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PubMedDate not supplied

[Effect of cardiopulmonary bypass on selected neutrophil functions in children with cyanotic congenital heart disease].

BACKGROUND: Cardiopulmonary bypass (CPB) is known to induce several pathogenic responses in cardiovascular surgery. Children suffering from cyanotic congenital heart disease (CCHD) have been assumed to have a deficiency in their defence mechanisms. The preoperative clinical condition (hypoxemia and circulation disorders) could initiate post CPB inflammatory response. The nature of the participation of neutrophils and its time course in the post CPB inflammatory response, however, is less clear. THE AIM of our study was to investigate alterations in expression of adhesion molecules and L-selectin on neutrophils, and soluble adhesion molecules levels in CCHD children. MATERIALS AND METHODS: 23 infants and children with congenital heart disease, scheduled for primary corrective surgical repair; were enrolled. Expression of CD11b. CD18, and CD62L on neutrophils was determined by cytometry. Additionally, serum concentrations of soluble ELAM-1, L-selectin were measured using enzyme-linked immunoassay. Blood samples of children with CCHD were collected at the following times: before anaesthesia, at the initiation of CPB, after 30 minutes of CPB, at the end of CPB, and 24 hours and 72 hours after surgery. RESULTS: The expression of CD11b molecule on neutrophils was increased significantly at the start of CPB and returned to the baseline after 24 hrs. In all patients, preoperative CD11b expression on neutrophils correlated with hypoxemia. Reduced CD62L expression on neutrophils and soluble L-selectin concentration were observed at the end of CPB. CONCLUSION: We conclude, that cardiac surgery with CPB has a suppressive effect on adhesion molecules expression in children with CCHD, These findings also suggest that the type of neutrophil response to cardiac surgery appears to depend on preoperative clinical condition.

CD11b Antigen↗
PubMed2002

[Frequency of postoperative fever in children with congenital heart disease undergoing cardiovascular surgery and associated risk factors].

OBJECTIVES: To determine the frequency of postoperative fever in children with congenital heart disease who undergo cardiovascular surgery, and the risk factors associated. PATIENTS AND METHODS: In a prospective cohort study, 100 children under the age of less than 9 years were followed-up during hospitalization in order to detect fever after cardiac surgery. Preoperative, perioperative, and postoperative variables were assessed to determine their relationship with postoperative fever. The cases were patients who developed fever. Multivariate analysis was used, and the odds ratio (OR) and 95% confidence intervals (95% CI) were calculated. RESULTS: The frequency of postoperative fever was 46%. Fever appeared within 24 hours of surgery in 56% cases. In 32/46 (70%) cases, fever remitted within 72 hours. Fever was more common in patients who underwent open-heart surgery than in those treated with a closed technique (28 vs. 18, P = 0.045). Prolonged extracorporeal circulation (OR = 1.024; 95% CI, 1.004-1.045), aortic cross-clamping (OR = 2.83; 95% CI, 1.21-6.61) and postoperative infections (OR = 24.07; 95% CI, 7.2-75.0) were the risk factors associated with the development of postoperative fever. CONCLUSIONS: Postoperative fever is common in children with congenital heart disease. The identification of risk factors associated to the development of fever should help clinicians to identify the cause of fever in this group of patients.

Age Factors↗
PubMed2006

Coronary artery anatomy in children with congenital heart disease by computed tomography.

AIMS: To evaluate electron beam computed tomography (EBCT) for recognition of coronary artery patterns in children with congenital heart diseases. METHODS: Institutional review board approval was obtained; informed consents were not required. A total of 226 children diagnosed with Tetralogy of Fallot (n=122), double outlet right ventricle (n=52), transposition of the great arteries (n=34), and congenitally corrected transposition (n=18) who had undergone cardiac EBCT at our institution between 1995 and 2002 were identified. Iodinated contrast medium was injected with arterial phase acquisition. The two radiologists and one pediatric cardiologist that interpreted the EBCT images and cardiac angiograms, respectively, were blinded to each other's results. Surgical and cardiac angiogram findings were compared to the EBCT results. Descriptive statistics were used to compare efficacy. RESULTS: Numerous aberrant patterns were clearly identified on the EBCT images. Pattern IX occurred in most patients with Tetralogy of Fallot or double outlet right ventricle. Patterns I and 0 are the most common coronary artery types in transposition of the great arteries and congenitally corrected transposition, respectively. Overall diagnostic accuracy for all disease groups was 82.7%. The diagnostic accuracy of the coronary arterial anatomy by EBCT increased with older age, and was more than 90% in individuals aged over 3 months. CONCLUSION: EBCT is effective for identification of the coronary anatomy of children with specific congenital heart diseases, except for neonates and small infants less than 3 months of age.

