Abstract：
OBJECTIVE: Shone\'s complex comprises of a combination of congenital cardiac anomalies causing obstructions in the left ventricle\'s inflow and outflow tracts. This systematic review aims to evaluate the clinical features and surgical outcomes of Shone\'s complex.
METHODS: An electronic literature search of PubMed and Scopus was performed to identify relevant studies related to the presentation, management, and outcomes of Shone\'s complex. Two reviewers independently performed selection. Data on study characteristics, participant demographics, interventions, outcomes, and follow-up durations were extracted and analyzed.
RESULTS: A total of 691 papers were identified, with 18 studies included in the final analysis. The majority of the studies (n = 12) focused on the pediatric age group. The most common clinical presentations were coarctation of the aorta (n = 17) and mitral stenosis (n = 12). Surgical interventions often involved staged approaches, prioritizing outflow before inflow obstructions. Mitral valve repair was preferred over replacement due to better long-term outcomes (n = 8). Biventricular repair was recommended due to improved postoperative outcomes, but often needed reoperations. Reoperations were common, primarily due to recurrent coarctation (n = 10), subaortic stenosis (n = 8), and mitral valve dysfunction (n = 7). Pulmonary hypertension (n = 10) and arrhythmias (n = 11) were significant complications. Most patients were in modified Ross/NYHA functional class 1 on follow-up. Mortality rates ranged from 4 to 28%, with better outcomes associated with early and strategic surgical interventions.
CONCLUSIONS: Early diagnosis and biventricular repair were associated with better outcomes while transplantation was often an eventuality. Standardized diagnostic criteria, long-term follow-up, and consensus guidelines are needed to improve the management of this congenital heart disease.
METHODS: An electronic literature search of PubMed and Scopus was performed to identify relevant studies related to the presentation, management, and outcomes of Shone\'s complex. Two reviewers independently performed selection. Data on study characteristics, participant demographics, interventions, outcomes, and follow-up durations were extracted and analyzed.
RESULTS: A total of 691 papers were identified, with 18 studies included in the final analysis. The majority of the studies (n = 12) focused on the pediatric age group. The most common clinical presentations were coarctation of the aorta (n = 17) and mitral stenosis (n = 12). Surgical interventions often involved staged approaches, prioritizing outflow before inflow obstructions. Mitral valve repair was preferred over replacement due to better long-term outcomes (n = 8). Biventricular repair was recommended due to improved postoperative outcomes, but often needed reoperations. Reoperations were common, primarily due to recurrent coarctation (n = 10), subaortic stenosis (n = 8), and mitral valve dysfunction (n = 7). Pulmonary hypertension (n = 10) and arrhythmias (n = 11) were significant complications. Most patients were in modified Ross/NYHA functional class 1 on follow-up. Mortality rates ranged from 4 to 28%, with better outcomes associated with early and strategic surgical interventions.
CONCLUSIONS: Early diagnosis and biventricular repair were associated with better outcomes while transplantation was often an eventuality. Standardized diagnostic criteria, long-term follow-up, and consensus guidelines are needed to improve the management of this congenital heart disease.
摘要：
目的：Shone\'s复合体包括导致左心室流入和流出道阻塞的先天性心脏异常的组合。本系统评价旨在评估Shone’s复合体的临床特征和手术结果。
方法：对PubMed和Scopus进行了电子文献检索，以确定与演示文稿相关的相关研究，管理,以及肖恩情结的结果。两名审阅者独立进行选择。有关研究特征的数据，参与者人口统计，干预措施，结果，并提取和分析随访持续时间。
结果：共鉴定出691篇论文，最终分析中包括18项研究。大多数研究（n=12）集中在儿科年龄组。最常见的临床表现是主动脉缩窄（n=17）和二尖瓣狭窄（n=12）。手术干预通常涉及分阶段的方法，优先考虑流入阻塞之前的流出。二尖瓣修复术优于置换术，因为长期效果更好（n=8）。由于改善了术后结果，建议进行双心室修复，但往往需要重新手术。再操作很常见，主要是由于反复缩窄(n=10)，主动脉瓣下狭窄（n=8），和二尖瓣功能障碍（n=7）。肺动脉高压（n=10）和心律失常（n=11）是明显的并发症。大多数患者在随访时处于改良的Ross/NYHA功能等级1。死亡率从4%到28%不等,与早期和战略性手术干预相关的更好的结果。
结论：早期诊断和双心室修复与更好的结果相关，而移植通常是一种可能性。标准化诊断标准，长期随访,和共识指南需要改善这种先天性心脏病的管理。
方法：对PubMed和Scopus进行了电子文献检索，以确定与演示文稿相关的相关研究，管理,以及肖恩情结的结果。两名审阅者独立进行选择。有关研究特征的数据，参与者人口统计，干预措施，结果，并提取和分析随访持续时间。
结果：共鉴定出691篇论文，最终分析中包括18项研究。大多数研究（n=12）集中在儿科年龄组。最常见的临床表现是主动脉缩窄（n=17）和二尖瓣狭窄（n=12）。手术干预通常涉及分阶段的方法，优先考虑流入阻塞之前的流出。二尖瓣修复术优于置换术，因为长期效果更好（n=8）。由于改善了术后结果，建议进行双心室修复，但往往需要重新手术。再操作很常见，主要是由于反复缩窄(n=10)，主动脉瓣下狭窄（n=8），和二尖瓣功能障碍（n=7）。肺动脉高压（n=10）和心律失常（n=11）是明显的并发症。大多数患者在随访时处于改良的Ross/NYHA功能等级1。死亡率从4%到28%不等,与早期和战略性手术干预相关的更好的结果。
结论：早期诊断和双心室修复与更好的结果相关，而移植通常是一种可能性。标准化诊断标准，长期随访,和共识指南需要改善这种先天性心脏病的管理。
