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At least 937 records · Page 52Linked to original sources
PubMed2006

[The effects of coronary abnormalities on reconstruction of right ventricular outflow tract in patients with complex congenital heart disease].

OBJECTIVE: To explore the effects of coronary abnormalities on reconstruction of right ventricular outflow tract in patients with complex congenital heart disease. METHODS: Eighty patients, including tetralogy of Fallot 14 cases and double outlet right ventricle (DORV) 4 cases with anomalous coronary artery, underwent surgical treatment from April 1989 to May 2004. The coronary diseases included single left or right coronary artery in 11 cases, left anterior descending coronary artery originating from right coronary artery in 6 cases, vascular plexus on right outflow tract in 1 case. One-stage correction was undergone in 17 cases, palliative procedure in 1 case. The reconstruction methods were as follows: mobilizing coronary artery and expanding incision under coronary artery with pericardial patches in 4 cases; repairing ventricular septal defect (VSD) via the incision beneath the coronary artery and expanding right ventricular outlet tract (RVOT) and pulmonary via longitudinal incision over the coronary artery in 3 cases. VSD were repaired via right atrium and minimal incision on RVOT plus incision on pulmonary were made to expand RVOT. Trunk of pulmonary were anastomosis with RVOT in 3 cases. Homograft valved aorta were used in 3 cases. RESULTS: One case died of serious low cardiac output syndrome postoperatively. There was no critical complication of hemorrhage and respiratory tract. Oxygen saturation rose from 68.0% to 82.0% after treated by palliative procedure. Seventeen cases were followed from 10 months to 8 years, 1 case suddenly died with no clear cause. Three cases were with residual leak, 3 with residual obstruction. CONCLUSIONS: Surgical procedure should be selected according to the characteristics of coronary disease with complex congenital heart disease.

Adolescent↗
PubMed2025

Association of MTHFD1 G1958A (rs2236225) gene polymorphism with the risk of congenital heart disease: a systematic review and meta-analysis.

BACKGROUND: We did this study to better clarify the correlations of methylenetetrahydrofolate dehydrogenase 1 (MTHFD1)-G1958A (rs2236225) gene polymorphism with the risk of congenital heart diseases (CHD) and its subgroups. METHODS: Relevant articles were searched in PubMed, Web of Science, Cochrane Library, Embase, CNKI, VIP database and Wanfang DATA until October 2023. We will use odds ratios (ORs) and 95% confidence intervals (CIs) to examine the potential associations of MTHFD1- G1958A gene polymorphism with CHD and its subgroups. RESULTS: We included a total of 9 eligible studies, encompassing 1917 children with CHD, 1863 healthy children, 1717 mothers of the children with CHD and 1666 mothers of healthy children. In our study, the meta-analysis of fetal group revealed no significant association between any of the five genetic models for the MTHFD1-G1958A polymorphism and the risk of CHD. Subgroup analysis showed that associations between the MTHFD1-G1958A polymorphism and Tetralogy of Fallot (TOF) risk in the homozygote model (AA vs. GG, OR = 2.82, 95%CI [1.16, 6.86], P = 0.02) and recessive model (AA vs. GG + GA, OR = 3.09, 95%CI [1.36, 7.03], P = 0.007). In addition, the MTHFD1-G1958A polymorphism was associated with the risk of CHD in racial subgroup, increasing the risk of CHD in Caucasians. In maternal analysis, 2 genetic models of MTHFD1-G1958A polymorphism increased the risk of CHD: the heterozygote model (GA vs. GG, OR = 1.22, 95%CI [1.04, 1.42], P = 0.01), and the dominance model (GA + AA vs. GG, OR = 1.17, 95%CI [1.01, 1.34], P = 0.03). CONCLUSIONS: The fetal MTHFD1-G1958A (rs2236225) gene polymorphism increase their risk of TOF. The maternal MTHFD1-G1958A polymorphism has a strong correlation with the risk of CHD, and there are racial differences in this correlation. Compared with GG genotype, the GA genotype increases the risk of CHD.

Humans↗
PubMed2002

[Pulmonary plasma catecholamine levels and pulmonary hypertension in congenital heart disease].