Adolescent↗
PubMed1985

Congenital heart disease as a significant factor in the morbidity of children with Down's syndrome.

A comparison of children with Down's syndrome classified according to having congenital heart disease has shown no differences in contact with the general practitioner, new episodes of respiratory illness, acute admission to hospital or mortality over the period of 1981. It is suggested that the prognostic significance of coincidental congenital heart disease in Down's syndrome be questioned.

Child↗
PubMed1994

[Congenital heart diseases in Sør-Trøndelag. Incidence, diagnosis, course and treatment].

In the county of Sør-Trøndelag, Norway, 145 children with congenital heart disease were detected among 14,194 live-born children from 1 January 1987 to 31 December 1990. This represents an incidence of 10.2 per thousand live-born children. The children were followed for six to 42 months (mean 2.38 years). Ventricular septal defect was detected in 71 (49%) cases. Isolated ventricular septal defect was found in 56 (39%) cases, the muscular type being the most frequent (three times that of the membranous type). Other congenital defects were found in 28 cases (19%), either as chromosome aberrations, syndromes or extracardiac malformations. 13 (9%) children died. None had constant symptoms of congestive heart failure by the end of the study. 34 (23%) cases were referred from mother and child clinics. 8 (5.5%) cases were detected prenatally. Compared with the results of other studies, the total incidence of congenital heart disease was high, and mortality and incidence of other coexistent congenital defects were low. It seems that small and less severe heart defects were detected more frequently in this study. The difference was most marked for incidence of muscular ventricular septal defect, atrial septal defect and pulmonary stenosis. The probable reasons for these results are an almost complete register of cases, well established postnatal care and a short distance to a pediatric cardiologist.

Child, Preschool↗
PubMed1975

Cyanotic congenital heart disease: Surgical techniques and pitfalls.

A review is presented of the currently employed approaches and techniques for the treatment of several forms of cyanotic congenital heart disease. Emphasis is placed on unusual associated defects which require special approaches for management. The question of definitive repair in infancy, as opposed to early palliation and later repair, is particularly pertinent to these conditions. It is evident that considerable progress has been made during the thirty-year evolution of surgery for congenital heart disease, but there are many advances yet to be made.

Child, Preschool↗
PubMed1992

Down syndrome: molecular mapping of the congenital heart disease and duodenal stenosis.

Down syndrome (DS) is a major cause of congenital heart and gut disease and mental retardation. DS individuals also have characteristic facies, hands, and dermatoglyphics, in addition to abnormalities of the immune system, an increased risk of leukemia, and an Alzheimer-like dementia. Although their molecular basis is unknown, recent work on patients with DS and partial duplications of chromosome 21 has suggested small chromosomal regions located in band q22 that are likely to contain the genes for some of these features. We now extend these analyses to define molecular markers for the congenital heart disease, the duodenal stenosis, and an "overlap" region for the facial and some of the skeletal features. We report the clinical, cytogenetic, and molecular analysis of two patients. The first is DUP21JS, who carries both a partial duplication of chromosome 21, including the region 21q21.1-q22.13, or proximal q22.2, and DS features including duodenal stenosis. Using quantitative Southern blot dosage analysis and 15 DNA sequences unique to chromosome 21, we have defined the molecular extent of the duplication. This includes the region defined by DNA sequences for APP (amyloid precursor protein), SOD1 (CuZn superoxide dismutase), D21S47, SF57, D21S17, D21S55, D21S3, and D21S15 and excludes the regions defined by DNA sequences for D21S16, D21S46, D21S1, D21S19, BCE I (breast cancer estrogen-inducible gene), D21S39, and D21S44. Using similar techniques, we have also defined the region duplicated in the second case occurring in a family carrying a translocation associated with DS and congenital heart disease. This region includes DNA sequences for D21S55 and D21S3 and excludes DNA sequences for D21S47 and D21S17.(ABSTRACT TRUNCATED AT 250 WORDS)

Blotting, Southern↗
PubMed1977

[An explanation of ventilation problems in congenital heart disease with left to right shunt].

