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At least 649 records · Page 36Linked to original sources
PubMed2006

Development of the MAGIC congenital heart disease catheterization database for interventional outcome studies.

As the field of catheter-based therapies for congenital heart disease continues to expand, we lack the evidence-based data to make appropriate therapeutic decisions in the catheterization laboratory. A stumbling block to the determination of evidence-based therapies is our inability to simply and reliably share outcome data across multiple centers. We investigated whether a commonly used congenital heart disease catheterization database program (PedCath) could be used as an automatic catheterization data submission tool to a central database for outcome analysis. To test the feasibility of such a tool for collaborative outcomes research we formed a national group of seven congenital heart disease centers, the Mid-Atlantic Group of Interventional Cardiology, to warehouse and analyze catheterization data. We successfully modified PedCath to transfer the results of catheter-based therapies on 256 therapeutic procedures for atrial septal defect (ASD) closure, coarctation of the aorta angioplasty and stenting, and pulmonary and aortic balloon valvuloplasties over a 13-month pilot period. Short-term follow-up within the 13-month period was received on 31 patients. This study demonstrated the successful development of a simple process, requiring minimal data entry for investigators from around the world to share cardiac catheterization data for long-term outcome determination of catheter-based therapies for congenital heart disease.

Adolescent↗
PubMed1994

Relationship between hemodynamic changes and blood hormone concentrations after cardiac surgery in children with congenital heart disease.

OBJECTIVES: To evaluate changes in atrial natriuretic factor in relation to modifications in hemodynamic variables, and to evaluate hormones determining salt and water metabolism after heart surgery in children with congenital heart disease. DESIGN: Prospective study. SETTING: Medical-surgical pediatric intensive care unit (ICU) in a tertiary referral center. PATIENTS: Sixty-five infants and children admitted to the pediatric ICU with congenital heart disease who underwent cardiac surgery with extracorporeal circulation. The control group consisted of 48 children undergoing noncardiac surgical interventions, without a history of cardiac disease and with normal hemodynamic variables. MEASUREMENTS AND MAIN RESULTS: Patients with congenital heart disease were distributed into two groups, according to hemodynamic variables. Group 1 consisted of patients with normal pulmonary blood flow (pulmonary/systemic blood flow ratio of < 2, pulmonary arterial systolic pressure of < 35 mm Hg, pulmonary arterial systolic pressure/aortic systolic pressure ratio of < 0.35). Group 2 consisted of patients with relatively increased pulmonary blood flow (pulmonary/systemic blood flow ratio of > 2.5, pulmonary arterial systolic pressure of > 65 mm Hg, pulmonary arterial systolic pressure/aortic systolic pressure ratio of > 0.75). Blood samples were obtained 24 hrs before, and 24 and 48 hrs after surgery. Hormone determinations included plasma concentrations of atrial natriuretic factor, vasopressin, plasma renin activity, corticotropin, cortisol, and aldosterone. Hemodynamic measurements were recorded simultaneously with blood sampling. Twenty-four hours before surgery, plasma concentrations of atrial natriuretic factor were higher in congenital heart disease patients than in the control group (67.36 +/- 93.44 vs. 15.11 +/- 8.29 pmol/L) (p < .001). Plasma concentrations of atrial natriuretic factor were 57.12 +/- 82.20 and 74.63 +/- 101.03 pmol/L in groups 1 and 2, respectively. Before surgery, in group 2 patients, there was a significant correlation between mean right atrial pressure and atrial natriuretic factor (r2 = .64; p < .001), vasopressin (r2 = .55; p < .001), cortisol (r2 = .43; p < .01), corticotropin (r2 = .53; p < .01), and aldosterone (r2 = .57, p < .001). None of these correlations were observed in group 1. However, 24 hrs after surgery, we found a significant correlation between diastolic blood pressure and atrial natriuretic factor in group 1. Plasma concentrations of atrial natriuretic factor did not change significantly after surgery. After surgery, the correlation between mean right atrial pressure and the various hormone concentrations persisted at 24 hrs (p < .05), but not at 48 hrs. Twenty-four hours after surgery, patients with a central venous pressure of > 7 mm Hg showed higher concentrations of atrial natriuretic factor than those patients with central venous pressures of < 7 mm Hg (p < .05). However, there was a large overlap of atrial natriuretic factor values between the two groups. CONCLUSIONS: Patients with congenital heart disease have increased plasma concentrations of atrial natriuretic factor. During the early postoperative period, in patients with a more severe degree of congenital heart disease, the increase in central venous pressure correlated with higher plasma concentrations of atrial natriuretic factor. High pulmonary blood flow was associated with an increase in plasma renin activity and serum aldosterone, and an increased secretion of atrial natriuretic factor.

