兒童心肌致密化不全20例臨床分析
發(fā)布時(shí)間：2019-04-21 12:59
【摘要】：目的：探討兒童心肌致密化不全（noncompaction of the ventricularmyocardium,NVM）的臨床特點(diǎn)及治療效果。 方法：對(duì)2007年9月至2012年11月期間收治的20例NVM患兒的臨床表現(xiàn)、輔助檢查（X線胸片、心電圖、超聲心動(dòng)圖、心臟磁共振等）、臨床治療以及隨訪結(jié)果等資料進(jìn)行回顧性分析，并對(duì)部分患兒進(jìn)行隨訪。 結(jié)果：20例NVM患兒中，有家族史者2例，以心功能不全為主要臨床表現(xiàn)者16例（80%）,心律失常10例（50%）,無(wú)血栓及血栓事件發(fā)生；胸部X線檢查示心臟擴(kuò)大者12例（60%），20例患兒超聲心動(dòng)圖均提示心臟NVM典型表現(xiàn)，其中單純左室病變17例，雙室病變2例，合并先天性心臟病11例（55%），合并有擴(kuò)張性心肌�。╠ilatedCardiomyopathy,DCM）3例（15%）及心內(nèi)膜彈力纖維增生癥（endocardial fibroelastosis,EFE）4例（20%）；20例NVM首次住院治療后，16例好轉(zhuǎn)出院，4例放棄治療；20例NVM患者平均隨訪7.86個(gè)月（1月-15月），其中5例死亡，主要死因?yàn)樾牧λソ呒又兀?例長(zhǎng)期隨訪者心功能不全的臨床癥狀明顯緩解，改良Ross評(píng)分較前降低，定期復(fù)查心臟彩超提示心功能改善尚可，左室射血分?jǐn)?shù)（leftventricular ejection fraction，EF）改善明顯，差異有統(tǒng)計(jì)學(xué)意義（P＜0.05）；左室短軸縮短率(Shortening Fraction, FS)較治療前增加，收縮末期左室內(nèi)徑（left ventricular diameter，LVD）、右室內(nèi)徑（right ventricular diameter,RVD）及非致密化心肌厚度/致密化心肌厚度比值（ratio of noncompacted to compacted myocardiallayers，NC/C）較治療前縮小，但差異均無(wú)統(tǒng)計(jì)學(xué)意義（P＞0.05）。 結(jié)論：兒童NVM以心功能不全為主要表現(xiàn)，超聲心動(dòng)圖是診斷NVM的主要手段；NVM在經(jīng)積極內(nèi)科對(duì)癥治療后臨床癥狀可得到緩解，短期心臟功能改善可，，但總體預(yù)后較差。
[Abstract]:Objective: to investigate the clinical features and therapeutic effects of myocardial densification in children (noncompaction of the ventricularmyocardium,NVM). Methods: from September 2007 to November 2012, the clinical manifestations and auxiliary examination (X-ray chest film, electrocardiogram, echocardiography, cardiac magnetic resonance, etc.) of 20 children with NVM were analyzed. Clinical treatment and follow-up results were retrospectively analyzed, and some children were followed up. Results: of the 20 children with NVM, 2 had family history, 16 (80%) had cardiac insufficiency, 10 (50%) had arrhythmias, and no thrombus and thrombus events occurred. Chest X-ray examination showed cardiac enlargement in 12 cases (60%). Echocardiographic findings in 20 cases showed typical manifestations of cardiac NVM, including 17 cases of simple left ventricular lesions, 2 cases of biventricular lesions, and 11 cases of congenital heart disease (55%). There were 3 cases (15%) with dilated cardiomyopathy (dilatedCardiomyopathy,DCM) and 4 cases (20%) with endocardial elastosis (endocardial fibroelastosis,EFE). After the first hospitalization of 20 cases of NVM, 16 cases were improved and 4 cases gave up treatment, and 20 cases of NVM were followed up for an average of 7.86 months (January-15 months), 5 of them died due to the aggravation of heart failure. The clinical symptoms of 8 long-term follow-up patients with cardiac insufficiency were relieved obviously, and the modified Ross score was lower than that before. Regular reexamination of heart color Doppler suggested that cardiac function could be improved, and left ventricular ejection fraction (leftventricular ejection fraction,EF) could be improved obviously, and left ventricular ejection fraction (LVEF) was improved. The difference was statistically significant (P < 0.05). Left ventricular short axis shortening rate (Shortening Fraction, FS) increased before treatment, and left ventricular inner diameter (left ventricular diameter,LVD), right ventricular diameter (right ventricular diameter,RVD) and undensified myocardial thickness / densified myocardial thickness ratio (ratio of noncompacted to compacted myocardiallayers,) were increased at the end of systolic period. NC/C was smaller than that before treatment, but there was no significant difference (P > 0.05). Conclusion: the main manifestation of NVM in children is cardiac insufficiency, and echocardiography is the main method to diagnose NVM. The clinical symptoms of NVM can be relieved after active medical treatment, and the short-term cardiac function can be improved, but the overall prognosis is poor.
