PDF(Pubmed)
Abstract：
BACKGROUND: Supravalvular aortic stenosis (SVAS) is an uncommon congenital abnormality that presents with intimal thickening of the aortic media at the sinotubular junction. Given the congenital nature of the disease, patients usually become symptomatic in childhood.
METHODS: A 48-year-old man developed symptomatic SVAS in middle age. A patch aortoplasty with a bovine pericardial patch was performed. His postoperative course was uneventful, and echocardiography revealed a significant decrease in peak velocity and pressure gradient.
CONCLUSIONS: SVAS, a congenital heart disease with an incidence of 1 in 20,000 live births, is often linked to Williams syndrome but can also occur independently. Isolated SVAS is generally less severe and may not show symptoms in childhood. Its narrowing often stabilizes after growth, but in this middle-aged patient, symptoms appeared later in life. SVAS usually presents as discrete thickening above the sinuses of Valsalva or as diffuse narrowing along the ascending aorta. Surgical relief is the common treatment, with flap plasty using various patch techniques. This patient, having discrete stenosis and intact aortic valve function, underwent single-patch expansion. Key to this surgery is avoiding coronary artery stenosis, by considering coronary orifice location and other cardiac anomalies. A bovine pericardial patch was chosen for its bleeding control benefits.
CONCLUSIONS: Although SVAS progression in middle age is quite rare, it can be successfully corrected with detailed and selected surgical procedures.
METHODS: A 48-year-old man developed symptomatic SVAS in middle age. A patch aortoplasty with a bovine pericardial patch was performed. His postoperative course was uneventful, and echocardiography revealed a significant decrease in peak velocity and pressure gradient.
CONCLUSIONS: SVAS, a congenital heart disease with an incidence of 1 in 20,000 live births, is often linked to Williams syndrome but can also occur independently. Isolated SVAS is generally less severe and may not show symptoms in childhood. Its narrowing often stabilizes after growth, but in this middle-aged patient, symptoms appeared later in life. SVAS usually presents as discrete thickening above the sinuses of Valsalva or as diffuse narrowing along the ascending aorta. Surgical relief is the common treatment, with flap plasty using various patch techniques. This patient, having discrete stenosis and intact aortic valve function, underwent single-patch expansion. Key to this surgery is avoiding coronary artery stenosis, by considering coronary orifice location and other cardiac anomalies. A bovine pericardial patch was chosen for its bleeding control benefits.
CONCLUSIONS: Although SVAS progression in middle age is quite rare, it can be successfully corrected with detailed and selected surgical procedures.
摘要：
背景：主动脉瓣上狭窄（SVAS）是一种罕见的先天性异常，表现为窦管交界处主动脉中膜的内膜增厚。鉴于这种疾病的先天性，患者通常在儿童时期出现症状。
方法：一名48岁男性在中年时出现症状性SVAS。进行了带有牛心包贴片的贴片主动脉成形术。他的术后进展顺利，超声心动图显示峰值速度和压力梯度显着降低。
结论：SVAS，先天性心脏病，发病率为2万活产婴儿中的1例，通常与威廉姆斯综合征有关，但也可以独立发生。孤立的SVAS通常不太严重，并且在儿童时期可能不会出现症状。它的收窄往往在增长后趋于稳定，但是在这个中年病人身上，症状出现在以后的生活中。SVAS通常表现为Valsalva窦上方的离散增厚或沿升主动脉的弥漫性狭窄。手术缓解是常见的治疗方法，使用各种补片技术进行皮瓣成形术。这个病人,具有离散的狭窄和完整的主动脉瓣功能，进行了单补丁扩展。手术的关键是避免冠状动脉狭窄,通过考虑冠状动脉口位置和其他心脏异常。选择牛心包贴片是为了其出血控制益处。
结论：尽管中年时SVAS进展相当罕见，它可以成功地纠正与详细和选定的外科手术。
方法：一名48岁男性在中年时出现症状性SVAS。进行了带有牛心包贴片的贴片主动脉成形术。他的术后进展顺利，超声心动图显示峰值速度和压力梯度显着降低。
结论：SVAS，先天性心脏病，发病率为2万活产婴儿中的1例，通常与威廉姆斯综合征有关，但也可以独立发生。孤立的SVAS通常不太严重，并且在儿童时期可能不会出现症状。它的收窄往往在增长后趋于稳定，但是在这个中年病人身上，症状出现在以后的生活中。SVAS通常表现为Valsalva窦上方的离散增厚或沿升主动脉的弥漫性狭窄。手术缓解是常见的治疗方法，使用各种补片技术进行皮瓣成形术。这个病人,具有离散的狭窄和完整的主动脉瓣功能，进行了单补丁扩展。手术的关键是避免冠状动脉狭窄,通过考虑冠状动脉口位置和其他心脏异常。选择牛心包贴片是为了其出血控制益处。
结论：尽管中年时SVAS进展相当罕见，它可以成功地纠正与详细和选定的外科手术。
