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PubMed2001

Hypothetical framework for a relationship between maternal thyroid function, nausea and vomiting of pregnancy, and congenital heart disease.

Our objective was to investigate whether a relationship exists among maternal thyroid function, nausea and vomiting of pregnancy, and congenital heart disease. A Medline search from 1966 to the present was conducted to look for reports on the existence of this relationship. The results were supplemented by abstract searches and personal communication with relevant authors. Our search found independent evidence that maternal hyperthyroidism is related to increased rates of nausea and vomiting of pregnancy, which in turn is significantly related to a decrease in the incidence of congenital heart disease. Early evidence indicates that the converse may be true: maternal hypothyroidism and thyroid replacement therapy are associated with an increase in congenital heart disease in children. The potential relationship between maternal thyroid function, nausea and vomiting of pregnancy, and thyroid replacement therapy needs further study. We propose a case-control study of children presenting for echocardiography to elicit specific information regarding the pregnancy.

Female↗
PubMed2000

The association between congenital heart disease and Down syndrome in prenatal life.

OBJECTIVE: To assess the relationship between congenital heart disease (CHD) and Down syndrome (DS) in utero. DESIGN: Retrospective case series. SUBJECTS: Fifty-two fetuses with a cytogenetic diagnosis of DS managed at our Fetal Cardiology Unit in the study period. In particular, two populations of fetuses with DS were studied: a group of 41 DS fetuses referred to our unit for fetal echocardiography due to the chromosomal anomaly and a second group of 274 fetuses referred because of suspected CHD, 11 of which were found to have DS. METHODS: All fetuses were submitted to detailed ultrasound evaluation of fetal anatomy. Associated extracardiac anomalies, and presence and type of CHD, were recorded for all fetuses. Karyotyping was obtained by means of cordocentesis or amniocentesis. Necropsy or neonatal echocardiograms were sought for confirmation of the prenatal diagnosis. RESULTS: In the group of 41 fetuses with known DS, the incidence of CHD was 56% ([atrioventricular septal defect (AVSD) 44%, ventricular septal defect (VSD) 48%], the remainder having other heart defects). Conversely, considering the incidence of DS in fetuses with CHD, 43% of all AVSDs (53% of AVSD with normal visceral situs) were associated with DS, whereas none of the 39 cases of VSD was associated with trisomy 21. Ventricular septal defects were diagnosed only in fetuses referred to our center with a known diagnosis of aneuploidy. CONCLUSIONS: We have confined that more than half of the fetuses with DS bear a CHD, which is an AVSD in 44% of cases. Conversely, 43% of fetuses with an AVSD have trisomy 21. For VSDS, the situation is controversial, due to the relatively low detection level of this heart defect at the routine mid-trimester obstetric scan.

Abnormalities, Multiple↗
PubMed2006

[Transcatheter treatment of congenital heart disease].

For more than 20 years, interventional cardiac catheterization has considerably increased in the therapeutic management of simple congenital heart disease in childhood. It is possible to correct pulmonary or aortic valvar stenosis, to close a persistent shunt as patent arterial duct or atrial septal defect. Sometimes, it can replace surgical repair and can be proposed as a first line treatment. Interventional cardiac catheterisation has several advantages for the patient: no thoracotomy, no scar, shorter hospital stay, less painful, lower morbidity and reduced cost. These techniques have also benefited from miniaturization and evolution of the occluders with time. Gene therapy, tissue engineering and new imaging modality (MRI, endovascular echo) will be the future of interventional cardiac catheterization which will occupy a more important place in the treatment of congenital heart disease in children.

Angioplasty, Balloon↗
PubMed2003

Implantable cardioverter defibrillator lead complications and laser extraction in children and young adults with congenital heart disease: implications for implantation and management.

INTRODUCTION: Implantable cardioverter defibrillators (ICDs) are being implanted for primary and secondary prevention of sudden death in children and young adults with congenital heart disease. Over time, ICD leads adhere to venous endothelium and endocardium. Lead removal, when necessary, often requires disruption of this fibrous tissue. METHODS AND RESULTS: We retrospectively reviewed and analyzed our experience with ICD lead extraction in children and young adults with congenital heart disease. From April 1999 through January 2002, 14 patients underwent 15 lead extraction procedures to remove 21 leads (17 ICD leads and 4 pacing or sensing leads). Seven patients had surgically corrected structural heart disease (5 transposition of the great arteries with atrial switch repair and 2 corrected tetralogy of Fallot). Mean patient age at extraction was 17.9 +/- 5.7 years (range 9-32), and mean duration of lead implantation was 42.0 +/- 18.9 months (range 15-75). Fourteen of 15 procedures were performed for lead fracture or failure. A laser sheath was used for 20 of 21 lead extractions. Twenty of 21 leads (95%) were completely extracted. There were three instances of blood loss requiring transfusion. There were no major complications or deaths. CONCLUSION: Young congenital heart disease patients with an ICD are at risk for growth-related lead distortion. The use of a laser sheath is safe and effective for ICD lead extraction in congenital heart disease patients, despite coil adherence and altered anatomy. It may be advisable to avoid dual-coil leads in patients with the potential for future growth.