Plasma catecholamines may play a role in the pathogenesis of pulmonary hypertension in congenital heart disease with increased pulmonary blood flow. At cardiac catheterization, blood samples were obtained before and after passage of the lung in patients with congenital heart disease with normal pulmonary blood flow (n=39), elevated pulmonary blood flow with normal pulmonary pressure and normal pulmonary vascular resistance (PVR) in patients with atrial septal defect (ASD) (n=57) or ventricular septal defect (VSD) (n=12), and increased pulmonary blood flow, pressure and vascular resistance in patients with ASD, VSD or both (n=22), or in patients with primary pulmonary hypertension (n=4). Plasma catecholamines were determined by reversed phase high performance liquid chromatography and electrochemical detection (HPLC-ECD). In patients with elevated PVR elevated norepinephrine levels (NE) were observed. In contrast, epinephrine levels (E) were not associated with the development of pulmonary hypertension. Patients with primary pulmonary hypertension had the highest levels of NE while patients with associated Down's syndrome had significantly lower epinephrine levels. Pulmonary plasma norepinephrine levels are increased in patients with elevated pulmonary vascular resistance. Whether this phenomena is the cause or sequalae of pulmonary hypertension needs further examination. In the future, plasma catecholamines may serve as a diagnostic feature or may result in further therapeutic options.

Adolescent↗
PubMed1999

Elevation of atrial natriuretic peptide prohormone. Hemodynamic background of the elevation of N-terminal natriuretic peptide prohormone in children with congenital heart disease.

We postulated previously that variables related to pulmonary flow are independent predictors of levels of atrial natriuretic peptide in children with congenital heart disease. The aim of this study was to test this hypothesis in relation to other hemodynamic and clinical variables. During catheterization we measured the levels of plasma N-terminal atrial natriuretic peptide prohormone in the plasma of 68 children with congenital heart disease. All had undergone complete clinical, echocardiographic and invasive hemodynamic investigations. The influence on the prohormone was analyzed for 10 different variables in a multiple linear regression model. The variability could be explained in large parts (adjusted R2 =77.2%) by variations in atrial pressures or sizes, together with the degree of excessive pulmonary blood flow and signs of heart failure. A value for atrial natriuretic peptide prohormone above 800 pmol/l predicted hemodynamic imbalance (defined as elevated pressures in left or right atrium or the pulmonary arteries, and/or Qp/Qs > 1.5) with a specificity of 94%, a sensitivity of 73%, a positive likelihood ratio of 12.2, and a negative likelihood ratio of 0.29. In conclusion, variables related to pulmonary blood flow are influential determinants of the levels of atrial natriureic peptide in children with congenital heart disease. Atrial pressures, and symptoms of heart failure are also of major importance.

Adolescent↗
PubMed2003

Arrhythmia and conduction disturbances in patients with congenital heart disease during pregnancy: multicenter study.

The incidence, manifestation and management of arrhythmia in congenital heart disease (CHD) during pregnancy were evaluated in a multicenter study. Of 126 pregnancies in patients with CHD in 17 institutions from January 1991 to December 2000, 29 cases of pregnancy in 27 patients after cardiac repair (mean age, 29+/-4.9 years) were identified with arrhythmias (supraventricular tachyarrhythmia (SVT) in 15, ventricular tachycardia (VT) in 9, high-grade atrioventricular block in 4 and sick sinus syndrome in 3) (group A) and 29 control pregnancies from 29 patients with repaired CHD and no arrhythmias (group B). SVT tended to require anti-arrhythmic medication more than VT (10/15 vs 2/9, p=0.04). Nine different types of anti-arrhythmic agents were successfully administered without maternal complications. There were no maternal deaths in either group. In the comparison of group A with group B, there was lower cardiac functional class (8/29, p=0.04), higher incidence of polysplenia (4/29, p=0.04), and higher incidence of low-birthweight infant (7/29, p=0.02) in the former. It appears that there is a high prevalence of arrhythmias during pregnancy in patients with repaired CHD. Patients with CHD and low cardiac functional class and/or polysplenia could have arrhythmia during pregnancy that results in a low-birthweight infant. Meticulous care for these patients during pregnancy is recommended.

Arrhythmias, Cardiac↗
PubMed2001

Recognising examples of congenital heart disease.

With improved treatments now available, many children with congenital heart disease are living into adulthood. Increasingly, it is more important for the nursing team to be familiar with the more common conditions that may be encountered.

Heart Defects, Congenital↗
PubMed1991

Successful intracardiac electrical conversion of atrial flutter in patients with complex congenital heart disease.