Adolescent↗
PubMed1994

Reproductive issues for the woman with congenital heart disease.

As the number of women with congenital heart disease continues to rise, information regarding reproduction is and will become an important part of their follow-up care. Until now the focus of reproduction has been on pregnancy outcomes. Practitioners must become better informed about issues of fertility, contraception, and childbearing to better advise their patients.

Adult↗
PubMed1979

Aortic vasa vasorum in cyanotic congenital heart disease.

Three cases for longstanding untreated cyanotic congenital heart disease are presented. In addition to collateral circulation from bronchial, intercostal, and inferior phrenic arteries, thoracic aortic vasa vasorum were visualized. We believe this may represent an additional collateral pathway to the lungs. The angiography of these cases is presented.

Adolescent↗
PubMedDate not supplied

Pulse oximetry in children with congenital heart disease: effects of cardiopulmonary bypass and cyanosis.

The objective of this prospective, observational study with consecutive sampling was to assess the reliability, bias, and precision of Nellcor N-395 (N) and Masimo SET Radical (M) pulse oximeters in children with cyanotic congenital heart disease and children with congenital heart disease recovering from cardiopulmonary bypass-assisted surgery admitted to a cardiovascular operating suite and pediatric intensive care unit at a tertiary care community hospital. Forty-six children with congenital heart disease were studied in 1 of 2 groups: (1) those recovering from cardiopulmonary bypass with a serum lactic acid > 2 mmol/L, and (2) those with co-oximetry measured saturations (SaO(2)) < 90% and no evidence of shock. Measurements of SaO(2) of whole blood were compared to simultaneous pulse oximetry saturations (SpO(2)). Data were analyzed to detect significant differences in SpO(2) readout failures between oximeters and average SpO(2) - SaO(2) +/- 1 SD for each oximeter. A total of 122 SaO(2) measurements were recorded; the median SaO(2) was 83% (57 - 100%). SpO(2) failures after cardiopulmonary bypass were 41% (25/61) for N versus 10% (6/61) for M (P < .001). There was a significant difference in bias (ie, average SpO(2) - SaO(2)) and precision (+/- 1 SD) between oximeters (N, 1.1 +/- 3.3 vs M, -0.2 +/- 4.1; P < .001) in the postcardiopulmonary bypass group but no significant difference in bias and precision between oximeters in the cyanotic congenital heart disease group (N, 2.9 +/- 4.6 vs M, 2.8 +/- 6.2; P = .848). The Nellcor N-395 pulse oximeter failed more often immediately after cardiopulmonary bypass than did the Masimo SET Radical pulse oximeter. SpO2 measured with both oximeters overestimated SaO2 in the presence of persistent hypoxemia.

Cardiopulmonary Bypass↗
PubMed1991

The adult with congenital heart disease: cardiac catheterization as a therapeutic intervention.

The adult with congenital heart disease who undergoes cardiac catheterization at the present time is most likely to have complex heart disease and is left with clinically important sequelae or residual defects, ventricular dysfunction or arrhythmias. Residual defects such as paravalvular leaks, coronary fistulas and pulmonary artery narrowings may be corrected with transcatheter techniques. Patients with simple forms of congenital heart disease (for example, atrial septal defect, patent ductus arteriosus, aortic valve stenosis, pulmonary valve stenosis) will go to the catheterization laboratory for treatment, not diagnosis. Certain lesions previously considered benign (for example, patent foramen ovale) may require definitive interventional therapy to reduce the risk of stroke from paradoxic embolism.

Adult↗
PubMed2004

Contraception for the adolescent and young adult with congenital heart disease.

