Unidad Integrada de Cardiopatías Congénitas del Adolescente y Adulto Vall d'Hebron-Sant Pau, Departamento de Cardiología, Hospital Universitario Vall d'Hebron, Barcelona, Spain; Departamento de Cardiología, Hospital de la Santa Creu I Sant Pau, Barcelona, Spain.
Unidad de Cardiopatías Congénitas del Adulto, Departamento de Cardiología, Hospital Universitario y Politécnico La Fe, Valencia, Spain; Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), Spain.
Rev Esp Cardiol (Engl Ed). 2024 Jan;77(1):6-16. doi: 10.1016/j.rec.2023.03.001. Epub 2023 Mar 9.
There is scarce information on patients with single ventricle physiology (SVP) and restricted pulmonary flow not undergoing Fontan circulation. This study aimed to compare survival and cardiovascular events in these patients according to the type of palliation.
SVP patient data were obtained from the databases of the adult congenital heart disease units of 7 centers. Patients completing Fontan circulation or developing Eisenmenger syndrome were excluded. Three groups were created according to the source of pulmonary flow: G1 (restrictive pulmonary forward flow), G2 (cavopulmonary shunt), and G3 (aortopulmonary shunts±cavopulmonary shunt). The primary endpoint was death.
We identified 120 patients. Mean age at the first visit was 32.2 years. Mean follow-up was 7.1 years. Fifty-five patients (45.8%) were assigned to G1, 30 (25%) to G2, and 35 (29.2%) to G3. Patients in G3 had worse renal function, functional class, and ejection fraction at the first visit and a more marked ejection fraction decline during follow-up, especially when compared with G1. Twenty-four patients (20%) died, 38 (31.7%) were admitted for heart failure, and 21 (17.5%) had atrial flutter/fibrillation during follow-up. These events were more frequent in G3 and significant differences were found compared with G1 in terms of death (HR, 2.9; 95%CI, 1.14-7.37; P=.026) and atrial flutter/fibrillation (HR, 2.9; 95%CI, 1.11-7.68; P=.037).
The type of palliation in patients with SVP and restricted pulmonary flow not undergoing Fontan palliation identifies distinct profiles. Patients palliated with aortopulmonary shunts have an overall worse prognosis with higher morbidity and mortality.
关于未行 Fontan 环术的单心室生理患者和肺血流量受限患者，相关信息较为匮乏。本研究旨在根据姑息治疗的类型，比较这些患者的生存率和心血管事件。
从 7 家中心的成人先天性心脏病科数据库中获取单心室生理患者的数据。排除完成 Fontan 环术或发生艾森曼格综合征的患者。根据肺血流量来源将患者分为三组：G1 组（限制性肺前向血流）、G2 组（腔肺分流术）和 G3 组（主-肺分流术±腔肺分流术）。主要终点为死亡。
共纳入 120 例患者，首次就诊时的平均年龄为 32.2 岁，平均随访时间为 7.1 年。55 例（45.8%）患者被分到 G1 组，30 例（25%）分到 G2 组，35 例（29.2%）分到 G3 组。G3 组患者在首次就诊时肾功能、心功能分级和射血分数较差，随访期间射血分数下降更明显，与 G1 组相比差异更显著。24 例（20%）患者死亡，38 例（31.7%）因心力衰竭住院，21 例（17.5%）在随访期间发生房性心动过速/心房颤动。G3 组这些事件更常见，与 G1 组相比，死亡（HR，2.9；95%CI，1.14-7.37；P=.026）和房性心动过速/心房颤动（HR，2.9；95%CI，1.11-7.68；P=.037）的发生率差异均有统计学意义。
未行 Fontan 环术的单心室生理合并肺血流量受限患者的姑息治疗类型可确定不同的特征。行主-肺分流术姑息治疗的患者总体预后较差，发病率和死亡率较高。
