N-terminal pro-B-type natriuretic peptide levels in acute versus chronic left ventricular dysfunction

OBJECTIVES: To determine whether acute left ventricular dysfunction (LVD) causes significantly higher elevation of N-terminal pro-B-type natriuretic peptide (NT-proBNP) levels than comparable chronic LVD. STUDY DESIGN: Plasma levels of NT-proBNP were measured in 10 pediatric patients diagnosed with acute LVD, in 7 pediatric patients with stable chronic dilated cardiomyopathy (DCM) and comparable levels of echocardiographic dysfunction, and during 5 episodes of acute exacerbation in patients with heart failure. Levels were compared using Mann-Whitney and analysis of variance for rank tests. RESULTS: Plasma levels of NT-proBNP were excessively elevated in patients with acute LVD in the first 24 to 48 hours of hospitalization (median level, 65,600 pg/mL), and were significantly higher than those in patients with chronic DCM (median level, 1125 pg/mL; P < .0001). NT-proBNP levels decreased in the subsequent days in 83% of patients with serial measurements. The NT-proBNP levels were lower In 5 episodes of acute exacerbation than in acute LVD (median level, 7185 pg/mL; P < .003). CONCLUSIONS: Acute LVD is associated with elevated NT-proBNP level in children.     

Cardiac Troponin T: A Marker in the Diagnosis of Acute Myocarditis in Children

This study was conducted to assess the use of serum cardiac troponin T (cTnT) level as a noninvasive indicator to diagnose acute myocarditis in children. Noninvasive conventional methods often fail to diagnose myocarditis, A median cTnT level of 0.088 ng/ml (0.04–3.11) was reported in pediatric patients with acute myocarditis in our previous study. Hence, we attempted to determine the cutfoff level of cTnT to diagnose acute myocarditis in children. Pediatric patients with clinically suspected myocarditis or dilated cardiomyopathy (DCM) and a control group were recruited. History, physical examination, elctrocardiogram, chest roentgenogram, echocardiogram, cTnT level, and/or endomyocardial biopsy and clinical course were studied. The gold standard to diagnose acute myocarditis was endomyocardial biopsy proved according to the Dallas criteria and/or recovery from cardiovascular problems within 6 months of follow-up. Forty-three patients were admitted due to cardiovascular problems from primary myocardial dysfunction. Twenty-four patients were diagnosed as acute myocarditis (group 1), 19 were idiopathic chronic DCM (group 2), and 21 patients had moderate to large ventricular septal defect and congestive heart failure (group 3). Median cTnT level was statistically higher in (group 1) compared to groups 2 and 3. Ejection fraction (EF) and left ventricular end diastolic dimension (LVEDd) z score of acute myocarditis were 38.5% (range, 21–67) and 1.3 (range, –0.8–3.0), respectively, which were significantly better than DCM [28.0% (range, 17–45) and 6.0 (range, 2.0–10.0)]. The cutoff point of cTnT level to diagnose acute myocarditis was 0.052 ng/ml (sensitivity, 71%; specificity, 86%). cTnT level, EF, and LVEDd z score did not predict short-term outcomes of patients. In acute myocarditis, cTnT level and EF were significantly higher and LVEDd z score was significantly lower than in DCM. However, the three parameters had no significant effect on outcomes of the patients. Our data show that cardiac a cTnT level of 0.052 ng/ml is an appropriate cutoff point for the diagnosis of acute myocarditis.     

The Efficacy of Mitral Valve Surgery in Children with Dilated Cardiomyopathy and Severe Mitral Regurgitation

Severe mitral regurgitation predicts poor outcomes in adults with left ventricular dysfunction. Frequently, adult patients now undergo initial mitral valve surgery instead of heart transplant. Pediatric data are limited. This study evaluates the efficacy of mitral valve surgery for severe mitral regurgitation in children with dilated cardiomyopathy. This is a single-institution experience in seven children (range, 0.5–10.9 years) with severe mitral regurgitation and dilated cardiomyopathy who underwent mitral valve surgery between January 1988 and February 2005, with follow-up to January 2006. Children with dilated cardiomyopathy had a depressed fractional shortening preoperatively (24.4% ± 6.1%) that remained depressed (22.9% ± 7.6%) 1.3 ± 1.2 years after surgery (p = 0.50). Left ventricular end-diastolic (6.5 ± 1.5 to 4.8 ± 1.8 z-scores, p < 0.01) and end-systolic (6.8 ± 1.5 to 5.5 ± 2.1 z-scores, p < 0.05) dimensions improved. Hospitalization frequency had a median decrease of 6.0 hospitalizations per year (p < 0.02). Three patients were transplanted 0.2, 2.4, and 3.5 years after surgery. There was no perioperative mortality. Mitral valve surgery in children with dilated cardiomyopathy was performed safely and improved symptoms, stabilizing ventricular dysfunction in most patients. Mitral valve surgery should be considered prior to heart transplant in children with dilated cardiomyopathy and severe mitral regurgitation.     

