[Indications for surgical treatment of acquired and congenital heart defects. (I)].
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Heart Defects, Congenital↗
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[What may be expected from the surgical correction of heart defects].
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Heart Defects, Congenital↗
[Surgical possibilities in congenital heart defects with only one ventricle--especially in hypoplastic left heart syndrome].
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Fontan Procedure↗
Influence of prenatal diagnosis on congenital heart defects.
The impact of prenatal detection of congenital heart defects (CHD) using the four-chamber screening examination cannot be accurately ascertained because of the wide range of detection rates that affect the cost associated with it. Assuming a screening ultrasound cost of $200 per examination, recent studies in which examiners not trained in fetal echocardiography obtained and interpreted the four-chamber view only identified 5.3% of CHD, for a cost of $476,190 per malformation. When the four-chamber screening examination was performed by an individual trained in fetal echocardiography, the detection rate increased to 55%, for a cost of $45,454 per malformation. This resulted in a savings of 90%, or $430,736. Because individuals trained in fetal echocardiography are not available to perform and interpret all of the heart screening examinations, another approach is for the fetal echocardiographer to review one to two minute video clips of the four-chamber and outflow tracts screening examination obtained by the individual performing the fetal screening examination. It is estimated that at a charge of $30 per video clip review, the cost to detect 50% of CHD would be $7,500 per defect. This would result in a reduction of 98% for the detection of CHD using current screening methods. This approach would increase the detection rate of CHD by 10-fold, remove the liability of missing CHD from the untrained individual performing the screening examination, and provide revenue to tertiary centers in which individuals skilled in fetal echocardiography could maximize their diagnostic skills.
Cost-Benefit Analysis↗
Prenatal exposures and congenital heart defects in Down syndrome infants.
The purpose of this study was to determine whether there are important differences in maternal and environmental prenatal risk factors between liveborn Down syndrome infants with congenital heart defects and Down syndrome infants without heart defects. Using a case control study design, we evaluated the risk associated with maternal illness, drug ingestion, substance usage, and chemical exposures in the home or workplace. The period of risk selected was 3 months before and 3 months after the last menstrual period, because cardiac development occurs early, before the mother may become aware of her pregnancy. Because fetal survival in Down syndrome may be more vulnerable to various exposures, controls were selected who also had trisomy 21. Of 171 infants studied, 89 were cases with congenital heart disease, and 82 were controls without heart disease. All interviews were performed by one nurse practitioner using a structured standardized questionnaire. Cases and controls had similar maternal ages, family incomes, parental education levels, and contraceptive practices before pregnancy. No differences were found between case and control mothers for maternal illness, medication use, or consumption of caffeinated beverages, cigarettes, or alcohol. Reporting of recreational drug usage was infrequent, may reflect underreporting, and did not differ between cases and controls. Maternal exposures were commonly reported for pesticides (50%), hair dyes (22%), craft paints (8%), varnishes (7%), and solvents (3.5%). However, in none of the categories was maternal exposure significantly more prevalent among case mothers than among control mothers. The failure of this study to identify risk factors for cardiac malformations may be attributable to the small differences in reported frequencies reducing statistical power or to the possibility that cardiac malformation in Down syndrome is a direct result of chromosomal duplication.
Case-Control Studies↗
Transthoracic intracardiac catheters in pediatric patients recovering from congenital heart defect surgery: associated complications and outcomes.
