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At least 1,171 records · Page 65Linked to original sources
PubMed1993

Delayed onset of alcuronium effect in children with cyanotic congenital heart disease.

The effect of alcuronium dichloride (0.3 mg/kg) was studied in seven normal children (Group A), nine with acyanotic congenital heart disease (Group B) and eight with cyanotic disease (Group C). The onset of action was recorded using an integrated electromyograph and blood samples taken for later estimation of plasma concentrations of the drug. The mean time (SD) taken to 75% suppression of twitch height was 1.3(0.8), 1.7(1.0) and 3.8(2.8) minutes, respectively, in each of the three groups. This was significantly slower in Group C compared with both other groups (P < 0.05). While six of the Group A patients and seven from Group B reached 95% paralysis within ten minutes, only two of the cyanosed children achieved this level of relaxation. However, if times to 95% relaxation were extrapolated, there was no significant difference between the groups at 4.5(3.9), 5.8(5.7) and 10.9(6.5) minutes respectively. There was a weak but statistically significant relationship between haematocrit and time to 75% blockade. Maximum twitch depression was similar in all three groups with plasma concentrations at this time being 1.6(0.7), 1.8(0.5) and 2.3(1.4) micrograms/ml respectively. Again, there was no statistically significant difference between these values. These results confirm that the clinical onset of relaxation is delayed in children with cyanotic congenital heart disease, possibly because of delayed distribution of alcuronium.

Alcuronium↗
PubMed1992

Discordance between abdominal and atrial arrangement in a case of complex congenital heart disease.

The report describes a rare case of a complex congenital heart disease wherein the splenic status and the relationship of the suprarenal abdominal great vessels strongly suggested right isomerism, but atrial morphology demonstrated usual atrial arrangement. It emphasises the increasing recognition that the arrangement of the abdominal organs need not accurately reflect atrial arrangement.

Abnormalities, Multiple↗
PubMed2006

Neurologic events in neonates treated surgically for congenital heart disease.

OBJECTIVE: The incidence of acute neurologic events prior to discharge in neonates with congenital heart disease (CHD) was determined and peri-operative characteristics predictive of a neurologic event were identified. STUDY DESIGN: A retrospective chart review over 1 year was conducted of infants <1 month of age with a diagnosis of CHD. Outcomes were measured by the occurrence of an acute neurologic event defined as electroencephalogram (EEG)-proven seizure activity, significant hypertonia or hypotonia, or choreoathetosis prior to hospital discharge. Stepwise logistic regression identified variables most likely to be associated with an acute neurologic event. RESULTS: Surgical intervention occurred in 95 infants who were admitted with a diagnosis of CHD. The survival rate was 92%. Of the survivors, 16 (17%) had an acute neurologic event, with 19% of events occurring preoperatively. Factors associated with neurologic events included an elevated nucleated red blood cell (NRBC) count, an abnormal preoperative brain imaging study, and a 5-min Apgar score <7 (P<0.05). CONCLUSIONS: Neonates with CHD have a significant risk of neurologic events. Preoperative brain imaging, the 5-min Apgar score, and initial serum NRBC counts may identify infants at highest risk for central nervous system injury.

Acute Disease↗
PubMed2003

Present scenario of device closure for congenital heart diseases.

There has been tremendous advances in the treatment of congenital heart diseases following the development of transcatheter closure devices in the last decade. Device-closures of patent ductus arteriosus, atrial septal defect and ventricular septal defects, as in use today, have been highlighted in this article along with a brief discussion in the subject.

Cardiovascular Surgical Procedures↗
PubMed1999

Paired ventricular pacing: an alternative therapy for postoperative junctional ectopic tachycardia in congenital heart disease.

Junctional ectopic tachycardia (JET) is one of the most life-threatening postoperative arrhythmias in children with congenital heart disease, and medical management is difficult. Paired ventricular pacing (PVP) may provide a safe alternative mode of management. We evaluated the safety and efficacy of PVP for the management of postoperative JET in patients with congenital heart disease. A retrospective collection of data was done from 1981-1995. PVP was successfully tried in five postoperative patients (age range: 37 days to 22 years, median: 10 months). Onset of JET was 3-60 hours (mean +/- SD, 19 +/- 23 hours) postoperatively. The maximal JET rate was 261 +/- 39 beats/min. PVP was used as the first line of management in three patients and was successful in all patients. It resulted in an instantaneous increase in blood pressure from 66 +/- 9 to 94 +/- 15 mmHg (42% increase) and was required for 12 +/- 14 hours (range 2-36 hours). No complications were noted. Therefore, in our experience, this is a safe alternative modality for the control of postoperative JET.