The problems associated with the ventilation of children who have congenital heart disease with left to righ shunts and pulmonary hypertension are more likely to be acquired than due to the congenital malformation. Haemodynamic, angiographic, bronchoscopic and bronchographic studies demonstrated that ventilation problems arose because of compression of the bronchus by the pulmonary artery. The bronchial compression should be treated by surgery to the heart defect without removing the lung. The intervention should be early to obtain maximum benefit and to avoid irreversible damage to the bronchus.

Bronchi↗
PubMed1988

In utero ultrasound diagnosis of congenital heart disease.

Two hundred and one pregnancies considered at high risk for congenital heart disease (CHD) underwent fetal cross-sectional echocardiographic (CSE) examination. In 190 cases a structurally normal heart was correctly predicted; seven cases of CHD were identified, but in two of them the specific diagnosis was inaccurate. One false positive diagnosis of a small ventricular septal defect was made. There were three false negative diagnoses: two of ventricular septal defects and one of aortic coarctation. We conclude that severe heart malformations can be reliably identified or excluded by CSE in utero, but important anatomical details may be missed. Therefore, prognosis should be based only on the structures identified to multiple-scan planes.

Echocardiography↗
PubMed1999

Stent implantation to create interatrial communications in patients with complex congenital heart disease.

A restrictive interatrial communication can complicate the management of complex congenital heart disease. The purpose of this report is to present a new technique to achieve a patent and reliable interatrial communication by using an endovascular stent. A stent was successfully implanted across a fenestrated extracardiac conduit in two patients with low cardiac output after Fontan operations and across the interatrial septum in a patient with double inlet left ventricle and severe left atrioventricular stenosis. The procedures were uncomplicated and all patients showed immediate hemodynamic improvement. Cathet. Cardiovasc. Intervent. 47:310-313, 1999.

Child, Preschool↗
PubMed2005

Longitudinal development of psychopathology in an adult congenital heart disease cohort.

BACKGROUND: Little is known about the development of psychopathology in patients with congenital heart disease (ConHD) from adolescence into adulthood. In this study, the course of psychopathology in a cohort of ConHD adults, who received their first heart surgery in childhood, was determined longitudinally over a 10-year-period. METHODS: At both the first (1989-1991) and second (2000-2001) follow-up, patients (n=251, aged 20-32 years) completed questionnaires during a psychological examination. Parallel instruments were used to measure psychopathology in adolescence and adulthood. RESULTS: Young ConHD female patients showed higher levels of psychopathology compared to the reference group. Larger differences in psychopathology were found between younger (20-27 years) and older (28-32 years) patients compared to the reference group; the younger patients showed more psychopathology than the older patients. Also, larger differences in psychopathology were found between male and female patients compared to the reference group; female patients showed more psychopathology than male patients. Parents and partners of patients evaluated the patients' behavior as more deviant from the norm than patients themselves. There was a stronger decline of psychopathology in ConHD patients with increasing age compared to reference groups. Female and younger patients showed more variability in trajectories of psychopathology than did male and older patients. CONCLUSIONS: Assessment and treatment of psychopathology at a younger age is highly desirable in ConHD patients. Young adult ConHD female patients deserve special attention since they might be hampered by disease-specific uncertainties.

Adolescent↗
PubMed1995

Behavioral and physiological response during feeding in infants with congenital heart disease: a naturalistic study.

Twenty mother-infant dyads, ten with congenital heart disease (CHD) and ten controls, were studied during a naturally occurring feeding episode. Heart and respiratory rate data from a Hewlett Packard Infant Monitor were recorded on a computer while the feeding was videotaped for later scoring. No significant relationship was found between potent disengagement cues and changes in either heart or respiratory rate during feeding. However, the presentation of subtle disengagement cues were observed to be significantly more frequent among infants with CHD. A wide range of individual differences were noted in presentation of disengagement cues and heart and respiratory rates in both groups. No discernable pattern of heart rate or respiratory rate increase or decrease related to subtle or potent disengagement cures was found in either the infants with CHD or the healthy controls.