OBJECTIVES: To characterize transthoracic intracardiac catheter uses and associated morbidities in pediatric patients recovering from congenital heart defect surgery and to identify potential risk factors associated with their use. DESIGN: Prospective data collection and review. SETTING: An 18-bed pediatric intensive care unit (PICU) in a tertiary care university hospital. PATIENTS: All pediatric patients between October 1, 1996, and September 30, 1997, who were recovering from congenital heart defect surgery and had transthoracic intracardiac catheters in place. MEASUREMENTS AND MAIN RESULTS: Catheter use, associated morbidity, necessary interventions, and risk factors for complications of catheter use were identified. During this period, 523 catheters (276 right atrial, 155 left atrial, 68 common atrial, and 24 right ventricular or pulmonary artery catheters) in 351 PICU patients were studied. Mean age was 23.1+/-45.1 months (median, 4.98 months); 138 patients (39.3%) were <3 months old. The rate of occurrence of bleeding with catheter removal (mediastinal output in the hour after removal that was more than twice the previous average hourly output) was 36.7%, and bleeding occurred more frequently with left atrial catheters (47%; odds ratio, 2.0; p < .05). However, interventions after catheter removal were required for only 8.3% (42/504) of catheters removed, and hemodynamic compromise occurred with the removal of only 2.6% (13/504) of catheters. Interventions included fluid resuscitation (35 cases), pleural drainage (three cases), catheter wiring for retention (one case), chest tube suctioning (two cases), and surgical removal (one case). No associated deaths occurred. In a multivariate logistic regression analysis, age <3 months (odds ratio, 4.74), catheter location (left atrial: odds ratio, 4.97; pulmonary artery: odds ratio, 12.48), and platelet count of <50,000 (odds ratio, 8.59) were identified as risk factors associated with a need for intervention after catheter removal (p < .05). Other complications included blood cultures positive for organisms (1.5%), thrombus (0.6%), and catheter nonfunction (10.9%). Prematurity was a risk factor for thrombus and nonfunction. CONCLUSIONS: Use of transthoracic intracardiac catheters in pediatric patients is safe. Young infants and pediatric patients with thrombocytopenia or with catheters in the left atrial or pulmonary artery position have a greater need for interventions after catheter removal, warranting added precautions.
Adolescent↗
Pathologic lung function in children and adolescents with congenital heart defects.
Lung function tests (i.e., spirometry, flow volume, and body plethysmography) were performed in 213 patients (age 6-21 years, mean 11.3 years) with hemodynamically significant congenital heart defects: atrial septal defect, ventricular septal defect (VSD), tetralogy of Fallot, aortic stenosis and coarctation of the aorta. We measured lung vital capacity, total lung capacity (TLC), residual volume (RV), the percentage ratio of the latter two measurements (%RV/TLC), maximal expiratory flow rates at 25% and 50% of vital capacity, and specific airway conductance. Pulmonary restriction dominated in patients with tetralogy of Fallot; pulmonary hyperinflation was more frequent in patients with VSD and coarctation of the aorta; and obstruction of the airways was observed most frequently in patients with tetralogy of Fallot. In conclusion, we found a range of pathologic lung function parameters in patients with hemodynamically significant congenital heart defects.
Adolescent↗
Genetic variation in the mitochondrial enzyme carbamyl-phosphate synthetase I predisposes children to increased pulmonary artery pressure following surgical repair of congenital heart defects: a validated genetic association study.
Increased pulmonary artery pressure (PAP) can complicate the postoperative care of children undergoing surgical repair of congenital heart defects. Endogenous NO regulates PAP and is derived from arginine supplied by the urea cycle. The rate-limiting step in the urea cycle is catalyzed by a mitochondrial enzyme, carbamoyl-phosphate synthetase I (CPSI). A well-characterized polymorphism in the gene encoding CPSI (T1405N) has previously been implicated in neonatal pulmonary hypertension. A consecutive modeling cohort of children (N=131) with congenital heart defects requiring surgery was prospectively evaluated to determine key factors associated with increased postoperative PAP, defined as a mean PAP>20 mmHg for at least 1h during the 48h following surgery measured by an indwelling pulmonary artery catheter. Multiple dimensionality reduction (MDR) was used to both internally validate observations and develop optimal two-variable through five-variable models that were tested prospectively in a validation cohort (N=41). Unconditional logistic regression analysis of the modeling cohort revealed that age (OR=0.92, p=0.01), CPSI T1405N genotype (AC vs. AA: OR=4.08, p=0.04, CC vs. AA: OR=5.96, p=0.01), and Down syndrome (OR=5.25, p=0.04) were independent predictors of this complex phenotype. MDR predicted that the best two-variable model consisted of age and CPSI T1405N genotype (p<0.001). This two-variable model correctly predicted 73% of the outcomes from the validation cohort. A five-variable model that added race, gender and Down's syndrome was not significantly better than the two-variable model. In conclusion, the CPSI T1405N genotype appears to be an important new factor in predicting susceptibility to increased PAP following surgical repair of congenital cardiac defects in children.
