Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “HEART DISEASE, CONGENITAL”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

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

At least 163 records · Page 9Linked to original sources
PubMed2006

Low arterial saturation is associated with increased sensitivity to activated protein C in children with congenital heart disease.

OBJECTIVES: Children with congenital heart disease experience both hemorrhagic and thrombotic complications. In this report the authors test the hypothesis that hypoxemia is associated with altered sensitivity to activated protein C (aPC) in pediatric patients with congenital heart lesions. DESIGN: A retrospective genetic registry review, with statistical evaluation of factors contributing to the aPC ratio. SETTING: Large university hospital. PARTICIPANTS: Cohort of 92 children with congenital heart disease undergoing cardiac catheterization procedures. INTERVENTIONS: The authors measured the aPC ratio at cardiac catheterization and evaluated the contribution of independent variables using linear regression and classification tree approaches. Independent variables included age, gender, use of aspirin, history of thrombosis, room air arterial saturation, factor VIII:C levels, presence of congestive heart failure, and heterozygosity for factor V Leiden. MEASUREMENTS AND MAIN RESULTS: At univariate analysis, factor V Leiden, female gender, room air arterial saturation, age greater than 6 months, and plasma factor VIII:C levels were associated with a lower aPC ratio (resistance to aPC). At stepwise linear regression, arterial saturation, factor VIII:C level, female gender, and factor V Leiden were independently associated with a lower aPC ratio, and these variables explained about 49% of the variability in aPC ratio. The classification tree approach confirmed the dependence of aPC ratio on factor V genotype and arterial saturation. CONCLUSIONS: The aPC ratio in this population is associated with hypoxemia, independent of factors previously observed in adults. Further studies are under way to determine how aPC resistance or sensitivity may independently affect perioperative hemostasis in this population.

Adolescent↗
PubMed1975

Visualization and measurement of the main bronchi by tomography as an objective indicator of thoracic situs in congenital heart disease.

When investigating complex congenital heart disease, determination of atrial situs is essential. Pathological studies have demonstrated that the best predictor of atrial situs is thoracic situs. To assess thoracic situs, bronchial tomography was performed in 92 patients with congenital heart disease. Sixty-four of these, without abnormalities of situs or cardiac position, formed 'normal' controls. The lengths of the left and right main bronchi were measured. When these were related to age, and the results analyzed statistically, linear discriminant equations resulted giving a chance of only 0.09% of misclassifying a bronchus of unknown morphology. The lowest ratio between bronchial lengths (BLR) in any individual was 1.71:1. These results were then used to assess thoracic situs in 17 patients with abnormal situs or CARDIAC POSITION. In 7 (2 with situs inversus), abdominal and thoracic situs agreed. Of 6 patients with bilateral left lung, 3 had an interrupted inferior vena cava. Of 4 patients with presumptive asplenia, 2 had bilateral right lung, but two had thoracic lateralization, one solitus and one inversus. The highest BLR in thoracic isomerism was 1.4:1. This emphasizes the complex interrelation of splenic status, thoracic, and abdominal situs, but demonstrates the value of bronchial measurement particularly in apparent situs indeterminatus.

Adolescent↗
PubMed2005

Illness understanding in adults with congenital heart disease.

BACKGROUND: Adult patients with congenital heart disease need information regarding their clinical diagnosis, medications and side effects, endocarditis prophylaxis, reproductive issues, employment, future surveillance, treatments, and possible reoperations. Accurate understanding of chronic illness in these patients is associated with less distress, less confusion, improved satisfaction with medical care, better compliance with treatment, and a better emotional status, all key factors for good health-related quality of life. The aim of the present study was to assess the level of knowledge that adult patients with congenital heart disease followed in our Center have about their heart condition. METHODS: A questionnaire on knowledge about congenital heart disease was sent by mail to 200 adults affected by a cardiac congenital disease chosen randomly from all patients regularly followed in our department. RESULTS: Patients had good knowledge about the treatment received, the importance of follow-up, the prognosis of their condition, and the possibility of taking part in physical activities. The patients receiving drug treatment were moderately knowledgeable about their treatment. The anatomy of the heart defect, factors contributing to the onset of endocarditis, the impact of smoking and alcohol, and the possible inheritance of the heart condition were poorly understood by the patients. Most of female patients knew that the oral contraceptive pill was the most appropriate method of birth control; most of them were aware that pregnancy would cause additional risks to their health. Multiple logistic analysis showed that four correct answers were related to the age of the patient. CONCLUSIONS: Overall the results indicated that the educational efforts of all the staff (physicians and nurses) have given encouraging results, but there are still significant gaps in knowledge that need more educational work.

