Pediatric Heart Center, Justus-Liebig University Clinic, Giessen, Germany; Department of Pediatric Cardiology and Congenital Heart Disease, German Heart Centre, Munich, Germany.
Pediatric Heart Center, Justus-Liebig University Clinic, Giessen, Germany.
J Thorac Cardiovasc Surg. 2019 Aug;158(2):523-531.e1. doi: 10.1016/j.jtcvs.2018.11.144. Epub 2019 Mar 9.
Pulmonary endothelial dysfunction due to a loss of pulsatile pulmonary blood flow is thought to be a major contributor in the development of increased pulmonary vascular resistance (PVR) in patients with a Fontan circulation. We sought to evaluate pulmonary endothelial dysfunction by vasodilator response to acetylcholine in children and adolescents with Fontan hemodynamics.
Twenty-one Fontan patients with a median age of 10.7 years (range, 3.4-30.0 years) underwent invasive cardiac catheterization, including intra-arterial Doppler and pressure measurements in a segmental pulmonary artery. Pulmonary flow reserve (PFR) was quantified as the change of average peak velocity in response to acetylcholine infusion. Local PVR was assessed by the ratio of local pressure and blood flow velocity.
Average peak velocity significantly increased from 16.4 ± 6.6 cm/s to 20.1 ± 6.4 cm/s (P = .002) resulting in a mean PFR of 1.28 ± 0.37. Local PVR dropped from 0.72 ± 0.32 mm Hg/cm/s to 0.57 ± 0.20 mm Hg/cm/s (n = 19; P = .01). A significant relationship was found between baseline local PVR and PFR (r = 0.73; P = .0006) as well as between PFR and the percent decrease in local PVR (r = 0.85; P < .0001). In addition, percentage decrease in local PVR correlated with New York Heart Association functional class, whereas neither PFR nor local PVR correlated with global PVR.
The assessment of PFR and local PVR may help to evaluate adverse pulmonary vascular remodeling in Fontan patients as an adjunct to the usual catheterization protocol, particularly given the difficulties in assessing global PVR in these patients. Future research is required to study whether impaired local pulmonary endothelial function may be associated with Fontan failure.
由于搏动性肺血流丧失导致的肺内皮功能障碍被认为是法洛四联症患者肺血管阻力（PVR）增加的主要原因。我们试图通过评估法洛四联症患者的血管舒张反应来评估肺内皮功能障碍。
21 名法洛四联症患者，中位年龄 10.7 岁（范围 3.4-30.0 岁），接受了有创性心导管检查，包括节段性肺动脉内动脉多普勒和压力测量。肺血流储备（PFR）通过乙酰胆碱输注时平均峰值速度的变化来量化。局部 PVR 通过局部压力和血流速度的比值来评估。
平均峰值速度从 16.4 ± 6.6cm/s 显著增加到 20.1 ± 6.4cm/s（P =.002），平均 PFR 为 1.28 ± 0.37。局部 PVR 从 0.72 ± 0.32mmHg/cm/s 降至 0.57 ± 0.20mmHg/cm/s（n = 19；P =.01）。在基线局部 PVR 和 PFR 之间（r = 0.73；P =.0006）以及 PFR 和局部 PVR 降低百分比之间（r = 0.85；P <.0001）发现显著的关系。此外，局部 PVR 降低百分比与纽约心脏协会功能分级相关，而 PFR 和局部 PVR 均与全球 PVR 不相关。
PFR 和局部 PVR 的评估可以帮助评估法洛四联症患者的不良肺血管重塑，作为常规心导管检查方案的辅助手段，特别是考虑到这些患者评估全球 PVR 的困难。需要进一步研究来研究局部肺内皮功能障碍是否与法洛四联症失败有关。