Carbamoyl-Phosphate Synthase (Ammonia)↗
Maternal occupation and congenital heart defects: a case-control study.
This paper reports a Swedish analysis of the possible effect of maternal occupation on the occurrence of congenital heart defects. The analysis included 3324 infants (1108 cases and 2216 controls) born during the period 1982-1986. Information on maternal occupation was retrieved using two sources: the 1985 census and the Medical Birth Registry. A comparison between these two sources using the information on mothers of 756 infants born in 1986 showed a good concordance although 11% of the women were reported with a different employment status. No association appeared between the type of maternal occupation and the occurrence of infant congenital heart defects although some odds ratios were slightly above 1.
Case-Control Studies↗
[Diagnosis of congenital heart defects in childhood].
Echocardiographic diagnosis of inborn developmental defects of the heart in children differs from the approach used in adults. Examination from the subcostal and suprasternal space is of utmost importance. On using "deductive echocardiography" individual segments of the heart are gradually assessed in the two-dimensional echocardiographic picture. On evaluating pathological changes (particularly borderline values), the use of Doppler signal and one-dimensional imaging is equally important in children as it is in adults.
Child↗
[Hypothermia treatment of junctional ectopic tachycardia after surgical repair of congenital heart defects].
Junctional ectopic tachycardia after surgical repair of congenital heart defects is associated with high mortality. Usually, it is transitory and resolves spontaneously, but a long period with very fast heart rate and without atrio-ventricular synchrony contraction may cause low cardiac output. Treatment with common anti-arrhythmic drugs is often uneffective both in restoring sinus rhythm and in reducing heart rate. Since hypothermia is known to decrease cardiac automaticity, two infants, aged 4 and 10 months, with junctional ectopic tachycardia and low cardiac output after surgical repair of the atrio-ventricular septal defect were treated with hypothermia after unsuccessful pharmacological attempts to control the arrhythmia. Generalized hypothermia was induced with cooling-blankets and ice packs. Rectal temperature initially dropped to 30 degrees and was subsequently maintained at between 33.8 and 34.2 degrees with the heart rate between 130 and 140 m beats per minute. Trans-oesophageal atrial pacing at a higher rate allowed for sequential atrio-ventricular contraction. Signs of low cardiac output were quickly resolved. One patient was warmed-up after 12 hours of hypothermia and remained in sinus rhythm. In the other patient, the arrhythmia recurred after rewarming and a further 30-hour period of hypothermia was required. Sinus rhythm was maintained thereafter. Both patients are in stable sinus rhythm 20 and 22 months after surgery. Our experience supports the use of hypothermia as a means to control post-operative junctional ectopic tachycardia resistant conventional anti-arrhythmic drugs.
Electrocardiography↗
First-year survival of infants born with congenital heart defects in Arkansas (1993-1998): a survival analysis using registry data.
BACKGROUND: In the United States and other developed nations, birth defects are the leading cause of infant mortality. Congenital heart defects (CHDs) are among the most prevalent and fatal of all birth defects. Here we report the survival probability of infants born with CHDs in Arkansas and examine the impact of multiple malformations on survival. METHODS: Birth and death certificate records were linked to birth defects registry data for infants born with CHDs from January 1993 through December 1998 in Arkansas. Both neonatal and first-year survival probabilities were estimated. These were computed non-parametrically using Kaplan-Meier's product limit method. A Cox proportional-hazards model was used to evaluate the relative importance of additional malformations on survival. RESULTS: A total of 1,983 infants with CHDs were included in this study. The neonatal survival probability for this cohort was 94.0% (95% CI: 93.0%, 95.1%), and the first-year survival probability was 88.2% (95% CI: 86.8%, 89.6%). The presence of hypoplastic left heart syndrome conferred the greatest reduction in survival, whereas infants with pulmonic valve stenosis and infants with ventricular septal defects had the highest first-year survival. Infants with multiple CHDs had decreased survival compared to those with isolated heart defects. Survival was also adversely affected by the presence of congenital abnormalities in other body systems. CONCLUSIONS: Neonatal and first-year survival of infants with CHDs varies by both the type of cardiac malformation and the presence of additional cardiac and non-cardiac malformations. Further work will focus on the effects of maternal and infant characteristics on survival.
