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.     

Comparison of Heart Failure in Children with Enterovirus 71 Rhombencephalitis and Cats with Norepinephrine Cardiotoxicity

The mechanism of heart failure in patients with enterovirus 71 rhombencephalitis (brain stem encephalitis) remains unknown. Our previous reports hypothesized that a catecholamine storm induced by rhombencephalitis may account for the heart failure. The aim of this study was to develop a novel feline model of norepinephrine cardiotoxicity and compare the resulting heart failure to that in children with enterovirus 71 rhombencephalitis. Nine of 75 children (12%) with enterovirus 71 rhombencephalitis (5 boys and 4 girls; age, 4–28 months; median age, 16 months) were complicated with left ventricular hypokinesia (ejection fraction, 31 ± 9%). Six cats (weight, 3.03 ± 0.64 kg) were administered intravenous norepinephrine 30 μg/kg/min for 3 hours. Echocardiography assessed the left ventricular diameter and function before and after the administration of norepinephrine. Pathology studies included hematoxylin and eosin stain and in situ terminal deoxyribonucleotidyl transferase-mediated dUTP nick end-labeling assay. In the feline model, norepinephrine induced significant left ventricular dilatation (end diastolic diameter from 1.18 ± 0.19 to 1.62 ± 0.22 cm, p = 0.001; endsystolic diameter from 0.54 ± 0.09 to 1.36 ± 0.32 cm, p = < 0.001) and hypokinesia (ejection fraction from 87.5 ± 4.1 to 35.2 ± 16.3%, p = 0.001). Heart specimens from 4 patients and six cats showed similar pathology findings, including myocardial hemorrhage, cardiomyocyte apoptosis, and coagulative myocytolysis, which is characterized by sarcoplasmic coagulation, granulation, vacuolization, myofibrillar waving, and disruption. Both groups showed no significant inflammatory reaction. In conclusion, heart failure in patients with enterovirus 71 rhombencephalitis is similar to that in cats with norepinephrine cardiotoxicity. Norepinephrine cardiotoxicity may play a role in the pathogenesis of heart failure in enterovirus 71 rhombencephalitis.     

Adrenomedullin and Nitrite Levels in Children with Dilated Cardiomyopathy

Dilated cardiomyopathy (DCM) is an important cause of chronic congestive cardiac failure in children. In patients with idiopathic DCM, endothelium vasomotor function is disturbed. There are many studies on the roles of nitric oxide (NO) and adrenomedullin (AM) in adult patients with DCM. However, to our knowledge, no studies have investigated the level of AM and NO in children with idiopathic DCM. We determined plasma and urinary AM and total nitrite concentrations in children with idiopathic DCM and investigated the correlation between these and other clinical and laboratory findings. Eleven patients with DCM, ranging in age from 5 month to 14 years, were compared to 10 healthy age- and sex-matched controls. Plasma (pmol/ml) and urinary (pmol/mg creatinine) AM levels were significantly lower than in the healthy controls (19.55 ± 2.36 vs 51.61 ± 7.22 and 28.29 ± 20.66 vs 68.87 ± 40.23, respectively; p < 0.001). Plasma and urinary AM levels were negatively correlated with ejection fraction (EF) and fractional shortening (FS). The plasma (µmol/L) and urinary nitrite levels (µmol/mg creatinine) were not different between patients and controls [50.90 ± 17.50 vs 53.40 ± 26.05 (p > 0.05) and 1.98 ± 1.24 vs 2.75 ± 1.68 (p > 0.05), respectively]. In our study, the first to analyze AM and nitrite levels in children with DCM, plasma and urinary AM levels were found to be decreased. A possible explanation for this reduction could be depletion of the viable myocyte population. However, this hypothesis must be clarified by further studies.     

Hemodynamic Abnormalities in Fetuses with Congenital Heart Disease

The purpose of this investigation was to assess left and right ventricular function, volume ejection fraction, combined stroke volume, and combined ventricular output in the human fetus with congenital heart disease compared to the normal healthy fetus. Seventy-two fetuses with a variety of in utero diagnosed congenital cardiac defects were compared with a control group of fetuses with structurally normal hearts matched for race, maternal age, and gestational age. We demonstrated significant hemodynamic changes in the fetus with congenital heart disease. There was a significant (p < 0.0001) decrease in the volume ejection fractions, biventricular stroke volume, and cardiac output in the congenital heart disease group compared to matched controls. Our findings suggest that hemodynamic abnormalities in the fetus with congenital heart disease are present before birth and we speculate that myocardial reserve may not be adequate to respond to hemodynamic stressors such as birth or heart surgery.     