【學(xué)位授予單位】：重慶醫(yī)科大學(xué)
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2013
【分類號(hào)】：R725.4
[Abstract]:Objective: to investigate the clinical features and therapeutic effects of myocardial densification in children (noncompaction of the ventricularmyocardium,NVM). Methods: from September 2007 to November 2012, the clinical manifestations and auxiliary examination (X-ray chest film, electrocardiogram, echocardiography, cardiac magnetic resonance, etc.) of 20 children with NVM were analyzed. Clinical treatment and follow-up results were retrospectively analyzed, and some children were followed up. Results: of the 20 children with NVM, 2 had family history, 16 (80%) had cardiac insufficiency, 10 (50%) had arrhythmias, and no thrombus and thrombus events occurred. Chest X-ray examination showed cardiac enlargement in 12 cases (60%). Echocardiographic findings in 20 cases showed typical manifestations of cardiac NVM, including 17 cases of simple left ventricular lesions, 2 cases of biventricular lesions, and 11 cases of congenital heart disease (55%). There were 3 cases (15%) with dilated cardiomyopathy (dilatedCardiomyopathy,DCM) and 4 cases (20%) with endocardial elastosis (endocardial fibroelastosis,EFE). After the first hospitalization of 20 cases of NVM, 16 cases were improved and 4 cases gave up treatment, and 20 cases of NVM were followed up for an average of 7.86 months (January-15 months), 5 of them died due to the aggravation of heart failure. The clinical symptoms of 8 long-term follow-up patients with cardiac insufficiency were relieved obviously, and the modified Ross score was lower than that before. Regular reexamination of heart color Doppler suggested that cardiac function could be improved, and left ventricular ejection fraction (leftventricular ejection fraction,EF) could be improved obviously, and left ventricular ejection fraction (LVEF) was improved. The difference was statistically significant (P < 0.05). Left ventricular short axis shortening rate (Shortening Fraction, FS) increased before treatment, and left ventricular inner diameter (left ventricular diameter,LVD), right ventricular diameter (right ventricular diameter,RVD) and undensified myocardial thickness / densified myocardial thickness ratio (ratio of noncompacted to compacted myocardiallayers,) were increased at the end of systolic period. NC/C was smaller than that before treatment, but there was no significant difference (P > 0.05). Conclusion: the main manifestation of NVM in children is cardiac insufficiency, and echocardiography is the main method to diagnose NVM. The clinical symptoms of NVM can be relieved after active medical treatment, and the short-term cardiac function can be improved, but the overall prognosis is poor.
【學(xué)位授予單位】：重慶醫(yī)科大學(xué)
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2013
【分類號(hào)】：R725.4
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相關(guān)期刊論文 前10條
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3 袁t
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