19例儿童重症心肌炎诊治体会

目的 探讨儿童重症心肌炎临床特点及治疗方法。方法 对我院2005年1月至2012年1月收治的19例儿童重症心肌炎（重症心肌炎组）发病特点、临床表现、诊治经过及预后进行回顾性分析,选择同期在我院体检的正常健康儿童23例为对照组。采用ELISA法检测心肌肌钙蛋白（cardiac troponin,CTn）-Ⅰ及血清氨基末端脑利钠肽前体（N-terminal pro-brain natriuretic peptide,NT-proBNP）水平,应用彩色多普勒超声心动图检查了解左室射血分数和左室短轴缩短率变化。结果 重症心肌炎组患儿CTn-Ⅰ为（18.67 ±12.31） ng/ml,显著高于正常对照组[（0.02 ±0.01） ng/ml],差异有统计学意义（P＜0.05）。与急性期相比,病程第7天CTn-Ⅰ为（0.55±0.24） ng/ml,呈逐渐下降趋势,第14天基本接近正常[（0.06±0.03） ng/ml],差异有统计学意义（P＜0.05）。重症心肌炎组NT-proBNP较对照组明显增高[（3 067.26 ±902.79） pg/ml vs （80.04±17.79） pg/ml,P＜0.05]。与急性期相比,病程第7天NT-proBNP为（648.63±342.37） pg/ml,病程第14天基本接近正常[（213.58±129.51） pg/ml]（P＜0.05）。重症心肌炎组患儿左室射血分数[（52.63±6.98）％vs（71.39±2.41）％]及左室短轴缩短率[（32.1±2.97）％vs（40.04±2.31）％]明显低于正常对照组,差异均有统计学意义（P均＜0.05）。结论 儿童重症心肌炎起病急,病情重,病死率高,在综合治疗基础上早期应用肾上腺素皮质激素和丙种球蛋白,必要时安装临时起搏器,可改善预后。     

扩张型心肌病患儿血清一氧化氮含量变化及其与左室功能关系

为探索扩张型心肌病 (DCM )患儿血清一氧化氮 (NO)含量的变化及其与左室功能的关系 ,检测26例DCM患儿血清NO水平及左室射血分数 (LVEF) ,并以34例健康儿童血清NO含量作为对照。结果显示 ,DCM患儿血清NO含量较正常儿童明显增高 (P<0.01) ,并与LVEF呈显著负相关 (r= -0.80,P<0.01)。提示DCM患儿的心功能下降可能与NO过度生成有关     

The effect of treatment with angiotensin-converting enzyme inhibitors on survival of pediatric patients with dilated cardiomyopathy

Summary Outcome in 81 pediatric patients with dilated cardiomyopathy was reviewed to assess whether treatment with angiotensin-converting enzyme (ACE) inhibitors affected survival. Age at onset was 3.6±0.6 years. Twenty-seven children (group 1) were treated with ACE inhibitors. Conventional therapy was used in the remaining 54 patients (group 2). There were no significant differences between the two groups in age at onset, left ventricular shortening fraction, left ventricular end-diastolic pressure, or mean pulmonary artery pressure. Patients treated with ACE inhibitors had a significantly better survival during the first year (p<0.05) with continuation of this trend throughout the second year (p=0.06). Beyond 2 years there was a tendency toward better survival in ACE inhibitor-treated patients, but the differences were no longer significant (p=0.14). These data, along with observations in adult patients with chronic cardiac failure, indicate that converting enzyme inhibitors have a beneficial effect on prolonging survival of infants and children with severe left ventricular dysfunction from dilated cardiomyopathy.     

