Zheng Wayne C, d'Udekem Yves, Grigg Leeanne E, Zentner Dominica, Cordina Rachael, Celermajer David S, Buratto Edward, Konstantinov Igor E, Lee Melissa G Y
Heart Research, Clinical Sciences, Murdoch Children's Research Institute, Melbourne, Australia.
Department of Cardiac Surgery, The Royal Children's Hospital, Melbourne, Australia.
Int J Cardiol Congenit Heart Dis. 2023 Apr 5;12:100457. doi: 10.1016/j.ijcchd.2023.100457. eCollection 2023 Jun.
Patients with single ventricle (SV) without Fontan palliation are uncommon, and their long-term outcomes remain unclear.
Retrospective study of 35 adult patients with SV without Fontan from two tertiary centers. Primary outcome was mortality.
Median age at first follow-up was 31 years (IQR: 20-40). Most common defect was double inlet left ventricle (34%), and 69% had left ventricular morphology. Patients were unoperated (46%), had systemic-to-pulmonary artery shunt (31%) or bidirectional cavopulmonary shunt (23%) as final palliation. Most common reasons for not progressing to Fontan palliation were pulmonary vascular disease (54%), patient refusal (17%), Fontan takedown (14%), and hypoplastic pulmonary arteries (11%). Baseline mean hemoglobin was 195 ± 29 g/L, mean O saturation 83 ± 6.9%, and 4 patients in NYHA Class III‒IV. After a mean follow-up of 10 ± 8.3 years, there were 9 deaths with heart failure being the leading cause (n = 6). Age-adjusted survival of these adult SV survivors was 73% and 53% at 40 and 50 years of age, respectively. Deceased patients more frequently had renal impairment (50% vs 0%) and QRS prolongation (75% vs 16%) at baseline (all p < 0.05). During follow-up, 40% had a new arrhythmia (atrial: n = 14, ventricular: n = 3), 34% had one or more hospitalizations for heart failure, and 17% had a stroke. A greater proportion of patients with pre-existing or new atrial/ventricular arrhythmia died compared to those without (42% vs 6%, p = 0.02).
Patients with SV without Fontan have high mortality and a substantial burden of cardiovascular complications, particularly arrhythmia. QRS prolongation and renal impairment were associated with mortality.
未接受Fontan姑息治疗的单心室（SV）患者并不常见，其长期预后仍不清楚。
对来自两个三级中心的35例未接受Fontan手术的成年SV患者进行回顾性研究。主要结局是死亡率。
首次随访时的中位年龄为31岁（四分位间距：20 - 40岁）。最常见的缺陷是双入口左心室（34%），69%患者具有左心室形态。患者未接受手术（46%），最终姑息治疗采用体肺分流术（31%）或双向腔肺分流术（23%）。未进展至Fontan姑息治疗的最常见原因是肺血管疾病（54%）、患者拒绝（17%）、Fontan手术拆除（14%）和肺血管发育不全（11%）。基线时平均血红蛋白为195±29 g/L，平均血氧饱和度为83±6.9%，4例患者为纽约心脏协会（NYHA）Ⅲ - Ⅳ级。平均随访10±8.3年后，有9例死亡，心力衰竭是主要死因（n = 6）。这些成年SV幸存者在40岁和50岁时的年龄调整生存率分别为73%和53%。死亡患者在基线时更常出现肾功能损害（50%对0%）和QRS波增宽（75%对16%）（所有p < 0.05）。随访期间，40%患者出现新发心律失常（房性：n = 14，室性：n = 3），34%患者因心力衰竭住院1次或多次，17%患者发生中风。与无既往或新发房性/室性心律失常的患者相比，有此类情况的患者死亡比例更高（42%对6%，p = 0.02）。
未接受Fontan手术的SV患者死亡率高且心血管并发症负担重，尤其是心律失常。QRS波增宽和肾功能损害与死亡率相关。
