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.

SourceResearch connectionFrom yearThrough year

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

At least 847 records · Page 47Linked to original sources

PubMed2005

Long-term and developmental outcomes of children with complex congenital heart disease.

As short-term survival of complex congenital heart disease continues to improve dramatically with advances in medical and surgical treatment, further efforts must be made to understand the long-term outcomes of our efforts. As survival continues to improve, cardiovascular morbidity and, equally importantly, neurodevelopmental and social outcomes must be a continual focus in our treatment of these complex patients. Further study of these effects is underway, and more is certainly warranted. Understanding should lead to modification of current techniques and management strategies, all with the ultimate goal of improving our patients' quality of life.

Child↗

PubMedDate not supplied

Effects of pentoxifylline (Trental) on blood flow, viscosity, and oxygen transport in young adults with inoperable cyanotic congenital heart disease.

Four polycythemic young adults with inoperable cyanotic congenital heart disease were treated for 12 weeks with 20 mg/kg/day of pentoxifylline in an attempt to increase pulmonary blood flow by improving red blood cell demorability and decreasing whole blood viscosity. Treatment caused a rise in mean arterial oxygen saturation from 75-82%, a fall in the mean oxygen extraction coefficient from 47-40%, and a fall in mean oxygen consumption from 186-169 ml/min/m2. Pentoxifylline decreased whole blood, but not plasma, viscosity at all hematocrits over a range of shear rates. Two patients had significant bleeding episodes during treatment.

Adolescent↗

PubMed1975

Silent anomalies of the urinary tract and congenital heart disease.

Postangiocardiographic screening of infants and children with congenital heart disease for silent anomalies of the urinary tract yielded an incidence of 7.7 percent in 260 so studied. The incidence was 29 percent in 21 babies who came to autopsy and had been studied at catheterization but without this screening. Patients with atrial septal defect had the highest incidence of urinary tract anomalies, while those with ventricular septal defect and tetralogy had an average incidence. Some of the lesions discovered were potentially significant even though silent. They were found with sufficient frequency to warrant routine use of this screening technique at cardiac catheterization.

Abnormalities, Multiple↗

PubMed2005

[The role of echocardiography as an isolated method for indicating surgery in patients with congenital heart disease].

OBJECTIVE: To determine the diagnostic accuracy of echocardiography for indicating surgery without catheterization in patients with congenital heart disease through a prospective analysis and comparison of the echocardiographic diagnosis with the intraoperative findings, or invasive study, or both. METHODS: From February 2000 to January 2001, 493 patients with congenital heart diseases indicated for surgery were followed up. They underwent echocardiography with color-flow mapping for a therapeutic decision. The results were compared with the findings of surgery or catheterization, or both, when the latter were performed for diagnostic reasons. RESULTS: Of the patients studied, 94.3% (465 cases) underwent congenital heart disease correction based only on echocardiographic findings, without a diagnostic catheterization. The invasive study was performed for diagnostic reasons in 28 (5.6%) patients, the surgical treatment was performed in more than 95% of the patients, and therapeutic catheterization was performed in 3.6%. The echocardiographic findings were confirmed in 464 (94.1%) patients, which showed the high accuracy of the method. False-positive findings occurred in 8 (1.6%) patients, and false-negative findings in 39 (7.9%). According to surgeons, no diagnostic error led to complications or adversely affected the surgical results. CONCLUSION: Echocardiography proved to be a sensitive and safe method for indicating surgery, making catheterization, often, unnecessary. The invasive study was restricted to diagnostic or therapeutic uses.

Adolescent↗

PubMed1999

Impact of rifampin on serum amiodarone concentrations in a patient with congenital heart disease.

A 33-year-old woman with congenital heart disease and atrial and ventricular arrhythmias, managed over the long term with an implantable cardioverter defibrillator, epicardial pacing system, and amiodarone, experienced an increase in palpitations and a shock from her defibrillator. Evaluation revealed decreases in amiodarone and desethylamiodarone serum concentrations from previous levels. Rifampin had been added to her therapy 5 weeks earlier. Increases in amiodarone and desethylamiodarone concentrations were observed after an increase in the amiodarone dosage and discontinuation of rifampin. The time course suggested that the addition of rifampin led to reductions in serum concentrations of both the drug and metabolite.