Increasing numbers of females born with congenital heart disease are reaching childbearing age. Practitioners involved in the management of the adolescents or adults must be aware not only of the risks of pregnancy associated with varying types of CHD but with other issues involving the reproductive cycle to provide appropriate care, counseling, and education.

Abortion, Induced↗
PubMed2003

[Safeguard and management of the heart vales in congenital heart disease procedure].

OBJECTIVE: To summarize the experience in performing reoperation of valve dysfunction after congenital heart disease procedure. METHODS: From 1994 to 2001 we reviewed the data of 13 patients with valve dysfunction after congenital heart disease operation, in which 8 patients after ventricular septal defect, 3 after atrioventricular canal and 2 after respectively tetralogy of Fallot and atrial septal defect were corrected. Before the first operation, 6 patients had presented the mild to moderate mitral regurgitation, 1 had aortic regurgitation. Other 6 patients had valves dysfunction occurring after the first operation, among them, 2 suffered from respectively residual shunt of the ventricular septal defect, 2 had anterior chordae rupture of tricuspid valve, one had an operative injured aortic valve and one had surviving of right ventricular outlet obstruction. Thirteen patients were reoperated, including mitral valve replacement in 6, tricuspid valve replacement in 2, aortic valve replacement in one, aortic valve replacement consists with mitral valve repair and tricuspid valve repair in one and tricuspid valve repair in 3. Concomitant procedures were performed. RESULTS: Low cardiac output occurred in 3 cases and there were 2 early deaths, due to cerebral air-embolism, respiratory and circulatory failure respectively. Other 11 cases discharged and were followed up well. CONCLUSIONS: It is important to safeguard and repair the valvular construction and function during the operation in congenital heart disease. Reoperation should be performed timely for obtaining recurrent and a good results.

Adult↗
PubMedDate not supplied

Congenital heart disease in pregnancy.

Care of pregnant patients with congenital heart disease requires understanding of the specific congenital defect, the nature of previous surgical correction, and the residua and sequelae. General risks and principles can be adduced in management decisions. In addition, lesion- and patient-specific details are important. There are only a few conditions that place patients at a high enough risk to advise that pregnancy be avoided under all circumstances (pulmonary vascular obstructive disease, Marfan syndrome with dilated aortic root, severe aortic stenosis, and severe systemic ventricular dysfunction). Preconception counseling, optimization of status, and meticulous multidisciplinary management during pregnancy and the postpartum period will improve outcomes.

Counseling↗
PubMed1992

Screening for congenital heart disease with the four-chamber view of the fetal heart.

OBJECTIVE: Our objective was to determine the efficacy of the four-chamber view of the fetal heart in routine ultrasonographic examination as a screening tool for congenital heart defects. STUDY DESIGN: A prospective cohort study compared the detection rate of congenital heart defects among 5336 pregnant women screened with the ultrasonographic four-chamber view of the fetal heart from 1987 through 1989 with that among 3680 patients examined ultrasonographically without the four-chamber view during the 2 preceding years (1985 through 1986). All patients were followed until delivery or termination of pregnancy, and clinical or autopsy confirmation of prenatal findings were obtained on all cases. RESULTS: The overall incidence of congenital heart diseases was 5.2 per 1000 (47/9016). During the years 1985 through 1986 15 neonates with congenital heart diseases were identified, seven of which were prenatally diagnosed (sensitivity 43%). During the period 1987 through 1989 a four-chamber view of the fetal heart was obtained in 95% of cases; 32 cases of congenital heart disease occurred, 26 of which were diagnosed antenatally (sensitivity 81%; p = 0.01). Two false-positive diagnoses were made during the second time period, giving a specificity of 99.9%. CONCLUSION: The four-chamber view of the fetal heart is easily obtained, does not significantly increase the duration of a routine ultrasonographic examination, and has an excellent sensitivity for the identification of congenital heart diseases.

Cohort Studies↗
PubMed1981

Inbreeding, mortality and genetic load in families with congenital heart diseases.