Abnormalities, Multiple↗
Echocardiographic diagnosis alone for the complete repair of major congenital heart defects.
OBJECTIVES: The study was done to determine the diagnostic accuracy of echocardiography alone in the preoperative diagnosis of children with major congenital heart defects undergoing primary complete repair. BACKGROUND: Although echocardiography is well established as the first-line imaging technique for the diagnosis of all forms of congenital heart disease, most institutions continue to perform cardiac catheterization prior to complete repair of more complex defects. METHODS: To determine the diagnostic accuracy of echocardiography alone and echocardiography plus catheterization, we reviewed the records of 503 children with major congenital heart defects who underwent primary complete repair at our institution between July 1992 and June 1997. We included children with transposition of the great arteries, tetralogy of Fallot, double-chamber right ventricle, interrupted aortic arch, aortic coarctation, atrioventricular septal defect, truncus arteriosus, aortopulmonary septal defect, and totally anomalous pulmonary venous return. We excluded children with less complex defects such as isolated shunt lesions, as well as those with the most complex defects that would require surgical palliation (e.g., functional univentricular heart). We defined major errors as those that increased the surgical risk and minor errors as those that did not. Errors in diagnosis were determined at surgery. RESULTS: Eighty-two percent of children (412 of 503) underwent surgery after preoperative diagnosis by echocardiography alone. There were 9 major (2%) and 10 minor errors in the echocardiography alone group and 7 major and 5 minor errors in the echocardiography plus catheterization group. The most common type of error was misidentification of coronary artery anatomy in patients with transposition of the great arteries. No error in either group resulted in surgical morbidity or mortality. CONCLUSIONS: This study suggests that echocardiography alone is an accurate tool for the preoperative diagnosis of major congenital heart defects in most children undergoing primary complete repair, and may obviate the need for routine diagnostic catheterization.
Adolescent↗
[Pulmonary hypertension and pulmonary vascular disease in congenital heart defects].
Pulmonary vascular disease (PVD) is a serious complication of several congenital heart defects (CHD). The post-tricuspid heart lesions, such as AVSD, VSD, PDA, TGA with VSD, Ao-pulmonary window, Truncus arteriosus, DORV and DILV (univentricular heart) with a high pressure and increased flow in the pulmonary circulation are earlier and more often the cause of PVD than such pretricuspid shunts as ASD or TAPVD. The pathogenesis of PVD is only partially known. The endothelial cell of the pre- and intraacinar arteries releases substances (eicosanoids and mitogens) which cause functional and structural changes in the wall of arteriols and precapillary arteries: media hypertrophy, intima proliferation, obliterations, and necroses. The number and size of small arteries is reduced. Advanced changes are irreversible. Subtle diagnostic tools are necessary to evaluate the clinical, hemodynamic and morphologic status of the pulmonary circulation. Clinical signs, ECG, echocardiogram, x-ray of the chest, cardiac catheterization and special angiograms of the lung vessels have to be performed and their results have to be viewed in a synopsis. The type of tapering of the small arteries in the wedge-angio, the transit time of contrast media in the digital function angio, and changes of pressure and flow under test conditions give further information. Biopsy and histologic studies are difficult and not without risk. Treatment means prevention of advanced changes of PVD. Earlier correction of operable defects, banding of the pulmonary artery in complex heart lesions can avoid the development of PVD. A potent dilator of the small pulmonary arteries, applicable orally and over a long time, is not available at present.(ABSTRACT TRUNCATED AT 250 WORDS)
Arterial Occlusive Diseases↗
[Differences in the incidence of congenital heart defects in boys and girls].