Adult↗

PubMed1986

Two-dimensional/Doppler echocardiography and its relationship to cardiac catheterization for diagnosis and management of congenital heart disease.

The impact of echocardiography on cardiac catheterization in an individual patient with congenital heart disease depends on a number of factors, including the availability of an echocardiographer who is experienced in congenital heart disease and of appropriate "state of the art" equipment, and willingness of clinicians and surgeons to accept echocardiographic data. The latter is influenced by factors such as completeness of the echocardiographic data, familiarity with tomographic cardiac anatomy, and the perceived risk of an invasive procedure. The combined use of echocardiography and catheterization will have an increasing impact on the management of these patients, significantly altering the indications and extent of a catheterization procedure. This will lead to a more goal-directed examination. The relationship between catheterization and 2-D/Doppler echocardiography should not be adversarial, but complementary. Increasingly, noninvasive techniques will support or complement the invasive procedure, particularly as echo technologies for invasive applications are further developed.

Cardiac Catheterization↗

PubMed1988

Chemical quality of maternal drinking water and congenital heart disease.

We undertook a case-control study to investigate the association between chemicals in maternal drinking water consumed during pregnancy and congenital heart disease in the offspring. Two hundred and seventy affected children and 665 healthy children were enrolled in the study. Information on contaminant levels in maternal drinking water was available from records of routine water analysis of samples taken from public taps in the communities where the mothers resided during pregnancy. Mothers provided information during a telephone interview on their health, pregnancy management, and demographic characteristics. Nine inorganic metals were analysed for detection of an association with congenital heart disease. The chemical exposures of particular interest were arsenic, lead, mercury and selenium. None of the chemicals was associated materially with an increase in the frequency of congenital heart disease overall. Arsenic exposure at any detectable level was associated with a threefold increase in occurrence of coarctation of the aorta (prevalence odds ratio = 3.4, 95% confidence interval = 1.3-8.9). Detectable traces of selenium in drinking water were associated with a lower frequency of any congenital heart disease than was observed among children exposed to drinking water not containing detectable levels of selenium (prevalence odds ratio = 0.62, 95% confidence limits = 0.40-0.97). A dose-response effect was observed over four levels of selenium exposure. Non-differential errors in the measurement and classification of exposure to contaminants routinely monitored in drinking water could account for lack of positive findings. In addition, most of the contaminant levels were below the maximum levels set by the Environmental Protection Agency, so that lack of evidence of effect may have been due to the low exposure levels in this population.

Arsenic↗

PubMed1999

[Interventions in congenital heart disease and their sequelae in adults].

