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PubMedDate not supplied

Thiamine deficiency in children with congenital heart disease before and after corrective surgery.

BACKGROUND: Malnutrition is common in children with congenital heart disease, while thiamine deficiency (TD) is common in malnutrition, in critically ill children, and in adults with congestive heart failure treated with loop diuretics. Our goal was to determine whether children with congenital heart disease had TD and whether treatment with loop diuretics is related to TD in these patients. METHODS: Twelve children with ventricular septal defect (VSD) treated with furosemide, and 10 children with tetralogy of Fallot (TOF) referred for corrective surgery were consecutively enrolled into a prospective study. Data were collected 24 hours before surgery and 5 days after surgery for nutrition evaluation, medications used, anthropometric measurements, and laboratory markers of malnutrition. Thiamine and pyridoxine deficiencies were evaluated using activated enzyme assays. RESULTS: Seven children (32% of patients) did not meet the recommended daily allowance (RDA) for calories and 18% of patients did not meet the RDA for thiamine intake. Anthropometric measurements were low in both groups, more so in those with VSD, although the difference did not reach statistical significance. Overall, 18% (1/12 with VSD and 3/10 with TOF) of children with congenital heart disease had thiamine deficiency before surgery. Three of the four children with TD had adequate intake of thiamine. Six children (27%) had TD 5 days postsurgery (3 children with VSD and 3 children with TOF). CONCLUSIONS: TD is common in children with congenital heart disease (CHD) referred for corrective surgery both before and after surgery. Our results suggest that neither diuretic treatment nor malnutrition can fully explain the development of TD in these children.

Anthropometry↗
PubMed1999

[Prevalence and distribution of congenital heart diseases in Indre-et-Loire. Evaluation of prenatal diagnosis (1991-1994)].

UNLABELLED: Congenital heart diseases are the most frequent malformation at birth. New technologies have improved diagnosis procedures (echocardiography and Doppler). The aim of our study was to evaluate the prevalence of congenital heart diseases, their different types, and the detection rate of antenatal diagnosis. METHODS: A retrospective study was performed for all infants with congenital heart disease (CHD), born between January 1st 1991 and December 31st 1994, and for all fetuses which died after disruption of pregnancy, in Indre-et-Loire (a French country). In all cases, CHD diagnosis was confirmed with echocardiography and Doppler. RESULTS: CHD prevalence in newborns was 9.8% and 10.4% for the total population including dead fetal material. A high proportion of septal defects (64.8%) was observed with muscular, isolated and small forms (< 3 mm) in 70.2% of cases. The prevalence of great vessels transposition (0.15%), left ventricular hypoplasia (0.11%), and atrioventricular septal defect (0.11%), were lower than in previous studies. The performance of antenatal diagnosis was estimated at 40.5% for the four years; the prevalence of detectable CHD was only 1.4/1000. The atrioventricular septal defect was the most frequently detected. CONCLUSION: Relative high prevalence of congenital heart disease in this French county is due to the high level of small septal defects. Prevalence of detectable CHD remains low, which explains in part the difficulties of improving the antenatal diagnosis.

Abortion, Induced↗
PubMed2005

Self-concept: comparison between school-aged children with congenital heart disease and normal school-aged children.

AIMS AND OBJECTIVES: The aim of this study was to evaluate and compare the self-concept of school-aged children with congenital heart disease to those of normal school-aged children. The primary objective was to analyse results of the Self-Concept Scale questionnaire administered to children with congenital heart disease aged 9-12 years. Sixty-four children with congenital heart disease (study group), and 71 without congenital heart disease (control group), completed the questionnaire. BACKGROUND: Little attention has focused on school-aged children with congenital heart disease who are in the important stages of developing self-concept. RESULTS: The mean score on the Physical self-concept of the Self-Concept Scale was significantly lower for the study group than the control group (P < 0.05). No significant differences were observed between children with congenital heart disease and normal children in terms of family self-concept, school self-concept, appearance self-concept, emotional self-concept and general self-concept for the Self-Concept Scale. CONCLUSIONS: Nurses should use the study findings to encourage positive self-concept development and improve their patient education about physical activity before the child is discharged. Thus, children with congenital heart disease could leave the hospital with a clear understanding of their body and condition, and how it affects daily life. RELEVANCE TO CLINICAL PRACTICE: The results of this study may provide more holistic views on self-concept for clinical nurses working with children who have congenital heart disease and their families and for school nurses working with elementary school children.