A total of 93 cases of congenital heart diseases (CHD) were studied for the effects of inbreeding on the incidence of these diseases. Sib mortality and genetic load in families with CHD were estimated. This study indicated (1) involvement of recessive genes in causation of CHD, (2) lack of inbreeding effects on sib mortality in CHD, and (3) the nature of genetic load to be segregational.

Consanguinity↗
PubMed1977

Horizontal interventricular septum in congenital heart disease: surgical considerations.

A patient with complex congenital heart disease associated with a horizontal interventricular septum is described. In addition the ventricles were inverted, both great vessels arose from the left-sided right ventricle in the dextrotransposed position, and he had a ventricular septal defect of the type usually seen in endocardial cushion defect. Correction was performed using a Rastelli type procedure with good hemodynamic results.

Child↗
PubMed2005

Clinical presentations of unoperated and operated adults with congenital heart disease.

The clinical presentation of adults with congenital heart disease (ACHD) is exceptionally diverse. Operated, unoperated acyanotic, and cyanotic patients often have similar physiologic characteristics, and thus shared clinical symptomatology. Five illustrative cases are included to highlight important considerations in the diagnosis and management of ACHD. This article focuses on epidemiology, natural history, clinical outcomes, and use of ancillary studies in commonly encountered cardiac defects.

Adult↗
PubMed1996

Serum insulin-like growth factor 1 in congenital heart disease.

Serum insulin-like growth factor 1 (IGF-1), insulin-like growth factor binding protein-3 (IGFBP-3), and a range of growth and nutritional variables were investigated in 62 infants with congenital heart disease and healthy controls. Infants with congenital heart disease were small, underweight, and had a reduced energy intake. Serum IGF-1 and IGFBP-3 concentrations were significantly reduced. Decreased IGF-1 and IGFBP-3 levels are observed in nutritional deficiency; similar findings in congenital heart disease suggest that undernutrition contributes to the poor growth of these infants. Serial measurements of serum IGF-1 and IGFBP-3 may be helpful in monitoring the effect of nutritional treatment in congenital heart disease.

Biomarkers↗
PubMed1998

Congenital heart disease: effects on the family.

Treatment of the child with congenital heart disease continues to challenge the medical profession. Although advances in technology have improved survival statistics over the past two decades, dealing with the effects on the family of the birth of a child with congenital heart disease has received minimal attention. Most nursing interventions focus on the immediate needs of the family related to the death of their child. Nurses must increase their awareness of the effects of the diagnosis itself on the family. To better help the family in crisis, nurses must understand the concept of death of the "normal" child and must also recognize the effects this diagnosis can have on personal relationships.

Adaptation, Psychological↗
PubMed1993

Prenatal diagnosis of congenital heart disease and fetal karyotyping.

OBJECTIVE: To determine the incidence of aneuploidy among fetuses with congenital heart disease diagnosed in utero. METHODS: From June 1988 through December 1991, 502 fetuses at risk for congenital heart disease underwent fetal echocardiography. Fetal karyotyping was performed whenever a cardiac anomaly was diagnosed. Autopsy reports, postnatal echocardiograms, and angiograms were obtained to confirm the diagnosis. RESULTS: Congenital heart disease was found in 31 of 469 fetuses with complete follow-up. Fifteen of these 31 fetuses (48%) were found to have an abnormal karyotype: five of 17 (29.4%) with isolated cardiac anomalies and ten of 14 (71.4%) with cardiac and extracardiac anomalies. Detected chromosomal abnormalities included six trisomy 21, four trisomy 18, four trisomy 13, and one triploidy 69,XXX. Atrioventricular septal defects and ventricular septal defects were the cardiac malformations most often associated with abnormal karyotypes (77 and 71%, respectively). CONCLUSIONS: The risk of aneuploidy associated with fetal cardiac anomalies is much greater than that associated with elevated maternal age; therefore, fetal karyotyping should be offered whenever a cardiac defect is diagnosed. Advanced gestational age should not represent a deterrent, because the discovery of a lethal trisomy in a fetus with a cardiac malformation can affect dramatically the prognosis and the obstetric and neonatal management. We believe that a screening view such as the four-chamber view should now be included routinely in obstetric ultrasound examinations.

Aneuploidy↗