Adult↗
PubMed1989

Continuous arteriovenous haemofiltration in the newlyborn with acute renal failure and congenital heart disease.

Newlyborn infants with congenital heart disease who develop acute renal failure are particularly difficult to treat. There are often complex associated medical problems and the mortality is high. Continuous arteriovenous haemofiltration (CAVH) provides a slow and gentle removal of fluid, together with the possibility of correcting metabolic abnormalities. We used CAVH in six newlyborn infants all with severe congenital heart disease, who developed acute renal failure early in life. In four patients it was necessary to insert a blood pump into the circuit to maintain adequate blood flow. CAVH alone, with or without a blood pump, was unable to reduce the plasma urea and creatinine, and in three of the infants, dialysis across the filter was required. CAVH was effective in controlling fluid balance. Although mortality remains high we feel CAVH has an important role in selected patients.

Acute Kidney Injury↗
PubMed1993

Radiofrequency ablation of accessory pathways associated with congenital heart disease including heterotaxy syndrome.

Congenital heart disease complicates the management of most accessory pathway-mediated tachycardias and also increases the challenge of radiofrequency ablation. Since 1990, radiofrequency ablation of accessory atrioventricular (AV) pathways has been attempted in 10 patients (age range 3.5 months to 30 years) with congenital heart disease: Ebstein's anomaly (n = 5), heterotaxy with AV discordance (n = 3), tetralogy of Fallot (n = 1) and total anomalous pulmonary venous return (n = 1). Eight patients had manifest Wolff-Parkinson-White syndrome and 2 had concealed pathways. Five patients had multiple pathways including 4 of the 5 with Ebstein's anomaly. Of 16 pathways total, 15 were associated with the tricuspid valve including all pathways in the patients with Ebstein's anomaly and heterotaxy. The His bundle area was identified in all patients and involved an anterior AV node in 2 of 3 with heterotaxy. Ablation was performed on the atrial side of the AV ring in all cases. No instance of AV block was encountered. Complete success was achieved in 6 patients including the 3 with heterotaxy. In 2 patients, manifest preexcitation was eliminated and clinical symptoms were greatly modified. The procedure was transiently successful in 1 patient who later had surgical interruption of the accessory pathway during tetralogy of Fallot repair. Ablation was unsuccessful in 1 patient. Thus, the overall success was 80%.

Adolescent↗
PubMed1976

Debate on congenital heart disease. Con: early total repair is not always preferable to palliative surgery in congenital heart disease.

While early one-stage repair of congenital heart lesions can now be done with low operative mortality, there are reasons to believe that sometimes a palliative procedure, followed later by complete repair, has some advantages. Some of the drawbacks to early repair such as presumably be abolished as better valves are produced, and better understanding of cardiopulmonary bypass may reduce operative myocardial damage. However, problems related to the very small size of some outflow tracts and pulmonary arteries may not be so easily overcome.

Age Factors↗
PubMed1995

Radical outcome method. A new approach to critical pathways in congenital heart disease.

BACKGROUND: Treatment of congenital heart disease has entered a new era of healthcare delivery and cost containment. Critical pathway method (CPM) has been previously demonstrated by us to produce a significant reduction in average length of stay (ALOS) in hospital of -44%. A new approach, radical outcome method (ROM), has produced comparable results that appear to improve over time. The dynamic nature is examined. METHODS AND RESULTS: Two hundred consecutive patients with congenital heart disease were treated by a single surgeon at a single health maintenance organization (HMO) facility. ROM was used in all patients. This method uses seven critical moments at which shortening rather than confirmation of the ALOS is possible. This process is completed by the second post-operative day. Overall mortality was 1%. The 200 patients were divided into two consecutive groups of 100 patients to determine the effectiveness of ROM over time. Fifty sets were matched. ALOS hospital decreased by 29 days (mean, 0.6 d/set), P < .003. Thirty sets who underwent cardiopulmonary bypass had a 16% decrease (P < .03), and 20 sets in whom nonbypass procedures were performed had a decrease of 16% (P < .02). ALOS in hospital for the 50 sets decreased from 3.7 to 3.1 days (-16%, P < .003). Outcome data demonstrated no significant difference. CONCLUSIONS: ROM, a proactive approach to hospital stay, is a dynamic process that reduces ALOS in hospital. This is achieved by both reducing negative variation in the standard CPM and allowing for positive variation. Outcome data confirm that this approach can reduce ALOS in hospital while providing optimal patient care and family satisfaction, a standard for the new era of healthcare delivery.