The advancements of cardiac surgery over the last decades led to larger numbers of patients with operated congenital heart diseases surviving into adulthood. In Germany it is estimated that over 120,000 adults have operated congenital heart diseases. Five to 7% of them will need yearly hospital admissions. Interventional procedures are additional tools used to treat these patients with various sequelae or residua (Table 1). In the following review we concentrate on 2 different interventional procedures: dilatation and stent implantations for treatment of stenosis and the different devices used for the closure of shunt lesions. For congenital valvular pulmonary stenosis, balloon dilatation is the therapy of choice regardless the age of the patient. Stent implantation for the treatment of peripheral pulmonary stenosis (e.g., after previous systemic pulmonary shunts) can decrease the need for redo surgery, which is accompanied with increased risk. Stent implantations proved also to be useful to treat stenoses after Mustard patch in patients with transposition of the great arteries, after Fontan procedures or dealing with the rare pulmonary venous stenosis. In contrast, dilatation of bioprosthesis and conduit stenosis are not promising. Balloon dilatation of valvular aortic stenosis is an accepted therapy in childhood up to adolescents. Table 2 compares a surgical series including many infants with critical aortic stenosis with a series of balloon dilatation in children and another one in adults regarding lethality, complications, and results. Table 3 illustrates the immediate and late results of balloon dilatation of aortic coarctation in 3 different studies. The high recurrence rate in infants made clinicians refrain from taking this age group for balloon dilatation. In children and adult patients, good results are reported (75% reduction of gradients). The complication rate is low (2.3 to 3.3%) and aneurysm formation rate seldom (1 to 7%). Stenosed aorto-pulmonary collaterals will rarely need balloon dilatation. Surgical closure of atrial septal defect is a low risk procedure with a very low rate of residual shunts (2%). Of the 5 available devices for transcatheter closure of atrial septal defect Type II, only 2 occluders are in use in Germany, the Clamshell and the Amplatzer device. The largest clinical studies of the different systems, their efficacy, complications and residual shunt rate are presented in Table 4. For the deployment of these occluders a TEE is always needed. There are many more systems in clinical use to close the patent arterial duct (PDA) (Table 5). The Ivalon plug as well as the Rashkind device have probably only historical value. Different types of coils (Gianturco, Cook detachable, PFM) are now in use worldwide. The reason for their widespread use, besides their easy application, is the fact that most coils are relatively cheap and need only small sheaths for deployment. Their further evaluation identified a residual shunt rate of 5% as well as a number of complications (embolization, hemolysis, stenosis of the left pulmonary artery) in 0 to 6%. For the large PDA the Amplatzer device has recently been introduced. An additional indication for the use of the different occluding devices are aorto-pulmonary collaterals, venovenous fistulae, pulmonary or coronary artery fistulae. Aorto-pulmonary collaterals are often associated with complex cardiac lesions and occasionally appear after palliative procedures. An excellent cooperation between adult and pediatric cardiologists is needed in order to offer the group of adults with congenital heart diseases an adequate and comprehensive management.

Adult↗

PubMedDate not supplied

Inhaled prostacyclin following surgical repair of congenital heart disease--a pilot study.

BACKGROUND: The development of additional therapies for the treatment of pulmonary hypertension would be a significant advancement in the treatment of congenital heart disease. Recently, studies have found inhaled prostacyclin (PGI2) is an effective pulmonary vasodilator, comparable with nitric oxide. In this prospective interventional pilot study, we examined the physiologic effects of inhaled PGI2 in children with congenital heart disease and pulmonary hypertension. METHODS: Six children (median age 6 months, range 5 to 21 months) with congenital heart disease and preoperative pulmonary hypertension (mean pulmonary artery pressure [MPAP] greater than 50% systemic) received a 15-minute course of inhaled PGI2 intraoperatively postrepair. The inhaled PGI2 was delivered by aerosolizing the IV formulation (Flolan, Glaxo-Wellcome) to achieve a dose of 50 ng/kg/min. Physiologic parameters measured during the medication period were compared with measurements taken during two 15-minute baseline periods before and after the medication period. RESULTS: Inhaled PGI2 significantly reduced the mean pulmonary artery pressure from 25 +/- 3 to 21 +/- 3 (p < 0.01) and improved the PaO2/FiO2 ratio from 275 +/- 181 to 433 +/- 285 (p = 0.01). There were no significant changes in systemic blood pressure, heart rate, or cardiac index. CONCLUSIONS: Children with congenital heart disease and pulmonary hypertension may benefit from inhaled PGI2. Inhaled PGI2 reduced pulmonary blood pressures and improved oxygenation in this small study. PGI2 acts through cyclic adenosine monophosphate mediated pulmonary vasodilation, a mechanism different from nitric oxide. In children with inadequate response to nitric oxide, inhaled PGI2 may be a useful alternative pulmonary vasodilator.

Administration, Inhalation↗

PubMed1980

Some epidemiological aspects of congenital heart disease in Denmark.

Information on 5249 children with congenital heart disease (CHD) in the age group 0-15 years was collected from all paediatric and cardiological departments in Denmark and, furthermore, from death certificates. The mean prevalence for CHD in Denmark was 6.1% during 11 years (1963-73); during the years 1972-73 it was 7.0%. The mean age at detection was 25.86 months; 36% were diagnosed within the first month of life and 63% within the first year. The sex distribution was; 52%, female, 48% male. No seasonal variation was found in the months of birth for individual cardiac anomalies. The largest group of CHD was ventricular septal defect, 24.0%, followed by persistent ductus arteriosus, 12.6%; atrial septal defect, 9.2%; coarctation of the aorta, 7.0%; pulmonary stenosis, 6.0%; and tetralogy of Fallot, 5.8%. The cumulated lethality for all 5249 children ws 25% at 1 month, 42% after 5 years, and 48% at the age of 15 years. CHD was found in 135 cases among 5835 siblings (2.3%).

