Prenatal chocardiography in diagnosis of fetal noncompaction of ventricular myocardium / 中国介入影像与治疗学

Objective: To investigate the value of prenatal echocardiography in diagnosis of fetal noncompaction of ventricular myocardium (FNVM). Methods: Echocardiographic data of 12 fetuses with FNVM were analyzed and compared with the pathological results. Results: All 12 fetuses were diagnosed with FVNM. Biventricular involvement was detected in 5 fetuses, left ventricle involvement was observed in 5 fetuses, while right ventricle involvement was found in 2 fetuses. FNVM mostly involved the left ventricular apical (n=10). Associated abnormalities of heart structures were noticed in 7 fetuses, while 5 fetuses were found with serous membrane fluid and 3 fetuses with arrhythmia. Eight fetuses were tested for single gene mutation and copy number variations, among them 6 were detected with pathogenic gene mutations, 1 with the likely pathogenic gene mutation KCNH2 and 1 with chromosomal microdeletion. Conclusion: FNVM can involve both the left and the right ventricles, which tends to combine with right sided lesions, arrhythmia and serous membrane fluid. Prenatal echocardiography may play an important role in the diagnosis, prognosis and parental counseling of FNVM.

Analysis of clinical characteristics of different degrees of myocardial noncompaction in children / 临床儿科杂志

Objectives To explore the clinical characteristics and prognosis of different degrees of noncompaction of ventricular myocardium (NVM) in children and to investigate the clinical significance of the ratio of the thickness of the non-compact myocardium to the dense myocardium (N/C). Methods The clinical data of the children with NVM were analyzed retrospectively. The differences between children with N/C > 2.0 and children with N/C 1.4~2.0 were compared. Results There were 26 children in each of the two groups. There was no significant difference in age, sex, the ratios of low birth weight and malnutrition, heart disease, and complication (P all > 0.05). When discharged, 8 cases were improved, 15 cases has no obvious improvement, and 3 cases died in N/C>2.0 group; while 18 cases were improved, 8 cases has no obvious improvement, and none died in N/C 1.4~2.0 group, and the effective rate of treatment between two groups was statistically difference (χ2=7.69,P=0.006). In 8 years of follow-up, the mortality rate in N/C > 2.0 group was higher than that in N/C 1.4~2.0 group, and the difference was statistically different (χ2=6.993, P=0.008). Conclusions The treatment efficiency was lower and the prognosis was worse when the N/C value was higher in children with NVM.

Analysis of clinical characteristics of different degrees of myocardial noncompaction in children / 临床儿科杂志

Objectives To explore the clinical characteristics and prognosis of different degrees of noncompaction of ventricular myocardium (NVM) in children and to investigate the clinical significance of the ratio of the thickness of the non-compact myocardium to the dense myocardium (N/C). Methods The clinical data of the children with NVM were analyzed retrospectively. The differences between children with N/C > 2.0 and children with N/C 1.4~2.0 were compared. Results There were 26 children in each of the two groups. There was no significant difference in age, sex, the ratios of low birth weight and malnutrition, heart disease, and complication (P all > 0.05). When discharged, 8 cases were improved, 15 cases has no obvious improvement, and 3 cases died in N/C>2.0 group; while 18 cases were improved, 8 cases has no obvious improvement, and none died in N/C 1.4~2.0 group, and the effective rate of treatment between two groups was statistically difference (χ2=7.69,P=0.006). In 8 years of follow-up, the mortality rate in N/C > 2.0 group was higher than that in N/C 1.4~2.0 group, and the difference was statistically different (χ2=6.993, P=0.008). Conclusions The treatment efficiency was lower and the prognosis was worse when the N/C value was higher in children with NVM.