Adolescent↗
PubMed2001

Abnormal rheology in cyanotic congenital heart disease--a factor in non-immune nephropathy.

OBJECTIVE: There are few data on non-immune nephropathy in cyanotic congenital heart disease reported in the literature. Rheologic changes have been suspected to be involved in the pathogenesis. This study was performed to describe the characteristics of renal functional and hemorheological abnormalities in a large group of patients with cyanotic congenital heart disease. PATIENTS AND METHODS: Thirty-five cyanotic patients with median oxygen saturation of 82% (range 38-92%), age 18 years (range 5-63 years), and 13 acyanotic controls with atrial septal defect, age 37 years (range 20-66 years) were included. Red cell indices, blood and plasma viscosity were analyzed. Renal function was evaluated with measurements of albumin, alpha1-microglobulin, transferrin, immunoglobuline, and N-acetyl-beta-D-glucosaminidase in the urine. RESULTS: Fifteen cyanotic patients and 1 control patient had pathologic albuminuria (p < 0.05). In cyanotic patients blood and plasma viscosity, erythrocyte count, hemoglobin, and hematocrit were elevated while mean corpuscular volume and mean corpuscular hemoglobin were decreased (p < 0.05). The possible impact of blood hyperviscosity on proteinuria is indicated by multiple regression analysis (odds ratio = 0.5, confidence intervals 22-130, p < 0.05). CONCLUSIONS: Our study shows a coincidence of elevated blood viscosity and proteinuria in patients with cyanotic congenital heart disease. This observation supports the hypothesis that impaired peritubular capillary blood flow with increased intraglomerular blood pressure may add to chronic glomerular dysfunction.

Adolescent↗
PubMed1987

Echocardiographic versus cardiac catheterization diagnosis of infants with congenital heart disease requiring cardiac surgery.

The success of noninvasive preoperative evaluation of infants with congenital heart disease using cardiac ultrasound depends not only on diagnostic accuracy, but also on risk of morbidity and mortality as compared with infants who undergo cardiac catheterization. Fifty-six infants (age 10 weeks or younger) with coarctation of the aorta (n = 16), coarctation with ventricular septal defect (n = 12), valvar aortic stenosis (n = 10) or total anomalous pulmonary venous connection (n = 18) were examined. Thirty-one underwent noninvasive preoperative assessment and 25 underwent evaluation including cardiac catheterization. Age, level and duration of support, pH, renal function, mortality, complications of cardiac catheterization and errors of diagnosis were compared. Significant differences between the 2 groups were more frequent preoperative use of prostaglandin E1 and shorter hospital stay in the noninvasively evaluated coarctation group. Of the infants with coarctation and ventricular septal defect, 1 who had cardiac catheterization required renal transplantation and 1 evaluated noninvasively required surgery at age 3 months for mitral stenosis not discovered on preoperative evaluation. One noninvasively evaluated infant with total anomalous pulmonary venous connection had a stenotic communication between the pulmonary venous confluence and the left atrium not detected by ultrasound. Surgery was successful in the latter 2 infants. Noninvasive preoperative diagnosis of some infants with congenital heart disease can be performed without increasing the risk of operative morbidity and mortality. Eliminating cardiac catheterization reduces hospital costs, decreases total numbers of catheterizations performed and influences the structure of training programs.

Cardiac Catheterization↗
PubMed1999

Increased expression of mast cell chymase in the lungs of patients with congenital heart disease associated with early pulmonary vascular disease.

