Division of Pediatric Cardiology, Department of Pediatric and Adolescent Medicine, Mayo Clinic College of Medicine, Rochester, Minnesota, USA.
Division of Cardiovascular Diseases, Department of Medicine, Mayo Clinic College of Medicine, Rochester, Minnesota, USA Division of Cardiovascular Radiology, Department of Radiology, Mayo Clinic College of Medicine, Rochester, Minnesota, USA.
Heart. 2016 Feb;102(3):216-22. doi: 10.1136/heartjnl-2015-308440. Epub 2015 Dec 23.
Patients with unoperated single ventricle (SV) rarely survive into adulthood with good functional status and may develop Eisenmenger's syndrome (ES). We report outcomes of a 30-year cohort of such patients.
Adult patients with unoperated SV were identified by searching the Mayo Clinic medical record from 1984 to 2014. Clinical data were collected and compared between patients with pulmonary stenosis (PS) and ES.
24 patients were identified (median peak-age 56 (31-77) years (11 ES, 13 PS); 22 had left ventricular morphology. 50-year transplant-free survival was 65% (95% CI 43 to 81). Median age at death was 55 years (31-77 years); 15 deaths (62%) occurred before oral pulmonary vasodilators were commercially available. Two-thirds of the cohort demonstrated preserved New York Heart Association functional class and median EF was 60% (49% to 62%). The majority of patients to survive into the fifth decade exhibited anatomy of double-inlet LV (DILV) with PS.
Selected patients with unoperated SV with PS and ES can survive with good functional class up to the eighth decade with good medical management. DILV/PS appears to be the ideal phenotype for advanced survival. Our outcomes may be considered when such patients with SV having 'balanced' physiology are evaluated for Fontan palliation. However, additional prospective study will be necessary to verify this assertion.
未经手术的单心室（SV）患者很少能在成年后保持良好的功能状态并生存下来，且可能发展为艾森曼格综合征（ES）。我们报告了这一 30 年队列患者的结局。
通过检索 1984 年至 2014 年梅奥诊所的医疗记录，确定未经手术的 SV 成年患者。收集临床资料并比较肺动脉瓣狭窄（PS）和 ES 患者之间的差异。
共确定了 24 例患者（中位峰值年龄 56（31-77）岁（11 例 ES，13 例 PS）；22 例患者具有左心室形态。无移植 50 年生存率为 65%（95%CI 43 至 81）。中位死亡年龄为 55 岁（31-77 岁）；在口服肺血管扩张剂商业化之前，有 15 例患者（62%）死亡。该队列的三分之二患者保持纽约心脏协会心功能分级良好，且中位 EF 为 60%（49%至 62%）。大多数能存活到第五个十年的患者表现出 PS 合并双入口左心室（DILV）的解剖结构。
经过精心管理，选择具有 PS 和 ES 的未经手术的 SV 患者，可在良好的功能状态下生存至第八个十年。DILV/PS 似乎是晚期生存的理想表型。在对具有“平衡”生理特征的 SV 患者进行 Fontan 姑息治疗评估时，可考虑我们的结果。但是，需要进一步的前瞻性研究来验证这一结论。