Sixteen patients presenting on 21 occasions with atrial flutter in association with complex congenital heart disease were treated by intracardiac stimulation techniques combined with activation mapping. Nineteen episodes of atrial flutter were successfully converted to sinus rhythm. In the remaining two episodes atrial fibrillation was induced with spontaneous conversion to sinus rhythm within 12 hours in one episode and immediate DC cardioversion to sinus rhythm in the other. Intracardiac stimulation techniques were highly successful in this group and allowed reliable conversion to sinus rhythm without general anaesthesia and high energy cardioversion. In patients with atrial flutter associated with congenital heart disease intracardiac stimulation techniques should be tried first.

Adolescent↗
PubMed1994

Malabsorption in infants with congenital heart disease under diuretic treatment.

Intestinal absorption and its relationship to increased total body water was studied in seven infants with congenital heart disease receiving regular diuretics due to congestive heart failure. All infants and six age-matched healthy controls were studied for a 3-d period during which time all food intake was recorded, stools were collected, and total body water content (TBW) and extracellular water were measured. All the anthropometric measurements were lower in the infants with congenital heart disease compared with healthy controls. Energy and fat excretion in the stools were significantly increased in the group of patients, but when expressed as a percentage of daily caloric intake or as a percentage of the specific intake (e.g. fat excretion/fat intake x 100), no statistical differences were found. TBW as a percentage of body weight was increased in our patients compared with our controls (84.95 +/- 5.82% versus 68.65 +/- 4.60%; p = 0.01) and so was extracellular water as a percentage of predicted (200.0 +/- 18.6% versus 100.9 +/- 7.2%; p = 0.001). A positive correlation was found between energy and fat excretion as a percentage of the intake and TBW as a percentage of predicted; energy and fat malabsorption did not exceed 8% in the patients with the highest body water content (120% of predicted). It is concluded that malabsorption is not a significant factor in failure to thrive of patients with congenital heart disease who are receiving regular diuretics. Based on the significant negative correlation between excess body water and fat and calorie absorption, however, it is suggested to monitor TBW in patients who fail to gain weight.

Body Water↗
PubMed1994

Physical activity and congenital heart disease.

With advances in the diagnosis and management of congenital heart disease (CHD), the need has arisen for the assessment and counseling of patients as to appropriate physical activity levels through adulthood. Considerations to be evaluated before making exercise and athletic activity recommendations to the patient with CHD are outlined. Recommended guidelines of the American Heart Association are presented for recreational and occupational activities. Selected congenital heart defects are reviewed to address potential negative effects of incorrect recommendations.

Adolescent↗
PubMed1992

Factors associated with choreoathetosis after cardiopulmonary bypass in children with congenital heart disease.

BACKGROUND: Choreoathetosis (CHO) after congenital heart surgery has been described since 1960. Risk factors and patient outcome have not been well defined. METHODS AND RESULTS: As our complexity of cases increased and management of pH on cardiopulmonary bypass (CPB) evolved, we noted the appearance of CHO among patients beginning in 1986. We reviewed the hospital course and follow-up of all 19 affected children, including eight younger patients (median age, 4.3 months) who developed a mild transient form of CHO, all of whom survived and had complete resolution of CHO; seven of these eight patients had deep hypothermic circulatory arrest (DHCA). Eleven older patients (median age, 16.8 months) developed severe persistent CHO; 11 had DHCA, 10 were cyanotic, seven had anatomic pulmonary atresia, and three others were physiologically analogous with a systemic to pulmonary artery shunt-dependent circulation. Five of six patients who had pertinent preoperative angiography had systemic to pulmonary collateral vessels arising from the head and neck arteries. Mortality in severe patients was 36% (four of 11); the seven survivors showed improvement, but only one had a normal neurological examination after 60 months of follow-up. When severe CHO patients were compared with 17 age- and diagnosis-matched patients without neurological complications, no differences were found in CPB or DHCA times, arterial blood gases, or hematocrits. Time from onset of CPB to onset of DHCA (time to shutoff) was shorter in the severe persistent CHO group than for comparison patients (22 +/- 7 versus 40 +/- 29 minutes, p = 0.053). CONCLUSIONS: Factors that may be associated with the development of severe persistent CHO include 1) age beyond early infancy, 2) cyanotic heart disease with systemic to pulmonary collaterals, particularly those arising from the head and neck vessels, and 3) the duration of the cooling period used in conjunction with deep hypothermic circulatory arrest. We advocate earlier reparative surgery, precise preoperative diagnosis and preoperative or intraoperative control of systemic to pulmonary artery collaterals, and further study of pH effects during CPB on development of CHO.

Athetosis↗
PubMed2003

When to order cardiovascular magnetic resonance in adults with congenital heart disease.