血清嗜铬粒蛋白A、尾加压素Ⅱ在小儿慢性心力衰竭中的变化及意义

目的探讨嗜铬粒蛋白A(CgA)、尾加压素Ⅱ(UⅡ)在慢性心力衰竭(CHF)患儿血清中的变化及意义。方法选取58例CHF患儿为心衰组,其中心内膜弹力纤维增生症17例,扩张型心肌病41例;另选取门诊健康体检儿童20例为对照组。采用酶联免疫吸附法(ELISA法)测定血清CgA及UⅡ水平;采用双向侧流免疫法测定氨基末端脑钠肽前体(NT-proBNP)水平;超声心动图测定心室重塑指标;Pearson相关或Spearman秩相关分析血清CgA、UⅡ与心室重塑的相关性。结果心功能Ⅱ级患儿的血清CgA、NT-proBNP水平与对照组的差异无统计学意义(P0.05);CgA、NT-proBNP水平在心功能Ⅲ级、Ⅳ级患儿中高于对照组,并且随着心功能损害加重而升高(P0.05)。UⅡ浓度在心功能Ⅱ级、Ⅲ级、Ⅳ级患儿中均低于对照组,并且随着心功能损害加重而逐渐降低,差异有统计学意义(P0.05)。心内膜弹力纤维增生症与扩张型心肌病患儿间血清CgA、UⅡ水平的差异无统计学意义(P0.05)。血清CgA浓度分别与左心室质量分数(LVMI)、NT-proBNP、心功能分级成正相关(r分别为0.279、0.649及0.778,P0.05),与左室射血分数(LVEF)、左室短轴缩短率(LVFS)、UⅡ成负相关(r分别为-0.369、-0.322及-0.718,P0.05)。血清UⅡ分别与NT-proBNP、心功能分级成负相关(r=-0.472、-0.591,P0.05),而与LVMI、LVEF、LVFS无明显相关性(P0.05)。结论 CgA可能参与CHF患儿心室重塑,血清CgA和UⅡ有可能为心衰的诊断和心功能判断提供参考。     

小儿扩张型心肌病并心力衰竭红细胞分布宽度与心功能关系研究

目的 观察扩张型心肌病（DCM）并发心力衰竭患儿红细胞分布宽度（RDW）水平的变化,并探讨其与常用心功能评价指标的相关性。方法 收集武汉市妇女儿童医疗保健中心2012年1月至2014年12月收治的并发心力衰竭的DCM患儿68例, 按改良Ross评分法, 将其分为轻度、 中度、 重度心力衰竭组, 选取健康体检儿童50名作为对照组, 比较各组间RDW水平的差异, 并将DCM患儿RDW与血浆N-端脑利钠肽前体（NT-proBNP）、 左室射血分数（LVEF）进行相关性分析。结果 DCM合并心力衰竭组RDW水平较对照组明显升高,差异有统计学意义（P＜0.01）。心力衰竭轻度、中度、重度患儿间NT-proBNP水平逐级升高,LVEF逐级下降,差异有统计学意义（P均＜0.01）,而心力衰竭各组间RDW水平差异无统计学意义。RDW与NT-proBNP、LVEF均无显著相关性（r分别为0.144、-0.158,P均＞0.05）。结论 RDW升高有助于小儿DCM合并心力衰竭的诊断,但其能否作为小儿心力衰竭严重程度的判断指标尚不能明确。     

Utility of B-Type Natriuretic Peptide in Differentiating Congestive Heart Failure from Lung Disease in Pediatric Patients with Respiratory Distress

