Robinson Joshua D, Del Nido Pedro J, Geggel Robert L, Perez-Atayde Antonio R, Lock James E, Powell Andrew J
Department of Cardiology, Children's Hospital Boston, Boston, Massachusetts, USA.
Am J Cardiol. 2010 Aug 1;106(3):426-9. doi: 10.1016/j.amjcard.2010.03.045.
We report 4 teenage patients who, after successful palliation of severe aortic valve stenosis by balloon aortic valvuloplasty in early infancy, presented within the previous 3 years with significant left ventricular (LV) diastolic heart failure. All patients had remained asymptomatic until their teenage years and had normal or hyperdynamic LV systolic function on presentation, with limited residual aortic valve stenosis and regurgitation. All underwent echocardiography, cardiac catheterization, and cardiac magnetic resonance evaluation. One notable common feature in the 4 patients was the presence of a confluent layer of LV subendocardial hyperenhancement demonstrated by late gadolinium enhancement technique. Histopathology was available for 2 patients, which documented significant LV endocardial fibroelastosis. One patient who underwent endocardial fibroelastosis resection in conjunction with aortic valve replacement had late clinical improvement. In conclusion, this group of patients may represent an important emerging clinical entity and merits close clinical surveillance, with prospective assessment of diastolic function.
我们报告了4例青少年患者，他们在婴儿早期通过球囊主动脉瓣成形术成功缓解严重主动脉瓣狭窄后，在过去3年内出现了明显的左心室舒张性心力衰竭。所有患者在青少年期之前一直无症状，就诊时左心室收缩功能正常或亢进，残余主动脉瓣狭窄和反流有限。所有患者均接受了超声心动图、心导管检查和心脏磁共振评估。这4例患者一个显著的共同特征是延迟钆增强技术显示左心室心内膜下有一层融合性强化。2例患者有组织病理学检查结果，证实存在明显的左心室心内膜弹力纤维增生症。1例同时接受心内膜弹力纤维增生症切除术和主动脉瓣置换术的患者临床症状后期有所改善。总之，这组患者可能代表一种重要的新出现的临床实体，值得密切临床监测，并对舒张功能进行前瞻性评估。
