Eisenmenger Syndrome in Adults: Treatment Pattern and Prognostic Factors in the Advanced Pulmonary Vasodilator Era.

Patients with Eisenmenger syndrome (ES) have a higher mortality rate than patients with simple congenital heart disease (CHD). To determine factors associated with death in the era of advanced pulmonary vasodilator treatment, we analyzed the characteristics of adult ES patients depending on underlying CHD. Simple septal defects and patent ductus arteriosus were classified as simple CHD, and other conditions were classified as complex CHD. Sixty-seven adult ES patients (50.7% women) were reviewed retrospectively. CHD was diagnosed at a median of 10.0 years of age and ES was diagnosed at 18.6 years. Thirteen patients (19.4%) died; the median age was 38.6 years (IQR 32.2-47.8). In a multivariate analysis, patients with SpO2 < 85% had a higher mortality rate than others [hazard ratio (HR) 9.7; 95% confidence interval (CI) 1.002-95.2, p = 0.05]. In simple CHD patients, those with a low platelet count (< 100 × 109/L) or low SpO2 (< 85%) were at a higher risk of death than those without (HR 16.32, 95% CI 1.25-2266.31, p = 0.032; and HR 38.91, 95% CI 3.44-5219.41, p = 0.001, respectively). Advanced pulmonary vasodilators were used more in survivors than in non-survivors (48.1% vs. 15.4%, p = 0.032). Low SpO2 and platelet count were related to mortality in adult ES, especially in those with simple CHD. Therefore, careful attention should be paid to the care of adult ES patients with this tendency; active pulmonary vasodilator treatment should be considered.

Congenital heart disease at Laos Children's Hospital: Two year experience.

BACKGROUND: Management of congenital heart disease (CHD) in developing countries is challenging because of limited access to health-care facilities and socioeconomic limitations. The aim of this study was to describe the recent experience with CHD at Children's Hospital, Vientiane, Laos, the only pediatric referral hospital in the country. METHODS: From July 2013 to November 2015, 1009 echocardiograms were carried out in 797 individuals who visited Children's Hospital, in whom CHD was identified in 213. Demographic characteristics, echocardiography and age at first CHD diagnosis on echocardiogram were compared by residential area. RESULTS: Among the 213 patients, the most frequent anomalies, in descending order, were ventricular septal defect, atrial septal defect, patent ductus arteriosus, and tetralogy of Fallot. Moderate or severe CHD requiring surgery or intervention was detected in 137 patients; median age at initial diagnosis was 6.0 months (IQR, 1.5-29.8). Among those with moderate or severe CHD, 89 patients were from rural areas and 48 from the capital area. The proportion of patients from rural areas older than 1 and 3 years at diagnosis was 46.1% and 32.6%, respectively. In contrast, patients from the capital area were diagnosed at a significantly earlier age: 16.7% at age >1 year and 6.2% at age >3 years (both P < 0.001 vs rural areas). CONCLUSIONS: Diagnosis of CHD requiring surgery is delayed in Laos, especially in rural areas. Better education and training of local medical staff, and greater access to medical advice are required to improve CHD care in Laos.

Impaired Vascular Function of the Aorta in Adolescents with Turner Syndrome.

To investigate intrinsic vascular abnormalities of the ascending aorta before adulthood in Turner syndrome patients (TS), we compared 25 adolescent TS patients (mean age 14.6 ± 3.4 years) and 16 healthy controls from a university hospital. Blood pressure and other vascular indices were evaluated using echocardiography such as aortic strain, stiffness index, distensibility, and pulse wave velocity. Aortic strain (0.1237 vs. 0.1865, respectively; P = 0.003) and aortic distensibility (0.0049 vs. 0.0081, P = 0.002) were significantly lower in the TS group than in the controls. The fractional area change on velocity vector imaging was also lower in TS patients (29.05 vs. 36.19, P = 0.002). These findings were still observed after adjustment for age, body mass index, and systolic blood pressure. The aortic stiffness index was greater in the TS patients than in the control group (6.79 vs. 3.34, P = 0.02). The pulse wave velocity and ascending aorta diameter did not significantly differ between the TS and control groups. Blood pressures were significantly higher in the TS patients than in the controls (systolic, 120.4 vs. 108.4 mmHg, P = 0.001; diastolic 71.5 vs. 61.7 mmHg, P < 0.001). Although the dimensional changes in the aorta were not clearly observed in adolescents with TS, the elastic properties of the aorta were significantly decreased in TS patients as compared to control subjects.