Plasma B-type natriuretic peptide (BNP) has been reported to be helpful in differentiating cardiac from pulmonary etiologies of dyspnea in adults. We investigated whether BNP concentration could be applied similarly in children with respiratory distress. BNP levels were measured using a rapid immunoassay in 49 infants and children presenting with acute respiratory distress. The patients history, symptoms, physical exam, chest x-ray, and an echocardiogram were used to identify patients with congestive heart failure (CHF) from noncardiac causes of respiratory distress. Results are reported as mean ± SD. Patients with CHF (n = 23) had BNP levels of 693.0 ± 501.6 pg/ml, significantly higher than those of the group of patients with lung disease (n = 26), whose BNP was 45.2 ± 64.0 pg/ml (p < 0.001). There was no significant difference in age between the two groups (29.7 ± 59.3 vs 13.1 ± 22.6 months; p = 0.12). A BNP level of 40 pg/ml was 84% accurate in differentiating CHF from pulmonary disease. Fifteen of 23 CHF patients had ventricular volume overload from left-to-right shunting congenital heart defects and 8/23 had left ventricular systolic dysfunction. Age-adjusted comparison of the two subgroups of CHF patients revealed that children with left ventricular systolic dysfunction had significantly higher mean BNP levels than those with left-to-right shunts (1181 ± 487 vs 433 ± 471 pg/ml, p = 0.0074). We conclude that BNP level is of value in differentiating cardiac from pulmonary causes of respiratory distress in children.     

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??Objective To observe the changes of red cell distribution width ??RDW?? in children with heart failure secondary to dilated cardiomyopathy??DCM????and to explore the correlations between RDW and the common parameters of cardiac function. Methods Totally 68 children with heart failure secondary to DCM were enrolled. According to the modified Ross scoring system??the children were divided into 3 subgroups?? mild??moderate and severe heart failure subgroups. Fifty healthy children were selected as control group. RDW levels were compared among the groups. The correlation analysis was performed between RDW and plasma N-terminal brain natriuretic peptide ??NT-proBNP??as well as left ventricular ejection fraction??LVEF??. Results RDW levels in children with heart failure secondary to DCM were significantly higher than the control group ??P??0.01??.With increasing heart failure grade??NT-proBNP levels were elevated gradually and LVEF were decreased gradually ??both P??0.01????RDW levels had no significant difference among the heart failure groups??P??0.05??. RDM was not correlated with NT-proBNP or LVEF??r??0.144??r??-0.158??P??0.05??. Conclusion In children with DCM??RDW can be used as a new diagnostic marker of heart failure??but it can not reflect the severity of heart failure.     

Clinicopathologic Characteristics of Hypertrophic Cardiomyopathy Detected During Mass Screening for Heart Disease

Between 1981 and 1992 a total of 10 patients with hypertrophic cardiomyopathy (HCM) were detected by mass screening for heart disease in Tokyo's Adachi Ward. Four were first grade elementary school children and six were first grade junior high school adolescents. Two-dimensional echocardiography at the initial evaluation revealed asymmetric septal hypertrophy in four patients, diffuse hypertrophy of the left ventricle in five, and poor left ventricular contractility with wall thinning in one (dilated phase). Three of the five patients with diffuse hypertrophy progressed to asymmetric septal hypertrophy during the average 4-year follow-up period. The degree of septal thickness and the left ventricular wall thickness index were significantly less than in those of young adult controls (12 ± 3 versus 21 ± 9 mm, p < 0.05; and 22 ± 4 versus 28 ± 16 mm, p < 0.05, respectively). Right ventricular endomyocardial biopsy specimens obtained from 9 of the 10 patients showed features typical of HCM (e.g., myocyte hypertrophy with myofibril disarray) in five patients and atypical features (mainly interstitial fibrosis with perivascular cell infiltration) in another four. One patient with dilated phase disease died of congestive heart failure 6 months after the initial evaluation. These results indicate that HCM detected during mass screening is a mild form of the disease and may have atypical pathologic features, such as interstitial fibrosis and perivascular cell infiltration, mimicking the sequela of chronic myocarditis.     

Circulating cardiac troponins levels and cardiac dysfunction in children with acute and fulminant viral myocarditis

Objectives: To study the correlation between cardiac Troponins blood levels and degrees of cardiac dysfunction in children with acute and fulminant viral myocarditis and to study their prognostic role in predicting the outcomes and risk of having dilated cardiomyopathy. Methodology: Troponin I & T blood levels were measured in 65 children with acute or fulminant viral myocarditis. The cardiac functions of RV & LV were assessed by Doppler echocardiography. Results: The levels of cTnI & CTnT were significantly higher in patients with fulminant myocarditis than in controls and children with acute myocarditis (p < 0.05 & <0.001* respectively). The cardiac functions were significantly impaired in fulminant myocarditis than in acute myocarditis (p < 0.001*). There were negative correlations between the cardiac troponins levels and the cardiac functions measured by echocardiography in children with acute and fulminant myocarditis. There were 3 deaths (7.5%), and 10 (25%) children developed dilated cardiomyopathy in acute myocarditis while there were eight deaths (32%) and one patient (4%) who developed dilated cardiomyopathy in fulminant myocarditis group. Conclusion: Cardiac troponins levels can predict the severity of myocarditis and the prognosis on the short‐term level. Fulminant myocarditis was associated with higher levels of both cTn I & cTn T than acute myocarditis. Despite that fulminant myocarditis has a more aggressive course, the risk of developing cardiomyopathy was less than in acute myocarditis.     