Cardiopulmonary Bypass↗
PubMed1988

Determinants of plasma atrial natriuretic factor concentrations in congenital heart disease.

Seventeen children with congenital heart disease undergoing elective cardiac catheterization were studied to define potential hemodynamic and echocardiographic determinants of plasma atrial natriuretic factor (ANF) concentrations. ANF concentrations in the right ventricle and femoral vein were measured by radioimmunoassay. Right ventricular levels were significantly higher than femoral venous levels (p less than 0.01), but the correlation between the 2 was high (r = 0.87, p less than 0.001). Stepwise multiple linear regression analysis demonstrated that right ventricular ANF concentrations were predicted by a combination of indexed right atrial area measured by 2-dimensional echocardiography, right atrial pressure, pulmonary resistance and heart rate (multiple r2 = 0.83). Femoral venous ANF concentrations were predicted by indexed right atrial area and systolic pulmonary artery pressure (multiple r2= 0.79). It was concluded that there are significant independent predictors of right ventricular and femoral venous plasma ANF concentrations in children with congenital heart disease. The right atrial area indexed by body surface area was the only significant predictor of both central and peripheral plasma ANF concentrations in the multivariate analysis.

Atrial Natriuretic Factor↗
PubMed1995

Extracorporeal membrane oxygenation and the treatment of critical pulmonary hypertension in congenital heart disease.

Certain forms of congenital heart disease (CHD) confer a high risk for the development of severe pulmonary hypertension before and after corrective cardiac surgery. Extracorporeal membrane oxygenation (ECMO) has theoretical benefits in the treatment of this complication in that it assures oxygenation, corrects acid-base balance and provides haemodynamic support at the same time as allowing lung rest from ventilation. We examined our experience of the 117 children and neonates supported with ECMO between November 1989 and July 1993. Of these, five received support for critical pulmonary hypertension associated with congenital heart disease. They comprised three who had undergone surgical repair of CHD, one whose total anomalous pulmonary venous drainage was diagnosed and corrected whilst on ECMO and one neonate with functional pulmonary atresia. Pulmonary artery pressure (PAP) was estimated by Doppler echocardiography in all patients and confirmed invasively in two. The median systolic PAP was 46 (range 42-65) mmHg prior to ECMO. The median ratio of pulmonary to systemic arterial pressure (PAP/SAP) was 0.75 (0.70-0.92). Following ECMO of 16-120 h duration, the median systolic PAP was 34 (30-49) mmHg with PAP/SAP 0.50 (0.35-0.60). All patients survived and there were no complications related to ECMO. Extracorporeal membrane oxygenation is an effective treatment in critical pulmonary hypertension and should be considered in all patients in whom this is refractory to conventional measures.

Acid-Base Equilibrium↗
PubMed1977

A new prenatal cardiac diagnostic device for congenital heart disease.

A unique case of congenital heart disease and complete heart block diagnosed by fetal electrocardiogram (ECG) and phonocardiogram recorded initially at 32 weeks gestation is presented. The slow, regular fetal heart rate of approximately 50 beats/min with bizarre QRS and a diamond-shaped murmur beginning with the fetal QRS complex made a diagnosis of complete heart block (CHB) and associated congenital heart disease highly probable. Direct fetal electrocardiograms (FECG) recorded during labor and vaginal delivery at term confirmed the diagnosis of completel atrioventricular block. Cardiac catheterization at 1 day of age showed multiple congenital heart anomalies. The infant developed signs of congestive heart failure and transvenous pacemaker was successfully placed; however, he died at 9 days of age of sepsis and renal failure.

