Department of Pediatrics and Pediatric Cardiology, Kitasato University.
Department of Pediatrics, Iwate Medical University.
Int Heart J. 2021 Mar 30;62(2):448-452. doi: 10.1536/ihj.20-461. Epub 2021 Mar 17.
While the advancement of perioperative management has expanded Fontan candidacy, not all patients have a successful postoperative course. Our case was a right isomerism patient who could not leave the ICU due to high central venous pressure and low output syndrome. Initial observation of the monitor ECG showed his rhythm to be supraventricular, however, an echocardiogram indicated simultaneous contraction of the atrium and ventricle, implying a junctional rhythm. While neither central venous pressure nor blood pressure improved with temporary pacing, better central venous and pulmonary venous blood flow patterns during pacing unraveled its positive impact. The patient successfully left the ICU after permanent pacing implantation. Hemodynamic study revealed a beneficial impact of atrial pacing in securing cardiac output and ventricular preload, lowering central venous pressure, and shortening blood transit time, which is partly attributed to the optimization of the fenestration function in reservation of the preload. Our case emphasizes the significant advantage of atrial pacing in a failing Fontan patient with junctional rhythm by reducing venous congestion and maximizing the benefit of fenestration.
尽管围手术期管理的进步扩大了 Fontan 手术的适应证范围，但并非所有患者都能成功完成术后康复。我们的病例是一位右位异构患者，由于中心静脉压高和低心输出量综合征，他无法离开重症监护病房。最初监测心电图显示他的节律为室上性，但超声心动图显示心房和心室同时收缩，提示交界性节律。虽然临时起搏并不能改善中心静脉压和血压，但起搏时更好的中心静脉和肺静脉血流模式揭示了其积极的影响。患者在植入永久性起搏后成功离开重症监护病房。血流动力学研究显示，心房起搏可通过增加心输出量和心室前负荷、降低中心静脉压和缩短血液通过时间来改善心脏功能，这在一定程度上归因于优化房间隔缺损的保留功能来增加前负荷。我们的病例强调了在出现交界性节律的 Fontan 衰竭患者中，心房起搏通过减少静脉淤血和最大限度地发挥房间隔缺损的益处，具有重要的优势。
