscr/rjac495. eCollection 2022 Nov.
In patients with critical left ventricular outflow tract obstruction but adequately sized ventricles, the treatment of choice is biventricular repair. Several options have been proposed, including neonatal Yasui or Ross-Konno operation. However, each of these procedures carries a high mortality risk, especially in syndromic neonates. Here, we report the case of a patient with 22q11.2 deletion syndrome and a diagnosis of interrupted aortic arch type B2, ventricular septal defect and left ventricular outflow tract obstruction. As a means to avoid high-risk neonatal surgery in this patient, we pursued a strategy of delayed biventricular repair involving initial hybrid Norwood palliation followed by a Yasui-type operation at 3 months. Although this strategy turned out to be successful, proactive monitoring for the development of ductal stent stenosis during follow-up after the hybrid procedure remains crucial to prevent hemodynamic complications such as cardiac failure and systemic hypoperfusion.
对于左心室流出道严重梗阻但心室大小合适的患者，首选治疗方法是双心室修复。已经提出了几种选择，包括新生儿安井手术或罗斯-孔诺手术。然而，这些手术中的每一种都有很高的死亡风险，尤其是在患有综合征的新生儿中。在此，我们报告一例患有22q11.2缺失综合征的患者，诊断为B2型主动脉弓中断、室间隔缺损和左心室流出道梗阻。为了避免该患者进行高风险的新生儿手术，我们采取了延迟双心室修复策略，包括最初的杂交诺伍德姑息手术，随后在3个月时进行安井式手术。尽管这一策略最终取得了成功，但在杂交手术后的随访期间，积极监测导管支架狭窄的发生对于预防心力衰竭和全身灌注不足等血流动力学并发症仍然至关重要。