Radiofrequency catheter ablation for supraventricular tachycardia: a comparison study of children aged 0-4 and 5-9 years.

BACKGROUND: The safety and effectiveness of radiofrequency catheter ablation (RFCA) for supraventricular tachycardia (SVT) in young children was investigated. METHODS: Ninety-five children who underwent RFCA procedures were stratified according to age (group 1, 0-4 years, n = 24; group 2, 5-9 years, n = 71) and were evaluated retrospectively. RESULTS: Among the 95 patients, atrioventricular reentrant tachycardia was 78.9%, atrioventricular nodal reentrant tachycardia was 10.5%, and ectopic atrial tachycardia was 8.4%. The acute success rate of RFCA was 97.9% and the recurrence rate was 11.6%. RFCA was performed for different main reasons in each group, including drug-resistant tachycardia (37.5% in group 1 vs 7% in group 2; P = 0.001) and symptomatic tachycardia (4.2% in group 1 vs 57.7% in group 2; P < 0.001). There was no significant difference in success rate, recurrence rate, and procedure and fluoroscopy duration between the two groups. The acute success rates did not differ significantly between patients with a single accessory pathway (AP) and those with multiple APs; however, failure or recurrence was more common in the patients with multiple APs (38.5% vs 11.5%; P = 0.01). The multiple APs were found frequently on the right side (P = 0.005). Septal dyskinesia with left ventricular dysfunction in Wolff-Parkinson-White syndrome and tachycardia-induced cardiomyopathy improved after RFCA. CONCLUSIONS: RFCA was found to be effective and safe for SVT in young children.

A novel mutation of the TAZ gene in Barth syndrome: acute exacerbation after contrast-dye injection.

A 14-month-old boy was transferred because of dilated and hypertrophied left ventricle, neutropenia, and developmental delay. After checking computed tomographic angiography with contrast-dye, the patient showed acute exacerbation and finally died from multi-organ failure despite intensive cares. From genetic analysis, we revealed that the patient had Barth syndrome and found a novel hemizygous frame shift mutation in his TAZ gene, c.227delC (p.Pro76LeufsX7), which was inherited from his mother. Herein, we report a patient with Barth syndrome who had a novel mutation in TAZ gene and experienced unexpected acute exacerbation after contrast dye injection for computed tomographic angiography.

Sudden cardiac arrest during anesthesia in a 30-month-old boy with syndactyly: a case of genetically proven Timothy syndrome.

Timothy syndrome, long QT syndrome type 8, is highly malignant with ventricular tachyarrhythmia. A 30-month-old boy had sudden cardiac arrest during anesthesia induction before plastic surgery for bilateral cutaneous syndactyly. After successful resuscitation, prolonged QT interval (QTc, 0.58-0.60 sec) and T-wave alternans were found in his electrocardiogram. Starting ß-blocker to prevent further tachycardia and collapse event, then there were no more arrhythmic events. The genes KCNQ1, KCNH2, KCNE1 and 2, and SCN5A were negative for long QT syndrome. The mutation p.Gly406Arg was confirmed in CACNA1C, which maintains L-type calcium channel depolarization in the heart and other systems.

Long QT syndrome: a Korean single center study.

The long QT syndrome (LQTS) is a rare hereditary disorder in which affected individuals have a possibility of ventricular tachyarrhythmia and sudden cardiac death. We investigated 62 LQTS (QTc ≥ 0.47 sec) and 19 family members whose genetic study revealed mutation of LQT gene. In the proband group, the modes of presentation were ECG abnormality (38.7%), aborted cardiac arrest (24.2%), and syncope or seizure (19.4%). Median age of initial symptom development was 10.5 yr. Genetic studies were performed in 61; and mutations were found in 40 cases (KCNQ1 in 19, KCNH2 in 10, SCN5A in 7, KCNJ2 in 3, and CACNA1C in 1). In the family group, the penetrance of LQT gene mutation was 57.9%. QTc was longer as patients had the history of syncope (P = 0.001), ventricular tachycardia (P = 0.017) and aborted arrest (P = 0.010). QTc longer than 0.508 sec could be a cut-off value for major cardiac events (sensitivity 0.806, specificity 0.600). Beta-blocker was frequently applied for treatment and had significant effects on reducing QTc (P = 0.007). Implantable cardioverter defibrillators were applied in 6 patients. Congenital LQTS is a potentially lethal disease. It shows various genetic mutations with low penetrance in Korean patients.

