PDF(Pubmed)
Abstract：
Shone complex (SC) is a rare congenital heart disease characterized by four obstructive anomalies, including parachute mitral valve (PMV), left atrial supra-valvular ring, subaortic stenosis, and coarctation of the aorta. Typically, SC manifests early in life. However, we encountered a 52-year-old female with a history of hypertension diagnosed at 26 years and left-sided weakness poststroke. She presented with worsening dyspnea and palpitations, prompting a thorough investigation. Echocardiography revealed a heavily calcified bicuspid aortic valve with severe aortic stenosis and parachute mitral valve with severe mitral stenosis and preserved ejection fraction, raising suspicions regarding the presence of SC. Cardiac catheterization, aortic-angiography, and noncontrast chest computed tomography (CT) revealed abrupt occlusion of the postductal aorta, giving a picture of aortic coarctation with well-established collateral vessels including prominent right and left internal mammary arteries. So, she was diagnosed with an incomplete SC at the age of 52. Shone complex is a rare congenital heart disease that typically presents in early childhood, but late presentations due to misdiagnosis or incomplete work up are possible. This case emphasizes the rarity of late presentations of SC and highlights the importance of early diagnosis and intervention to improve outcomes. An incomplete SC should be considered in adult patients presenting with left-sided obstructive lesions.
摘要：
Shone复合体（SC）是一种罕见的先天性心脏病，以四种阻塞性异常为特征，包括降落伞二尖瓣（PMV），左心房瓣膜上环,主动脉瓣下狭窄,和主动脉缩窄.通常,SC表现在生命的早期。然而,我们遇到了一名52岁女性,在26岁时诊断出高血压病史,并在卒中后出现左侧无力.她出现了恶化的呼吸困难和心悸,促使进行彻底的调查。超声心动图显示严重钙化的二叶主动脉瓣伴严重主动脉瓣狭窄，降落伞二尖瓣伴严重二尖瓣狭窄，射血分数保留。怀疑SC的存在。心导管插入术,主动脉血管造影术,非对比胸部计算机断层扫描（CT）显示导管后主动脉突然闭塞，提供一张主动脉缩窄的图片，包括突出的左右乳内动脉。所以,她在52岁时被诊断出患有不完整的SC.Shone复合体是一种罕见的先天性心脏病，通常出现在儿童早期，但由于误诊或未完成的工作而导致的延迟报告是可能的。该病例强调了SC晚期表现的罕见性，并强调了早期诊断和干预对改善预后的重要性。对于患有左侧阻塞性病变的成年患者，应考虑不完整的SC。