Cardiovascular magnetic resonance (CMR), where available, contributes to the informed management of patients with congenital heart disease. In contrast to echocardiography, CMR becomes easier as patients grow. It is versatile and gives unrestricted access to the heart and intrathoracic vessels, providing functional and structural information. Its relative strengths are discussed, and examples are given of congenital conditions in which it provides clinically important information. CMR can prevent the need for diagnostic catheterization or expedite intervention if indicated, enabling planned, directed procedures. In our practice, CMR is used for serial follow-up, investigation of altered symptoms or signs, planning of transcatheter or surgical interventions, and for baseline assessment after surgery. As CMR becomes more widely available, it will contribute increasingly to the lifelong management of patients with congenital heart disease.

Adult↗
PubMed1988

Infective endocarditis in children with congenital heart disease: the changing spectrum, 1965-85.

Thirty-five episodes of infective endocarditis in 35 children with congenital heart disease, from January 1965 to December 1984, were reviewed. The incidence of infective endocarditis in cyanotic congenital heart defects increased with a concomitant decrease in the frequency of lesions with left to right shunt. The incidence of postoperative endocarditis decreased from 1:52 during the first decade to 1:1033 during the second decade. The time interval between onset of symptoms and the establishment of diagnosis and treatment shortened from 7.8 +/- 3.2 weeks (mean +/- SD) to 2.1 +/- 0.9 weeks. During the latter decade, positive blood cultures were obtained in 100% of patients, as compared with 60% during the former decade (P less than 0.05). Echocardiography demonstrated vegetations in 70% of the cases. Over the entire time period, streptococcus viridans was the most frequent pathogen (46%) and staphylococcus aureus second most frequent (17%). Six patients died, all were under two years of age and all had congestive heart failure. Early surgical correction of the congenital heart disease may offer the best form of prevention.

Adolescent↗
PubMed1995

Prenatal diagnosis of congenital heart disease; prospects and problems.

Routine fetal echocardiography has been submitted as an antenatal screening test for congenital heart disease despite a wide range of efficacy reported. Hence, evaluation of the variable results of prenatal ultrasound screening programs was pursued. As the studies appeared to have a heterogeneous design, the originally reported figures have been corrected for one plausible prevalence of cardiovascular anomalies. Clinical setting (general hospitals vs. teaching hospitals), case ascertainment (retrospective vs. prospective; follow-up on (suspected) cases only vs. routine follow-up) and length of follow-up (< 1 year vs. a longer period) all have similar effects on the reported yield of cases and sensitivity of the proposed screening test. Notably, a relatively low yield of cases causes a seemingly high sensitivity, whereas a higher yield of cases causes a seemingly low sensitivity. Additional factors of study design, e.g. case definition, diagnostic procedures and appliances used, were usually not described in detail, yet may also have affected the results. Evaluating diagnostic tests apparently demands a rigorous study design to overcome biased results. Furthermore, no sufficient evidence could be found meriting current routine prenatal screening for congenital heart disease.

Female↗
PubMed1996

Treatment of children with congenital heart disease and growth retardation with recombinant human growth hormone.

Seven prepubertal short children with congenital heart disease were treated with recombinant human growth hormone (GH). Although complete surgical correction was performed for their heart disease at least 2 years before the start of GH therapy, improvement in growth was less than expected in these children. They received 0.5 IU kg-1 week-1 of GH daily for 2 years or more. The growth rate increased from a mean of 4.3 cm year-1 before treatment to a mean of 7.8 cm year-1 in the first year and to a mean of 6.3 cm year-1 in the second year of treatment. Their mean standardized height improved from -3.41 +/- 0.78 to -2.54 +/- 0.62 after 2 years. The mean height age difference minus the bone age difference became positive in these children. We conclude that recombinant GH increases the growth rate in children with congenital heart disease and prepubertal growth retardation.

Child↗
PubMed1982

Reversible hypertrophic osteoarthropathy associated with cyanotic congenital heart disease.

We treated two patients with congenital cyanotic heart disease (CCDH) in whom associated hypertrophic osteoarthropathy (HOA) developed. Both were severely cyanotic and hypoxic for many years before HOA was fully manifested. Clinical and roentgenographic indications of bone and joint lesions disappeared completely shortly after corrective of palliative surgery. There appears to be an etiologic connection between HOA and CCHD.

Adolescent↗
PubMed1982

Pregnancy and its outcome in women with and without surgical treatment of congenital heart disease.