Adult↗
PubMed2004

Satisfaction with care: a study of parents of children with congenital heart disease and parents of children with other diseases.

AIM: We compared parents of children with congenital heart disease (PCCHD, n=1092) with parents of children with other diseases (PCOD, n=112) regarding satisfaction with their children's care (SCC). We also examined the association between parental/patient characteristics and SCC. METHOD: The parents completed a questionnaire about such areas as satisfaction with care, children's health status, and financial situation. The design was cross-sectional and data were gathered over 20 consecutive days. RESULTS: The univariate and multivariate analyses showed that PCCHD were more satisfied with their children's medical care and waiting period for treatment of their ill children than PCOD, although the difference was only modest. Furthermore, mothers were less satisfied with staff attitudes than fathers, with the lowest satisfaction among mothers of children with CHD. However, the multivariate analysis indicated that less satisfaction with care was more associated with decreasing child age, unemployment, financial burden of disease, social isolation and psychological distress than with children's diseases, their severity and parental gender. CONCLUSION: We corroborated some previous findings and may have provided new insights regarding determinants of SCC among parents. Interventions to improve SCC may need to address issues of parental psychological distress, socialization, and financial burden of illness. Possible ways of achieving this are discussed. Finally, research in a longitudinal format is needed to further scrutinize determinants of parental SCC.

Adult↗
PubMed1980

Congenital hepatic fibrosis with congenital heart disease. A family study with ultrastructural features of the liver.

A family with congenital hepatic fibrosis (CHF) and congenital heart disease (CHD) is presented. The consanguineous healthy parents gave birth to 12 children of whom 10 survived. One son had CHF and CHD, one daughter had CHF and a second daughter had CHD. Three other siblings probably had small a ventricular septal defect and another one probably had mild pulmonary valve stenosis. Development of portal hypertension and hypersplenism necessitated performing shunt operation on both siblings suffering from congenital hepatic fibrosis. Ultrastructural findings were giant mitochondria with large laminar inclusions in hepatocytes, and excess of villi and whorls of membranes and collagen fibrils between hepatocytes.

Adolescent↗
PubMed1991

Influence of colour Doppler echocardiography on the ultrasonic assessment of congenital heart disease: a prospective study.

OBJECTIVE: To evaluate the additional information provided by colour Doppler in the ultrasonic assessment of congenital heart disease. PATIENTS AND METHODS: A prospective study of 215 children (age range 1 day-16 years) presenting with clinical signs of congenital heart disease. RESULTS: Colour Doppler was essential for the diagnosis of an anomalous left coronary artery and altered the management of a patient initially diagnosed as having cardiomyopathy. Colour Doppler provided extra information, but without major impact on management, in the following: the diagnosis of ventricular septal defects associated with other defects, of multiple ventricular septal defects, of anomalous pulmonary venous drainage, and of mild mitral regurgitation; the demonstration of site of coarctation, of stenotic or hypoplastic pulmonary artery branches, of unobstructed flow through a right atrial membrane, and of left ventricle to right atrium regurgitation; the assessment of the width of the duct and of flow through the patent foramen ovale in transposition and tricuspid atresia; the differentiation of pulmonary atresia from critical pulmonary stenosis and the measurement of maximum velocity of tricuspid regurgitation. CONCLUSIONS: Ideally all patients should undergo colour Doppler studies before cardiac surgery to ensure a more accurate diagnosis. However, since the additional information provided does not affect the management in most patients, machines without colour Doppler can provide a satisfactory service in paediatric cardiology centres in countries where resources are limited.

Abnormalities, Multiple↗
PubMedDate not supplied

Plasma nitric oxide products correlate with cardiac index of congenital heart disease.

We wished to determine the relationship between circulating levels of nitric oxide (NO) and cardiac index (CI) in children with congenital heart diseases. We measured the plasma levels of nitrate/nitrite (NO(x)), the stable end products of NO production as well as tumor necrosis factor-alpha (TNF-alpha), atrial natriuretic peptide (ANP), and brain natriuretic peptide in relation to various parameters determined simultaneously. The plasma NO(x) levels correlated negatively with CI (r = -0.541, p < 0.05). No correlation was observed between NO(x) and cardiac output. TNF-alpha correlated with NO(x) levels (r = 0.593, p < 0.005) but not with either CI or cardiac output. Plasma levels of ANP and TNF-alpha were higher in atrial septal defect than those in the control group (p < 0.001 and p < 0.05, respectively). Elevated plasma NO(x) could explain the increased basal release of endothelial NO due to high pulmonary blood flow. Plasma NO(x) correlate negatively with CI in young patients with left-to-right shunt congenital heart diseases.

