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PubMed2004

Phospholipid composition of myocardium in children with normoxemic and hypoxemic congenital heart diseases.

Samples of myocardial tissue were obtained during cardiac surgery from children operated for different types of normoxemic and hypoxemic congenital heart diseases. The phospholipid composition was analyzed by thin layer chromatography. The concentration of total phospholipids (PL), phosphatidylcholine and phosphatidylethanolamine (PE) was found lower in atrial tissue of both normoxemic and hypoxemic groups in comparison with the ventricles. When comparing the difference between hypoxemic and normoxemic defects, hypoxemia was found to increase the concentration of total PL, PE and phosphatidylserine in ventricles and total PL and PE in the atria. The increased level of particular phospholipid species may represent adaptive mechanisms to hypoxemia in children with congenital heart diseases.

Child↗
PubMed2001

Cardiopulmonary transplantation for congenital heart disease in the adult.

BACKGROUND: Patients surviving into adulthood with congenital heart disease (CHD) often succumb to progressive cardiopulmonary dysfunction. For these patients transplantation is often considered. METHODS: We performed a retrospective review of 69 adults (age >18 years) with CHD transplanted between 1984 and 1999. RESULTS: We evaluated 31 heart-lung (HLTxp), 30 lung (LTxp), and 8 heart (HTxp) transplants performed in 22 men and 47 women with CHD. Mean age was 37 +/- 10 years with a mean follow-up of 3.1 +/- 3.5 years. A concomitant cardiovascular procedure was performed in 1 HLTxp, 23 LTxp, and 2 HTxp. Early mortality (>30 days) was 26% (8/31) for HLTxp, mostly due to bleeding. Early LTxp mortality was 23% (7/30), largely due to graft failure. One and 3-year survival was similar in adults transplanted for CHD and adults transplanted for other disease. Early mortality among HTxp recipients was 50% (4/8) from rejection or technical complications. Survival for patients undergoing HLTxp versus LTxp with cardiac repair was similar. When examined by era, the survival of patients transplanted for CHD between 1992 and 1999 was greater than that of patients transplanted between 1984 and 1991. CONCLUSIONS: Adults undergoing HLTxp and LTxp for CHD can expect survival comparable to that of non-CHD adults. In the presence of a reparable cardiac lesion, LTxp with cardiovascular repair for CHD is an attractive option, optimizing organ allocation. Specific technical concerns are discussed. Survival of adults undergoing cardiopulmonary transplantation for CHD has improved over time.

Adult↗
PubMed2001

Structural abnormalities of great arterial walls in congenital heart disease: light and electron microscopic analyses.

BACKGROUND: Great arteries in congenital heart disease (CHD) may dilate, become aneurysmal, or rupture. Little is known about medial abnormalities in these arterial walls. Accordingly, we studied 18 types of CHD in patients from neonates to older adults. METHODS AND RESULTS: Intraoperative biopsies from ascending aorta, paracoarctation aorta, truncus arteriosus, and pulmonary trunk in 86 patients were supplemented by 16 necropsy specimens. The 102 patients were 3 weeks to 81 years old (average, 32+/-6 years). Biopsies were examined by light (LM) and electron (EM) microscopy; necropsy specimens by LM. Positive aortic controls were from 15 Marfan patients. Negative aortic controls were from 11 coronary artery disease patients and 1 transplant donor. Nine biopsies from acquired trileaflet aortic stenosis were compared with biopsies from bicuspid aortic stenosis. Negative pulmonary trunk controls were from 7 coronary artery disease patients. A grading system consisted of negative controls and grades 1, 2, and 3 (positive controls) based on LM and EM examination of medial constituents. CONCLUSIONS: Medial abnormalities in ascending aorta, paracoarctation aorta, truncus arteriosus, and pulmonary trunk were prevalent in patients with a variety of forms of CHD encompassing a wide age range. Aortic abnormalities may predispose to dilatation, aneurysm, and rupture. Pulmonary trunk abnormalities may predispose to dilatation and aneurysm; hypertensive aneurysms may rupture. Pivotal questions are whether these abnormalities are inherent or acquired, whether CHD plays a causal or facilitating role, and whether genetic determinants are operative.

