PubMed Health⌕ Search

SEARCH · PubMed Health

Results for “HEART DISEASE, CONGENITAL”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 55 records · Page 3Linked to original sources
PubMed2006

[Prevalence of congenitial kidney and urinary tract anomalies in neonates and infants with congenital heart disease].

INTRODUCTION: Congenital heart diseases are the most frequent anomalies and one of the most important reasons of mortality and morbidity in neonates and infants. Coincidence of additional malformation aggravates prognosis. The frequency of incidence of other anomalies accompanying congenital hart diseases is defined as 11 to 45%. AIM: Estimation of the frequency of a coincidence of congenital kidney anomalies on the basis of the ultrasound examination (US) in children with congenital heart disease in first year of life. MATERIAL AND METHODS: Abdominal US were done in 350 neonates and infants hospitalized in Department of Pediatric Cardiology Medical University of Gdansk in the first twenty-four hours after diagnosis of congenital heart disease. Examinations were performed in 187 male (53%) and 163 female (47%) in average age 54.9 days (range from 1 to 349 days). RESULTS: In examined population congenital kidney anomalies were found in 4.9% of children from which 18% represent hipoplasia, and another 18% dysplasia. 15.3% of examined patients were diagnosed by dilatation of collecting system, whereof 3% mild, 7% moderate, and 5% severe degree. CONCLUSIONS: 1. The frequency of incidence of kidney anomalies and collecting system dilatation in the examined population of neonates and infants with congenital heart disease accounts for 20.2%. 2. Abdominal ultrasound screening with carefully examination of urinary tract should be performed in neonates and infants with congenital heart disease.

Abnormalities, Multiple↗
PubMed2006

[Pulmonary hypertension associated with congenital heart disease].

INTRODUCTION: Congenital heart diseases are among the most common congenital malformations at birth with an incidence of 8/1,000 live births. These defects are characterized by a heterogeneous group of abnormal defects and connections between the cardiac chambers and vessels with different haemodynamic consequences and hence, varying need for follow-up and interventions. The most common forms are congenital cardiac shunts (i.e.: ventricular septal defects, atrial septal defects, patent ductus arteriosus) that account for almost 60% of the malformations. STATE OF THE ART: Pulmonary hypertension remains a major complicating factor of many types of congenital heart disease characterized by a systemic to pulmonary shunt either by causing increased morbidity and mortality during or immediately after surgical repair, or even preventing complete repair for those with advanced pulmonary vascular disease. Similarities in histological lesions between congenital heart disease and idiopathic pulmonary arterial hypertension suggest similarities in pathobiological pathways and thus pulmonary arterial hypertension associated with congenital cardiac shunt has been classified in the group 1 of the classification of Venice. Even if these complex mechanisms are not completely elucidated, the role of hemodynamics (shear stress) in the development of pulmonary vascular disease has been clearly demonstrated. PERSPECTIVES AND CONCLUSION: Until recently, patients with advanced pulmonary vascular disease following congenital systemic to pulmonary shunt had no specific treatment and were managed empirically. Because of the similarities with idiopathic pulmonary arterial hypertension the use of novel therapies suggest new therapeutic possibilities for these patients. Randomized controlled trials are definitely needed to evaluate the safety and efficacy of these therapies in this very particular group of patients, both in the short and long term.

Ductus Arteriosus, Patent↗
PubMed2000

Chronic hypoxemia leads to reduced serum IGF-I levels in cyanotic congenital heart disease.

Cyanotic congenital heart disease in children commonly causes more pronounced growth retardation in comparison with acyanotic congenital heart disease. Chronic hypoxemia has been suggested as the cause of poor growth in these patients, but the relationship between serum IGF-I levels and chronic hypoxemia is unclear. Serum IGF-I concentrations, oxygen saturation and nutritional status were evaluated in 29 patients with cyanotic congenital heart disease, and serum IGF-I levels were compared with a group of 20 well-nourished, age-matched control children to assess the relationship between IGF-I levels and chronic hypoxemia. The nutritional status of each patient was determined by using anthropometric parameters and calorie and protein intake ratios. The patients were divided into malnourished and well-nourished groups (21 and 8 patients, respectively) according to their nutritional status. Serum IGF-I concentrations were measured in the two patient groups and the controls. The malnourished group had the lowest IGF-I levels (48.14 +/- 21.8 ng/ml, p<0.05). However, the well-nourished group's IGF-I levels were significantly lower than the control subjects' despite improved nutritional status (85.5 +/- 30.2 and 107 +/- 19.7 ng/ml, respectively, p<0.05). In addition, we found a positive correlation between serum IGF-I levels and oxygen saturation of the patients (r=0.402, p<0.05). These findings indicate that chronic hypoxemia has a direct or indirect effect to reduce serum IGF-I concentrations and this may be a cause of the increased growth failure in patients with cyanotic congenital heart disease.