Comparative Study Between the Patients With Noncompaction of Ventricular Myocardium and Dilated Cardiomyopathy Combining Hypertrabeculation / 中国循环杂志

Objective: To explore the clinical and echocardiography characteristics between noncompaction of ventricular myocardium (NVM) and dilated cardiomyopathy (DCM) combining hypertrabeculation in order to distinguish NVM from DCM. Methods: Our research included 2 groups of patients: NVM group,n=31 and DCM combining hypertrabeculation group, n=50. The basic information as gender, age, family history, symptoms, ECG, plasma levels of BNP and echocardiography were recorded and examined in all patients; the size of cardiac chambers, myocardium, endocardium and hemodynamics were particularly focused. The trabeculation was analyzed by 17 segments method. Results:①Compared with NVM group, the patients in DCM combining hypertrabeculation group had the worse cardiac classiifcation, higher plasma levels of BNP (P 2.③The patients in DCM combining hypertrabeculation group had the less trabeculation segments (5.56 ± 1.56) and the apical was seldom involved, patients had the lower ration of NC/C as (1.91± 0.42), there was at most 1 segment with NC/C > 2. All P Conclusion: Echocardiography is a simple, practical and noninvasive method to distinguish NVM from DCM. NVM could be diagnosed by obvious left ventricular apex involvement with NC/C >2 in at least 2 segments of free ventricular walls.

Clinical features of noncompaction of ventricular myocardium in children / 临床儿科杂志

ObjectiveTo assess the clinical features of noncompaction of ventricular myocardium (NVM) in children. Methods The clinical data from ifve children with NVM were analyzed, including clinical manifestations, ultrasonic cardio-gram(UCG), electrocardiogram, chest X-ray, the process of diagnosis and treatment, and follow up.Results In ifve patients (3 boys and 2 girls) aged 3 months to 12 years, four of them were hospitalized because of pneumonia with heart failure. None of the ifve patients had thromboembolism. Heart enlargement was observed in all ifve patients on chest X-ray. By 2D ultrasonography, 2 patients had cardiac apex involvement, and 3 patients showed involvement of left ventricular wall and interventricular septum. In one patient, congenital partial defect of the endocardial cushion was observed. All patients were followed up for 3 to 36 months. One patient died of heart failure, 3 patients survived, and one patient was lost to follow-up.Conclusions The common clinical manifestation of NVM is the heart failure. UCG is preferred for NVM diagnosis. In children with refractory pneumonia and heart failure, NVM should be suspected.

Clinical and prognosis of the children with noncompaction of ventricular myocardium / 国际儿科学杂志

Noncompaction of ventricular myocardium (NVM) is reported as a genetic disease characterized by excessively prominent trabeculations and deep intertrabecular recesses.The etiology and pathogenesis are unclear.NVM is a rare disease with poor prognosis.The purpose of this areide is to review NVM manifestations and prognosis,to enhance the awareness of NVM,to improve the diagnosis and treatment,to ensure a better life quality and to increase the lifetime of NVM patients.

Assessment of left ventricular volume and function in patients with left ventricular non-compaction by contrast-enhanced three-dimensional echocardiography / 中华超声影像学杂志

Objective To evaluate the value of echo‐contrast RT‐3DE for assessment of left ventricular volume and function in patients with left ventricular non‐compaction(LVNC) .Methods Twenty‐one patients of LVNC were involved and underwent non‐enhanced and contrast‐enhanced RT‐3DE to evaluate left ventricular end‐diastolic volume (LVEDV) ,left ventricular end‐systolic volume (LVESV) ,left ventricular ejection fraction (LVEF) .The endocardial border definition of LV was graded for each of the 16 LV segments as follows :0 = border invisible ,1 = border visualized only partially ,and 2 = complete visualization of the border .Three image‐quality groups (good ,fair ,and uninterpretable) were identified . Results ①Duringcontrast‐enhancedRT‐3DE,ascomparedwithnon‐enhancedRT‐3DE,thenumberof segments with complete visualization of the endocardial border increased significantly (55% vs 82% ,P <0.01) ,and the number of patients with a good‐quality echocardiogram increased significantly (33% vs 81% , P <0.01) .②Contrast‐enhanced RT‐3DE provided significantly larger values of LVEDV ( P < 0 0.1) and LVESV ( P < 0 0.1) as compared with non‐enhanced RT‐3DE ,the values of LVEF were not statistically different between the two techniques ( P =0.07) .③Intra‐and inter‐observer agreement for assessment of LV volumes and systolic function improved during contrast‐enhanced RT‐3DE ,as compared with non‐enhanced RT‐3DE .Conclusions Contrast‐enhanced RT‐3DE can increase the prevalence of good‐quality echocardiograms and significantly improve the reproducibility of LV volumes and function measurements .