Adolescent↗
PubMedDate not supplied

Neuropathology in patients with congenital heart disease and Down syndrome.

This report examines the relationship between congenital heart disease (CHD) and neuropathological findings in three groups of patients: Down syndrome (45 cases), isolated CHD (296 cases), and CHD with multiple anomalies (92 cases). The increase in brain weight in Down syndrome was similar to control standards up to 1 year of age, after which it was less than normal. Among the three groups, there were differences in frequency in cyanotic CHD, history of operation, and macroscopic and microscopic brain malformations. The incidence of calcification in the brain was increased in Down syndrome. Nine children out of the total cohort had cerebrovascular abnormalities. Although CHD is frequent in Down syndrome, the cerebrovasculature is spared; only infrequent minor abnormalities of the circle of Willis were detected.

Abnormalities, Multiple↗
PubMed2004

Effect of proximity to hazardous waste sites on the development of congenital heart disease.

The authors sought to determine whether the risk of congenital heart disease (CHD) was greater for the children of mothers who lived close to a hazardous waste site (HWS) than for those who lived farther away. All cases (n = 1283) of confirmed CHD, and a random sample of 2,292 controls, born in Dallas County, Texas, from 1979-1984 were linked with 276 HWSs present during the study. The authors ascertained locations of households and determined the distance to the nearest HWS. They obtained odds ratios (ORs) for CHD. A 20% increased risk for CHD was found in association with maternal residence within 1 mile of an HWS (OR = 1.2, 95% confidence interval = 1.1-1.4). The results of this study suggests that a statistically significant additional risk for CHD is associated with maternal proximity to an HWS.

Adult↗
PubMed1996

[The effect of rehabilitation training on postoperative recovery of children with congenital heart disease].

In this study, seven stages rehabilitation exercise were set up according to the rehabilitation training principles. 83 post-operative congenital heart disease children whose age ranging from 4 approximately 12 years were divided into two groups. The results showed that there was a significant difference between rehabilitation training group and control group in the time of ventilator weaning, intensive care, and mean hospitalization. The time of ventilator wearing was 7.34 hours shorter in the study group than that of control group. The time of under intensive care and hospitalization were also 2.04 days and 3.24 days shorter respectively. It is suggested that rehabilitation training has a good effect on post-operative children with congenital heart disease.

Child↗
PubMedDate not supplied

[Systolic and diastolic function of heart ventricles and their remodeling in adult patients with congenital heart disease in phase of Eisenmenger's syndrome].

55 patients with congenital heart diseases (CHD) in phase of Eisenmereger's syndrome (ES) (11 patients with ductus arteriosus (DA), 28--ventricular septal defect (VSD), 16--atrial septal defect (ASD)) at age of 14-53 (mean 29.7+/-3.2) were examined. Heart failure corresponded to I-II functional classes (NYHA) in 28 (50.9%) patients, III-IV--in 27 (49.1%). Diagnosis of ES was confirmed by ECG, Doppler-Echo-CG, catheterization and ventriculography. Moderate right ventricle systolic dysfunction was revealed and confirmed by its dilation and deterioration of inferior vena cava (IVC) (for 156.0% in comparision with healthy people, P<0.001) in patients DA and ASD collapse after inspiration. Unchanged indices of Vmax and Accmax in PA and IVC diameter showed good compensatory potential of RV. There was the initial stage of LV systolic dysfunction (decrease EF for 7.0%, Vmax for 16.7% and Accmax for 21.4%, P<0.05). Diastolic dysfunction of both ventricles in patients with ES is unidirectional and corresponds to relaxation type.

Adolescent↗
PubMed2001

Palliated congenital heart disease.