Child↗
PubMed2003

Soluble P-selectin and thrombomodulin-protein C-Protein S pathway in cyanotic congenital heart disease with secondary erythrocytosis.

INTRODUCTION: The aim of the study was to elucidate the roles of soluble P-selectin and thrombomodulin (TM)-protein C-protein S pathway in the pathogenesis of coagulopathy or thrombosis in cyanotic congenital heart disease (CCHD) with secondary erythrocytosis, and their correlations with hematocrit (Hct) value. MATERIALS AND METHODS: We studied 27 patients (age: 4.8 to 34.9, median 15) with cyanotic congenital heart disease complicated by secondary erythrocytosis (hematocrit >45%) and 26 patients with acyanotic congenital heart disease (ACHD). Plasma levels of P-selectin, beta-thromboglobulin (beta-TG), platelet factor 4 (PF4), thrombomodulin, protein S and activity of protein C were compared between the two groups, and the relationships between these indices and hematocrit value were evaluated. RESULTS: Plasma levels of P-selectin, beta-thromboglobulin and platelet factor 4 [mean (S.D.)] were significantly high in cyanotic patients comparing with acyanotic patients [138 (70.1) vs. 82.5 (28.7), p<0.001; 94.4 (74.0) vs. 54.9 (19.7), p<0.01; 45.4 (48.7) vs. 22.7 (11.9), p=0.020, respectively]. Those of thrombomodulin and protein S and activity of protein C were significantly low in cyanotic patients comparing with acyanotic patients [22.1 (9.69) vs. 34.3 (27.4), p=0.029; 90.7 (15.1) vs. 112 (21.4D), p<0.0001; 88.8 (19.7) vs. 106 (27.7), p<0.01, respectively]. P-selectin (r=0.445, p=0.001) and beta-thromboglobulin (r=0.311, p=0.025) correlated positively, and platelet count (r=-0.418, p=0.0015), protein C (r=-0.322, p=0.018) and protein S (r=-0.368, p=0.007) correlated negatively with hematocrit. CONCLUSIONS: Chronic platelet activation and suppression of the thrombomodulin-protein C-protein S pathway might play an important role in coagulopathies identified in patients with erythrocytosis. Hematocrit is an important determinant of such abnormalities.

Adolescent↗
PubMed1994

Should cardiac transplantation for congenital heart disease be delayed until adult age?

The number of pediatric heart transplantations for complex congenital heart disease has increased over the last years, but little experience has been reported in adolescent and adult populations. Between 1987 and 1992, 14 patients (mean age 33.2 years, range 14 to 51 years) were transplanted in our institution because of structural congenital heart disease (n = 9) or other rare disorders of the endomyocardial morphogenesis (n = 5). The main diagnosis included transposition of the great arteries, congenitally corrected transposition of the great vessels, left superior vena cava, tricuspid atresia with right ventricular hypoplasia, double outlet right ventricle with transposition, left ventricular sinusoidal malformation and right ventricular dysplasia. In several cases there were additional intracardiac malformations, including ventricular septum defect, atrial septum defect as well as different forms of pulmonary stenosis. Seven patients had undergone one or more palliative repairs that consisted of modified Blalock-Taussig shunts, Glenn's cavopulmonary anastomosis, Waterstone shunt, Blalock-Hanlon atrioseptectomy and Brock pulmonary valvotomy. Two patients had undergone Senning procedure for transposition of the great arteries. The donor cardiectomy was modified in order to include complete inflow and outflow tissue in the explant and transplantation could be performed without prosthetic material in all patients; deep hypothermic cardiac arrest was never necessary in this series. There was no early or late mortality after a mean follow-up of 37 months (range 4 to 74 months); postoperative echocardiography and cardiac catheterization demonstrated perfect anatomical and functional results in all patients. Adolescent and adult patients with complex congenital cardiac diseases can be transplanted with a very low perioperative risk, even after several prior operative procedures.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1998

Anomalous origin of coronary arteries and risk of sudden death: a study based on an autopsy population of congenital heart disease.

