Kuhn Miriam, Hornung Andreas, Ulmer Heidi, Schlensak Christian, Hofbeck Michael, Wiegand Gesa
Department of Pediatric Cardiology, University Children's Hospital, Hoppe-Seyler-Str. 1, 72076, Tuebingen, Germany.
Department of Thoracic and Cardiovascular Surgery, University Hospital Tuebingen, Tuebingen, Germany.
Pediatr Cardiol. 2018 Apr;39(4):810-817. doi: 10.1007/s00246-018-1824-9. Epub 2018 Feb 3.
There are many complex cardiac malformations that are characterized by a functionally univentricular physiology. Staged surgical repair according to the Fontan principle separates the systemic and pulmonary circulations by connecting the systemic venous return to the pulmonary arteries. However, long-term follow-up studies demonstrate a gradual deterioration of cardiac function, particularly from the second or third decade. Noninvasive evaluation of the cardiac function is, therefore, important in the follow-up of these patients. The cardiac index (CI) is a reliable hemodynamic parameter and represents an important marker of cardiac function. We compared CI values determined by cardiac MRI (CMRI) with values obtained by noninvasive inert gas rebreathing (IGR; Innocor® system). Sixteen patients (age range: 7.2-32.7 years) with functionally univentricular hearts (UVH) following total cavopulmonary connection (TCPC) were compared with 12 healthy subjects (age range: 8.5-18.6 years). The standard treadmill protocol of the German Society of Pediatric Cardiology was used for exercise testing. CI was determined at rest and at two standardized submaximal exercise levels. In all subjects, CI increased under exercise conditions, but the values were significantly lower in patients with UVH. There was no significant difference between patients with UVH and predominantly right- or left-ventricular morphology. In comparison with CMRI measurements, the CI values obtained by the IGR method tended to be lower, with a mean difference of 1.02 l/min/m. Noninvasive measurement of CI with the IGR method is feasible at rest and during exercise, and appears to be suited for routine determination of CI in patients with UVH following TCPC.
存在许多以功能性单心室生理为特征的复杂心脏畸形。根据Fontan原则进行的分期手术修复通过将体静脉回流与肺动脉相连来分离体循环和肺循环。然而,长期随访研究表明心脏功能会逐渐恶化,尤其是在第二个或第三个十年。因此,心脏功能的无创评估在这些患者的随访中很重要。心脏指数(CI)是一个可靠的血流动力学参数,代表心脏功能的一个重要指标。我们比较了心脏磁共振成像(CMRI)测定的CI值与无创惰性气体再呼吸(IGR;Innocor®系统)获得的值。将16例(年龄范围:7.2 - 32.7岁)在全腔静脉肺动脉连接术(TCPC)后患有功能性单心室心脏(UVH)的患者与12名健康受试者(年龄范围:8.5 - 18.6岁)进行比较。采用德国儿科心脏病学会的标准跑步机方案进行运动测试。在静息状态和两个标准化的次最大运动水平下测定CI。在所有受试者中,运动时CI均升高,但UVH患者的值显著更低。UVH患者中以右心室或左心室形态为主的患者之间无显著差异。与CMRI测量相比,IGR方法获得的CI值往往更低,平均差异为1.02 l/min/m²。采用IGR方法对CI进行无创测量在静息和运动时都是可行的,似乎适用于TCPC术后UVH患者CI的常规测定。