Impact of Pulmonary Arterial Elastance on Right Ventricular Mechanics and Exercise Capacity in Repaired Tetralogy of Fallot.

BACKGROUND AND OBJECTIVES: Pathophysiological changes of right ventricle (RV) after repair of tetralogy of Fallot (TOF) are coupled with a highly compliant low-pressure pulmonary artery (PA) system. This study aimed to determine whether pulmonary vascular function was associated with RV parameters and exercise capacity, and its impact on RV remodeling after pulmonary valve replacement. METHODS: In a total of 48 patients over 18 years of age with repaired TOF, pulmonary arterial elastance (Ea), RV volume data, and RV-PA coupling ratio were calculated and analyzed in relation to exercise capacity. RESULTS: Patients with a low Ea showed a more severe pulmonary regurgitation volume index, greater RV end-diastolic volume index, and greater effective RV stroke volume (p=0.039, p=0.013, and p=0.011, respectively). Patients with a high Ea had lower exercise capacity than those with a low Ea (peak oxygen consumption [peak VO2] rate: 25.8±7.7 vs. 34.3±5.5 mL/kg/min, respectively, p=0.003), while peak VO2 was inversely correlated with Ea and mean PA pressure (p=0.004 and p=0.004, respectively). In the univariate analysis, a higher preoperative RV end-diastolic volume index and RV end-systolic volume index, left ventricular end-systolic volume index, and higher RV-PA coupling ratio were risk factors for suboptimal outcomes. Preoperative RV volume and RV-PA coupling ratio reflecting the adaptive PA system response are important factors in optimal postoperative results. CONCLUSIONS: We found that PA vascular dysfunction, presenting as elevated Ea in TOF, may contribute to exercise intolerance. However, Ea was inversely correlated with pulmonary regurgitation (PR) severity, which may prevent PR, RV dilatation, and left ventricular dilatation in the absence of significant pulmonary stenosis.

Long QT syndrome and dilated cardiomyopathy with SCN5A p.R1193Q polymorphism: cardioverter-defibrillator implantation at 27 months.

Cardiac sodium channel dysfunction associated with the SCN5A gene presents with mixed phenotypes, including long QT syndrome type 3, sinus node dysfunction, and dilated cardiomyopathy (DCM). We report a Korean case of an overlap syndrome of cardiac sodium channelopathy with SCN5A p.R1193Q polymorphism, treated by the placement of an intrapericardial implantable cardioverter-defibrillator (ICD) at the age of 27 months. Although the patient received two appropriate life-saving shocks for ventricular fibrillations, he eventually died of DCM progression. However, this case shows that intrapericardial ICD implantation is feasible in young children with a high risk for sudden cardiac death.

Clinical significance of thrombosis in an intracardiac blind pouch after a Fontan operation.

The univentricular heart after the Fontan operation may have a blind pouch formed by the pulmonary stump or rudimentary ventricle according to the anatomy before surgery. Thrombosis in an intracardiac blind pouch of patients with a univentricular heart is a hazardous complication. Because only a few reports have described this complication, the authors evaluated the clinical significance of thrombosis in an intracardiac blind pouch of a univentricular heart. They performed a retrospective review of medical records from August 1986 to December 2007. Four patients were confirmed as having thrombosis in a pulmonary artery stump and one patient as having thrombosis in a rudimentary ventricle shown by cardiac computed tomography (CT). This represents 1.85% (5/271) of patients with ongoing regular follow-up evaluation after the Fontan operation. The median age at diagnosis was 14.2 years. Two of the five patients were taking aspirin and one patient was taking warfarin when they were identified for the development of thrombosis. None of the patients demonstrated thrombosis in the Fontan tract or venous side of the circulation. Brain magnetic resonance imaging (MRI) showed that three patients had cerebral infarction and one patient had suggestive old ischemia. Three patients with thrombus in the pulmonary stump underwent pulmonary artery stump thrombectomy and pulmonary valve obliteration. One patient with thrombus in the rudimentary ventricle underwent ventricular septal defect (VSD) closure with thrombectomy. Thrombus in a blind pouch could cause systemic thromboembolism despite little blood communication. Therefore, surgical modification of the pulmonary stump and VSD closure of the rudimentary ventricle are required to reduce the risk of later thrombus formation. Clinicians should not overlook the possibility of thrombus in a ligated pulmonary artery stump or a rudimentary ventricle after the Fontan operation, which may increase the risk of embolic stroke for patients with single-ventricle physiology.

Idiopathic cardiomyopathies in Korean children. - 9-Year Korean Multicenter Study-.

