CAJ | 학술논문

目的：分利用速度向量成像（VVI）技术比较原发性心脏淀粉样变性（AL-CA）及肥厚型心肌病（HCM）患者左室各节段以及室壁的内膜（ENDO）、中层（MYO）、外膜下（EPI）心肌长轴收缩期峰值应变（LSsys）的差异。方法入选20例经过组织活检证实的AL-CA患者，20例非对称性HCM患者以及年龄匹配的20例健康人作为对照组，收集临床资料并分别进行常规二维心脏超声检查和VVI技术分析。常规二维超声用于评价左室壁厚度、左房室腔大小以及收缩舒张功能。VVI技术用于分析左室各节段以及各室壁的ENDO、MYO、EPI心肌LSsys特征。AL-CA和HCM患者还进行了心脏核磁共振（CMR）以评价其心肌延迟强化（LGE）特征。结果 AL-CA和HCM两组在临床症状、体征上相似，而且两组左室壁厚度、左房内径、E/A比值、室间隔E/e'比值以及颗粒样回声的比例上均高于正常对照组。AL-CA组左室各节段及室壁ENDO LSsys较HCM和正常对照组均明显减低，且内外膜LSsys之差（ENDO-EPI LSsys）在AL-CA组各个室壁均较正常对照组明显减低，而HCM组各节段LSsys及各室壁ENDO-EPI LSsys变异性大，各室壁显示出明显的不均一性。LGE同样显示出与AL-CA和HCM室壁ENDO-EPI LSsys一致的特征：AL-CA组以各室壁心内膜下弥漫性延迟强化为特征，而HCM以区域性分布的多发斑片状延迟强化为特征。结论 AL-CA以左室心内膜下各节段LSsys以及各室壁ENDO-EPI LSsys均明显减低为特征；而HCM节段和室壁变化变性大。因此， VVI技术可以为鉴别AL-CA与HCM提供帮助。
목적：분이용속도향량성상（VVI）기술비교원발성심장정분양변성（AL-CA）급비후형심기병（HCM）환자좌실각절단이급실벽적내막（ENDO）、중층（MYO）、외막하（EPI）심기장축수축기봉치응변（LSsys）적차이。방법입선20례경과조직활검증실적AL-CA환자，20례비대칭성HCM환자이급년령필배적20례건강인작위대조조，수집림상자료병분별진행상규이유심장초성검사화VVI기술분석。상규이유초성용우평개좌실벽후도、좌방실강대소이급수축서장공능。VVI기술용우분석좌실각절단이급각실벽적ENDO、MYO、EPI심기LSsys특정。AL-CA화HCM환자환진행료심장핵자공진（CMR）이평개기심기연지강화（LGE）특정。결과 AL-CA화HCM량조재림상증상、체정상상사，이차량조좌실벽후도、좌방내경、E/A비치、실간격E/e'비치이급과립양회성적비례상균고우정상대조조。AL-CA조좌실각절단급실벽ENDO LSsys교HCM화정상대조조균명현감저，차내외막LSsys지차（ENDO-EPI LSsys）재AL-CA조각개실벽균교정상대조조명현감저，이HCM조각절단LSsys급각실벽ENDO-EPI LSsys변이성대，각실벽현시출명현적불균일성。LGE동양현시출여AL-CA화HCM실벽ENDO-EPI LSsys일치적특정：AL-CA조이각실벽심내막하미만성연지강화위특정，이HCM이구역성분포적다발반편상연지강화위특정。결론 AL-CA이좌실심내막하각절단LSsys이급각실벽ENDO-EPI LSsys균명현감저위특정；이HCM절단화실벽변화변성대。인차， VVI기술가이위감별AL-CA여HCM제공방조。
Objective To analyze the endocardial, myocardial, and epicardial longitudinal systolic strain (LSsys) in the left ventricle (LV) segments and walls in patients with cardiac involvement due to primary amyloidosis (AL-CA) and hypertrophic cardiomyopathy (HCM). Methods Twenty patients with biopsy-proven AL-CA, 20 with asymmetric HCM, and 20 age-matched healthy volunteers were analyzed for their clinical characteristics and underwent conventional echocardiography for evaluating LV wall thickness, left atrial and ventricle size, systolic and diastolic function and 2-dimensional velocity vector imaging for evaluating the endocardial, myocardial and epicardial LSsys of the LV segments and walls. AL-CA and HCM patients also underwent cardiac magnetic resonance to evaluate the late gadolinium enhancement (LGE) features. Results Compared with the control group, AL-CA and HCM groups, with similar clinical symptoms and physical signs, both showed increased LV wall thickness, left atrial diameter, E/A ratio, septal E/e' ratio and the prevalence of granular sparkling. LV segments and walls endocardial LSsys were significantly lower in AL-CA patients than in HCM patients and the control subjects. The endocardial-epicardial LSsys difference in all the left ventricle walls were significantly smaller in AL-CA group than in the control group, but this difference appeared variable in HCM group. The LGE also presented with different features in AL-CA and HCM:AL-CA group showed subendocardial LGE in almost all the LV walls, but HCM group showed patchy LGE with a regional, multifocal distribution. Conclusion AL-CA is characterized by a significantly reduced endocardial LSsys in the LV segments and an uniform decrease of the endocardial-epicardial LSsys difference in all the LV walls, but the changes in HCM appear variable, and 2-dimensional velocity vector imaging is therefore a useful modality to differentiate AL-CA from HCM.