The molecular mechanism involved in pulmonary vascular disease (PVD) associated with congenital heart disease (CHD) remains uncertain. Evidence suggesting that angiotensin converting enzyme plays an important role in pulmonary vascular pathology led us to hypothesize that mast cell chymase, another angiotensin I converting enzyme, also had the potential to contribute to the development of PVD in CHD. Twenty-three patients 3 mo to 45 yr of age with atrial or ventricular or both septal defects with increased pulmonary arterial blood flow and pressure, with pulmonary vascular resistance ranging from 1.3 to 8.1 units/m(2), were studied. Mast cells and mast cell chymase were immunohistochemically identified in the lung biopsy tissues obtained during corrective surgery. There was a significant difference in numbers of total mast cells between patients (n = 23) and control subjects (n = 10) with normal pulmonary circulation (p < 0.01). Moreover, chymase-containing mast cells in the lung tissues of patients with CHD showed striking differences from those of control subjects. In the patients, 72% of lung mast cells contained chymase, compared with only 15% in control subjects (p < 0.0001). Chymase-containing mast cells predominantly appeared in the media and adventitia of vessel walls. Importantly, angiotensin II was immunohistochemically detected in perivascular lesions where chymase was present, but not in the lesions where chymase was sparsely seen. Furthermore, the number of chymase-containing mast cells was correlated with pulmonary vascular resistance (r = 0.64). These findings suggest a possible role of mast cell chymase in the development of early-stage PVD in patients with CHD.

Adult↗
PubMed1978

Apex echocardiography. A two-dimensional technique for evaluating congenital heart disease.

We have evaluated apex echocardiography, using an 80 degrees phased array sector scanner, in 368 patients with congenital heart disease. With the patient lying with the left side dependent, the transducer is placed over the apex of the heart and cross sectional images are obtained in the plane perpendicular to the cardiac septa and through the orifices of the mitral and tricuspid valves. In this view, the chambers are side by side and both atria and ventricles are separated by their respective septa and atrioventricular valves. Defects in the region of the septa can be detected. Congenital defects involving the atrioventricular valves, such as endocardial cushion defects, tricuspid atresia, and Ebstein's anomaly, can be defined. The location of the baffle after Mustard's operation for aortopulmonary transposition and intra-atrial structures, such as the membrance in cor triatriatum, can be seen. The position of the apex of the heart can be located in dextro, levo, or mesocardia by definition of the apex image. The relative size of the ventricular septum can be identified with the apex image. We have found this technique to be valuable in patients with congenital heart disease who are undergoing cross sectional echocardiography.

Adolescent↗
PubMed1996

Lead extraction in young patients with and without congenital heart disease using the subclavian approach.

Pacemaker lead removal using interlocking stylets and dilator sheaths has greatly reduced the need for major surgical intervention when lead extraction is required. Previous reports have shown the utility of this method in older patients, most of whom have anatomically normal hearts. The purpose of this study is to report the results of this technique in young patients with and without congenital heart disease. There were 13 patients (M:F = 7:6) aged 9-26 years (median 13). Congenital heart disease was present in 8 of 13 patients. A total of 17 leads required removal; they had been implanted for 54 +/- 24 months (range 19-94). Leads were removed from the left subclavian vein (13) or right subclavian vein (4) only. Seventeen of 18 leads were completely removed and one partially retained in the left subclavian vein. New leads were implanted from the same vein in 11 of 13 patients. Interlocking stylets and metal or flexible dilator sheaths were used in all cases except two. There was one surgical complication: a late wound dehiscence, which was easily managed. No patient required a transfusion, and there was no structural damage noted in any patient on the postoperative echocardiogram. We conclude that lead removal using interlocking stylets and dilator sheaths from the subclavian approach is an effective technique that can be used in young patients, including those with congenital heart disease.

Adolescent↗
PubMed1979

Prevalence of congenital heart disease in black schoolchildren of Soweto, Johannesburg.

A survey conducted by cardiologists in Soweto, Johannesburg, provided an opportunity of assessing the frequency of congenital heart disease in black schoolchildren. Among 12,050 schoolchildren aged 2 to 18 years, 48 had a congenital heart defect, yielding a prevalence of 3.9 per 1000. Only in 2- to 6-year-old children did the prevalence exceed that of rheumatic heart disease. The distribution of the types of defects was largely similar to that reported in other surveys with a predominance (52%) of ventricular septal defects. Two unusual findings were the unexplained absence of persistent ductus arteriosus in these children, and the detection of 5 children with situs inversus (1 in 2410). In all but one child, the congenital heart defect was first discovered during the survey. Despite the limitations of a prevalence study, it can be concluded that congenital heart disease is at least as common in this South African black community as in Caucasians.

Adolescent↗
PubMed2000

Detailed mapping of a congenital heart disease gene in chromosome 3p25.

