Di Molfetta A, Iacobelli R, Guccione P, Di Chiara L, Rocchi M, Cobianchi Belisari F, Campanale M, Gagliardi M G, Filippelli S, Ferrari G, Amodeo A
Department of Pediatric Cardiology and Cardiac Surgery, Pediatric Hospital Bambino Gesù, Piazza Sant'Onofrio, 4, 00165, Rome, RM, Italy.
Faculty of Biomedical Engineer, Rome University Campus Bio-medico, Via Álvaro del Portillo, 21, Rome, RM, Italy.
Pediatr Cardiol. 2017 Dec;38(8):1613-1619. doi: 10.1007/s00246-017-1704-8. Epub 2017 Aug 22.
Hyperplastic left heart syndrome (HLHS) patients are palliated by creating a Fontan-type circulation passing from different surgical stages. The aim of this work is to describe the evolution of ventricular energetics parameters in HLHS patients during the different stages of palliation including the hybrid, the Norwood, the bidirectional Glenn (BDG), and the Fontan procedures. We conducted a retrospective clinical study enrolling all HLHS patients surgically treated with hybrid procedure and/or Norwood and/or BDG and/or Fontan operation from 2011 to 2016 collecting echocardiographic and hemodynamic data. Measured data were used to calculate energetic variables such as ventricular elastances, external and internal work, ventriculo-arterial coupling and cardiac mechanical efficiency. From 2010 to 2016, a total of 29 HLHS patients undergoing cardiac catheterization after hybrid (n = 7) or Norwood (n = 6) or Glenn (n = 8) or Fontan (n = 8) procedure were retrospectively enrolled. Ventricular volumes were significantly higher in the Norwood circulation than in the hybrid circulation (p = 0.03) with a progressive decrement from the first stage to the Fontan completion. Ventricular elastances were lower in the Norwood circulation than in the hybrid circulation and progressively increased passing from the first stage to the Fontan completion. The arterial elastance and Rtot increased in the Fontan circulation. The ventricular work progressively increased. Finally, the ventricular efficiency improves passing from the first to the last stage of palliation. The use of ventricular energetic parameters could lead to a more complete evaluation of such complex patients to better understand their adaptation to different pathophysiological conditions.
左心发育不全综合征（HLHS）患者通过不同手术阶段建立Fontan型循环来进行姑息治疗。本研究的目的是描述HLHS患者在姑息治疗不同阶段（包括杂交手术、诺伍德手术、双向格林分流术（BDG）和Fontan手术）中心室能量学参数的变化。我们进行了一项回顾性临床研究，纳入了2011年至2016年期间接受杂交手术和/或诺伍德手术和/或BDG手术和/或Fontan手术治疗的所有HLHS患者，收集了超声心动图和血流动力学数据。测量数据用于计算能量变量，如心室弹性、外部和内部功、心室 - 动脉耦合以及心脏机械效率。2010年至2016年，共有29例HLHS患者在接受杂交手术（n = 7）、诺伍德手术（n = 6）、格林分流术（n = 8）或Fontan手术（n = 8）后接受了心导管检查，并进行了回顾性登记。诺伍德循环中的心室容积显著高于杂交循环（p = 0.03），从第一阶段到Fontan手术完成逐渐减少。诺伍德循环中的心室弹性低于杂交循环，从第一阶段到Fontan手术完成逐渐增加。Fontan循环中的动脉弹性和总阻力增加。心室功逐渐增加。最后，从姑息治疗的第一阶段到最后阶段，心室效率提高。使用心室能量学参数可以对这类复杂患者进行更全面的评估，以便更好地了解他们对不同病理生理状况的适应性。
