心肌致密化不全研究进展

世界卫生组织(WHO)[1]与中国心肌病诊断与治疗建议工作组[2]将原发性心肌病分类和命名为扩张型心肌病(DCM)、肥厚型心肌病(HCM)、致心律失常性右室心肌病(ARVC)、限制型心肌病(RCM)和未定型心肌病五类.病毒性心肌炎演变为扩张型心肌病属继发性,左室心肌致密化不全纳入未定型心肌病.心肌致密化不全(NVM)是以心室内异常粗大的肌小梁和交错的深隐窝为特征的一种与基因相关的遗传性心肌病.     

孤立性心肌致密化不全1例报告并文献复习

心肌致密化不全（noncompaction of ventricular myocardium,NVM）是胚胎早期心内膜心肌发育不良导致的一种罕见的先天性心肌病,具有家族发病倾向,2006年美国心脏病协会将其归类为原发性心肌病中的遗传性心肌病。NVM可并发室间隔缺损、左右流出道梗阻等其它心脏畸形,也可单独存在,     

心肌致密化不全1例

1病史简介患者45岁,男性,幼时起即有反复发作心悸,20年前体检发现“心脏病”,诊治不详,尚能胜任日常工作。因阵发性心悸、胸痛、呼吸困难伴咳嗽1个月,夜间不能平卧2d于2006年2月22日入院。体查:血压105/80mmHg,脉搏120次/min,双肺可闻中量湿啰音,心界扩大,心音略低,心尖区可闻第四心音奔马律,末闻明显杂音,双下肢轻度水肿。查肝、肾功能正常,血糖正常,甘油三脂:3.13mmol/L,总胆固醇:7.46mmol/L,高密度脂蛋白胆固醇:1.24mmol/L,低密度脂蛋白胆固醇:4.80mmol/L。心脏彩超示左房内径44mm,左室内径57mm,室壁运动不协调,二尖瓣、三尖瓣轻度返流,EF:38%。冠状动脉造影未见明显冠状动脉狭窄。心电图示不完全性左束支传导阻滯牡缤偶发室性早搏,偶发房性早搏,短阵房性心动过速,持续6s~10s,伴ST-T改变,无相关症状。诊断为扩张型心肌病,予卡托普利、倍他乐克、利尿剂、洋地黄等治疗,症状可缓解,但仍有反复发作。住院期间两次心悸、胸痛等症状发作时心电图记录到与症状相关的持续3min~5min的阵发性房性心动过速伴ST-T改变,心动过速可自行停止,症状同时缓解...     

成人右室心肌致密化不全伴肺动脉栓塞1例并文献复习

心肌致密化不全(noncompaction of ventricular myocardium,NVM)是一种罕见的先天性心肌病,又称海绵状心肌或心肌窦状隙持续状态.有家族遗传倾向.可单独存在,也可并存于其他的先天性心脏病.左室心肌致密化不全临床较常见,但右心室心肌致密化不全尚罕见.现报道1例右室心肌致密化不全伴肺动脉栓塞病例,并复习国内外相关文献,探讨右室心肌致密化不全的诊断标准及其并发症的危险因素.     

心肌致密化不全心肌病伴慢性心力衰竭患者的临床研究

目的 目前致密化不全心肌病影像学诊断标准基于心肌致密层与非致密层比值、非致密心肌厚度或肌小梁数目。患者临床表现包括无症状到心律失常、心力衰竭、血栓栓塞甚至心源性猝死。本研究旨在观察致密化不全心肌病患者临床特征及其对预后的影响。 方法 回顾性研究2001年1月—2016年12月中国科学技术大学附属第一医院(安徽省立医院)收治的致密化不全心肌病伴心力衰竭患者60例(研究组),同期住院的扩张型心肌病71例患者作为对照组,分析致密化不全心肌病临床特征,评价其对患者预后的影响。 结果 与对照组比较,致密化不全心肌病患者男性比例高(68.33%vs. 47.89%,P=0.018),更易出现左束支传导阻滞(56.67%vs. 29.58%,P=0.002)和右束支传导阻滞(6.67%vs. 0.00%,P=0.043),心电图碎裂QRS波发生率(23.33%vs. 8.45%,P=0.018)增加,以室性心律失常事件作为终点的logistic多因素回归分析显示,男性(P=0.028)是室性心律失常发生的重要因素。以室性心律失常和/或栓塞为复合终点的logistic多因素回归分析显示,高血压(P=0.024)是影响复合终点的重要因素。 结论 心肌致密化不全患者易出现心电异常,其中男性是室性心律失常的重要影响因素,若伴有高血压,将增大栓塞事件发生率。      