In the state of Connecticut, 233 women with congenital heart defects were prospectively followed up through 482 pregnancies that resulted in 372 infants who were examined frequently during their first 3 years of life. Approximately half of the women had undergone cardiac surgery and they were compared with the women without operation. There was no maternal mortality, and no patient had infective endocarditis, brain abscess or a cerebrovascular accident. The proportion of pregnancies resulting in live births did not differ significantly in mothers with and without cardiac surgery; the average live birth rate was 77 percent in all. However, the number and size of live-born infants was much greater in mothers who had become acyanotic as a result of reparative surgery than in the still cyanotic women, whether or not they had had palliative surgery. In cyanotic women, placental size was abnormally large in relation to birth weight, which was abnormally low. When the mothers were classified according to cardiac function, there was a significant difference between the number of infants born alive to mothers in good to excellent status and the number born to mothers in fair to poor condition. The latter had a significant increase in interrupted pregnancies as well as in cardiovascular complications during pregnancy. The total group had a 16.1 percent incidence rate of infants with congenital heart disease. This rate was corrected to 14.2 percent by removal of seven mothers, two with Noonan's syndrome, one with hypertrophic cardiomyopathy and four with a family history of congenital heart defects.

Abortion, Spontaneous↗
PubMed2006

Intensive care outcome of adult patients operated on for congenital heart disease.

OBJECTIVE: To describe the ICU outcome and the most frequent complications observed in adult patients operated on for a congenital heart disease. DESIGN AND SETTING: Retrospective analysis of prospectively collected data and chart review in an adult cardiovascular ICU of a university hospital. PATIENTS: 156 patients older than 15 years with congenital heart disease undergoing cardiac surgery between June 2001 and June 2005. RESULTS: According to the initial cardiac malformation, patients were divided in four groups with different operative risk based on the Euroscore: those diagnosed bicuspid aortic valve (n = 73) had a score of 5, those with tetralogy of Fallot (n = 33) 5.5, those with simple cardiac defect (n = 26) 3, and those with complex malformations (n = 24) 6. Only two patients (one with tetralogy of Fallot and one with complex malformations) died during the hospitalization (1.2%). CONCLUSION: Euroscore clearly overestimates the risk of surgery in this population of adults with congenital heart disease. Mortality and morbidity were low in those diagnosed bicuspid aortic valve, tetralogy of Fallot, or simple cardiac defect, justifying early surgery for incipient complications. Patients with complex congenital defect require prolonged ICU stay, sometimes with mechanical cardiac support, but their overall good outcome justifies these efforts.

Adolescent↗
PubMed2005

Changing practice of cardiac surgery in adult patients with congenital heart disease.

OBJECTIVES: To review 13 years' data from a unit for grown ups with congenital heart disease (GUCH) to understand the change in surgical practice. METHODS: Records were reviewed of patients over 16 years of age undergoing surgery between 1 January 1990 and 31 December 2002 in a dedicated GUCH unit. Patients with atrial septal defects were included but not those with Marfan's syndrome or undergoing a first procedure for bicuspid aortic valves. Three equal time periods of 52 months were analysed. RESULTS: Of 474 operations performed, 162 (34.2%) were repeat operations. The percentage of repeat operations increased from 24.8% (41 of 165) in January 1990-April 1994 to 49.7% (74 of 149) in September 1998-December 2002. Mortality was 6.3% (n = 30). The median age decreased from 25.4 years (interquartile range 18.7) in January 1990-April 1994 to 23.9 (interquartile range 17.3) in September 1998-December 2002 (p = 0.04). The proportion of patients with a "simple" diagnosis decreased from 45.4% (74 or 165) in January 1990-April 1994 to 27.5% (41 of 149) in September 1998-December 2002 (p = 0.013). Pulmonary valve replacements in operated tetralogy of Fallot increased from one case in January 1990-April 1994 to 23 cases in September 1998-December 2002 and conduit replacement increased from five cases to 17. However, secundum atrial septal defect closures decreased from 35 cases to 14 (p < 0.0001). The estimated cost (not including salaries and prosthetics) incurred by an adult patient with congenital heart disease was pound2290 compared with pound2641 for a patient undergoing coronary artery bypass grafting. CONCLUSION: Despite the impact of interventional cardiology, the total number of surgical procedures remained unchanged. The complexity of the cases increased particularly with repeat surgery. Nevertheless, the patients do well with low mortality and the inpatient costs remain comparable with costs of surgery for acquired disease.

Adolescent↗