Adolescent↗

PubMed1978

Intestinal function in infants with severe congenital heart disease.

We evaluated digestive tract function in 21 young infants with severe congenital heart disease. One group had congestive heart failure and ventricular septal defect or single ventricle; the other had cyanosis and transposition of the great arteries. Enteric protein loss was excessive in eight patients, and steatorrhea was found in five. These abnormalities were mild and not related to the type or severity of the cardiac lesion. Available evidence points to a need of these babies for calories in excess of normal requirements for weight. The present study suggests that in designing dietary regimens for these very sick patients, their potential for defective gastrointestinal function must be considered. Because no consistent pattern of abnormalities in apparent, each patient who fails to thrive may deserve gastroenterologic evaluation.

Body Weight↗

PubMed1997

Molecular genetics of congenital heart disease.

This review of advances made toward understanding the molecular basis of congenital heart disease covers studies on subjects ranging from atrioventricular septal defects to zebrafish models. Genetically abnormal mice with atrioventricular septal defects have abnormal endocardial cushion development with the delayed appearance of mesenchymal cells and certain critical adhesion proteins. The prevalence of 22q11 deletions among patients with the conotruncal defects was estimated at 8% to 17%. Deletions were rare among patients lacking typical DiGeorge syndrome (DGS) or velocardiofacial (VCF) dysmorphic features, and more common in tetralogy of Fallot with pulmonary atresia than tetralogy of Fallot alone. Studies with patients with unusual 22q11 defects revealed that regional effects on several genes seem to underlie these complex phenotypes. A second DGS/VCF region on chromosome 10p13 was defined molecularly. Laterality defects (heterotaxy) have been associated with connexin43 mutations, and mice lacking connexin43 developed pulmonary atresia with intact ventricular septum. Three other groups failed to find connexin43 mutations in heterotaxy patients, suggesting genetic heterogeneity. Studies of cardiac looping with lower vertebrates revealed the critical role of the notochord. Ellis-van Creveld syndrome, an autosomal dominant skeletal dysplasia with atrial septal defects, and familial total anomalous pulmonary venous return, an autosomal dominant trait with reduced penetrance, were genetically linked to chromosomal bands 4p16 and 4p13-q12, respectively. The zebrafish has emerged as an important model for the study of the earliest stages of the cardiovascular system, and the miles apart and gridlock mutants, which have failure of heart tube fusion and aortic atresia, respectively, are discussed.

Animals↗

PubMed1985

Postoperative exercise rehabilitation benefits children with congenital heart disease.

Physical fitness was assessed in children with congenital heart disease using seven tests designed to measure cardiovascular endurance, strength, flexibility and co-ordination. Test scores for age-matched healthy children were significantly higher than preoperative scores for patients. Postoperatively, the patients were divided into a control group and a physical training group. The training group was subsequently divided into compliant and non-compliant subgroups. Patients who complied with a simple six-week home program of physical activity training after surgery improved their test scores significantly (p less than .001) and were not significantly different from normal children. The scores of the non-compliant group remained below normal (p less than .01). When retested six months postop, the scores of all patient groups had improved; however, while there was no significant difference between the healthy and compliant groups, the scores of the non-compliant and control groups remained significantly below those of the healthy children (p less than .01).

Adolescent↗

PubMed1994

Children with congenital heart disease: a nutrition challenge.