BACKGROUND: Idiopathic cardiomyopathies (CMPs) are an important heterogeneous group of diseases. With the advance of therapeutic strategies, epidemiologic data on CMP have become very important, but only a few have been reported in Asian children. We conducted a retrospective epidemiologic study of primary CMP in Korean children. METHODS AND RESULTS: Using a multicenter survey, we studied primary CMP among Korean children from January 1998 to December 2006 based on classification (2006) of CMP by the American Heart Association. A total of 277 primary CMP patients were reported from 17 cardiovascular centers. The average annual occurrence of new cases of primary CMP was 0.28 per 100,000 Korean children younger than 15 years of age (95% confidence interval (CI) 0.24-0.31). Dilated CMP (DCMP) was 66.43%, hypertrophic CMP (HCMP) 23.47%, restrictive CMP (RCMP) 6.50% and others 3.61%. The point prevalence of primary CMP at the end of the study was estimated as 2.11/100,000 (95%CI 1.83-2.43), DCMP 1.39/100,000, HCMP 0.51/100,000, RCMP 0.16/100,000 and others 0.04/100,000. Survival rates over 9 years were 69.8% in DCMP, 90.3% in HCMP, and 47.2% in RCMP. CONCLUSIONS: Recent point prevalence of childhood primary CMP in Korea was estimated as 2.11/100,000. Further epidemiologic study with a nationwide survey is necessary.

Significance of circulating hepatocyte growth factor in protein-losing enteropathy after Fontan operation.

The purpose of this study was to measure serum hepatocyte growth factor (HGF) and elucidate the relationship between HGF and protein-losing enteropathy (PLE) after Fontan operation (FO). Ten patients with PLE (mean age 15.7 ± 8.7 years) who underwent FO were enrolled. Control group 1 comprised 20 patients without PLE after FO, and control group 2 comprised 10 patients with nephrotic syndrome (NS). Serum HGF, vascular endothelial growth factor, albumin, and random stool alpha-1 antitrypsin concentration were measured. Transthoracic echocardiography was completed. Serum HGF level was significantly greater in the PLE patients (0.61 ± 0.27 ng/ml) after FO than in the two control groups (0.41 ± 0.12 ng/ml [P = 0.024] for the Fontan group without PLE and 0.26 ± 0.12 ng/ml [P = 0.002] for the patients with NS). Serum albumin of patients with PLE (2.82 ± 0.96 g/dl) showed significantly lower levels compared with those of patients without PLE after FO (4.30 ± 0.37 g/dl, P < 0.001) but significantly greater levels compared with patients with NS (1.91 ± 0.33 g/dl, P = 0.019). Patients with greater serum HGF levels showed significant correlation with lower serum albumin level (P = 0.006, r = -0.495). Because serum HGF levels were significantly greater in patients with PLE after FO, HGF may play a role in the development of PLE after FO.

Pregnancy outcomes in patients with structural heart disease: a single center experience.

BACKGROUND: In women, pregnancy is a period of relatively drastic hemodynamic change in a short period of time. Most pregnant women adapt well to these gradual hemodynamic changes. However, in women with congenital heart disease or other structural heart disease, adaptation to theses sudden hemodynamic changes is difficult, and heart failure or arrhythmia can get aggravated. This study shares our experiences on the outcomes of pregnancy in patients with structural heart disease. METHODS: From January 2007 to December 2016, we reviewed the medical records of all pregnant women with structural heart disease who received obstetric care at the Sejong General Hospital. RESULTS: During the study period, 103 pregnancies were observed in 79 women with structural heart disease. Of the 103 pregnancies, 55 were primiparous and 48 were multiparous. Echocardiography performed before pregnancy revealed that 52 patients had moderate to severe valvular regurgitation and 38 patients had moderate to severe valvular stenosis; 22 patients had mechanical valves and 5 patients had pulmonary hypertension. Overall, there were 9 maternal cardiac events, 7 obstetric events and 19 neonatal events. Pulmonary embolic events occurred only in 1 case; 77 deliveries were made, and 26 pregnancies did not last. Among 77 deliveries, 55 patients delivered by cesarean section (C/S) (71.43%). C/S in 16 of 55 patients was performed due to the maternal hemodynamic risk. CONCLUSIONS: Overall complications associated with pregnancy in women with structural heart disease were very high at 28.16%. However, it is hoped that maternal and neonatal outcomes will be improved through careful observation and preparedness for anticipated complications.