Value of left ventricular contrast echocardiography in diagnosis of left ventricular myocardium noncompaction / 中华超声影像学杂志

Objective To assess the usefulness of left ventricular contrast echocardiography in diagnosis of left ventricular myocardium noncompaction.Methods Contrast echocardiography was done in ten patients who were diagnosed or suspected with left ventricular noncompaction by common transthoracic echocardiography,for further study of the trabecular muscles extent,the continuity of the endocardium,the compact myocardium thickness,and the contrast agent in the trabecula recessus.Results By contrast echocardiography,noncompaction myocardium thickness can be perspicuously observed,the turgor of the contrast agent was vividly detected in the trabecular recessus.Especially for the measurement of compaction myocardium,the contrast echocardiography was more accurate than in the condition of the common echocardiography.Conclusions Left ventricular contrast echocardiography can be used in the diagnosis of left ventricular noncompaction,it was a good added method of conventional echocardiography.

Isolated Noncompaction of Ventricular Myocardium: a Magnetic Resonance Imaging Study of 11 Patients

OBJECTIVE: To retrospectively summarize the cardiac magnetic resonance imaging (CMRI) findings of isolated noncompaction of ventricular myocardium (INVM). MATERIALS AND METHODS: Eleven patients (M:F = 9:2; mean age, 35 years) were evaluated. Steady-state free precession (SSFP), fast spin echo (SE) sequence, SSFP cine imaging, and delayed enhanced inversion recovery spoiled gradient echo (IR-SPGR) sequence were used for showing abnormal myocardium, measuring ratio of noncompacted/compacted myocardium layers (NC/C ratio), and detecting myocardial viability. The left ventricle was divided into nine segments and a NC/C ratio > 2.3 in diastole was used as cutoff value in diagnosing left INVM. The right ventricle was assessed qualitatively. RESULTS: Cardiac MRI indicated left INVM in seven patients, right INVM in one patient and biventricle INVM in three patients. Characteristic CMRI changes included prominent trabeculations, deep intertrabecular recesses and an increase in the NC/C ratio. The most frequently involved segments was left ventricular apex. Three patients had abnormal high signals within the trabecular structures on SE T2 weighted image. One ventricular aneurysm and one apical thrombus were also observed. Delayed enhancement was seen in six of nine patients with subendocardial and transmural patterns. CONCLUSION: There are CMRI features that might be characteristic for INVM.

Miocárdio não compactado de ventrículo esquerdo: relato de caso com estudo de necropsia / Noncompaction of ventricular myocardium: case report with autopsy

O miocárdio não compactado de ventrículo esquerdo é cardiomiopatia genética em decorrência de uma anormalidade da morfogênese endomiocárdica. Quando presente, geralmente está associado a outras anomalias cardíacas congênitas, sendo raro seu achado isolado sem outras alterações estruturais cardíacas associadas. Desde seu primeiro relato em 1990, a não compactação do miocárdio vem sendo amplamente estudada a fim de se estabelecerem caráter genético, história natural, métodos de diagnóstico, repercussões hemodinâmicas e prognóstico. O objetivo deste estudo foi relatar um caso de miocárdio não compactado isolado de ventrículo esquerdo com estudo de necrópsia.

Noncompaction of the ventricular myocardium is a genetic cardiomyopathy due to an alteration in endomyocardial morphogenesis. When present, it is commonly associated with other congenital cardiac diseases, whereas it is rare as an isolated finding without other associated cardiac structural alterations. Since its first report in 1990, the noncompaction of the ventricular myocardium has been widely studied in order to establish its genetic character, natural history, diagnostic methods, hemodynamic repercussions and prognosis. This study aims to report the case of an isolated noncompaction of the left ventricular myocardium with autopsy.

Long-term outcomes and clinical analysis of 16 noncompaction of ventricular myocardium / 中国实用内科杂志

Objective To investigate the features and prognosis of noncompaction of ventricular myocardium (NVM) in Chinese.Methods The clinical features of 16 NVM patients were analyzed and 15 patients were followed prospectively.Results Familial occurrence were found in seven patients.Six came to hospital for heart failure and two for chest pain.Arrhythmias were found in six patients.Six patients were asymptomatic.Fifteen patients were followed prospectively for 52.06?8.46 months.In the follow-up,three patients died and heart failure occurred in one asymptomatic patients.One patients accepted permanent pacemaker.Five patients were still asymptomatic.Conclusion In general,the prognosis of NVM is poor,but the outcome is better in patients without symptom.The early diagnosis is important.