N-Terminal Pro-Brain Natriuretic Peptide as a Marker in Follow-Up Patients with Tetralogy of Fallot After Total Correction

Patients with tetralogy of Fallot (TOF) after total correction usually have residual pulmonary regurgitation resulting in right ventricular (RV) dilatation and dysfunction. This study was performed to evaluate N-terminal pro-brain natriuretic peptide (NT-proBNP) in predicting RV dilatation and RV dysfunction in TOF after total correction. Twenty-one patients with TOF after total correction (12 males and 9 females, 12.06 ± 2.54 years old) underwent echocardiography, cardiac magnetic resonance imaging (MRI), and blood sampling for NT-proBNP. Mean time after total correction was 7.59 ± 2.30 years. From cardiac MRI study, mean right ventricular end diastolic volume index (RVEDVi) was 148.36 ± 64.50 ml/m² and mean right ventricular ejection fraction (RVEF) was 35.50 ± 10.50%. Right ventricular dilatation was considered if RVEDVi was >108 ml/m² and RV dysfunction was considered if RVEF was <40%. A plasma NT-proBNP level of 115 pg/ml was identified by receiver operating characteristic analysis in predicting RV dilatation and/or dysfunction. At this value, the sensitivity and specificity for predicting RV dilatation, RV dysfunction, and both RV dilatation and dysfunction were 71 and 100%, 71 and 71%, and 83 and 78%, respectively. In conclusion, plasma NT-proBNP level may be helpful in follow-up patients. Plasma NT-proBNP levels >115 pg/ml can be used as a marker in the detection of RV dilatation and dysfunction. Presented at The Scientific Sessions, American Heart Association, Dallas, Texas, USA, November 2005.     

N-terminal pro-B-type natriuretic peptide: reference plasma levels from birth to adolescence. Elevated levels at birth and in infants and children with heart diseases

AIM: Determination of plasma levels of N-terminal pro-B-type natriuretic peptide (N-BNP) in infants and children with and without heart diseases. METHODS: Plasma N-BNP was measured in 78 infants and children without heart disease and in 55 infants and children with heart disease causing volume and pressure overload. Heart diseases included chronic dilated cardiomyopathy, acute left ventricular dysfunction, and congenital cardiac anomalies resulting in left and right ventricular volume or pressure overload. The Mann-Whitney rank-sum test and the ANOVA for ranks test were used to compare two or more groups, respectively. RESULTS: N-BNP levels were elevated in the first days of life but were not significantly different in children from 4 mo to 15 y old. The upper limit in children older than 4 mo with no heart disease was 349 pg/ml. In patients with heart disease, N-BNP levels were significantly higher than in control children (p < 0.0001). CONCLUSION: N-BNP levels are elevated in the first days of life and are stable from age 4 mo to adolescence. Elevated N-BNP levels reflect cardiac dysfunction in infants and children.     

Brain Natriuretic Peptide as a Hormonal Marker of Ventricular Diastolic Dysfunction in Children with Kawasaki Disease

Although an increased level of serum brain natriuretic peptide (BNP) has been reported in children in the acute phase of Kawasaki disease (KD), no precise relation was documented between the serum BNP level and left ventricular (LV) systolic function. We hypothesized that the increased BNP levels may be explained by diastolic abnormalities in those with KD. We prospectively studied 25 patients in the acute phase of KD. Patients with abnormal systolic function were excluded. Pediatric cardiologists making the assessment of LV diastolic function were blinded to the BNP levels. Doppler interrogation was applied to measure LV inflow velocities, which were transformed to z scores using control measurements obtained from 83 healthy subjects. In the patients, the BNP levels ranged from 2.0 to 450.0 pg/ml, with a mean of 54.0 ± 102.8 pg/ml. Six patients with abnormal velocities (> 2 SD in z score) showed significantly higher levels of BNP (152 ± 173 pg/ml) than those in the remaining patients (p < 0.01). The BNP levels correlated positively with diastolic atrial velocity in z score (r = 0.51, p < 0.05), and negatively with diastolic early velocity to atrial velocity ratio in z score (r = -0.75, p < 0.01). This study suggests that LV diastolic dysfunction may occur in some children in the acute phase of KD, causing an increased level of BNP.     