Dilated cardiomyopathy after pacemaker implantation in complete heart block

BACKGROUND: The aim of this study was to evaluate the clinical features of patients with congenital complete heart block (CCHB) who developed dilated cardiomyopathy (DCM) after pacemaker implantation (PMI) and to determine factors predicting DCM development. METHOD: A total of 15 patients were reviewed retrospectively. They were classified into two groups, one consisted of four patients who were diagnosed as having CCHB in utero or at birth and who developed DCM after PMI (DCM group) and the other consisted of 11 patients who did not (non-DCM group). RESULTS: Maternal autoantibodies were found in two of the DCM group and in five of the non-DCM group. Perfusion defects in myocardial imaging were detected in all DCM patients and in five non-DCM patients. DCM developed 2 to 43 months after PMI and three DCM patients died of heart failure 7 to 48 months after PMI. In pathological studies, endocardial or interstitial fibrosis was present in all DCM patients and in one of two in the non-DCM group. No significant differences between the two groups were found in age at PMI, atrial or ventricular rate, end-diastolic dimension and ejection fraction of the left ventricle before PMI, and width of QRS after PMI. CONCLUSION: Although it was suspected that the patients with CCHB had myocardial involvement before PMI, there was no significant factor predicting the risk of DCM after PMI. In addition to cardiac rhythm abnormalities, careful attention should be paid to cardiac function in CCHB patients after PMI.     

Percutaneous Transvenous Mitral Commissurotomy in Patients with Severe Mitral Stenosis and Acute Rheumatic Fever

There is a concern that percutaneous transvenous mitral commissurotomy (PTMC) performed during acute rheumatic fever (ARF) may result in adverse outcomes. We performed a retrospective study at a tertiary care center. Ten patients (ages 8–12 years) with severe symptomatic mitral stenosis and ARF who underwent PTMC (ARF group) were compared with 10 controls with similar mitral valve area (MVA). The procedure was successful in all patients. The median MVA increased from 0.7 to 1.5 cm² following PTMC in the ARF group and from 0.7 to 1.8 cm² in the control group [p = not significant (NS)]. The median MVA was 1.2 cm² in the study group compared to 1.6 cm² in the control group (p = 0.02) over a mean follow-up of 17.5 ± 11.7 months. Echocardiographic restenosis occurred in 4 patients (40%) in the ARF group compared to 1 patient (10%) in the control group (p = NS). The improvement in the New York Heart Association functional class was maintained in 8 patients of the ARF group and 9 patients of the control group. PTMC can be performed in children with ARF without an increase in procedural complications. However, the restenosis rates appear to be higher.     

Intravenous immunoglobulin in children with acute myocarditis and/or early dilated cardiomyopathy

We retrospectively studied medical records of all children with acute myocarditis and/or early DCM admitted to the Pediatric Critical Care Unit of our hospital between January 2010 and December 2012 were reviewed. 28 patients were included in the study, of which 12 were treated with IVIG (1 g/kg per day) for two days. The patients who received IVIG therapy had a higher left ventricular ejection fraction and a reduced left ventricular end diastolic diameter six months after treatment, as compared to children who had not received IVIG (P<0.001 and P=0.002, respectively).     

Outcome of acute lymphoblastic leukemia in children with down syndrome—Polish pediatric leukemia and lymphoma study group report

Children with Down syndrome (DS) have a 20-fold increased risk of developing leukemia compared with the general population. The aim of the study was to analyze the outcome of patients diagnosed with Down syndrome and acute lymphoblastic leukemia (ALL) in Poland between the years 2003 and 2010. A total of 1848 children were diagnosed with ALL (810 females and 1038 males). Of those, 41 (2.2%) had DS. The children were classified into three risk groups: a standard-risk group—14 patients, an intermediate-risk group—24, a high-risk group—3. All patients were treated according to ALLIC 2002 protocol. The median observation time of all patients was 6.1 years, and in patients with DS 5.3 years. Five-year overall survival (OS) was the same in all patients (86% vs 86%, long-rank test, p = .9). The relapse-free survival (RFS) was calculated as 73% in patients with DS and 81% in patients without DS during a median observation time (long-rank test, p = .3). No statistically significant differences were found in the incidence of nonrelapse mortality between those two groups of patients (p = .72). The study was based on children with ALL and Down syndrome who were treated with an identical therapy schedule as ALL patients without DS, according to risk group. This fact can increase the value of the presented results.     