The group of patients with palliated complex forms of congenital heart disease presents a challenging and difficult management problem during the adolescent years. In patients not considered to be candidates for more fully palliated procedures that separate the circulations, a bidirectional caval pulmonary shunt, often associated with a systemic to pulmonary shunt, may provide significant palliation for several more decades. However, there remain a significant number of patients who, after some years, may develop increasing problems associated with myocardial failure and the development of serious atrial arrhythmias. Interventional cardiac catheterization combined with newer surgical techniques may return many of these patients to more satisfactory hemodynamic states. However, some patients during their adolescent years may eventually require cardiac transplantation for the long-term management of their complex congenital cardiac defects.

Adolescent↗
PubMed1995

Incidence and predictors of postextubation laryngeal edema in pediatric patients with congenital heart disease.

Laryngeal edema developed in 10.1% of studied patients with congenital heart disease after cardiac surgery. The 181 patients were divided into two groups; those with laryngeal edema (group 1) and those without laryngeal edema (group 2). The mean ages in group 1 and 2 were 10 and 22.9 months. Group 1 patients were younger on average than those of group 2 (p < 0.05). The differences in the cardiopulmonary bypass time and anesthesia time between the two groups were not statistically significant. The duration of intubations and ventilatory support before and after the onset of laryngeal edema and the period of the ICU stay were longer in group 1 than in group 2 (P < 0.05). A predictor of postextubation laryngeal edema was not found in our patients from above mentioned parameters. We conclude that the higher incidence of laryngeal edema may be due to young age (most were under 1 year of age), and duration of intubation and ventilatory support.

Adolescent↗
PubMed2000

Incidence and spectrum of congenital heart disease in Oman.

We analysed the incidence and spectrum of congenital heart disease (CHD) in the Sultanate of Oman from 1994 to 1996. CHD was detected in 992 of 139,707 live births (incidence 7.1/1000 live births). The common CHDs were ventricular septal defect (24.9%), atrial septal defect (14.4%) and patent ductus arteriosus (10.3%). The frequency of atrioventricular septal defects (5.9%) was higher than reported from other countries. Age at diagnosis was under 1 month in 38% and 1-12 months in 40%. Cyanotic CHD was found in 21.7% of the whole group and 35% of neonates. Although this was a hospital-based study, we believe we included almost all the infants and children with CHD in the country. The incidence and pattern of CHD in Oman were similar to those reported from developed countries in Europe and America, except for a higher frequency of atrioventricular septal defects. The high prevalence of consanguinity in the country did not affect the overall incidence of CHD.

Health Services↗
PubMedDate not supplied

Enteral nutritional support by percutaneous endoscopic gastrostomy in children with congenital heart disease.

One of the major problems of children with severe congenital heart disease (CHD) is their poor nutritional status. Among other consequences, it influences the surgical outcome. Retrospectively we present our experience with percutaneous endoscopic gastrostomy (PEG) in 15 children with CHD. This technique allows enteral nutritional support without the disadvantages related to long-term nasogastric tube feeding. Major complications were absent, and minor complications were rare both at PEG insertion, which was performed under deep sedation, and during feeding via PEG tube. In 4 of the 8 children who were followed for at least 6 months the age-matched body weight increased more than one standard deviation. In 2 other patients it increased more than 0.5 standard deviations. In 7 children the tube was removed after 2.5 to 42 months since enteral support was no longer necessary. Apart from initial reservations the parental acceptance of PEG was good. We conclude that the PEG is a safe and reliable technique to support enteral nutrition in children with severe CHD.

Enteral Nutrition↗
PubMed1985

Noninvasive evaluation of newborns with suspected congenital heart disease.

In the distressed newborn, the presence or absence of congenital heart disease (CHD) may be difficult to determine on clinical grounds alone. We prospectively studied 104 infants who were younger than 7 days of age and in whom CHD was suspected. The presence of CHD was detected noninvasively in 64 infants. The other 40 infants were considered not to have CHD. None of these 40 infants underwent cardiac catheterization for the purpose of excluding CHD, although three (7.5%) were subsequently found to have incidental cardiac lesions. In nine (14%) of the patients with CHD, the noninvasive diagnosis was incomplete. In four patients, ventricular septal defects were not detected; in another patient, a suspected ventricular septal defect was not confirmed at catheterization. In addition, three abnormalities of the great vessels were misdiagnosed noninvasively. We conclude that cardiac catheterization is not necessary to exclude significant CHD in the distressed newborn resulting from noncardiac causes, but significant diagnostic errors regarding anomalies of the aortic arch and ventricular septal defects may occur, based on the noninvasive evaluation alone.