Utilidad de la terapia intracoronaria con células progenitoras en pacientes con miocardiopatía dilatada: ¿puente o alternativa al trasplante cardiaco?

Introduction and objectivesSome paediatric publications have recently raised the value of intracoronary therapy with autologous bone marrow-derived progenitor cells (APCs) in children with dilated cardiomyopathy (DCM) and heart failure. We describe the usefulness of this treatment in two infants with severe DCM and heart failure, who had been transferred to our hospital for cardiac transplant evaluation.Patients and methodsThe first patient was a 3 months old male weighing 4 kg. The second was a 4 months old male weighing 5 kg. At the time of admission, both were in poor clinical condition (NYHA IV), with severe dilation and systolic dysfunction (ejection fraction [EF]<30%) of the left ventricle and marked elevation of NT-proBNP, requiring treatment with mechanical ventilation and inotropic iv infusion. After mobilization with G-CSF for 4 days, APCs were obtained from peripheral blood by leukocytapheresis, administering them by a slow intracoronary bolus injection using a stop-flow technique (6.15x106 CD34-positive cells/Kg in the first patient, and 10.55x106 CD34-positive cells/Kg in the second).ResultsSince the first week after the procedure, clinical status of patients improved and echocardiography showed a decrease in left ventricular dilation. A month later, there was a significant improvement in EF (> 40%) and NT-proBNP levels, subsequently maintained throughout the follow-up. However, four months later in the first patient, the left ventricle dilated again and its function slightly worsened, but without any significant impact in his clinical status.ConclusionsIntracoronary therapy with APCs can be an alternative in children, especially infants, with DCM and heart failure. It can reduce the waiting list mortality, improve clinical status and provide more time on the waiting list to receive a suitable organ, or even to make transplantation unnecessary.     

Dyssynchronous ventricular contraction in Wolff–Parkinson–White syndrome: a risk factor for the development of dilated cardiomyopathy

Emerging evidence suggests that significant left ventricular dysfunction may arise in right-sided septal or paraseptal accessory pathways (APs) with Wolff–Parkinson–White syndrome, even in the absence of recurrent or incessant tachycardia. During 1 year and 9 months, we identified four consecutive female children with median age of 8 years diagnosed as having dilated cardiomyopathy (DCM) combined with overt right-sided APs several years ago. Incessant or recurrent tachycardia as the cause of DCM could be excluded. Anti-heart failure chemotherapy did not produce satisfactory effects. The patients underwent radiofrequency ablations (RFCAs). This report describes the clinical and echocardiographic characteristics of the cases before and after the ablation. Dyssynchronous ventricular contraction was observed in all patients. The locations of the APs were the right-sided anteroseptum and the free wall (n?=?2 each). All patients received successful RFCAs. Their physical activities and growth improved greatly, and the echocardiographic data demonstrated that their left ventricular (LV) contraction recovered to synchrony shortly after the ablation and that their LV function recovered to normal gradually during the follow-up. Conclusions: A causal relationship between overt ventricular preexcitation and the development of DCM is supported by the complete recovery of LV function and reversed LV remodeling after the loss of ventricular preexcitation. Preexcitation-related dyssynchrony was probably the crucial mechanism. Not only right-sided septal or paraseptal but also free wall overt APs may induce LV dysfunction and even DCM. AP-induced DCM is an indication for ablation with a good prognosis.     