Child↗
PubMed1985

Electroencephalographic abnormalities in children with congenital heart disease.

Ninety-eight consecutive patients with clinically suspected congenital heart disease were prospectively studied with electroencephalographic (EEG) recordings before cardiac catheterization. Twenty-five patients had abnormal EEGs. Fifty-five patients had acyanotic heart disease and normal neurologic examination results, of whom 15 had abnormal EEGs. Thirteen had spikes or spike and wave discharges and two had mildly abnormal EEGs. Twenty-seven patients had cyanotic heart disease and normal neurologic examination results, of whom four had abnormal EEGs. Seven patients (8%) had abnormal neurologic examination results, of whom four had abnormal EEGs. There was a higher incidence of seizures with increasing age. Based on their medical history two children with acyanotic heart disease had had seizures without EEG abnormalities. Five children with normal catheterization findings and four children with Down's syndrome (two with abnormal EEGs) were excluded from the total of 98. These findings suggest that subclinical nervous system involvement may occur in congenital heart disease.

Brain↗
PubMed2001

Intravascular and intracardiac stents used in congenital heart disease.

Intravascular or intracardiac stenoses occur in many forms of congenital heart disease or after attempted surgical repair. Although balloon dilation is one option for management, restenosis can occur due to elastic recoil immediately after the procedure. To address to such stenotic lesions, many reports support implanting endovascular stents to provide a framework for vessel expansion. Both balloon-expandable fixed tubular mesh stainless steel devices, and self-expandable stents have had an extensive clinical application. In pediatric patients, stents are used for a variety of stenoses, such as systemic venous obstruction pathways (eg, Mustard, Fontan baffle, or bidirectional cavopulmonary connections), pulmonary artery, right ventricular to pulmonary conduits, aortic coarctation, the arterial duct, aorticopulmonary collaterals, or postoperative systemic to pulmonary shunts. Because of improvements in device profile, implantation rates have increased. Complications such as stent fracture, migration, aneurysm formation, and in-stent restenosis occur but only rarely. This latter event may be because of intimal hyperplasia and/or continued vessel (and patient) growth related to the stent diameter. As such, some instances require redilation to manage the acquired lesion. Stent application has importantly altered management algorithms in congenital heart disease.

Adolescent↗
PubMed2005

Surgical outcomes in congenital heart disease: expectations and realities.

The past 50 years of congenital heart surgery has produced enormous progress. Current results surpass expectations. Yet there are important residual problems in patients growing up after heart surgery for congenital heart disease. Our system of care must evolve to care for these people throughout their lives. The evolution of congenital heart surgery has reached a point in time when we should extend care to patients in under serviced emerging countries. Development of local expertise will be required within those countries that are willing to commit resources to an organized program of caring for people with congenital heart disease. Database technology is an essential tool for ensuring and improving quality of care in every congenital heart centre. Both Registry and Academic databases have much to offer in improving care for future patients. Yet overzealous privacy laws threaten the knowledge base provided by computerized databases. We need to guide our legislators in ensuring that the valuable resource provided by database technology is not lost.

Confidentiality↗
PubMed1991

Applications of intravascular scanning and transesophageal echocardiography in congenital heart disease: tradeoffs and the merging of technologies.

