Department of Medicine, Bangalore Medical College and Research Institute, K.R Road, Bangalore, 560002, Karnataka, India.
Gen Thorac Cardiovasc Surg. 2024 Oct;72(10):621-640. doi: 10.1007/s11748-024-02067-1. Epub 2024 Aug 2.
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.
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.
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.
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.
Shone 综合征由一系列先天性心脏畸形组成，导致左心室流入道和流出道阻塞。本系统评价旨在评估 Shone 综合征的临床特征和手术结果。
对 PubMed 和 Scopus 进行电子文献检索，以确定与 Shone 综合征的表现、治疗和结局相关的研究。两名评审员独立进行选择。提取并分析研究特征、参与者人口统计学、干预措施、结局和随访时间的数据。
共确定了 691 篇论文，最终有 18 项研究纳入分析。大多数研究（n=12）侧重于儿科年龄组。最常见的临床表现为主动脉缩窄（n=17）和二尖瓣狭窄（n=12）。外科干预通常采用分期方法，优先处理流出道阻塞。由于长期预后更好，二尖瓣瓣膜修复优于置换（n=8）。由于术后结局改善，推荐双心室修复，但往往需要再次手术。再次手术很常见，主要是因为复发性主动脉缩窄（n=10）、主动脉瓣下狭窄（n=8）和二尖瓣瓣膜功能障碍（n=7）。肺动脉高压（n=10）和心律失常（n=11）是严重的并发症。大多数患者在随访时处于改良 Ross/NYHA 功能分级 1。死亡率范围为 4%至 28%，早期和策略性手术干预与更好的结局相关。
早期诊断和双心室修复与更好的结局相关，而移植通常是最后的手段。需要标准化的诊断标准、长期随访和共识指南，以改善这种先天性心脏病的管理。