心肌致密化不全1例报道

心肌致密不全(noncompaction ventricular my-ocardium,NVM)是一种罕见的先天性心室肌发育不全性的心肌病。现将笔者2007年3月诊断的1例NVM报道如下,并结合文献对该病进行总结。1临床资料患者,女性,47岁。近2年以来,反复出现胸闷、心悸、进行性乏力,并且逐步进展为夜间不能平卧     

成人左室心肌致密化不全1例及文献复习

<正>左室心肌致密化不全(LVNC)作为一种临床较少报道的病例,目前没有明确指南提出其病因、诊断及治疗、预后等,现报告1例LVNC患者并总结该疾病文献资料。1 临床资料患者男性,52岁,主因"活动后胸闷、气短3年,加重伴咳嗽、咳痰10d"于2018年2月7日入院,10d前于当地医院诊断"肺部感染",治疗后不缓解,3d前加重。查体:血压150/90mmHg(1mmHg=0.133kPa),心率78次/min,双肺呼吸音粗,双下肺叩浊,可     

成人心肌致密化不全4例

2002年9月至2003年8月心功能不全住院治疗的4例患者.临床表现、胸部x线、心电图及超声心动图特点见表1.     

左心室心肌致密化不全合并脑栓塞1例

1病例介绍 患者,男,45岁。因劳累后胸闷、憋气3年,突发左侧肢体活动不利2d入院。入院时查体：血压110／70mmHg,头颅无异常;两肺呼吸音清;心界向左侧扩大,心率90次／min,心律绝对不齐,心音低钝,各瓣膜听诊区未闻及杂音;腹软,肝脾未触及;双下肢无浮肿;左侧肢体肌力Ⅱ级,     

孤立性心肌致密化不全3例

孤立性心肌致密化不全(isolated noncompaction of ventricular myocardium，INVM)是胚胎心肌致密化停止所致的特殊类型的心肌病，我们发现3例，报告如下：     

孤立性心室肌致密化不全一例报告

1 临床资料患者,男,56岁,因反复发作的胸闷、气短于2002年入院,患者缘于2000年2月出现劳力时胸闷、气短,未经治疗,此后上述症状反复发作,于2002年10月上述症状加重,休息时也有胸闷、气短症状,逐渐出现夜间阵发性呼吸困难和端坐呼吸,于当地医院诊断为扩张型心肌病,经治疗无明显好转后转院入我科.入院后给予强心、利尿、扩血管等药物治疗,患者胸闷、气短症状曾一度好转,心电图提示心房纤颤,二维超声心动图可在左室心尖部、前侧壁心内膜面探及多发性突入左室腔内的肌小梁,小梁之间可见深度不同的间隙.     

心肌致密化不全2例(附文献复习)

心肌致密化不全(noncompactionventricularmyocardium,NVM)又称海绵状心肌(spongymyocardium)或心肌窦状隙持续状态,临床上较为少见。近年来国内外对该病逐渐认识[1],笔者在临床上也见到2例,报告如下。1临床资料例1.女性,19岁。既往健康,无心血管病家族史,因近2个月来感活动后胸闷并进行性加重伴双下肢水肿而就诊。查体:BP10060mmHg(1mmHg=0.133kPa),双下肺可闻及湿性口罗音。心界向左下扩大,心音稍弱,心率80次min,律齐,心尖部可闻及2级收缩期杂音。肝脏肋下未触及,双下肢轻度水肿,余未见异常。心电图检查正常。心脏超声检查显示左房前…     

成人左室心肌致密化不全2例临床特征

左室心肌致密化不全(left ventricular noncompaction, LVNC)是少见的先天性心肌病变,美国心脏学会(AHA)2006年的心肌病分类建议中,将其划归为原发性、遗传性心肌病的一种类型[1].对LVNC的病因、病理目前已有一定了解,但对其临床表现、病程、预后等的认识尚有限.现将我院诊断的2例LVNC报告如下,并结合现有文献资料对该病的临床特征进行分析,以期增进临床医生对该病的认识.     

