孤立性心肌致密化不全患者左室收缩功能的相关因素分析

目的总结孤立性左室心肌致密化不全（ILVNC）患者的临床资料,并对其左室收缩功能的相关影响因素进行分析。方法连续入选自2006年1月至2010年9月经超声心动图确诊为ILVNC的患者共41例,总结其临床资料,并对可能影响该组患者左室射血分数（LVEF）的因素进行分析。结果 41例患者（男性35例）LVEF均值为31.41±11.21%;最常受累的心肌节段是心尖部（100%受累）,其次是心尖段外侧壁（92.7%受累）、心尖段下壁（82.9%受累）和心尖段前壁（70.7%受累）。相关性分析表明,LogNT-proBNP、左室舒张末内径（LVEDD）和室壁运动评分指数（WMSI）与LVEF均呈负相关,r分别为-0.441（P=0.007）、-0.357（P=0.022）以及-0.714（P=0.000）,具有显著的统计学意义;受累节段数目和NC/C比值则与LVEF无显著相关性;WMSI与受累节段数目呈正相关（r=0.362,P=0.020）,与NC/C比值无显著相关性（P=0.254）。结论 ILVNC患者LVEF左室整体收缩功能与NT-proBNP及LVEDD呈负相关,而与受累心肌节段数目、NC/C比值无明显关联。     

心肌致密不全

心肌致密不全是一种罕见的心肌病,是胚胎生长过程中心内膜心肌发育缺陷所造成。现就心肌致密不全的原因、诊断以及治疗进行综述。     

An unusual cause of heart failure identified by echocardiography in an octogenarian

Non-compaction of the ventricular myocardium is a rare congenital cardiomyopathy that carries an unfavorable prognosis. It is associated with a high incidence of progressive heart failure, thromboembolism and malignant arrhythmias. Echocardiography currently remains the imaging modality most commonly used for diagnosis. We describe an unusual case of isolated non-compaction of the left ventricle in an octogenarian male to highlight the need for echocardiography in all patients with suspected heart failure, in order to determine aetiology, prognosis and assess treatment.     

Left Ventricular Noncompaction

According to the World Health Organization classification of cardiomyopathies, left ventricular noncompaction is still an unclassified cardiomyopathy. In 2006, the American Heart Association classified this entity as a primary cardiomyopathy of genetic origin. In 2008, the European Society of Cardiology updated the classification scheme similar to the World Health Organization classification. At present, there is no consensus on the diagnostic criteria, and diagnosis is based on the morphologic features identified by cardiac imaging studies or at autopsy. Due to lack of standardization of the diagnostic criteria and little awareness of this condition among clinicians, the true prevalence of this disease is not clear. There is no specific therapy for this condition. However, it seems prognosis is much better than initially reported. The current status of diagnosis, prognosis, and management of isolated noncompaction in adults is discussed in this review.     

Ventricular noncompaction: Over or under diagnosis?

Ventricular noncompaction (VNC) describes a cardiomyopathy characterized by excessive myocardial trabeculations and deep intertrabecular recesses. Detection rates are increasingly rising and the frequency of NVC has changed from a disease that is thought to be rare to that described as ‘relatively common’. However, there had been a number of un-answered questions regarding the clinical and echocardiographic features of VNC. In this review we discuss the frequency of VNC and emphasize its distinctive clinical features and associations.     

Association of Wolff-Parkinson-White With Left Ventricular Noncompaction Cardiomyopathy in Children

BackgroundWolff-Parkinson-White (WPW) has been associated with left ventricular noncompaction (LVNC) in children. Little is known about the prevalence of this association, clinical outcomes, and treatment options.MethodsRetrospective review of subjects with LVNC. LVNC was defined by established criteria; those with congenital heart disease were excluded. Electrocardiograms (ECGs) were reviewed for presence of pre-excitation. Outcomes were compared between those with isolated LVNC and those with WPW and LVNC.ResultsA total of 348 patients with LVNC were identified. Thirty-eight (11%) were found to have WPW pattern on ECG, and 84% of those with WPW and LVNC had cardiac dysfunction. In Kaplan–Meier analysis, there was significantly lower freedom from significant dysfunction (ejection fraction ≤ 40%) among those with WPW and LVNC (P < .001). Further analysis showed a higher risk of developing significant dysfunction in patients with WPW and LVNC versus LVNC alone (hazard ratio 4.64 [2.79, 9.90]). Twelve patients underwent an ablation procedure with an acute success rate of 83%. Four patients with cardiac dysfunction were successfully ablated, 3 having improvement in function.ConclusionWPW is common among children with LVNC and is associated with cardiac dysfunction. Ablation therapy can be safely and effectively performed and may result in improvement in function.     