This chapter will review the evolving role of intravascular ultrasound imaging and transesophageal echo in the care of children, infants and adults with congenital heart disease. The technologies relevant to congenital heart disease applications differ from those involving coronary disease since the intravascular structures imaged often involve visualization of large vessels and cardiac chambers. On the other hand, the requirements for transesophageal echo in children with congenital heart disease involve intraoperative (surgical) and imaging procedures in the catheterization laboratory which are performed for monitoring interventional catheterization therapy. As such, whereas the intravascular devices needed for pediatric cases involve lower frequency and sometimes larger catheters, the requirements for transesophageal echocardiography require higher frequency and smaller esophagoscopes. Applications of intravascular imaging including sizing of congenital stenoses, dilation of coarctation and valvular stenoses, imaging of intrapulmonary thrombi and monitoring of placement of ASD 'button' devices in the heart will be reviewed. The intraoperative transesophageal uses for monitoring infant surgery include procedures for tetralogy repair, transposition repair and repair of AV septal defects and other complex congenital heart disorders. Both of these 'invasive' methods of echocardiography have an important and evolving role in the management of congenital heart disease in children and infants.

Adult↗
PubMed1983

[Outcome of patients undergoing open-heart surgery for congenital heart disease in early childhood].

Corrective surgery for congenital heart disease is performed on progressively younger children in order to restore normal haemodynamics during infancy and to avoid the disadvantages of palliative operations. The authors consider the long-term outcome of children who have been operated for complete transposition of the great vessels--by physiological correction or by anatomical correction--for tetralogy of Fallot, interventricular communication, atrio-ventricular channel and aortic stenosis. The sequelae and complications of these various operations are reviewed and the authors stress, in particular, the risk of delayed arrhythmias and disturbances of ventricular function. The risk of delayed complications justifies long-term follow-up of all these patients, even those who are leading a normal life, i.e. the majority of them.

Cardiac Surgical Procedures↗
PubMed2005

Combination therapy of prostacyclin for pulmonary hypertension in congenital heart disease.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a recognized complication of congenital heart disease. Despite differences in etiology and pathophysiology, successful therapy for idiopathic PAH may benefit in patients with congenital heart disease. We theorized that combination of oral and aerosolization prostacyclin will benefit this group of patients in long-term. MATERIAL AND METHOD: The study design was single group and open label study with intention to treat for patients with congenital heart disease with pulmonary artery (PA pressure) more than 50% of systemic pressure. All patients were given a combination of orally given beraprost sodium and inhalation of iloprost for 12 months. Data were collected prospectively consisting of functional class, O2 saturation, 6-minute walk test and right ventricular systolic pressure (RVSP). RESULTS: There were 23 patients with an average right ventricular systolic pressure (+/- SD) of 94.8 +/- 14.5 mmHg and with average age of 27.8 +/- 14.9 years (2.5 to 50 years). The average oxygen saturation was 87.9 +/- 7 %. There were 12 patients with post surgical repair or cardiac catheterization interventional procedure and 11 with and Eisenmenger's syndrome. There were significant improvement of 6-minute-walk test from an average of 268 +/- 70 meters to 308 +/- 57 meters at the end of 12 months. The functional class of patients was also improving. However, there were no significant different in oxygen saturation. CONCLUSION: Combination therapy of oral and inhalation of aerosolized vasodilators is a fascinating concept in the therapy of pulmonary hypertension. Treated patients showed an improvement in exercise capacity and right ventricular systolic pressure without a worsening in oxygen saturation.

Administration, Inhalation↗
PubMed1983

Comparative hemodynamic effect of metrizamide and Renografin 76 in infants with congenital heart disease.

Twenty patients under the age of 2 years with suspected congenital heart disease received alternately Renografin 76 and metrizamide for angiocardiography. The dose was 2.0 ml/kg per injection for both contrast media into the left ventricle. Metrizamide induced slightly lesser change in heart rate, peak systolic pressure, and peak end-diastolic pressures. Serum osmolality, hematocrit, and serum electrolytes were affected equally by the contrast media. Metrizamide was well tolerated by the neonates with congenital heart disease and its radiopacity was adequate for diagnostic purposes. At the doses administered, metrizamide does not seem to have any great advantage over Renografin 76 for angiocardiography in infants with severe congenital heart disease.