Safety and Efficacy of Carvedilol Therapy for Patients with Dilated Cardiomyopathy Secondary to Muscular Dystrophy

Background By the age of 20 years, almost all patients with Duchenne’s or Becker’s muscular dystrophy have experienced dilated cardiomyopathy (DCM), a condition that contributes significantly to their morbidity and mortality. Although studies have shown carvedilol to be an effective therapy for patients with other forms of DCM, few data exist concerning its safety and efficacy for patients with muscular dystrophy. This study aimed to evaluate the safety and efficacy of carvedilol for patients with DCM. Methods A clinical trial at an outpatient clinic investigated 22 muscular dystrophy patients, ages 14 to 46 years, with DCM and left ventricular ejection fraction (LVEF) less than 50%. Carvedilol up-titrated over 8 weeks then was administered at the maximum or highest tolerated dose for 6 months. Baseline and posttreatment cardiac magnetic resonance imaging (CMR), echocardiography, and Holter monitoring were recorded. Results Carvedilol therapy was associated with a modest but statistically significant improvement in CMR-derived ejection fraction (41% ± 8.3% to 43% ± 8%; p < 0.02). Carvedilol also was associated with significant improvements in both the mean rate of pressure rise (dP/dt) during isovolumetric contraction (804 ± 216 to 951 ± 282 mmHg/s; p < 0.05) and the myocardial performance index (0.55 ± 0.18 to 0.42 ± 0.15; p < 0.01). A trend toward improved shortening fraction, E/E’ ratio, and isovolumetric relaxation time also was observed. Two patients had runs of nonsustained ventricular tachycardia exceeding 140 beats per minute (bpm) before carvedilol administration. Ventricular tachycardia exceeding 140 bpm was not observed after carvedilol therapy. Carvedilol was well tolerated, and no serious adverse events were identified. Conclusions Carvedilol therapy appears to be safe for patients with DCM secondary to muscular dystrophy and produces a modest improvement in systolic and diastolic function.     

The highest mortality rates in childhood dilated cardiomyopathy occur during the first year after diagnosis

Aim

The aim of the study was to assess the incidence, mortality and morbidity of dilated cardiomyopathy (DCM) and noncompaction of the left ventricle (LVNC) in Swedish children.

Methods

We reviewed hospital records of all children with dilated cardiomyopathy (DCM) or left ventricular noncompaction cardiomyopathy (LVNC) up to the age of 18 in the healthcare region of western Sweden from 1991 to 2015.

Results

In total, 69 cases (61% males) were identified. The combined incidence of DCM and LVNC was 0.77 (95% CI 0.59‐0.96) per 100 000 person years. Children were divided into six groups, and their outcomes were analysed depending on their aetiology. Idiopathic DCM was reported in 43%, and familial dilated and left ventricular noncompaction aetiology was present in 32%. DCM due to various diseases occurred in 8%. DCM associated with neuromuscular diseases was present in 16%. The overall risk of death or receiving transplants in children with idiopathic and familial DCM was 30% over the study period, and 21% died in the first year after diagnosis.

Conclusion

The combined incidence of DCM and LVNC was similar to previous reports. Most children with idiopathic DCM presented during infancy, and mortality was highest during the first year after diagnosis.     

Myocardial scintigraphy after pacemaker implantation for congenital complete atrioventricular block

Patients with isolated congenital complete atrioventricular block (CCAVB) occasionally develop dilated cardiomyopathy (DCM), despite early pacemaker implantation. However, the etiology of the DCM and its relationship to permanent ventricular pacing are not fully understood. Twenty-five patients with CCAVB underwent ^99m technetium (Tc) myocardial perfusion scintigraphy. Five patients were studied before and after pacing, providing a total of 30 image sets, which were divided into three groups; group 1: CCAVB before pacemaker implantation (PMI) (n = 11); group 2: CCAVB after PMI who did not subsequently develop DCM (n = 13); group 3: CCAVB after PMI who subsequently developed DCM (n = 6). Perfusion defects on single-photon-emission computed tomography (SPECT) were identified in group 1, 0 of 11 patients; group 2, 85% of patients; and group 3, 100% of patients. In groups 2 and 3, in patients with right ventricular pacing, the perfusion defects were mainly in the septum or between the apex and septum. On 20 segments’ polar maps, the distribution of %uptake showed a similar pattern in groups 2 and 3, the degree of decreased %uptake and the number of segments with decreased %uptake being more severe in group 3. “Artificial” left bundle branch block (LBBB) pattern myocardial contraction induced by right ventricular pacing decreased myocardial perfusion around the apex and septum. Some patients with CCAVB will develop left ventricular dysfunction caused by artificial LBBB-induced interventricular asynchrony.