Distal deletion of chromosome 3p25-pter (3p- syndrome) produces a distinct clinical syndrome characterised by low birth weight, mental retardation, telecanthus, ptosis, and micrognathia. Congenital heart disease (CHD), typically atrioventricular septal defect (AVSD), occurs in about a third of patients. In total, approximately 25 cases of 3p- syndrome have been reported world wide. We previously analysed five cases and showed that (1) the 3p25-pter deletions were variable and (2) the presence of CHD correlated with the proximal extent of the deletion, mapping a CHD gene centromeric to D3S18. To define the molecular pathology of the 3p- syndrome further, we have now proceeded to analyse the deletion region in a total of 10 patients (five with CHD), using a combination of FISH analysis and polymorphic markers, for up to 21 loci from 3p25-p26. These additional investigations further supported the location of an AVSD locus within 3p25 and refined its localisation. Thus, the critical region was reduced to an interval between D3S1263 and D3S3594. Candidate 3p25 CHD genes, such as PMCA2 (ATP2B2), fibulin 2, TIMP4, and Sec13R, were shown to map outside the target interval. Additionally, the critical region for the phenotypic features of the 3p- phenotype was mapped to D3S1317 to D3S17 (19-21 cM). These findings will accelerate the identification of the 3p25 CHD susceptibility locus and facilitate investigations of the role of this locus in non-syndromic AVSDs, which are a common form of familial and isolated CHD.

Chromosome Mapping↗
PubMed1978

Pharmacologic dilatation of the ductus arteriosus with prostaglandin E1 in infants with congenital heart disease.

Prostaglandin E1 (PGE1) has been used for successful palliation in nine infants with congenital heart disease. Seven patients had pulmonary atresia with ductal-dependent pulmonary blood flow. In this group, systemic O2 saturation increased from a mean value of 45% to 79% after infusion of PGE1, and surgical palliation was successfully done with the infants in stable condition, without hypoxemia or acidemia. An additional patient with coarctation of the aorta had marked ductal dilatation after PGE1 infusion as indicated by umbilical artery pulse pressure. The coarctation was repaired, with the infant in stable condition. The final patient had neonatal tricuspid insufficiency with right to left atrial shunting. Systemic O2 saturation was improved after PGE1 infusion, though the ductus was closed. The improved oxygenation was believed to be due to a reduction in pulmonary vascular resistance by PGE1. Prostaglandin E1 provides a powerful new tool for palliation of critical congenital heart disease in infants whose ductal constriction can markedly influence their clinical status.

Ductus Arteriosus, Patent↗
PubMed1998

Measured versus predicted oxygen consumption in children with congenital heart disease.

OBJECTIVE: To compare measured and predicted oxygen consumption (VO2) in children with congenital heart disease. DESIGN: Retrospective study. SETTING: The cardiac catheterisation laboratory in a university hospital. PATIENTS: 125 children undergoing preoperative cardiac catheterisation. INTERVENTIONS: VO2 was measured using indirect calorimetry; the predicted values were calculated from regression equations published by Lindahl, Wessel et al, and Lundell et al. Stepwise linear regression and analysis of variance were used to evaluate the influence of age, sex, weight, height, cardiac malformation, and heart failure on the bias and precision of predicted VO2. An artificial neural network was trained and used to produce an estimate of VO2 employing the same variables. The various estimates for VO2 were evaluated by calculating their bias and precision values. RESULTS: Lindahl's equation produced the highest precision (+/- 42%) of the regression based estimates. The corresponding average bias of the predicted VO2 was 3% (range -66% to 43%). When VO2 was predicted according to regression equations by Wessel and Lundell, the bias and precision were 0% and +/- 44%, and -16% and +/- 51%, respectively. The neural network predicted VO2 from variables included in the regression equations with a bias of 6% and precision +/- 29%; addition of further variables failed to improve this estimate. CONCLUSIONS: Both regression based and artificial intelligence based techniques were inaccurate for predicting preoperative VO2 in patients with congenital heart disease. Measurement of VO2 is necessary in the preoperative evaluation of these patients.

Calorimetry, Indirect↗
PubMed2001

Outcomes of neonates with congenital heart disease.

One of the most important advances of the past 10 to 15 years in the field of pediatric cardiology is the improvement in prognosis for neonates with complex congenital heart disease. During the past 18 months, several publications have addressed outcomes in neonates with congenital heart disease. Many of these reports demonstrate continuing improvement in preoperative, early postoperative, and late postoperative survival among patients with complex neonatal heart defects. Other reports shed substantial new light on late functional outcome, especially neurodevelopmental status. In addition to data on survival, morbidity, and functional status, we discuss developments in perioperative evaluation and management that are likely to further the trend toward improved outcome for neonates with complex congenital heart disease.

