Latus Heiner, Kruppa Pia, Hofmann Lucas, Reich Bettina, Jux Christian, Apitz Christian, Schranz Dietmar, Voges Inga, Khalil Markus, Gummel Kerstin
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.
Int J Cardiol. 2020 Jul 15;311:28-34. doi: 10.1016/j.ijcard.2020.01.065. Epub 2020 Jan 29.
Single ventricle (SV) patients with a Fontan circulation are at risk for functional deterioration. The aim of this study was to assess longitudinal Fontan hemodynamics using serial cardiovascular magnetic resonance (CMR) data and to study the impact of aortopulmonary collateral (APC) flow and type of SV morphology.
Forty-one Fontan patients (age at first CMR 13.4 ± 6.0 years) with two CMR examinations within a time interval of at least 4 years (follow-up 5.3 ± 0.9 years) were included. The protocol consisted of short-axis cine volumetry and 2-dimensional blood flow measurements in the inferior vena cava (IVC), superior vena cava (SVC) and ascending aorta (Ao). APC flow was calculated as Ao - (SVC + IVC). Myocardial strain/strain rate was assessed using feature tracking technique.
SV end-diastolic volume (p = 0.14) and ejection fraction (p = 0.70) remained constant. No significant changes in CMR derived myocardial strain/strain rate values were observed. Ao flow decreased (p = 0.01), IVC flow remained unchanged (p = 0.52), while SVC flow (p = 0.003) and APC flow (p = 0.006) decreased significantly. Patients with a systemic right ventricle (RV) showed unchanged APC flow and a further increase in SV size over time compared to patients with a systemic left ventricle.
Longitudinal CMR data in a cohort of clinically stable Fontan patients revealed no significant changes in SV dimensions and myocardial performance while APC flow decreased spontaneously. Patients with a systemic RV seem to be at risk for permanent SV volume overload through APCs and might therefore benefit from consequent interventional APC embolization.
接受Fontan循环的单心室（SV）患者存在功能恶化风险。本研究旨在利用系列心血管磁共振（CMR）数据评估Fontan循环的纵向血流动力学，并研究主肺动脉侧支（APC）血流及SV形态类型的影响。
纳入41例Fontan患者（首次CMR检查时年龄为13.4±6.0岁），在至少4年的时间间隔内进行了两次CMR检查（随访时间为5.3±0.9年）。检查方案包括短轴电影容积测定以及下腔静脉（IVC）、上腔静脉（SVC）和升主动脉（Ao）的二维血流测量。APC血流计算为Ao - （SVC + IVC）。使用特征追踪技术评估心肌应变/应变率。
SV舒张末期容积（p = 0.14）和射血分数（p = 0.70）保持不变。未观察到CMR得出的心肌应变/应变率值有显著变化。Ao血流减少（p = 0.01），IVC血流保持不变（p = 0.52），而SVC血流（p = 0.003）和APC血流（p = 0.006）显著减少。与具有体循环左心室的患者相比，具有体循环右心室（RV）的患者APC血流无变化，且SV大小随时间进一步增加。
一组临床稳定的Fontan患者的纵向CMR数据显示，SV尺寸和心肌功能无显著变化，而APC血流自发减少。具有体循环RV的患者似乎有通过APC导致永久性SV容量超负荷的风险，因此可能受益于相应的介入性APC栓塞。