Coronary arteries anomalies may be part of complex congenital malformations of the heart or be an isolated defect. In our anatomic collection of congenital heart disease, an isolated anomalous origin of coronary arteries was observed in 27 of 1,200 specimens (2.2%): left coronary artery from pulmonary trunk in five, origin from the wrong aortic sinus in 12 (both right and left coronary artery from the right sinus in four and from the left sinus in seven, left coronary artery from the posterior sinus in one), left circumflex branch from right aortic sinus or from very proximal right coronary artery in three, high takeoff of right coronary artery in three, stenosis of the coronary ostia attributable to valvelike ridge in four. In 16 (59%) patients (12 males and 4 females, age ranging from 2 months to 53 years; median, 14), the final outcome was sudden death; it occurred in all cases of left coronary artery origin from right aortic sinus, in 43% of right coronary artery origin from left aortic sinus, and in 40% of the left coronary artery from the pulmonary trunk. Sudden death was precipitated by effort in eight (50%) and was the first manifestation of the disease in eight (50%); previous symptoms consisted of recurrent syncope in four, palpitations in three, and chest pain in one. Five patients who died suddenly during effort were athletes. In conclusion, (1) more than half of our postmortem cases with anomalous origin of coronary arteries died suddenly, (2) all but two patients with sudden death had anomalous coronary artery origin from the aorta itself, (3) the fatal event was frequently precipitated by effort, (4) palpitations, syncope, and ventricular arrhythmias were the only prodromic symptoms and signs. Recognition during life of these coronary anomalies, by the use of noninvasive procedures, is mandatory to prevent the risk of sudden death and to plan surgical correction if clinically indicated.

Adolescent↗
PubMed1998

Effect of modified ultrafiltration in high-risk patients undergoing operations for congenital heart disease.

BACKGROUND: Modified ultrafiltration (MUF) after cardiopulmonary bypass (CPB) in children decreases body water, removes inflammatory mediators, improves hemodynamics, and decreases transfusion requirements. The optimal target population for MUF needs to be defined. This prospective, randomized study attempted to identify the best candidates for MUF during operations for congenital heart disease. METHODS: Informed consent was obtained from 100 consecutive patients with complex congenital heart disease undergoing operations with CPB. They were randomized into a control group (n = 50) of conventional ultrafiltration during bypass and an experimental group using dilutional ultrafiltration during bypass and venovenous modified ultrafiltration after bypass (MUF group, n = 50). Postoperative arterial oxygenation, duration of ventilatory support, transfusion requirements, hematocrit, chest tube output, and time to chest tube removal were compared between the groups stratified by age and weight, CPB technique, existence of preoperative pulmonary hypertension, and diagnosis. RESULTS: There were no MUF-related complications. In patients with preoperative pulmonary hypertension, MUF significantly improved postoperative oxygenation (445 +/- 129 mm Hg versus control: 307 +/- 113 mm Hg, p = 0.002), shortened ventilatory support (42.9 +/- 29.5 hours versus control: 162.4 +/- 131.2 hours, p = 0.0005), decreased blood transfusion (red blood cells: 16.2 +/- 18.2 mL/kg versus control: 41.4 +/- 27.8 mL/kg, p = 0.01; coagulation factors: 5.3. +/- 6.9 mL/kg versus control: 32.3 +/- 15.5 mL/kg, p = 0.01), and led to earlier chest tube removal. In neonates (< or =30 days), MUF significantly reduced transfusion of coagulation factors (5.4 +/- 5.0 mL/kg versus control: 39.9 +/- 25.8 mL/kg, p = 0.007), and duration of ventilatory support (59.3 +/- 36.2 hours versus 242.1 +/- 143.1 hours, p = 0.0009). In patients with prolonged CPB (>120 minutes), MUF significantly reduced the duration of ventilatory support (44.7 +/- 37.0 hours versus 128.7 +/- 133.4 hours, p = 0.002). No significant differences were observed between MUF and control patients for any parameter in the presence of ventricular septal defect without pulmonary hypertension, tetralogy of Fallot, or aortic stenosis. CONCLUSIONS: Modified ultrafiltration after CPB is safe and decreases the need for homologous blood transfusion, the duration of ventilatory support, and chest tube placement in selected patients with complex congenital heart disease. The optimal use of MUF includes patients with preoperative pulmonary hypertension, neonates, and patients who require prolonged CPB.

Blood Loss, Surgical↗
PubMed2006

Transition and transfer from pediatric to adult care of the young adult with complex congenital heart disease.

This article focuses first on the process of transition and transfer of care of young adults with complex congenital heart disease. It defines the transition process and briefly discusses its history. It reviews the important aspects of transition, outlines the key elements of a successful transition program, and provides a curriculum appropriate for the young adult with congenital heart disease. Finally, it identifies the barriers to transfer of care, discusses the importance of a policy on timing, outlines the components of adult provider services that may be needed, and reviews the steps to an orderly transfer process.

Adolescent↗