NT-proBNP as a Marker for Persistent Cardiac Disease in Children with History of Dilated Cardiomyopathy and Myocarditis

Children with myocarditis and dilated cardiomyopathy may recover clinically and echocardiographically. Plasma levels of the N-terminal segment of B-type natriuretic peptide prohormone (NT-proBNP), a sensitive marker for cardiac dysfunction, may reflect residual cardiac damage in these patients. The purpose of this study was to evaluate NT-proBNP status in pediatric patients with a history of myocarditis and dilated cardiomyopathy. Cardiac evaluation was performed and the levels of NT-proBNP were measured in 23 children who had a history of myocarditis or dilated cardiomyopathy. NT-proBNP levels were also measured in 56 age-matched control children. Nine of the 23 patients had evidence of left ventricular dysfunction (DCM group), whereas 14 had none (recovery). NT-proBNP levels were higher in the DCM group (3154 ± 2858 pg/ml) than in the recovery group (122 ± 75 pg/ml, p < 0.001) and the control group (113 ± 96 pg/ml, p < 0.001). There was no difference between the recovery and the control groups (p = 0.45), and none of the recovered patients had a NT-proBNP level higher than the upper limit of normal. The area under the receiver operating characteristics curve for the diagnosis of persistent left ventricular dysfunction was 0.984. NT-proBNP levels correlated with echocardiographically derived shortening fraction and with clinical score. NT-proBNP is a good marker for persistent left ventricular dysfunction in children who have had myocarditis or cardiomyopathy. In this group of patients, NT-proBNP levels are normal in children who recover echocardiographically, suggesting no residual hemodynamic abnormalities.     

Myocardial performance index combining systolic and diastolic myocardial performance in doxorubicin-treated patients and its correlation to conventional echo/Doppler indices

This study was designed to evaluate the utility of myocardial performance index (MPI) in anthracycline cardiotoxicity. The MPI measures the ratio of total time spent in isovolumic activity (isovolumetric contraction time and isovolumetric relaxation time) to the ejection time, thus giving a global index combining systolic and diastolic myocardial performance. In this study, MPI was measured in 35 doxorubicin-treated children (aged 108.5+/-55.31 months, 23 males and 12 females) in sinus rhythm and 32 age-matched controls, and it was compared with conventional Doppler echocardiographic parameters. The isovolumetric contraction time was prolonged (38.37+/-24.43 vs 26.37+/-15.53, p <0.02) and ejection time was shortened (231.91 +/- 28.87 vs 256.21+/-19.55, p<0.001) in doxorubicin-treated patients compared to that in normal children. The isovolumetric relaxation time did not show significant difference between patients and control group (60.11+/-10.92 vs 61.06+/-12.12, p>0.05). MPI was significantly increased in doxorubicin-treated patients compared with that in control groups (0.42+/-0.07 vs 0.34+/-0.06, p<0.001), and significant correlation was observed between MPI and fractional shortening, ejection fraction, and left ventricular end diastolic and end systolic diameters (respectively, r = -0.508, p <0.002; r = -0.532, p<0.001; r = 0.467 p<0.005; r=0.606, p<0.001). Also, a weak correlation was found between MPI and duration of the disease and patient ages (r = 0.393, p < 0.02; r = 0.379; p < 0.02). However, there was no correlation between MPI and cumulative doxorubicin dose (r = 0.311, p > 0.05) and diastolic Doppler parameters in doxorubicin-treated patients. We think that MPI may be a useful parameter in monitoring left ventricular dysfunction in anthracyline-treated patients.     

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.     