Noncompaction of the ventricular myocardium combined with polycystic kidney disease

Noncompaction of the ventricular myocardium (NVM) is a rare cardiac abnormality of unknown etiology. The condition is characterized by prominent and excessive trabeculations in a ventricular wall segment, with deep intertrabecular spaces perfused from the ventricular cavity. Polycystic kidney diseases are characterized by the formation of multiple cysts in the kidneys and liver and, less frequently, in the pancreas. Cardiovascular abnormalities including hypertension, mitral valve prolapse, and intracranial aneurysms are also frequently recognized. However, polycystic kidney disease and isolated ventricular noncompaction have not previously been correlated. Here, we describe one case of isolated noncompaction of ventricular myocardium with polycystic kidney disease, coupled with a progressive worsening of heart failure. We confirmed these abnormalities using contrast echocardiography, abdominopelvic computed tomography, and cardiac magnetic resonance imaging.     

肥厚型心肌病扩张期2例报告及文献复习

目的 :探讨肥厚型心肌病 (HCM)的命名伴心室扩张和心力衰竭的可能原因。方法 :报告本病 2例并结合文献复习进行分析。结果与结论 :1以 HCM扩张期命名较为恰当。 2其发生的可能原因是心肌缺血、酒精性心肌损害和病毒持续感染。 3预后较差。     

Preserved systolic function with isolated left ventricular noncompaction in an elderly patient.

Isolated left ventricular noncompaction (IVNC) is a rare congenital cardiomyopathy resulting from an arrest in normal endomyocardial embryogenesis. Clinical presentations of IVNC include systolic and diastolic dysfunction, systemic embolism and ventricular arrhythmias. In recent years there has been an increasing awareness of this anomaly; however, especially in elderly, clinical characterization and natural course of IVNC are still in question. In this case, we report a case of a 78-year-old patient with the diagnosis of IVNC with preserved left ventricular (LV) systolic function. To the best of our knowledge, this is the oldest IVNC case with preserved LV systolic function in the literature.     

同种异体骨髓间充质干细胞移植对扩张型心肌病心功能衰竭大鼠左心室功能的影响

目的探讨同种异体大鼠骨髓间充质干细胞移植在阿霉素诱导的扩张型心肌病心功能衰竭大鼠心脏内存活、分化的情况及对左心室功能的影响。方法雌性Wistar大鼠55只,随机分成正常对照组(n=10)、模型组(n=15)、诱导前移植组(n=15)和诱导后移植组(n=15)。体外分离培养雄性大鼠骨髓间充质干细胞,传至一代后用10μmol/L5-氮胞苷诱导4周,DAPI标记后,诱导前移植组移植诱导前的骨髓间充质干细胞,诱导后移植组移植诱导后的骨髓间充质干细胞;4周后检测左心室功能及移植细胞存活、分化情况。结果异体骨髓间充质干细胞移植4周后可存活并分化成心肌细胞,表达心肌细胞特异性蛋白;与模型组比较,细胞移植组左心室功能明显改善,且两细胞移植组间差异无显著性。结论同种异体骨髓间充质干细胞移植4周后可存活并分化成心肌细胞,对扩张型心肌病心功能衰竭大鼠的左心室功能有保护作用。     

Electrocardiographic Findings at Initial Diagnosis in Children with Isolated Left Ventricular Noncompaction

Background: The aim of this study was to comprehensively evaluate electrocardiographic (ECG) findings of isolated left ventricular noncompaction (IVNC) patients at initial diagnosis and to explore the correlation between them and the clinical, echocardiographic, and magnetic resonance imaging (MRI) findings. Methods: Twenty‐three patients diagnosed with IVNC by echocardiography and cardiac MRI between January 2006 and June 2010 were enrolled in this study. The patients were examined with standard ECG and 24‐hour Holter ECG. For comparison purpose, ECGs of 50 healthy children of similar ages and demographic characteristics were taken. Results: In 87% of patients, ECG abnormalities were found. The most frequently seen ECG findings were left ventricular hypertrophy, ST‐segment depression, and negative T wave related to abnormal repolarization particularly in DII, DIII, and V_4–6 leads, as well as prolonged PR and QTc intervals. No ECG features or patterns were found that were specific to the disease. In contrast to adult patients, while no intraventricular conduction defects (particularly in the left bundle brach) were found in any of our patients, 13% had considerable bradycardia and one required a pacemaker. The Holter ECG recordings showed supraventricular tachycardia attacks in two patients and a short ventricular tachycardia attack in one. Patients whose echocardiograms and MRI showed left ventricular systolic dysfunction and left ventricular dilatation had signs of left ventricular hypertrophy and repolarization abnormality on their ECGs, but there was no significant difference in PR, QRS, and QTc intervals. Conclusion: Regardless of how frequently left ventricular hypertrophy and repolarization abnormalities are found on IVNC patients’ initial ECGs, we think that they are not unique to the disease but are related to the severity of the cardiomyopathy. Ann Noninvasive Electrocardiol 2011;16(2):184–191