Anthropometry↗
PubMed2006

Clinical feasibility of free-breathing, gadolinium-enhanced magnetic resonance angiography for assessing extracardiac thoracic vascular abnormalities in young children with congenital heart diseases.

OBJECTIVE: Congenital heart diseases in pediatric patients are associated with a wide variety of extracardiac thoracic vascular abnormalities. This study analyzed the value of gadolinium-enhanced magnetic resonance angiography during quiet free breathing for assessing extracardiac thoracic vascular abnormalities in young children with congenital heart diseases. METHODS: Fifty-three children with congenital heart diseases (age range, 1 day-40 months; mean, 10.9 months) associated with extracardiac thoracic vascular abnormalities who had undergone both free-breathing, gadolinium-enhanced magnetic resonance angiography and cardiac catheterization, surgical intervention, or both within 2 weeks were reviewed. Diagnostic findings on gadolinium-enhanced magnetic resonance angiography among patients grouped according to 3 major conditions of conotruncal abnormalities (n = 33), aortic or venous abnormalities (n = 11), and pulmonary vascular abnormalities (n = 9), as well as associated extracardiac thoracic vascular abnormality findings, were compared with findings made by using cardiac catheterization, surgical intervention, or both. RESULTS: Extracardiac thoracic vascular abnormality findings on gadolinium-enhanced magnetic resonance angiography were similar to those on catheterization, surgical intervention, or both in patients with conotruncal abnormalities (124 vs 127, P = .083), aortic or venous abnormalities (36 vs 33, P = .083), and pulmonary vascular abnormalities (24 vs 25, P = .317). The overall sensitivity of gadolinium-enhanced magnetic resonance angiography for detection of these lesions identified by means of catheterization, surgical intervention, or both was 97.9%. However, gadolinium-enhanced magnetic resonance angiography revealed 11 additional extracardiac thoracic vascular abnormalities that were not found on cardiac catheterization. The kappa coefficient for the correlation of image quality and diagnostic value of gadolinium-enhanced magnetic resonance angiography by using a 4-point-scale (1 = nondiagnostic to 4 = excellent) assessed by 2 independent reviewers was excellent (mean score = 3.66), with superb interobserver agreement (kappa = 0.727-0.874). CONCLUSIONS: Free-breathing, gadolinium-enhanced magnetic resonance angiography is clinically feasible for detailed anatomic delineation and treatment planning of various extracardiac thoracic vascular abnormalities in young children with congenital heart diseases.

Cardiovascular Abnormalities↗
PubMed1994

[An evaluation of coronary artery lesions of Kawasaki disease and congenital heart disease using rotary three dimensional digital cardiovascular angiography].

Congenital heart disease and the coronary artery lesions of children suffering from Kawasaki disease were evaluated by cardiovascular angiography using a newly developed rotary three-dimensional digital angiography method, and the usefulness of the device was examined. This method enable the observation of lesions from 144 directions within a 180 degree range depicting an image from optimal directions. In addition, the radiation exposure during one angiography was about one fifth of that of conventional cineangiography. With regard to the lesions of the coronary artery, identification of the localization of the stenotic lesions were made possible, especially at bifurcations, or the stenotic lesions overlapping with other bifurcations or coronary artery aneurysms as well as the structure at the ostium of the left and right coronary arteries, which were difficult to identify using conventional coronary artery angiography. For the case of patient ductus arteriosus or major aortopulmonary collateral artery (MAPCA), separation and imaging of the overlap with other blood vessels through the three-dimensional observation became possible. This method is effective for the evaluation of the site, direction and morphology of these arteries. With regard to stenosis of the right ventricular outflow tract, the morphology and the degree of stenosis could be evaluated more accurately than by conventional cineangiography. In addition, the images matched well with the operative findings. This method was also effective for the diagnosis and evaluation of the stenosis at the main pulmonary artery and stenosis of the bifurcation of the right and left pulmonary arteries overlapping with the main trunk of the pulmonary artery.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1993

Inhaled nitric oxide in congenital heart disease.