Endothelial Dysfunction and Circadian Blood Pressure Rhythmicity in Young Heart Transplant Recipients

Blood pressure variability correlates with circadian rhythmicity in endothelium-derived nitric oxide (NO) production in adults. Young, hypertensive orthotopic heart transplant (OHT) patients have functional abnormalities in NO-dependent signaling pathways that lead to reduced NO bioavailability and endothelial dysfunction. Following acute intravenous infusion of L-arginine, the amino acid substrate for NO, OHT patients normalize blood pressure (BP) and endothelial function. However, the effects of chronic L-arginine infusion on circadian BP rhythmicity and endothelial function in OHT patients have not been described. Six OHT patients (9–29 years old), and seven healthy control subjects (19–28 years old) were admitted for 48 hours. Systolic, diastolic, and mean blood pressures (MBP) were recorded hourly. Urine samples were obtained to measure nitrates/nitrites (NO_X). Brachial artery flow-mediated vasodilatation (FMD; an index of endothelial function) and left ventricular ejection fraction (LVEF) were measured 0, 23, and 48 hours after admission. Intravenous L-arginine HC1 was infused continuously beginning 24 hours after admission in all subjects. The incidence (50%) and degree (12.0 ± 9.2%) of nocturnal MBP dipping was significantly less in OHT patients than control subjects. Furthermore, FMD was significantly reduced in OHT patients compared to controls (3.2 ± 1.1 vs 7.2 ± 3.1%, p = 0.01). L-Arginine infusion had no significant effect on 24-hour MBP, LVEF, or nocturnal dipping status in any subject; however, L-arginine normalized FMD in OHT patients (7.4 ± 1.8%). Circadian BP variability and endothelial function are impaired in young cardiac transplant patients with medically controlled hypertension, and L-arginine administration reverses endothelial dysfunction.     

Steroid Therapy and Cardiac Function in Duchenne Muscular Dystrophy

Duchenne muscular dystrophy leads to progressive deterioration in skeletal and cardiac muscle function. Steroids prolong ambulation and improve respiratory muscle strength. The authors hypothesized that steroid treatment would stabilize cardiac muscle function. Echocardiograms performed from 1997 to 2004 for 111 subjects 21 years of age or younger with Duchenne muscular dystrophy were restrospectively reviewed. The medical record was reviewed for steroid treatment. Untreated and steroids-treated subjects did not differ in age, height, weight, body mass index, systolic and diastolic blood pressure, or left ventricular mass. The shortening fraction was lower in the untreated group. Of those treated, 29 received prednisone and 19 received deflazacort. There was no difference in the shortening fraction between the two treated subgroups. Treated subjects not receiving steroids still had a normal shortening fraction, which was no different from the shortening fraction of those still receiving treatment. As compared with the treated subjects, the untreated subjects 10 years of age or younger were 4.4 times more likely to have a shortening fraction less than< 28% (p = 0.03), and the untreated subjects older than 10 years were 15.2 times more likely to have a shortening fraction less than< 28% (p < 0.01). This retrospective study suggests that the progressive decline in cardiac function of patients with Duchenne muscular dystrophy can be altered by steroid treatment. The effect appears to be sustained beyond the duration of treatment and independent of steroid type. This work is supported by a grant from the Parent Project Muscular Dystrophy.     

Left ventricular end-systolic wall stress to volume relationship before and after surgical closure of ventricular septal defect

Summary Left ventricular function was examined angiographically in 64 patients with ventricular septal defect and 13 postoperative patients with a preoperatively large shunt (postoperative group). The unoperated 64 patients were divided into three groups; small (left-to-right shunt ratio <35%), moderate (35%–50%), and large (>50%). The control group consisted of 27 patients with Kawasaki disease. For assessing left ventricular function, left ventricular shape and the end-systolic wall stress to end-systolic volume index ratio, as well as left ventricular ejection fraction were examined. Left ventricular ejection fraction was higher in the small-shunt group (p<0.05) than in the control group, but normal in the other groups. Left ventricular end-diastolic shape was normal only in the small-shunt group and more spherical in the other groups. The large-shunt group alone manifested more spherical left ventricular end-systolic shape and lower end-systolic wall stress to end-systolic volume index (p<0.001). These findings suggest that the left ventricular dysfunction is present in patients with a left-to-right shunt larger than 50%, but this change was reversible in patients who underwent early repair of ventricular septal defect.     