Heart Defects, Congenital↗
PubMed2006

[Effects of intraoperative hybrid procedures for the treatment of congenital heart disease].

OBJECTIVE: A hybrid operation is a joint procedure involving the interventional cardiologist and the cardiac surgeon concomitantly to optimize surgical management for complex congenital heart disease. We analyzed the feasibility and efficacy of this procedure in 20 patients with congenital heart disease. METHODS: Data from 20 patients who underwent intraoperative hybrid procedures from March to October 2005 were analyzed. Two groups were identified: (1) Balloon valvuloplasty group: pulmonary atresia with intact ventricular septum in neonate (n = 3) and severe pulmonary stenosis in infants (n = 4); (2) Device closure group: atrial septum defects (ASD) in child (n = 7), adult ASD (n = 3) and multiple ventricular septum defect (VSD, n = 3). Pulmonary balloon dilation under the guidance of echocardiography together with modified Blalock-Taussig shunt procedure and PDA ligation were applied to patients in balloon valvuloplasty group. For ASD patients, the occluder was placed via right atria after opening the chest either with median sternotomy (4 case) or with submaxillary minimally invasive incision (6 cases), PDA ligation was performed in 1 patient and 2 coronary artery bypass surgeries were performed in 2 adult ASD patients complicating CHD. For multiple VSD, perimembranous VSD was closed surgically and muscular VSD closed by occluder via tricuspid valve approach under cardiopulmonary bypass. RESULTS: All operations were successful and 1 infant received another conventional valvuloplasty due to the pulmonary restenosis after initial balloon valvuloplasty and 1 patient's muscular VSD was closed by conventional procedure after unsuccessful device closure. No device-related complications were found during follow-up (1-6 months). CONCLUSION: Our results showed that intraoperative hybrid procedure was feasible and effective in selected patients with congenital heart disease.

Adolescent↗
PubMed1977

Congenital heart disease in high school and college students.

The prevalence and relative frequency of congenital heart disease in high school and college students were investigated during the period April 1970-March 1976. Forty congenital lesions were found among 13,127 subjects which shows a rate of 3 per thousand. The ratio of congenital lesion to rheumatic heart disease was 4/1. Ventricular septal defect was the most common defect, found in 30% of all lesions followed by atrial septal defect (20%) and patent ductus arteriosus (17.5%). The prevalence and relative frequency in those investigated differed only slightly from the results obtained among children. The possibility of spontaneous closure of ventricular septal defect during adulthood strongly suggested itself when compared with our previous data on post middle age subjects.

Adolescent↗
PubMed1985

Systematic diagnostic method of two-dimensional echocardiography in congenital heart disease.

A two-dimensional echocardiographic (2-DE) method of segmental approach for diagnosis of congenital heart disease is presented. Firstly, the visceroatrial situs is determined, i.e., whether it is solitus, inversus, or ambiguous, by identifying the inferior vena cava to right atrium junction. Secondly, it is ascertained whether the ventricular loop is D-loop or L-loop. The ventricular loop is determined by identifying the internal structure of the right and left ventricles by 2-DE. Thirdly, the spatial relationships of the great arteries are determined by identifying the pulmonary artery and aorta. The pulmonary artery is identified by its branches, and the other artery is the aorta. Fourthly, the atrioventricular connection is determined as follows: (1) usual alignment, (2) criss-crossing, (3) straddling, (4) double inlet, and (5) unilateral atrioventricular valve atresia, by using an apical four-chamber echo view. Finally, the ventriculo-great arterial relationship is determined from the apical four-chamber echo view with a tilting maneuver or by echocardiographic recognition of the existence of the fibrous continuities. Following this echocardiographic "five-step approach," hemodynamic considerations should be included, and by integrating all the information the diagnosis of congenital heart disease is completed.

Animals↗
PubMed1985

Congenital heart disease in the adult.

On the basis of literary data and of their own series of 264 adult patients, who were in 1978-1982 subjected to invasive haemodynamic examination for congenital heart disease, 162 patients operated on, and 128 patients followed up for long-term periods after surgical treatment of congenital heart disease in adult age, the authors analyse the causes of late recognition of the disease, operative complications and long-term operative results. It is pointed out that a haemodynamically serious congenital heart disease is indicated for surgical correction even in adult age, and that age itself does not represent a limitation to cardiosurgical intervention. In adult age, it is necessary to anticipate a greater number of complications and a more severe post-operative course, but the long-term results are very good.

Adolescent↗