BACKGROUND: Congenital heart lesions may be complicated by pulmonary arterial smooth muscle hyperplasia, hypertrophy, and hypertension. We assessed whether inhaling low levels of nitric oxide (NO), an endothelium-derived relaxing factor, would produce selective pulmonary vasodilation in pediatric patients with congenital heart disease and pulmonary hypertension. We also compared the pulmonary vasodilator potencies of inhaled NO and oxygen in these patients. METHODS AND RESULTS: In 10 sequentially presenting, spontaneously breathing patients, we determined whether inhaling 20-80 ppm by volume of NO at inspired oxygen concentrations (FIO2) of 0.21-0.3 and 0.9 would reduce the pulmonary vascular resistance index (Rp). We then compared breathing oxygen with inhaling NO. Inhaling 80 ppm NO at FIO2 0.21-0.3 reduced mean pulmonary artery pressure from 48 +/- 19 to 40 +/- 14 mm Hg and Rp from 658 +/- 421 to 491 +/- 417 dyne.sec.cm-5.m-2 (mean +/- SD, both p < 0.05). Increasing the FIO2 to 0.9 without adding NO did not reduce mean pulmonary artery pressure but reduced Rp and increased the ratio of pulmonary to systemic blood flow (Qp/Qs), primarily by increasing Qp (p < 0.05). Breathing 80 ppm NO at FIO2 0.9 reduced mean pulmonary artery pressure and Rp to the lowest levels and increased Qp and Qp/Qs (all p < 0.05). While breathing at FIO2 0.9, inhalation of 40 ppm NO reduced Rp (p < 0.05); the maximum reduction of Rp occurred while breathing 80 ppm NO. Inhaling 80 ppm NO at FIO2 0.21-0.9 did not alter mean aortic pressure or systemic vascular resistance. Methemoglobin levels were unchanged by breathing up to 80 ppm NO for 30 minutes. CONCLUSIONS: Inhaled NO is a potent and selective pulmonary vasodilator in pediatric patients with congenital heart disease complicated by pulmonary artery hypertension. Inhaling low levels of NO may provide an important and safe means for evaluating the pulmonary vasodilatory capacity of patients with congenital heart disease without producing systemic vasodilation.

Administration, Inhalation↗
PubMed1982

Coeliac disease associated with congenital heart disease.

Six children with congenital heart disease are described in whom poor growth was found to be due to small-bowel villous atrophy; none was in heart failure and only one was severely cyanosed. Growth improved in all 6 on a gluten-free diet. Gluten enteropathy may be more common than is realised in children with congenital heart disease, and jejunal biopsy should be undertaken early in any patient with poor growth and no heart failure to exclude the coexistence of the condition.

Celiac Disease↗
PubMedDate not supplied

Pediatric heart transplantation: surgical considerations for congenital heart diseases.

Congenital anomalies of the atrium, of the pulmonary and systemic venous return, and of the great vessels are sometimes regarded as contraindications to heart transplantation. Among 10 children who underwent heart transplantation in our institution, five, aged 4 to 15 years and weighting 9 to 32 kg, previously operated on, had congenital anomalies. These lesions, encountered singly or in association, were single atrium (2), previous Mustard operation (1), hypoplastic left atrium (1), anomalous systemic venous return (3), anomalous pulmonary venous return (2), transposition or malposition of the great vessels (5), pulmonary artery hypoplasia (1). To enable correction of these lesions and to perform heart transplantation, the donor heart preparation was modified in four ways. The surgical techniques used for correcting these anomalies in this group were atrial septation, atrial enlargement, superior and inferior reroofing, double venous rerouting, septal realignment, full-length mobilization of the great vessels, and pulmonary artery reconstruction. All children survived operation and left the hospital in excellent condition. Follow-up ranged from 6 months to 3 years. All children are totally asymptomatic. As demonstrated by echocardiography, heart catheterization, and angiography, there are no stenoses, no shunts, and there is good atrial size and good orientation of the great vessels. On the basis of our experience, we conclude that with appropriate surgical techniques, most atrial, venous return, and intrapericardial great vessel anomalies are correctable at the time of orthotopic transplantation and that these techniques allow for a successful outcome in children.

Anastomosis, Surgical↗
PubMedDate not supplied

Contemporary thinking for congenital heart disease.

Understanding congenital heart disease (CHD) in terms of three physiologic categories provides the pediatric nurse with practical, effective interventions for sick children. Children with increased pulmonary blood flow require support for congestive heart failure (CHF) symptoms including medications, increased caloric intake, and organization of care. Children with significantly decreased pulmonary blood flow need surgical intervention to create a path for pulmonary blood supply. Children with obstruction to systemic blood flow require support for both CHF symptoms and poor ventricular contractility. Shifting the focus of understanding from cyanotic and acyanotic defects to these three physiologic categories improves the pediatric nurse's capability to plan and care for children with CHD.