Angiocardiography↗
PubMed1990

Extracorporeal membrane oxygenation for the circulatory support of children after repair of congenital heart disease.

We have treated 39 infants and children with congenital heart disease with extracorporeal membrane oxygenation during the past 5 years. Thirty-six were treated for low cardiac output or pulmonary vasoreactive crisis after repair of congenital heart defects. Twenty-two (61%) survived. Most patients were cannulated from the neck via the right internal jugular vein and the right common carotid artery. Six patients were cannulated from the chest, including three who had separate drainage of the left side of the heart with a left atrial cannula. Two of these patients survived and were the only survivors of the nine patients cannulated in the operating room because they could not be weaned from cardiopulmonary bypass after open cardiac operations. We also reviewed 312 patients (the predictor study series) having open cardiac operations before the availability of extracorporeal membrane oxygenation; 27 of these patients died. Data were collected at 1 and 8 hours postoperatively to determine if any parameters might predict early mortality. With these parameters used as criteria, patients who went on extracorporeal membrane oxygenation were as sick as those who died before extracorporeal membrane oxygenation was available. The most common complication was bleeding related to heparinization. The mean transfusion requirement in survivors was 1.50 +/- 1.13 ml/kg/hr, 5.63 +/- 7.0 ml/kg/hr in the nonsurvivors, and 7.46 +/- 8.29 ml/kg/hr in those cannulated in the operating room because they could not be weaned from bypass. Four children had intracranial hemorrhage, and two of them died. There was one late death. Nine of the 22 survivors are entirely normal. All survivors who do not have Down's syndrome are considered to have normal central nervous system function. We conclude that extracorporeal membrane oxygenation can improve survival in patients with both pulmonary artery hypertension and low cardiac output after operations for congenital heart disease.

Arrhythmias, Cardiac↗
PubMed2005

[Clinical information and management in patients with adult congenital heart disease undergoing non-cardiac surgery].

Untreated or palliated congenital heart disease has the strong impact on the outcomes of surgery for non-cardiovascular disease that accrues with age. However, excellent guidelines have not been published yet that provide ideal information and expert opinion for patients and doctors in assessing adult congenital heart disease (ACHD). In the global risk assessment for such a condition, American College of Cardiology--American Heart Association (ACC/AHA) guidelines can be helpful although developed exclusively from populations with acquired heart disease. The first necessity is to clarify the diagnosis The second is preoperative hemodynamic assessment according to the specific physiology and anatomy. The third is to evaluate the intensity of expected hemodynamic stress by proposed operation. The last is to elucidate co-morbid conditions. In addition to global risk assessment, Risk factors unique to ACHD must be carefully considered. Under complete assessment of these risk factors by the staff who have sufficient experience or training in the management of ACHD, the optimal, effective and meticurous cares can be provided to patients with ACHD encountered at noncardiac surgery.

Adult↗
PubMed2004

Applying standard therapies to new targets: the use of ACE inhibitors and B-blockers for heart failure in adults with congenital heart disease.

Increasingly, patients and clinicians are being confronted with congestive heart failure (CHF) as a late complication of congenital heart disease. However, medical management of heart failure in this patient group represents a challenge because of complex hemodynamics and a lack of evidence from large randomized controlled trials to guide therapy. This article will review the evidence of the use angiotensin converting enzyme inhibitors (ACEIs) and beta-blockers (BBs) in left heart failure, discuss the mechanisms of heart failure as they pertain to congenital heart disease and review the limited literature of the use of neurohormonal antagonists in congenital heart disease. Some recommendations for use of angiotensin converting enzyme inhibitors and beta-blockers in heart failure due various congenital heart lesions are offered. Well-designed clinical trials are urgently needed to extend the impressive reductions in morbidity and mortality achieved with neurohormonal blockade in left ventricular (LV) heart failure to adults with congenital heart disease.