L-type Ca(2+) channel facilitation mediated by H(2)O(2)-induced activation of CaMKII in rat ventricular myocytes.

The Ca(2+)-dependent facilitation (CDF) of L-type Ca(2+) channels, a major mechanism for force-frequency relationship of cardiac contraction, is mediated by Ca(2+)/CaM-dependent kinase II (CaMKII). Recently, CaMKII was shown to be activated by methionine oxidation. We investigated whether oxidation-dependent CaMKII activation is involved in the regulation of L-type Ca(2+) currents (I(Ca,L)) by H(2)O(2) and whether Ca(2+) is required in this process. Using patch clamp, I(Ca)(,L) was measured in rat ventricular myocytes. H(2)O(2) induced an increase in I(Ca,L) amplitude and slowed inactivation of I(Ca)(,L). This oxidation-dependent facilitation (ODF) of I(Ca)(,L) was abolished by a CaMKII blocker KN-93, but not by its inactive analog KN-92, indicating that CaMKII is involved in ODF. ODF was not affected by replacement of external Ca(2+) with Ba(2+) or presence of EGTA in the internal solutions. However, ODF was abolished by adding BAPTA to the internal solution or by depleting sarcoplasmic reticulum (SR) Ca(2+) stores using caffeine and thapsigargin. Alkaline phosphatase, beta-iminoadenosine 5'-triphosphate (AMP-PNP), an autophosphorylation inhibitor autocamtide-2-related inhibitory peptide (AIP), or a catalytic domain blocker (CaM-KIINtide) did not affect ODF. In conclusion, oxidation-dependent facilitation of L-type Ca(2+) channels is mediated by oxidation-dependent CaMKII activation, in which local Ca(2+) increases induced by SR Ca(2+) release is required.

Septal dyskinesia and global left ventricular dysfunction in pediatric Wolff-Parkinson-White syndrome with septal accessory pathway.

INTRODUCTION: Echocardiographic studies have shown that some patients with Wolff-Parkinson-White (WPW) syndrome have myocardial dyskinesia in the segments precociously activated by an accessory pathway (AP). The aim of the present study was to determine the extent to which the AP contributes to global left ventricular (LV) dysfunction. METHODS: Electrophysiological and echocardiographic data from 62 children with WPW (age at diagnosis = 5.9 +/- 4.2 years) were retrospectively analyzed. RESULTS: The left ventricular ejection fraction (LVEF) of patients with septal APs (53 +/- 11%) was significantly lower than that of patients with right (62 +/- 5%) or left (61 +/- 4%) APs (P = 0.001). Compared to patients with normal septal motion (n = 56), patients with septal dyskinesia (n = 6) had a reduced LVEF (61 +/- 4% and 42 +/- 5%, respectively) and an increased LV end diastolic dimension (P < 0.001 for both comparisons). Multivariate analysis identified septal dyskinesia as the only significant risk factor for reduced LVEF. All 6 patients with septal dyskinesia had right septal APs, and a preexcited QRS duration that was longer than that of patients with normal septal motion (140 +/- 18 ms and 113 +/- 32 ms, respectively; P = 0.045). After RFA there were improvements in both intraventricular dyssynchrony (septal-to-posterior wall motion delay, from 154 +/- 91 ms to 33 +/- 17 ms) and interventricular septal thinning (from 3.0 +/- 0.5 mm to 5.3 +/- 2.6 mm), and a significant increase in LVEF (from 42 +/- 5% to 67 +/- 8%; P = 0.001). CONCLUSION: The dyskinetic segment activated by a right septal AP in WPW syndrome may lead to ventricular dilation and dysfunction. RFA produced mechanical resynchronization, reverse remodeling, and improvements in LV function.

Clinical spectrum and prognostic factors of pediatric ventricular tachycardia.

