Division of Cardiology, Department of Pediatrics, Oregon Health & Science University, 707, SW Gaines Street, Mailcode: CDRC-P, Portland, OR, 97239, USA.
Division of Cardiology, Department of Pediatrics, University of California, Irvine, CA, USA.
Pediatr Cardiol. 2024 Jan;45(1):63-67. doi: 10.1007/s00246-023-03296-6. Epub 2023 Sep 23.
Sinus node dysfunction with concomitant junctional rhythm (JR) is frequently observed among Fontan patients and has been recognized as a contributor to heart failure. The impact and management of JR is unclear. A survey was mailed to all members of the Pediatric and Congenital Electrophysiology society (PACES) and members were asked to forward the questionnaire to their non-electrophysiology colleagues. Responses were received from 154 physicians (88 electrophysiologists (EP's) and 66 non-EP's (46 pediatric cardiologists and 20 adult congenital cardiologists). There were few differences in the response between EP's and non-EP's. Overall, 57% recommended an annual ambulatory ECG (AECG). A significant majority (80%) opted to continue to follow patients with significant periods of JR on AECG as long as the patients were asymptomatic, and showed no echocardiographic signs of cardiac decompensation. However, 84% would place a pacemaker in a patient with JR who was having open chest surgery for other reasons. Finally, pacemaker placement would be performed by 91% if a patient with JR showed signs of heart failure. Most congenital cardiologists would not recommend pacemaker placement in asymptomatic Fontan patients with JR. Further studies are needed on the Fontan population to determine the impact of SND and JR on longer term outcomes and to determine the role and optimal timing of pacemaker placement in these patients.
窦房结功能障碍伴交界性节律（JR）在 Fontan 患者中经常观察到，并且已被认为是心力衰竭的一个原因。JR 的影响和管理尚不清楚。一项调查邮寄给了儿科和先天性电生理学会（PACE）的所有成员，并要求成员将问卷转发给他们的非电生理科同事。共收到 154 名医生（88 名电生理学家（EP）和 66 名非 EP（46 名儿科心脏病学家和 20 名成人先天性心脏病学家）的回复。EP 和非 EP 的回答之间几乎没有差异。总体而言，57%的人建议每年进行一次动态心电图（AECG）。绝大多数（80%）选择继续在 AECG 上随访有明显 JR 期的患者，只要患者无症状，且没有超声心动图显示心脏失代偿的迹象。然而，如果 JR 患者因其他原因接受开胸手术，84%的人会为其放置起搏器。最后，如果 JR 患者出现心力衰竭迹象，91%的人会放置起搏器。大多数先天性心脏病专家不会建议在无症状的 Fontan 患者 JR 中放置起搏器。需要对 Fontan 人群进行进一步研究，以确定 SND 和 JR 对长期结果的影响，并确定这些患者起搏器放置的作用和最佳时机。