Persistent candiduria complicating intraureteral stenting: a case report and review of literature

Over the past 20 years, there has been a significant increase in the number of nosocomial infections. Nosocomial infections by fungal organisms, in particular Candida species, have shown the highest increase in incidence, currently being the leading pathogen in urinary tract infections and the fourth most common blood-born pathogen within hospitalized patients. This case report describes a patient with acute pancreatitis, complicated by acute renal failure, who developed nosocomial fungiuria and fungemia following bilateral ureteral stenting, and whose fungiuria persisted despite adequate antifungal treatment. Conditions resolved with removal of the intraureteral stents, which we believe, served as the nidus for her persistent fungiuria.     

Neurofibrosarcoma - complicating neurofibromatosis-I: case report and review of relevant literature

Summary: Neurofibromatosis I is a multi systemic genetic and progressive disorder. Malignancy is one of the several complications and frequency of neurofibrosarcoma is significantly higher in NF-I patients. Neurofibromatosis was noted at 2 years after birth and overtime became malignant for which a below the knee amputation was done at the age of 29 years. Malignant transformation probably occurred prior to excision of the tumour at 26 years. Recurrence within 18 months is suggestive of inadequate excision and of a slow growing tumour. Diagnosis was missed despite previous presentation to other hospitals. This case presentation and review of literature highlights the need for early diagnosis and follow up, education of the patients and their families and the need for histological diagnosis for lesion removed to achieve overall improvement in morbidity and mortality. Keywords: neurofibromatosis I, neurofibrosarcoma.     

Systemic cytomegalovirus infection complicating ulcerative colitis: a case report and review of the literature

Cytomegalovirus is a common infection worldwide and in the immunocompromised individual it can be a major cause of morbidity and mortality. In patients with inflammatory bowel disease cytomegalovirus infection has been described in both immunocompetent and immunocompromised individuals. A 34 year old man with an exacerbation of his colitis was diagnosed as having both cytomegalovirus colitis and hepatitis. The diagnosis was made on the classical appearance of "owl's eye" inclusion bodies on colonic and hepatic biopsies and, in addition, viral serology and polymerase chain reaction (PCR) analysis of the cytomegalovirus DNA copy number. Fourteen days of treatment with ganciclovir led to a prompt improvement in the symptoms of colitis, resolution of the pyrexia, normalisation of the liver function tests, and clearance of the virus, as measured by a negative cytomegalovirus DNA PCR. Cytomegalovirus infection is a potentially fatal complication of treatment induced immunosuppression in patients with inflammatory bowel disease. As in this case, infection may be systemic and not confined to the intestine. Prompt diagnosis using histology, serology, and PCR analysis allows prompt introduction of therapy and an improved prognosis.     

阿霉素性心肌病一例报告

1临床资料患者,女,36岁.因反复胸闷、气短半年余入院.患者于半年前出现胸闷、气短伴乏力,活动和夜间平卧时加重,咳较频,咳白色泡沫痰,在当地医院查心脏彩超提示全心扩大,射血分数(EF)为35%,B超示双侧心包积液,多次以"扩张性心肌病"治疗效不佳而转入我院.患者过去有急性淋巴细胞性白血病史,行化疗至今,1999年12月至2002年10月间曾用多柔比星(阿霉素)、环磷酰胺等化疗药物,阿霉素总量465 mg/m2.     

1例超高龄新型冠状病毒肺炎并发急性心力衰竭的抢救经验及文献回顾

2022年春季新型冠状病毒Omicron变异株在上海爆发了大流行。合并各类基础疾病的老年患者疫苗接种率较低,是本次流行的主要易感人群,且易进展为危重症。因此救治老年危重患者成为目前临床诊疗新型冠状病毒肺炎的难点之一。本报道是1例超高龄新型冠状病毒肺炎患者, 病程由轻症进展为重症,并发急性心力衰竭、心源性休克。笔者结合文献回顾性分析其病因和临床特征,分享基层医院诊疗高龄新型冠状病毒肺炎危重症的经验与得失,为救治新型冠状病毒肺炎危重症合并心血管并发症提供参考依据。      

Isolated biventricular noncompaction: a case report

Myocardial noncompaction （MN） is defined as an .unclassified congenital cardiomyopathy which is characterized by the presence of prominent ventricular trabeculations and deep intertrabecular recesses. It is assumed to occur as an arrest in normal endomyocardial morphogenesis and is mostly associated with other congenital cardiac malformations The left ventricle is usually involved; but, both ventricules can be affected.