BACKGROUND: There have been no studies on the clinical characteristics and prognostic factors of pediatric ventricular tachycardia (VT). METHODS AND RESULTS: Eighty-one patients with pediatric VT were studied retrospectively at a single center. The median follow-up period was 6.0 years (0.7-23.5 years). Patients were categorized into 6 groups: idiopathic VT (IVT, n=37), catecholaminergic polymorphic VT (CPVT, n=10), congenital heart disease-associated VT (n=15), myocarditis-associated VT (n=8), cardiomyopathy-associated VT (CMP-VT, n=5) and miscellaneous. The age distribution of VT had 2 peaks (infant and teenager). VT with left bundle branch block configuration was more frequently nonsustained than VT with right bundle branch block configuration (61% vs 8%). Although 22% were asymptomatic, 38% experienced syncope or seizure and 16% had cardiac arrest. The overall mortality rate was 7.4%. The expected life span without cardiac arrest was <4 years in the CMP-VT group and the 10-year survival rate in CPVT patients was approximately 55%. Onset at infancy, monomorphic type and transcatheter/surgical ablation were related to the successful resolution of VT. Logistic regression analysis revealed that CPVT, CMP-VT, polymorphic VT and sustained VT were significantly correlated with death or cardiac arrest. CONCLUSIONS: The clinical features and prognosis of pediatric VT differed with the VT type, clinical categories and onset age. Accurate diagnosis and proper treatment according to the clinical categories may improve the outcome.

Balloon valvuloplasty for neonatal critical pulmonary valvar stenosis with IVC interruption: pitfalls of the transumbilical approach.

Transcatheter balloon pulmonary valvuloplasty (BPV) is considered to be the treatment of choice for neonates with critical pulmonary valvar stenosis (PVS) or pulmonary valvar atresia with intact ventricular septum accompanied by reasonable right ventricular volume. The percutaneous femoral venous access is the most preferred route for BPV in most cardiac centers. We report herein the case of a newborn baby with critical PVS with inferior vena cava interruption, severe tricuspid regurgitation and a severely enlarged right atrium. We tried BPV through the transumbilical approach with difficulty, but he was successfully treated with the assistance of a coronary artery guiding catheter.

Cardiac resynchronization therapy for left ventricular dysfunction induced by chronic right ventricular pacing in a child.

Cardiac resynchronization therapy (CRT) has been proven its value in adult patients with congestive heart failure of low ejection fraction and wide QRS duration. Contrast to adult patients, CRT has been rarely applied for young patients. We report on a 9-yr-old boy with progressive left ventricular (LV) dilatation and dysfunction following chronic VVI pacemaker therapy for congenital complete atrioventricular block associated with maternal anti-SSA/Ro and SSB/La antibody. His LV dysfunction was improved after epicardially established CRT.

Jervell and Lange-Nielsen syndrome: novel compound heterozygous mutations in the KCNQ1 in a Korean family.

The Jervell and Lange-Nielsen syndrome (JLNS) is an autosomal recessive syndrome characterized by congenital deafness and cardiac phenotype (QT prolongation, ventricular arrhythmias, and sudden death). JLNS has been shown to occur due to homozygous mutation in KCNQ1 or KCNE1. There have been a few clinical case reports on JLNS in Korea; however, these were not confirmed by a genetic study. We identified compound heterozygous mutations in KCNQ1 in a 5-yr-old child with JLNS, who visited the hospital due to recurrent syncope and seizures and had congenital sensorineural deafness. His electrocardiogram revealed a markedly prolonged corrected QT interval with T wave alternans. The sequence analysis of the proband revealed the presence of novel compound heterozygous deletion/splicing error mutations (c.828-830 delCTC, p.S277del/c.921G>A, p.V307V). Each mutation in KCNQ1 was identified on the maternal and paternal side. With ß-blocker therapy the patient has remained symptom-free for three and a half years.

Dexrazoxane for preventing anthracycline cardiotoxicity in children with solid tumors.

This study attempted to assess the incidence and outcome of anthracycline cardiotoxicity and the role of dexrazoxane as a cardioprotectant in childhood solid tumors. The dexrazoxane group included 47 patients and the control group of historical cohort included 42. Dexrazoxane was given in the 10:1 ratio to doxorubicin. Fractional shortening and systolic and diastolic left ventricular diameters were used to assess the cardiac function. The median follow-ups were 54 months in the dexrazoxane group and 86 months in the control group. The mean cumulative doses of doxorubicin were 280.8+/-83.4 mg/m(2) in the dexrazoxane group and 266.1+/-75.0 mg/m(2) in the control group. The dexrazoxane group experienced significantly fewer cardiac events (27.7% vs. 52.4%) and less severe congestive heart failure (6.4% vs. 14.3%) than the control group. Thirteen cardiotoxicities including one cardiac death and 2 congestive heart failures occurred in the dexrazoxane group, and 22 cardiotoxicities including 2 cardiac deaths and 4 congestive heart failures, in the control group. Five year cardiac event free survival rates were 69.2% in the dexrazoxane group and 45.8% in the control group (P=0.04). Dexrazoxane reduces the incidence and severity of early and late anthracycline cardiotoxicity in childhood solid tumors.