Growth in children with congenital heart disease (CHD) is often compromised. For several decades, investigators have tried to identify the factors affecting growth in children with CHD. Cardiac malformations are undoubtedly responsible for malnutrition, which may range from mild undernutrition to severe failure to thrive (FTT). Malnutrition may then significantly undermine the outcome of corrective surgical operations and postoperative recovery. Mechanisms linking CHD to malnutrition may be related either to decreased energy intake and/or to increased energy requirements. Decreased energy intake can involve deficiencies of specific nutrients, or insufficient total caloric intake. Increased respiratory rate accompanying congestive heart failure may be responsible for increased energy requirements. Different types of cardiac malformations and consequent interventions may have different effects on growth and require diverse strategies. Most treatment strategies aim to facilitate "catch-up" growth, providing extra calories and protein that exceed the Recommended Dietary Allowance for age. However, there is no generally accepted set of guidelines that define appropriate caloric intake for catch-up growth. We attempt to identify the most important causes of malnutrition and highlight the most effective nutrition strategies for children with CHD.

Energy Intake↗

PubMed2004

Venovenous extracorporeal membrane oxygenation for cyanotic congenital heart disease.

BACKGROUND: Severe, refractory hypoxemia complicating uncorrected cyanotic congenital heart disease is a potentially lethal condition, even when urgent surgical intervention is undertaken. When a viral pneumonia initiates hypoxemia, the likelihood of a satisfactory outcome is further reduced. We examined our policy of venovenous extracorporeal membrane oxygenation support through the hypoxic event and performing delayed surgery, if required, to separate from extracorporeal membrane oxygenation. METHODS: A single institution, retrospective review of an Institutional Review Board approved database was undertaken. Over a 6-year period, 18 instances were identified for 17 patients who became acutely hypoxemic from either inadequate pulmonary blood flow (8 instances) or a viral pneumonia (10 instances) complicating their cyanotic heart disease. Demographics, duration of venovenous extracorporeal membrane oxygenation and outcomes are reported. RESULTS: The length of venovenous extracorporeal membrane oxygenation ranged from 13.5 to 362.5 hours (mean 130 +/- 121 hours). During 10 supports, operations were performed to facilitate weaning from support. In 7 patients, extracorporeal support was weaned during this surgery. Follow-up was obtained in all patients over a period ranging from 4 months to 7 years (mean 39.0 +/- 23.0 months). There were two late deaths due to sepsis 1.4 and 2.5 months after extracorporeal support. CONCLUSIONS: Venovenous extracorporeal membrane oxygenation allows time for the recovery of acute hypoxic insult and resolution of some viral pneumonia processes. Palliative surgical procedures may be safely undertaken during extracorporeal support. Viral pneumonia is a risk for prolonged support. Venovenous extracorporeal membrane oxygenation is useful in these high-risk patients.

Child, Preschool↗

PubMed1983

Surgical treatment for severe congenital heart diseases.

The surgical results in patients with severe congenital heart disease, who underwent surgical treatment between 1978 and 1981 at the National Cardiovascular Center, Japan, were analyzed. The surgical mortality rates were 4% for cases of ventricular septal defect associated with pulmonary hypertension in patients under 2 years of age, 16.7% for complete atrio-ventricular canal, 11% for coarctation of the aorta associated with ventricular septal defect, 40% for pure pulmonary atresia without Ebstein anomaly, 44% for total anomalous pulmonary venous return, 14.8% for transposition of the great arteries, 44% for double outlet right ventricle and 1.1% for tetralogy of Fallot. The surgical results have been improving and postoperative residua and sequelae have been decreasing through our persistent efforts. Today's main problem is a relatively poor performance in cases which need surgical intervention early in life, i.e., under 3 months of age. Therefore, we think that our effort to improve the surgical results for new-borns or very young infants is most necessary.

Aortic Coarctation↗

PubMed2003

A new echocardiographic approach in assessing pulmonary vascular bed in patients with congenital heart disease: pulmonary artery stiffness.