Adult↗
PubMed1994

Neutrophil activation and morbidity in young adults with cyanotic congenital heart disease.

Young adults with cyanotic congenital heart disease have a high incidence of respiratory and haemostatic problems. Activated neutrophils release vasoactive and chemotactic factors which result in endothelial injury, lung parenchymal damage and the activation of platelets and coagulation pathways. To investigate the contribution of neutrophil activation to morbidity in young adults with cyanotic congenital heart disease, plasma neutrophil elastase levels were measured in 25 cyanotic patients and the results compared to patients with acyanotic heart disease and normal controls. Neutrophil elastase levels were significantly elevated in the group with cyanotic congenital heart disease (P < 0.001). Platelet activation was significantly increased in the patients with cyanotic heart disease (P < 0.001). Platelet aggregation was impaired only in those with haematocrits greater than 0.50 (P < 0.02). Whole blood coagulation, as determined by thrombelastography, was within normal limits. The reason for neutrophil activation in patients with cyanotic congenital heart disease is unclear, but activated neutrophils may contribute to the respiratory and haemostatic problems common to these patients.

Adult↗
PubMed1998

Pregnancy and congenital heart disease--maternal and fetal outcome.

Two hundred and seventy five pregnancies in patients with congenital heart disease during 1980-1996 were analyzed retrospectively. Maternal and perinatal outcome was compared in 251 pregnancies of women with acyanotic and 24 pregnancies of women with cyanotic heart disease. Congenital heart disease was diagnosed during the index pregnancy in 26.1% of patients and the majority (88.4%) were in NYHA classes 1 and 2. Atrial septal defect (27.7%) was the most common lesion in women with acyanotic heart disease and the majority with cyanotic heart disease had Eisenmenger syndrome, 13 of 21 (61.9%). Sixty pregnancies occurred in patients with surgically corrected lesions (acyanotic, 56; cyanotic, 4). The incidences of abortions (8.3%), stillbirths (13.6%) and small for gestational age (SGA) (36.4%) were higher in cyanotic heart disease compared to acyanotic heart disease (stillbirth, 0.8%; SGA, 6.9%). There was a statistically significant difference in mean maternal age, mean gestational age and mean birth-weight in the surgically corrected and noncorrected lesions in both acyanotic and cyanotic heart disease. There was 1 maternal death in a woman with Eisenmenger syndrome.

Adult↗
PubMed2006

Pregnancy outcomes in women with congenital heart disease.

BACKGROUND: Pregnant women with congenital heart disease are at increased risk for cardiac and neonatal complications, yet risk factors for adverse outcomes are not fully defined. METHODS AND RESULTS: Between January 1998 and September 2004, 90 pregnancies at age 27.7+/-6.1 years were followed in 53 women with congenital heart disease. Spontaneous abortions occurred in 11 pregnancies at 10.8+/-3.7 weeks, and 7 underwent elective pregnancy termination. There were no maternal deaths. Primary maternal cardiac events complicated 19.4% of ongoing pregnancies, with pulmonary edema in 16.7% and sustained arrhythmias in 2.8%. Univariate risk factors included prior history of heart failure (odds ratio [OR], 15.5), NYHA functional class > or =2 (OR, 5.4), and decreased subpulmonary ventricular ejection fraction (OR, 7.7). Independent predictors were decreased subpulmonary ventricular ejection fraction and/or severe pulmonary regurgitation (OR, 9.0) and smoking history (OR, 27.2). Adverse neonatal outcomes occurred in 27.8% of ongoing pregnancies and included preterm delivery (20.8%), small for gestational age (8.3%), respiratory distress syndrome (8.3%), intraventricular hemorrhage (1.4%), intrauterine fetal demise (2.8%), and neonatal death (1.4%). A subaortic ventricular outflow tract gradient >30 mm Hg independently predicted an adverse neonatal outcome (OR, 7.5). Cardiac risk assessment was improved by including decreased subpulmonary ventricular systolic function and/or severe pulmonary regurgitation (OR, 10.3) in a previously proposed risk index developed in pregnant women with acquired and congenital heart disease. CONCLUSIONS: Maternal cardiac and neonatal complication rates are considerable in pregnant women with congenital heart disease. Patients with impaired subpulmonary ventricular systolic function and/or severe pulmonary regurgitation are at increased risk for adverse cardiac outcomes.

