Pediatric and Grown-up Congenital Cardiac Surgery, University of Bologna, S.Orsola-Malpighi Hospital, Bologna, Italy.
Eur J Cardiothorac Surg. 2012 Dec;42(6):996-1002. doi: 10.1093/ejcts/ezs202. Epub 2012 Apr 26.
To define the evolution of the single-ventricle (SV) heart in adult patients in terms of morbidity, mortality and quality of life.
Sixty-two patients with SV physiology and aged older than 16 years were retrospectively reviewed. Three patients (5%) were in natural history, one had received a Blalock-Taussig shunt, one a Waterstone anastomosis, one a pulmonary artery banding, three a bidirectional cavopulmonary anastomosis, eight a classic Fontan procedure and 46 a total cavopulmonary connection (TCPC). The morphology of the SV was left in 48 patients (77%), right in nine (14%) and indeterminable in five (8%). Thirty-three patients underwent magnetic resonance imaging (MRI) to assess ventricular mass (VM), ventricular systolic function, pulmonary artery branch diameter and potential thrombosis of the conduit. Cardiopulmonary exercise testing (CPTE) was carried out to evaluate exercise tolerance. The quality of life was monitored with two different specific tests, the Short Form-36 (SF-36) and the congenital heart disease-TNO/AZL adult quality of life (CHD-TAAQOL). The mean follow-up time was 8.0 ± 9.1 years.
Two of the three patients in natural history underwent primary TCPC. Re-interventions were necessary in seven patients (11%). Three patients (5%) died during follow-up. Five patients (8%) underwent cardiac transplantation. Protein losing enteropathy appeared in six (10%), while the arrhythmic disorder was detected in 13 patients. On the MRI, the mean end-diastolic ventricular volume was 106 ± 448 ml/m(2), the mean ejection fraction (EF) was 52.3 ± 10% and VM was 56 ± 22.1 g/m(2). On CPTE, the peak of oxygen uptake (peak VO(2)) was moderately impaired in 92% of patients, while 4% presented a severely impaired and 4% a normal peak of VO(2). No correlations were found among the peak of VO(2) and the quality-of-life evaluation.
Adult patients with SV are at high risk of reoperations and need of transplant and complications. Nevertheless, in the presence of a moderately reduced peak of VO(2) and a moderate reduction in the EF detected at the MRI, the results of the evaluation of daily quality of life are incredibly high.
从发病率、死亡率和生活质量的角度来描述成人单心室（SV）心脏的演变。
回顾性分析 62 例年龄大于 16 岁且具有 SV 生理学特征的患者。其中 3 例（5%）为自然病史，1 例接受了 Blalock-Taussig 分流术，1 例接受了 Waterstone 吻合术，1 例接受了肺动脉带缩术，3 例接受了双向腔肺吻合术，8 例接受了经典 Fontan 手术，46 例接受了全腔静脉-肺动脉连接术（TCPC）。48 例（77%）SV 形态为左心室，9 例（14%）为右心室，5 例（8%）形态不定。33 例患者接受了磁共振成像（MRI）检查以评估心室质量（VM）、心室收缩功能、肺动脉分支直径和管道潜在血栓形成。进行心肺运动试验（CPTE）以评估运动耐量。使用两种不同的特定测试，即 36 项简短健康调查问卷（SF-36）和先天性心脏病-TNO/AZL 成人生活质量（CHD-TAAQOL）来监测生活质量。平均随访时间为 8.0 ± 9.1 年。
自然病史组的 3 例患者中有 2 例接受了初次 TCPC。7 例患者（11%）需要再次干预。3 例患者（5%）在随访期间死亡。5 例患者（8%）接受了心脏移植。6 例（10%）出现蛋白丢失性肠病，13 例患者出现心律失常。在 MRI 上，平均舒张末期心室容积为 106 ± 448 ml/m2，平均射血分数（EF）为 52.3 ± 10%，VM 为 56 ± 22.1 g/m2。在 CPTE 上，92%的患者峰值摄氧量（peak VO2）中等受损，4%的患者峰值 VO2 严重受损，4%的患者峰值 VO2 正常。在峰值 VO2 与生活质量评估之间未发现相关性。
成人单心室患者再手术、移植和并发症的风险较高。然而，在 MRI 上检测到峰值 VO2 中度降低和 EF 中度降低的情况下，日常生活质量评估的结果令人难以置信地高。