The Effect of Carvedilol Treatment on Chronic Heart Failure in Pediatric Patients With Dilated Cardiomyopathy: A Prospective, Randomized-Controlled Study

This study aimed to evaluate the clinical efficacy and safety of carvedilol for pediatric patients with chronic heart failure caused by dilated cardiomyopathy. Seven pediatric medical centers participated in this prospective study. Pediatric patients (n = 89) were randomly divided into an experimental group (carvedilol treatment) and a control group (conventional treatment). The analysis excluded 12 patients lost during the follow-up period. Carvedilol was added to the therapy of the experimental group after at least 1 month of basic treatment with digoxin, an angiotensin-converting-enzyme inhibitor, and diuretics. The control patients received the same basic treatment but did not receive carvedilol. The initial dose of carvedilol was 0.1 (mg/kg day), and the dose was doubled every 2 weeks until the maximum tolerated dose or 0.8 (mg/kg day) was achieved. The tolerated dose was maintained for 6 months. The Ross scales and echocardiographic parameters including left ventricular diastolic diameter, left ventricular systolic diameter, left ventricular ejection fraction, left ventricular fractional shortening, and serous brain natriuretic peptide (BNP) concentration, as well as clinical progress were compared between the two groups. The Ross scales decreased by 11.94 % in the experimental group, which was more than in the control group (2.81 %). In addition, changes to other echocardiographic parameters in the experimental group also were superior to those in the control group. The serous BNP concentration in the experimental group decreased by 30.1 %, which also was more than the decrease (22.2 %) observed in the control group. Clinical improvement was demonstrated by 40 %, no change by 35 %, and clinical deterioration by 25 % of the patients in the experimental group, and by respectively 37.8, 27, and 35.2 % of the patients in the control group. These differences were not statistically significant. Only one patient demonstrated a severe adverse event, severe pulmonary infection, and complete atrioventricular block. None of the other carvedilol-treated patients experienced drug-related side effects. Carvedilol can be safely used for treatment of chronic heart dysfunction in pediatric patients with dilated cardiomyopathy. The results in this study showed an apparent improvement in the cardiac function of these patients. Additional clinical studies are required to determine the most favorable dosing levels and regimens of carvedilol before its safety and efficacy for the pediatric population can be determined conclusively.     

Chronic hepatitis B infection: long term comparison of children receiving interferon alpha and untreated controls

OBJECTIVES: To investigate the virological outcome of chronic hepatitis B (CH-B) in children who received interferon alpha (IFN) compared with no treatment. METHODS: Seventy-four children with CH-B (median age, 6.1 years; 44 boys) selected from a cohort of 158 cases were included and divided into two groups: IFN-treated (n = 37) and control (n = 37). The controls were matched with the treated children by baseline alanine aminotransferase (ALT) levels, sex and age. The Kaplan-Meier method was performed to estimate the time to clearance of hepatitis B e antigen (HbeAg) and hepatitis B surface antigen (HbsAg). RESULTS: Mean duration of follow-up was comparable in two groups (5.2 +/- 3.8 years in treatment group versus 5.2 +/- 3.7 years in control group, NS). HBeAg and HBsAg loss occurred in 20 (54.1%) and three treated children versus 13 (35.1%) and one untreated children (NS), respectively. The 7-year cumulative HBeAg and HBsAg clearance rates were 47.5% and 8.9% after the first visit in the treatment group versus 33.5% and 4.0% in untreated children (NS), respectively. Elevated baseline ALT (two times upper limit of normal) had a significant effect on the long-term cumulative rate of HBeAg seroconversion in treated patients (P = 0.01) but not in the untreated group. CONCLUSIONS: These findings show that the overall long-term virological outcome does not differ significantly between IFN-treated and untreated children but that a significant benefit of treatment on the long term rate of HBeAg seroconversion is obtained in children with higher baseline ALT levels.     

