Nederend Marieke, Egorova Anastasia D, van der Kley Frank, Kiès Philippine, Roest Arno A W, Schalij Martin J, Jongbloed Monique R M
CAHAL, Center for Congenital Heart Disease Amsterdam Leiden, Leiden University Medical Center, Leiden, the Netherlands.
Department of Cardiology, Leiden University Medical Center, Leiden, the Netherlands.
Int J Cardiol Congenit Heart Dis. 2023 Oct 11;14:100479. doi: 10.1016/j.ijcchd.2023.100479. eCollection 2023 Dec.
The Fontan operation resulted in improved survival of patients with congenital heart defects not equipped to sustain biventricular circulation. Long-term complications are common, such as veno-venous collaterals (VVC). The aim of this study was to evaluate patient characteristics, percutaneous treatment strategy and (short-term) outcomes in adult Fontan patients with VVC, and review literature to date.
In this single-centre retrospective observational cohort study, patients who underwent percutaneous VVC closure between 2017 and 2023 were identified.
Thirteen patients underwent percutaneous VVC closure (77 % female, age at intervention 24 ± 4 years, 77 % systemic left ventricle, 77 % extracardiac tunnel, median conduit size 16 [16-20]mm). Indications for closure were symptoms and/or significant exercise-related hypoxia. Mean Fontan pressure was 10±4 mmHg. The VVC originated from tributaries of the vena cava superior (VCS) and connected to pulmonary veins (8 VVC, 32 %), VCS to systemic atrium (3 VVC, 12 %), VCS to coronary sinus (3 VVC, 12 %) and tributaries of vena cava inferior to pulmonary veins (11 VVC, 44 %). Twenty-three VVC were occluded using coils and/or plugs. No periprocedural complications occurred. At first follow-up at least 6 months after closure (n = 11), 9 patients (82 %) reported symptom reduction. Saturation at rest and peak exercise increased significantly (96 ± 3 to 98 ± 1 %, p = 0.040; 89 ± 3 to 93 ± 5 %, p = 0.024, respectively). Exercise capacity remained unchanged.
VVC typically connect the tributaries of the vena cava inferior and/or superior with the pulmonary veins. Low Fontan pressures do not exclude the presence of VVC. Percutaneous closure of VVC is technically feasible, safe, and associated with symptom reduction and a significant rise in resting and exercise oxygen saturation.
Fontan手术提高了先天性心脏缺陷患者的生存率,这些患者无法维持双心室循环。长期并发症很常见,如腔静脉侧支循环(VVC)。本研究的目的是评估患有VVC的成年Fontan患者的特征、经皮治疗策略和(短期)结局,并回顾迄今为止的文献。
在这项单中心回顾性观察队列研究中,确定了2017年至2023年间接受经皮VVC封堵术的患者。
13例患者接受了经皮VVC封堵术(77%为女性,干预时年龄24±4岁,77%为体循环左心室,77%为心外隧道,导管中位尺寸16[16 - 20]mm)。封堵的指征是症状和/或与运动相关的严重低氧血症。平均Fontan压力为10±4 mmHg。VVC起源于上腔静脉(VCS)的分支并连接到肺静脉(8个VVC,32%)、VCS到体心房(3个VVC,12%)、VCS到冠状窦(3个VVC,12%)以及下腔静脉分支到肺静脉(11个VVC,44%)。使用线圈和/或封堵器封堵了23个VVC。未发生围手术期并发症。在封堵后至少6个月的首次随访(n = 11)中,9例患者(82%)报告症状减轻。静息和运动峰值时的血氧饱和度显著升高(分别从96±3%升至98±1%,p = 0.040;从89±3%升至93±5%,p = 0.024)。运动能力保持不变。
VVC通常连接下腔静脉和/或上腔静脉的分支与肺静脉。低Fontan压力并不排除VVC的存在。经皮封堵VVC在技术上是可行的、安全的,并且与症状减轻以及静息和运动时血氧饱和度显著升高相关。