OBJECTIVE: The state of pulmonary vascular bed in congenital heart disease is the predictor of the patients clinical condition, prognosis and outcome of surgical intervention. This study aims to investigate the condition of pulmonary vascular bed analyzing pulmonary artery stiffness by means of Doppler echocardiography. METHODS: Thirty-three patients (16 females, mean age 26+/-15 years) with various congenital heart diseases such as atrial septal defect (20 patients), ventricular septal defect (10 patients), patent ductus arteriosus (2 patients), atrioventricular septal defect (1 patient) were enrolled in this study. Systemic flow (Qs), pulmonary flow (Qp), systemic vascular resistance, pulmonary vascular resistance were calculated according to Fick method by using data obtained during left and right heart catheterization. Echocardiographically, pulmonary artery stiffness (PAS) was calculated by using maximal frequency shift (MFS) and acceleration time (AcT) of the pulmonary artery flow trace. PAS (kHz/sec)= MFS/AcT. RESULTS: Invasively, the average Qp/Qs, mean pulmonary artery pressure, and pulmonary vascular resistance were found as 2.58+/-1.25, 25+/-20 mmHg, and 135+/-217 dyn.sec.cm-5, respectively. Echocardiographically, PAS was found to be 33+/-17 kHz/sec. Pulmonary artery stiffness was correlated with mean pulmonary artery pressure (r=0.63; p<0.001) and pulmonary vascular resistance (r=0.55, p<0.001), while no relation was found with Qp/Qs. CONCLUSION: Estimation of pulmonary artery stiffness by using pulmonary flow maximal frequency shift and acceleration time obtained by means of Doppler echocardiography may give us an idea about the state of pulmonary vascular bed.

Adult↗

PubMed2006

[Diagnostic value of the currently used criteria and brain natriuretic peptide for diagnosing congestive heart failure in children with congenital heart disease].

OBJECTIVE: To improve the accuracy of diagnosis of heart failure (HF) has been the focus of research for a long time. The diagnosis for HF with congenital heart disease, however, is more difficult. The aim of the study was to evaluate the diagnostic criteria for HF in children and examine the value of plasma brain natriuretic peptide (BNP) and NT-proBNP for diagnosing HF in pediatric patients with congenital heart disease, and to look for the most valuable index for the diagnosis according to the multifactor analysis. METHODS: Totally 118 children with congenital heart disease were enrolled. They were diagnosed using modified Ross score, Qingdao criteria, NYU PHFI, and plasma BNP and NT-proBNP. According to modified Ross score as the referent criteria, other diagnostic criteria and plasma BNP and NT-proBNP were studied. The sensitivity, specificity and area of the ROC curve were examined. Logistic regression analysis was used to select the valuable index for diagnosing HF. RESULTS: (1) The value of each clinical criteria: 1 The sensitivity of Qingdao criteria for diagnosing HF was 47.9%. The specificity was 100% and the accuracy was 57.6%. 2 There were 52 patients younger than six months in whom 27 (51.9%) were breast fed. Only 25 children were measured with Ross score. The Ross score was positively correlated with the modified Ross score (r = 0.948). The area under the ROC curve of Ross score diagnosing HF was 0.985, and the sensitivity was 88%, while the specificity was 100%. 3 NYU PHFI score was positively correlated with the modified Ross score. The area under the ROC curve of the NYU PHFI diagnosing HF was 0.964, and the sum of sensitivity and specificity was favorite when > or = 8 was set as the cut-off point. If > 2 was set as cut-off point, it had a high sensitivity but a low specificity. The sensitivity of NYU PHFI was 100% > was set 2 as cut-point for diagnosing HF, but the specificity was 4.5%. (2) Plasma BNP and NT-proBNP were positively correlated with the modified Ross score, and increased with the severity of congestive HF. The area under the ROC curve of BNP was 0.880, and the cut-off line was > or = 349 pg/ml. The area under the ROC curve of NT-proBNP was 0.981, and the cut-off line was > or = 499 fmol/ml. (3) Logistic regression analysis showed that in multifactor analysis, only plasma concentration of NT-proBNP, dyspnea, tachycardia, tachypnea, failure to thrive were the independent predictors for diagnosing HF. (4) Plasma concentration of NT-proBNP incorporated with clinical criteria would improve its accuracy. CONCLUSION: All the clinical criteria commonly used were valuable for diagnosing HF in children with congenital heart disease, but each has its own limits, such as the low sensitivity of Qingdao, the low adaptation of Ross score because of the high breast-feeding rate in our country and the low specificity of NYU PHFI when > 2 was set as the cut-off point. Plasma concentrations of BNP and NT-proBNP were valuable for diagnosing HF in children with congenital heart disease, and NT-proBNP was the independent predictor for HF.

Adolescent↗