Cho Min Soo, Jang Sun-Joo, Sun Byung Joo, Jang Jeong Yoon, Ahn Jung-Min, Kim Dae-Hee, Song Jong-Min, Kang Duk-Hyun, Song Jae-Kwan
Cardiac Imaging Center, Asan Medical Center Heart Institute, University of Ulsan College of Medicine, Seoul, South Korea.
Cardiac Imaging Center, Asan Medical Center Heart Institute, University of Ulsan College of Medicine, Seoul, South Korea.
J Am Soc Echocardiogr. 2014 Sep;27(9):965-71. doi: 10.1016/j.echo.2014.05.016. Epub 2014 Jul 1.
Although surgery is recommended for pediatric patients with supracristal ventricular septal defects (sVSDs) to prevent progression of aortic regurgitation (AR), outcomes in adolescents and adults with sVSDs are not known.
In this retrospective observational study, clinical data without surgery were obtained in 60 patients with sVSDs (group 1; mean age, 36 ± 13 years), 120 age- and defect size-matched patients with perimembranous ventricular septal defects (group 2), and 52 patients with sVSDs who underwent surgery (group 3; mean age, 32 ± 11 years).
Aortic sinus wall prolapse (38% vs 3%, P < .0001) and moderate to severe AR (7% vs 0%, P = .012) were more frequently observed in group 1 than in group 2. Five, three, and two patients in group 1 had surgery during follow-up because of rupture of the aneurysm of the sinus of Valsalva, endocarditis, and heart failure, respectively. Group 1 had a lower 12-year clinical event-free (surgery and endocarditis) rate (76 ± 9% vs 94 ± 4%, P = .031) but an equivalent overall survival rate (100% vs 94 ± 3%, P = .143) compared with group 2. Patients with maximal prolapsing aortic sinus wall length > 7 mm showed a higher frequency of aneurysm of the sinus of Valsalva rupture than those with no prolapse or maximal prolapsing length ≤ 7 mm (80% [four of five] vs 2% [one of 55], P < .001). The event-free and overall survival rates were comparable between groups 1 and 3, with equivalent 10-year AR progression-free survival rates (94 ± 5% vs 91 ± 5%, P = .301).
Aneurysm of the sinus of Valsalva rupture, rather than AR progression, was the main clinical event. Watchful monitoring of patients with high-risk echocardiographic features may be a rational option.
尽管对于患有嵴上型室间隔缺损（sVSD）的儿科患者推荐进行手术以预防主动脉瓣反流（AR）进展，但青少年和成人sVSD患者的预后尚不清楚。
在这项回顾性观察研究中，收集了60例sVSD患者（第1组；平均年龄36±13岁）、120例年龄和缺损大小匹配的膜周部室间隔缺损患者（第2组）以及52例接受手术的sVSD患者（第3组；平均年龄32±11岁）的非手术临床数据。
第1组主动脉窦壁脱垂（38%对3%，P<.0001）和中重度AR（7%对0%，P=.012）的发生率高于第2组。第1组分别有5例、3例和2例患者在随访期间因瓦氏窦瘤破裂、心内膜炎和心力衰竭接受了手术。与第2组相比，第1组的12年无临床事件（手术和心内膜炎）发生率较低（76±9%对94±4%，P=.031），但总生存率相当（100%对94±3%，P=.143）。主动脉窦壁最大脱垂长度>7mm的患者瓦氏窦瘤破裂的频率高于无脱垂或最大脱垂长度≤7mm的患者（80%[5例中的4例]对2%[55例中的1例]，P<.001）。第1组和第3组的无事件生存率和总生存率相当，10年无AR进展生存率相当（94±5%对91±5%，P=.301）。
瓦氏窦瘤破裂而非AR进展是主要临床事件。对具有高危超声心动图特征的患者进行密切监测可能是一种合理的选择。