Cardiac Catheterization↗
PubMed2003

Prevalence of congenital heart diseases in oral cleft patients.

To establish the prevalence of congenital heart diseases (CHDs) in cleft patients, the type of cleft and the presence of a syndrome were coded in 220 patients. A Doppler echocardiogram with color-flow mapping (DE) was obtained in all patients. Mean age was 112.0 +/- 101.2 months (range, 1-576 months), and 56.8% (125) were males. Cleft lip and palate occurred in 144 patients (65.5%), cleft lip in 40 (18.2%), and cleft palate in 36 (16.4%). Cleft palates were more frequent among females. Twenty-four CHDs were diagnosed in 21 of 220 patients (9.5%): 7 mitral valve prolapses, 6 atrial septal defects, 4 patent ductus arteriosus, 3 ventricular septal defects, 2 cases of tetralogy of Fallot, 1 pulmonary stenosis, and 1 bicuspid aortic valve. The presence of CHD did not correlate with the type of cleft. Syndromes occurred in 28 patients (12.7%), and this association was higher among patients with a cleft palate.

Age Factors↗
PubMed2003

Magnetic resonance imaging guided cardiovascular interventions in congenital heart diseases.

The purpose of this article is to present some recent applications of diagnostic and interventional MRI in congenital heart disease. To date x-ray-based techniques have been the norm for most diagnostic and therapeutic applications. With the advent of ultrafast MRI and the development of MRI-compatible catheters and guide wires, the goal of achieving real-time guidance by MRI for interventions in congenital heart diseases has proven feasible. We briefly review the latest advances in cardiovascular MRI, and the development of MR-compatible devices for diagnostic and therapeutic applications such as ASD closure and pulmonary artery dilation.

Cardiac Catheterization↗
PubMedDate not supplied

Nebulized nitroglycerin in children with pulmonary hypertension secondary to congenital heart disease.

Pulmonary hypertension continues to be a major cause of morbidity and mortality, despite new treatments. Since inhaled nitric oxide has been reported to be effective in some cases, we investigated using nebulized nitroglycerine to treat pulmonary hypertension in children with congenital heart disease. Four children (ages 6-72 months) with severe pulmonary hypertension secondary to congenital heart disease (all with membranous ventricular septal defect, undergoing cardiac catheterization) were given 3 cc of nebulized normal saline over 10 min as placebo control, followed by nebulized nitroglycerine (20 micrograms/Kg in 3 cc normal saline). Normal saline administration did not elicit any change, but nitroglycerine administration resulted in the following changes (mean +/- SE, paired statistics): systolic pulmonary artery pressure from 68 +/- 8 to 53 +/- 6 at 10 min into treatment (P 0.006), mean pulmonary artery pressure 47 +/- 4 to 38 +/- 4 (P 0.005), heart rate 131 +/- 8 to 127 +/- 7 (P 0.13), systolic blood pressure 85 +/- 8 to 88 +/- 3 (P 0.7), mean blood pressure 59 +/- 6 to 63 +/- 4 (P 0.5). These results indicate that nebulized nitroglycerine may be an effective, easy to administer, inexpensive, and safe alternative for treatment of severe pulmonary hypertension in children with congenital heart disease, especially in areas where other treatments such as extracorporeal membrane oxygenation or inhaled nitric oxide are inaccessible.

Aerosols↗
PubMed1987

Electrophysiologic effects of intravenous verapamil in children after operations for congenital heart disease.

