Elling R, Stiller B, Grohmann J
Center for Pediatrics and Adolescent Medicine, University Medical Center Freiburg, Freiburg, Germany.
Department of Congenital Heart Defects and Pediatric Cardiology, Heart Center, University of Freiburg, Freiburg, Germany.
Catheter Cardiovasc Interv. 2015 Sep;86(3):463-6. doi: 10.1002/ccd.25782. Epub 2015 Jan 19.
Aortic regurgitation is a rare but dangerous condition in patients with hypoplastic left heart syndrome (HLHS). We report the case of a 2-year-old girl with HLHS with aortic/mitral stenosis (AS-MS) subtype after stage II palliation via the bilateral bidirectional Glenn (BBDG) procedure, when aortic regurgitation presented with the clinical manifestation of a paroxysmal coronary steal phenomenon provoked by exertion. Transcatheter closure of the left ventricular outflow tract (LVOT) with an Amplatzer(TM) Vascular Plug 4 minimized and finally abolished aortic reflux without compromising either coronary perfusion or atrioventricular (AV) conduction. Midterm follow-up over 3 years revealed complete disappearance of her symptoms, and follow-up catheterization displayed complete LVOT closure. This case illustrates the promising potential of modern cardiac catherization techniques in certain cases and emphasizes that precise assessment of the native aorta and coronary system is critical in HLHS patients.
主动脉瓣关闭不全在左心发育不全综合征（HLHS）患者中是一种罕见但危险的病症。我们报告了一例2岁患有HLHS且为主动脉/二尖瓣狭窄（AS-MS）亚型的女孩病例，该患儿在经双侧双向格林（BBDG）手术进行II期姑息治疗后，出现了劳力诱发的阵发性冠状动脉窃血现象临床表现的主动脉瓣关闭不全。使用Amplatzer™血管封堵器4经导管封闭左心室流出道（LVOT）可使主动脉反流最小化并最终消除，且不影响冠状动脉灌注或房室（AV）传导。超过3年的中期随访显示其症状完全消失，随访心导管检查显示LVOT完全封闭。该病例说明了现代心脏导管技术在某些病例中具有广阔前景，并强调对HLHS患者的天然主动脉和冠状动脉系统进行精确评估至关重要。