Abortion, Spontaneous↗
PubMed2005

Increased circulating calcitonin gene-related peptide in congestive heart failure caused by congenital heart disease.

Calcitonin gene-related peptide has potent vasodilatory and inotropic actions. The aim of this study was to characterize the changes in this peptide in children with varying degrees of heart failure secondary to congenital heart disease with left to right shunt and to assess its relationship to systolic pulmonary arterial pressure. Plasma calcitonin gene-related peptide levels were measured in 131 children including 13 healthy ones, 43 with various degrees of heart failure secondary to congenital heart disease, and 75 with congenital heart disease without heart failure. In patients with heart failure, calcitonin gene-related peptide concentrations were markedly elevated (15.8 +/- 2.1 pg/mL) as compared with healthy control subjects (7.0 +/- 0.8 pg/mL, P < 0.05) or patients with congenital heart disease but without heart failure (18.6 +/- 1.2 pg/mL, P < 0.01). Compared with the controls, there were highly significant stepwise increases in the calcitonin gene-related peptide levels in the mild (n = 15), moderate (n = 12), and severe (n = 16) heart failure subgroups by 1.5, 1.7 and 3.4 fold, respectively. The plasma calcitonin gene-related peptide levels also correlated directly with the pulmonary arterial systolic pressure (r = 0.515, P < 0.0001). The results of this study indicate that congestive heart failure secondary to congenital heart disease with increased pulmonary flow is associated with elevated levels of calcitonin gene-related peptide that are related to disease severity. Pulmonary overcirculation may play a role in upregulation of calcitonin gene-related peptide in congestive heart failure.

Blood Pressure↗
PubMed1993

Abnormalities in the biosynthesis of thromboxane A2 and prostacyclin in children with cyanotic congenital heart disease.

BACKGROUND: Children with cyanotic congenital heart disease and pulmonary outflow tract obstruction have shortened platelet survival times and are susceptible to thrombosis and organ infarction. Thromboxane A2 and prostacyclin have opposing actions on platelet aggregability and an imbalance in their biosynthesis might contribute to the pathophysiology of these complications. METHODS: Biosynthesis of thromboxane A2 and prostacyclin was investigated in 16 children (4-32 months, median 18 months) with cyanotic congenital heart disease and pulmonary outflow tract obstruction and compared with 16 healthy children of a similar age (6-34 months, median 24 months). Urinary excretion of 2,3-dinor-thromboxane B2 (a metabolite of thromboxane A2) and of 2,3-dinor-6-oxo-prostaglandin F1 alpha (a metabolite of prostacyclin) was measured. RESULTS: The children with cyanotic congenital heart disease and pulmonary outflow tract obstruction excreted more 2,3-dinor-thromboxane B2 than the healthy children: 916(163) compared with 592(122) ng/g creatinine (mean(SEM); 2p = 0.014). The ratio of excretion of 2,3-dinor-thromboxane B2 to 2,3-dinor-prostaglandin F1 alpha was greater in the patients than in the healthy control group (2.38(0.28) v 1.3(0.22)) (2p = 0.002). CONCLUSION: The balance between biosynthesis of prostacyclin and of thromboxane A2 is abnormal in children with cyanotic congenital heart disease and pulmonary outflow tract obstruction and favours platelet aggregation and vasoconstriction.

6-Ketoprostaglandin F1 alpha↗
PubMedDate not supplied

Tracheobronchial compression in acyanotic congenital heart disease.

Children with acyanotic congenital heart disease frequently develop respiratory difficulties such as atelectasis, pneumonia, or infantile lobar emphysema. In some cases, the cause of the respiratory difficulty is compression of the tracheobronchial tree by hypertensive dilated pulmonary arteries, since this type of heart disease frequently demonstrates large left-to-right intracardiac shunts. Sites of predilection for compression include the left main bronchus, the left upper lobe bronchus, the junction of the right bronchus intermedius and right middle lobe bronchus, and the left side of the distal trachea. Cardiac anomalies which predispose to this type of compression include ventricular septal defect, patent ductus arteriosus, interruption of the aortic arch, and tetralogy of Fallot. Pulmonary arteriopexy may relieve the tracheobronchial compression.

Bronchial Diseases↗