4,409 children born in Bohemia (1974-1983) with heart defect were distributed almost between 2,296 (52.1%) boys and 2,213 (47.9%) girls: The boy:girl ratio was 1.09:1. Boys prevailed in double outlet right ventricle (2.68:1), hypoplastic left heart (2.25:1), transposition of the great arteries (2.11:1), aortic stenosis (1.95:1), pulmonary atresia (1.55:1), tricuspid atresia (1.45:1), coarctation of the aorta (1.30:1) and the corrected transposition (1.25:1). Girls prevailed significantly in patent ductus arterious (1.66:1), Ebstein's anomaly of the tricuspid valve (1.57:1), truncus arterious (1.22:1), atrioventricular septal defect (1.17:1) and tetralogy of Fallot (1.12:1). The difference in the remaining heart defects was less than 10%.
Child↗
Congenital heart defect in a patient with deletion of chromosome 7q.
We describe a premature male infant with a terminal deletion of 7q [del(7) (pter----q34:)]. Manifestations include low birth weight, hypertelorism, bilateral cleft lip and palate, cryptorchidism, and a complex congenital heart defect. The latter consisted of hypoplasia of the main pulmonary artery, absent pulmonary valve, ventricular septal defect, and anomalous right pulmonary artery. We briefly review the spectrum of heart defects seen with chromosome 7 deletions, and comment on the incidence of this unusual heart lesion.
Chromosome Aberrations↗
Constant postoperative monitoring of cardiac output after correction of congenital heart defects.
A new method has been developed that permits constant postoperative monitoring of mean and phasic cardiac output in patients after correction of congenital heart defects. A miniature ultrasound probe is attached to the adventitia of the ascending aorta at the conclusion of the operative procedure. This is connected to the monitoring equipment by means of polyurethane-covered wires that exit the chest wall through a small stab wound. The probe can easily be removed by gentle traction when the patient's condition is stable. The technique was developed, validated, and refined in extensive animal studies, and this report describes the first series of 20 consecutive human implants, performed between August 1984 and September 1985, in which the absolute cardiac output determination obtained with the ultrasound probe at the time of its application was correlated with cardiac output as measured with a standard electromagnetic flow probe. Fourteen male and six female patients (mean age 5.5 years) were studied. Operations performed included eight atrial septal defect repairs, four procedures for tetralogy of Fallot, three ventricular septal defect repairs, three stenotic valve corrections, and two Senning operations. One operative death occurred, but no complications were related to probe application or removal. The average cardiac output in the 20 patients as measured with the ultrasound probe was 2.2 +/- 1.1 L/min (range 0.67 to 5.27 L/min). This is nearly identical to the results noted with the electromagnetic flow probe, where the mean cardiac output was 2.3 +/- 1.2 L/min (range 0.7 to 6 L/min). Regression analysis revealed a high linear correlation (r = 0.9) between the two techniques. A monitor can display the cardiac output trend with 1 minute updates, which greatly enhance management of intravenous drug therapy and volume administration. In conclusion, this new extraluminal removable probe allows virtually continuous monitoring of the postoperative cardiac output after correction of congenital heart defects and should become a standard technique in the postoperative care of these patients.
Adolescent↗
Dermatoglyphic indicators of congenital heart defects in Down's syndrome patients: a preliminary study.
In a preliminary study fingerprint patterns of Down's syndrome children were examined to determine correlations with congenital heart defects (CHD). The results demonstrate that the left hand digit ridge count minus the right hand digit ridge count, and number of ridges on the fifth digit of the left hand separate patients with CHD from those without heart defects (W/OHD). The method correctly classified 92% of the CHD group and 73% of the W/OHD group.
Dermatoglyphics↗