Predictors of Left Ventricular Performance After Valve Replacement in Children and Adolescents with Chronic Aortic Regurgitation

Aortic valve replacement has been recommended in patients who have severe symptoms, in patients with extreme left ventricle (LV) dilatation (end diastolic dimension >4 SD above normal) or LV ejection fraction <50%. However, the occurrence of advanced symptoms or severe LV dilatation raises concern about irreversible LV dysfunction. This study sought to determine the influence of preoperative symptoms, LV size and function on mortality, and postoperative LV performance in children and adolescence after valve replacement for aortic regurgitation (AR). A total of 49 patients 18 years old or younger (mean, 13.9 ± 3) who underwent valve replacement for chronic AR between 1991 and 2001 were followed up for 1–10 years (mean, 3.3 ± 2.1). Baseline and postoperative characteristics were compared between 13 patients (group 1) with extreme LV dilatation and 34 patients (group 2) with a lesser degree of LV enlargement. Preoperative low ejection fraction (p < 0.008), extreme LV dilatation (p < 0.05), and LV end systolic dimension >4 SD above normal (p < 0.05) were predictors of persistent LV dysfunction. Extreme LV dilatation (p < 0.0003), LV end systolic dimension (p < 0.0007), and reduced LV ejection fraction (p < 0.01) predicted persistent LV dilatation. In the setting of chronic AR, preoperative symptoms, LV systolic function, and LV internal dimensions are the main predictors for persistent LV dysfunction and dilatation. Surgical correction should be performed before LV systolic dysfunction and/or extreme LV enlargement occurs.     

Two decades of acute hematogenous osteomyelitis in children: are there any changes?

Our purpose was to compare the clinical course of acute hematogenous osteomyelitis (AHO) 20 years ago and today in the Department of Paediatric Surgery, Kaunas Medical University Hospital, Lithuania. Retrospective data analysis from patients aged 1–16 years with confirmed diagnosis of AHO was performed. The data were collected from 1982 to 2003. The incidence of AHO per year for 1,00,000 children (0–16 years) was analyzed for that period. The patients were divided into two groups—group A, treated in 1982–1983, and group B, treated in 2002–2003. The number of patients, patient age, duration of illness, complications, and length of hospital stay were compared using statistical methods for nonparametric data analysis (Mann–Whitney U test, chi-square criterion). Linear regression was used for incidence analysis. Population data were obtained from the Lithuanian Statistics Department. From 1982–2003, 758 patients were treated. The incidence of AHO increased from 1982. There was no statistically significant difference between the periods 1982–1983 and 2002–2003 in median patient age (10.36 and 10.72 years, respectively), in gender proportion (20.4% and 29.8% of the cases were girls), or in median duration of symptoms until admission (4 days and 3 days, respectively, p=0.058). Median hospital stay and duration of antibiotic therapy were longer in the period 1982–1983 (50 days and 43 days) than during 2002–2003 (29 days and 29 days). The differences in frequency of positive blood cultures (36.4% in group A and 64.9% in group B, p=0.046) and frequency of periosteal abscess (40.8% in group A and 19.3% in group B, p=0.015) were statistically significant. An increase in AHO incidence is seen when comparing contemporary data and the data from two decades ago, but nowadays the clinical course is less complicated and is marked by shorter hospital stays and shorter duration of antibiotic therapy.     

Morbidities in Patients with Hypoplastic Left Heart Syndrome

We sought to document morbidities and growth for patients with hypoplastic left heart syndrome (HLHS) to inform the initial surgical decision and understand healthcare needs. Data were obtained on 137 patients with HLHS, born between 1989 and 1994, who survived staged surgery (n = 62) or transplantation (n = 75) and had follow-up information available from four pediatric cardiac surgical centers. In patients with HLHS older than 1 year of age at follow-up, 93% experienced at least one major postsurgical morbidity. Morbidities depended on the surgery received. Hypertension, renal compromise, and abnormal infections were more common in transplanted patients than staged surgery patients. Staged surgery patients used more anticongestive medications and experienced more morbidities requiring interventional catheterization than did transplanted patients. Rejection was common for transplanted patients. On average these children spent 23 days per year in the hospital. Patients with HLHS were small for their age; 43% of staged surgery patients weighed below the third percentile at last information, compared to 19% of transplanted patients (p = 0.003). The median height percentile was the 10th in both groups. Normal activity level was reported in more transplanted patients (90%) than staged surgery patients (49%; p < 0.001). Trade-offs between mortality and morbidity outcomes can help inform the initial surgical decision.