Female↗
PubMed2006

Right-sided Maze procedure for atrial tachyarrhythmias in congenital heart disease.

BACKGROUND: Congenital heart disease (CHD) that causes right atrial dilatation is commonly associated with atrial flutter and/or fibrillation (AFl/F). To reduce late recurrence of AFl/F in patients undergoing repair of CHD, we utilized a concomitant right-sided maze procedure. METHODS: From 1993 to 2003, 99 patients with CHD and associated AFl/F underwent a concomitant right-sided maze procedure at the time of CHD repair. Ages ranged from 9 to 72 years (median, 43 years). Atrial flutter and/or fibrillation was paroxysmal in 81 and chronic in 18; duration ranged from less than 1 month to 39.5 years (median, 2.9 years). Primary cardiac diagnoses were Ebstein anomaly (n = 47), other congenital tricuspid regurgitation (n = 19), univentricular heart (n = 11), isolated atrial septal defect (ASD, n = 8), tetralogy of Fallot (n = 8), and other (n = 6). RESULTS: Other concomitant procedures included tricuspid valve repair or replacement (n = 70), ASD closure (n = 39), and pulmonary valve procedures (n = 18). There were 6 early deaths. At hospital dismissal, 83 patients were free of AFl/F and 63 were in sinus rhythm. Follow-up in 87 of the 93 early survivors extended up to 8 years (mean, 2.7 years). There were 4 late deaths, all from noncardiac causes. Of the 83 known late survivors, 77 (93%) were free of AFl/F. Eighty-two of the 83 survivors were in New York Heart Association class I or II. CONCLUSIONS: In patients with AFl/F associated with CHD, a concomitant right-sided maze procedure at the time of intracardiac repair is effective in reducing late recurrent AFl/F. Most patients enjoy an excellent quality of life.

Adolescent↗
PubMed2005

Comparison of the acute pulmonary vasodilating effect of beraprost sodium and nitric oxide in congenital heart disease.

BACKGROUND: Congenital heart disease patients who have pulmonary hypertension (PH) require an evaluation for pulmonary vascular reactivity before surgical repair. In the present study the acute pulmonary vasodilating effects of 100% oxygen (O2), beraprost sodium (BPS) and 40 ppm inhaled nitric oxide (iNO) during cardiac catheterization were compared. METHODS AND RESULTS: There were 90 patients who underwent cardiac catheterization for evaluation of PH (mean age, 16.5+/-16 years). The baseline mean pulmonary artery (mPA) pressure was 69.6+/-14.8 mmHg and the pulmonary arteriolar resistance (Rpa) was 13.8+/-8.3 Wood unit m2. Change in pulmonary vascular reactivity was defined as a decrease in mPA or Rpa>20% from baseline. The response to 100%O2, iNO and BPS during cardiac catheterization was 84%, 72.7% and 64%, respectively. Pair comparisons among each hemodynamic parameter showed no difference between the acute vasodilating effect of BPS and iNO. In some patients BPS showed a stronger effect than iNO in lowering Rpa. CONCLUSIONS: BPS has a similar pulmonary vasodilating effect to iNO and can be used as an acute pulmonary vasodilating agent during cardiac catheterization with potential benefits over iNO.

Adolescent↗
PubMed2002

Family stress, perceived social support and coping following the diagnosis of a child's congenital heart disease.

BACKGROUND: Congenital heart disease (CHD) is now estimated to be the second most prevalent chronic illness. A child's chronic illness may have effects that have pervasive consequences for family life. Recently, attention has focused on resiliency variables, especially social support and coping strategy, regulating the impact of stress. In the resiliency model of family stress, adjustment and adaptation, social support is viewed as one of the primary moderators or mediators between stress and well-being. AIMS: The purpose of this study was to explore the relationships of family stress, perceived social support, and coping and determine the resiliency factor associated with coping by families who have a child with chronic illness. DESIGN: In a secondary analysis of a large longitudinal study, the sample consisted of 92 families who had a child under age 12 who was newly diagnosed with CHD within the last 3-4 months. FINDINGS: Results from regression analysis revealed that perceived social support operated as a resiliency factor between family stress and both parental and family coping. Child and family characteristics appeared to be the important predictors of perceived social support and parental coping. Although perceived social support appeared to be an important predictor of parental and family coping, neither the moderating nor mediating model was supported in full but partial causal relations were confirmed. CONCLUSIONS: Findings provided evidence for the theoretical and empirical significance of perceived social support as a predictor of family coping. Further, these findings suggest that perceived social support is a factor influencing the resiliency of relatively high-risk groups of families who have a child with chronic illness.

Adaptation, Psychological↗