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三例同胞兄弟姐妹膜性主动脉瓣下狭窄的临床特征及手术结果:病例系列报道与文献综述

Clinical features and surgical outcomes of membranous subaortic stenosis in three siblings: a case series and literature review.

作者信息

Alaswad Mohammed, Almohamed Ahmad, Alsmoudi Hasan, Haydar Hasan, Karaja Saja, Barakat Lyne, Takkem Saleh

机构信息

Faculty of Medicine, University of Hama, Hama, Syria.

Department of Cardiology, Hama National Hospital, Hama, Syria.

出版信息

Ann Med Surg (Lond). 2024 Oct 22;86(12):6930-6935. doi: 10.1097/MS9.0000000000002675. eCollection 2024 Dec.

Abstract

INTRODUCTION

Subvalvular aortic stenosis (SAS) is a rare heart condition that can cause LVOT obstruction and lead to serious complications. While surgery is the main treatment, recurrence is a concern. This case series highlights the challenges in managing SAS in young patients.

CASE SERIES

Three young brothers presented with symptoms of shortness of breath and chest tightness. All were diagnosed with membranous SAS through echocardiography and Doppler ultrasound. Surgical intervention was performed to remove the obstruction, but all three patients experienced recurrence within 2 years.

DISCUSSION

This case series of three brothers suggests a genetic predisposition. Symptoms included exertional dyspnea and chest tightness, diagnosed via echocardiography. Surgical intervention is the primary treatment, but its timing is debated due to recurrence risks and complications.

CONCLUSIONS

This case series suggests a possible genetic predisposition to SAS. Despite successful initial surgery, the high recurrence rate highlights the need for improved management strategies and long-term follow-up. Further research is crucial to understand the causes of recurrence and optimize treatment for children with SAS.

摘要

引言

瓣下主动脉瓣狭窄(SAS)是一种罕见的心脏疾病,可导致左心室流出道梗阻并引发严重并发症。虽然手术是主要治疗方法,但复发是一个令人担忧的问题。本病例系列突出了年轻患者SAS管理中的挑战。

病例系列

三名年轻兄弟出现呼吸急促和胸闷症状。通过超声心动图和多普勒超声检查,他们均被诊断为膜性SAS。进行了手术干预以解除梗阻,但所有三名患者在2年内均出现复发。

讨论

这个三兄弟的病例系列提示了遗传易感性。症状包括劳力性呼吸困难和胸闷,通过超声心动图诊断。手术干预是主要治疗方法,但由于复发风险和并发症,其时机存在争议。

结论

本病例系列提示SAS可能存在遗传易感性。尽管初始手术成功,但高复发率凸显了改进管理策略和长期随访的必要性。进一步研究对于了解复发原因并优化SAS患儿的治疗至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ebff/11623858/be590e707f25/ms9-86-6930-g001.jpg

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