Adrenergic beta-Antagonists↗
PubMed2004

Polymorphism of methionine synthase gene in nuclear families of congenital heart disease.

OBJECTIVE: To investigate the relation of methionine synthase (MS) gene variation with congenital heart disease (CHD) phenotype. METHODS: One hundred and ninety three CHD patients (94 males and 99 females) and their biological parents (nuclear families) in Liaoning Province were selected as the case group, and another 104 normal persons (60 males and 44 females) and their parents without family history of birth defects as the control group. For all subjects the polymorphism of MS gene A2756G locus was examined by PCR-RFLP method. RESULTS: In offspring of the control group the frequencies of MS genotype (+/-) and allele (+) were 10.7% and 5.3%, without existence of homozygote. The MS genotype distribution and allele frequencies of CHD patients and their mothers were not significantly different from the control (P > 0.05). The frequency of allele (+) in case fathers (5.0%) was apparently lower than that in the control (9.1%, P = 0.060), and the odds ratio (OR) was 0.53 (95% CI: 0.25-1.09). There was no difference in parents' genotype combination between the two groups, and in genotype distribution among different types of CHD. Analysis of genetic transmission indicated that mutation allele (+) existed transmission disequilibrium in CHD nuclear families. The percentage of allele (+) transmitted from parents was lower than that allele (-) with OR 0.26 (95% CI: 0.11-0.60). CONCLUSION: MS gene variation in parents is associated with occurrence of CHD in offspring, and mutation allele (+) in parents may be related with the decrease of CHD risk in offspring.

5-Methyltetrahydrofolate-Homocysteine S-Methyltran↗
PubMed1991

Echocardiographic segmental approach to complex congenital heart disease in the neonate.

A segmental echocardiographic approach to complex congenital heart disease in the neonate allows accurate and precise assessment of intracardiac, as well as extracardiac, anomalies. Determination of abnormalities of atrial and visceral situs, as well as the cardiac situs, provides a fundamental background for the echocardiographic examination. Subsequently, a segmental approach allows precise determination of abnormalities of atrioventricular and ventricular great artery connections. Recent echocardiographic advances including high-frequency (7.5 and 10 MHz) and color flow imaging capabilities have dramatically improved our ability to accurately define abnormalities of anatomy and connection in the neonate. These advances now have expanded our previous echocardiographic criteria and allow application of the echocardiographic segmental approach to characterize complex congenital heart disease in the neonate.

Echocardiography↗
PubMedDate not supplied

New trends in the management of congenital heart disease.

Considerable improvements have been made in the diagnosis and treatment of congenital heart disease during the last decade. Many congenital heart lesions are now treated successfully during the neonatal period that previously were associated with high mortality. Improved echocardiographic imaging, catheterization techniques, and earlier surgical repair are factors that have resulted in greater success in the treatment of congenital cardiac disease. Diagnosis has been improved greatly with advancements in echocardiography and angiography. Better ultrasound technology combined with doppler techniques and transesophageal echocardiography allow more accurate preoperative assessment and therefore more successful surgical repair. Cardiac catheterization techniques have also improved and, when combined with treatment such as balloon angioplasty, have changed the treatment of certain cardiac anomalies such as pulmonary stenosis or coarctation of the aorta. Operative treatment of congenital heart disease has improved the short- and long-term survival of most infants with congenital cardiac anomalies. Improved cardiopulmonary bypass techniques, better suture material, and the ability to perform cardiac transplantation are examples of technology that allows earlier, more complete repair of these complex cardiac defects. Reviewed here are improvements in the treatment of four complex cardiac anomalies that occur in newborns and are associated with high mortality when left untreated. All four anomalies have undergone significant changes in the approach to their treatment with dramatic improvements in survival.

Aortic Valve Stenosis↗