To assess the effect of verapamil in children with congenital heart disease after surgical correction, an electrophysiologic study was undertaken in 19 patients, 0.8 to 15 years old. Atrial pacing and programmed atrial extrastimulation were performed before and 5 mn after intravenous administration of verapamil. A significant increase in conduction time and refractory periods was observed at the atrioventricular node level. Dual or accessory atrioventricular pathways, present in 14 cases, appear to have been suppressed in 9. The effect on the sinus node was of particular interest: The corrected sinus node recovery time was strongly prolonged by verapamil in 6 patients, 5 of whom had undergone surgery with extensive atrial reconstruction (Senning 2, anomalous pulmonary venous return 3) and 1 of whom had undergone correction of tetralogy of Fallot. Additional effects of verapamil were the disappearance of a sinus node entrance block in 8 patients and emergence of an ectopic pacemaker in 6. Although verapamil has electrophysiologic properties that should make it a useful drug in the treatment of reentry tachycardia, caution is indicated after extensive atrial surgery because sinus node depression may occur.

Adolescent↗
PubMed1984

Two-dimensional echocardiographic assessment of the aorta in infants and children with congenital heart disease.

To determine the accuracy of two-dimensional echocardiography in the identification of congenital anomalies of the aorta, we compared two-dimensional echocardiographic with angiographic results in 261 consecutive infants and children with congenital heart disease (age 1 day to 20 years, mean 3.3 years). Two-dimensional echocardiography was performed and interpreted without knowledge of angiographic results. Complete visualization of the ascending and descending aorta and aortic arch branches was possible by two-dimensional echocardiographic examination in suprasternal, parasternal, and subcostal views of 255 patients (98%). Identification of the esophagus during swallowing aided the diagnosis of anatomic characteristics of aortic arch. One or more significant aortic arch anomalies were present on angiograms of 116 of 255 patients (46%) and were detected by two-dimensional echocardiography in 110 (sensitivity 95%, 99% specificity). Anomalies detected by two-dimensional echocardiography/angiography were ascending aorta hypoplasia in four/four, truncus arteriosus three/three, right aortic arch 31/31, anomalous subclavian artery 11/16, coarctation 27/29, and patent ductus arteriosus 53/57. We conclude that two-dimensional echocardiography can be used to determine the anatomy of the aorta in most infants and children. In selected patients, two-dimensional echocardiography may eliminate the need for angiographic examination before surgery for congenital heart disease.

Adolescent↗
PubMed2004

Reoperations in adults with congenital heart disease: analysis of early outcome.

BACKGROUND: Grown-ups with congenital heart disease (GUCH) are an increasingly important group of patients, with many requiring reoperations because of late complications or residual defects, correction after palliation, further palliation or heart transplantation. METHODS: In order to identify perioperative risk factors, we have retrospectively analysed perioperative mortality and morbidity of 66 consecutive GUCH patients needing reoperations between July 1987 and December 2000 with a mean age of 28+/-12 (14.2-63.5) years and preoperative ejection fraction of 57+/-21%. Primary cardiac defects were LVOT pathology (17 patients), coarctation (10), Tetralogy of Fallot (TOF) (9), VSD (9), transposition of the great arteries (TGA) (7), Marfan syndrome (6), ASD (5) and others (3). RESULTS: Reoperations included various aortic valve procedures (28), aortic replacements (16), ASD/VSD closures (16), conduits (11), RVOT procedures (8), coarctation repair (5), cardiac transplantation (3) or others (8). Early mortality was 7.6%. Serious postoperative complications occurred in 24%. Presence of cyanosis, heart failure, VSD, TGA, pulmonary atresia, correction after palliative surgery and number of previous operations were preoperative risk factors and duration of operation, cardiopulmonary bypass and aortic cross-clamp, core temperature, low output syndrome, use of epinephrine, pneumonia and ARDS, renal failure, dialysis and stroke perioperative risk factors for fatal outcome. CONCLUSIONS: Reoperations in GUCH patients are mostly due to outflow tract lesions, coarctations and TOF. Perioperative risks remain important especially with cyanosis, TGA, pulmonary atresia and poor ventricular function. Therefore, close follow-up, timely referral for re-intervention and adequate perioperative management are mandatory to reduce perioperative risks and improve results.

Adolescent↗