Adult↗
PubMedDate not supplied

Contrast-enhanced magnetic resonance angiography of the great arteries in patients with congenital heart disease: an accurate tool for planning catheter-guided interventions.

BACKGROUND: Catheter-guided interventions are increasingly used for relief of lesions in patients with congenital heart disease. Exact anatomical imaging with measurement of the vascular structures is crucial in the planning of such interventions. This can be provided non-invasively and without radiation by contrast-enhanced MR angiography (CE-MRA). AIM: To evaluate the accuracy of the measurements of the vessels obtained by CE-MRA in comparison to those obtained by conventional X-ray angiography (XRA). METHODS: Measurements of the diameters of aorta and pulmonary arteries were performed retrospectively and blinded on the CE-MRA and XRA images, in comparable locations. The limits of agreement between the two methods were calculated. RESULTS: Twenty-one CE-MRA and XRA were performed in 20 children with congenital heart disease, median age 4 years (1 day-13 years), weight 18 kg (3.2-74 kg). The time interval between CE-MRA and XRA was 2.6 +/- 2.3 months. A total of 98 measurements, 38 of the aorta and 60 of the pulmonary arteries were performed on the images obtained by each technique. The correlation between CE-MRA and XRA measurements was excellent, r = 0.97, p < 0.0001. The mean difference between the two techniques was 0.018 +/- 1.1 mm; the limits of agreement -2.14 and + 2.18 mm. Similar agreement was found for measures of the aorta (r = 0.97, mean difference 0.20 +/- 1.08 mm) and of the pulmonary arteries (r = 0.97, mean difference 0.048 +/- 0.89 mm). CONCLUSIONS: CE-MRA provides accurate quantitative anatomical information, which highly agrees with XRA data, and can therefore be used for planning catheter-guided procedures.

Adolescent↗
PubMed2005

The adult with congenital heart disease: born to be bad?

The population of patients with adult congenital heart disease is approximately 800,000 in the U.S. Those with prior cardiac surgery often consider themselves "cured," although the majority faces a lifetime of problems including arrhythmias, ventricular dysfunction, and one or more re-operations. Even patients with repaired "simple" lesions such as an atrial septal defect may not have normal survival if they are repaired in adulthood. Patients with repaired coarctation may have premature cardiovascular complications including sudden cardiac death, myocardial infarction, and stroke. They also have aortic complications such as aneurysm and dissection, which result from a diffuse arteriopathy and continued hypertension that may be caused by underlying endothelial dysfunction. In addition, bicuspid aortic valve occurs in more than one-half of the patients with coarctation, so continued surveillance for significant aortic valvular heart disease is necessary. More complex lesions also pose problems after "total correction." Patients with repaired tetralogy of Fallot often have pulmonary regurgitation, which is frequently overlooked on clinical exam and echocardiography. Pulmonary valve replacement should be performed before the development of irreversible right ventricular dysfunction and an increased risk of ventricular tachycardia or sudden cardiac death. Because they are vulnerable to deterioration of systemic ventricular function, those with congenitally corrected transposition require special vigilance, usually with concomitant atrio-ventricular valve regurgitation. Late referral is common with a deleterious effect on long-term survival. These patients need lifelong follow-up and the residua and sequelae of their complex anomalies must be understood in order to provide optimum care.

Adult↗
PubMed2003

Comparison of different approaches for pediatric congenital heart diseases.

To compare the clinical results of different surgical approaches for congenital heart disease in pediatric patients, 1669 cases of atrial septal defect, ventricular septal defect, or tetralogy of Fallot, which were corrected from January 1999 to December 2001, were classified according to approach (sternotomy, ministernotomy, or minithoracotomy). In cases of ventricular septal defect, the incidence of pulmonary complications was significantly higher in the minithoracotomy group than in the full sternotomy or ministernotomy groups. In patients with tetralogy of Fallot, hemoglobin concentration was higher, oxygen saturation was lower, and more patients required a transanular patch in the sternotomy group than in the other groups, but the clinical results were similar. Patients with complex defects or severe pulmonary hypertension should undergo a full sternotomy.

Cardiac Surgical Procedures↗
PubMed1995

Bicuspid aortic valves in hearts with other congenital heart disease.

The bicuspid aortic valve is the most frequent congenital cardiac malformation; it may be isolated or associated with other congenital heart disease. The present investigation consists of a study of bicuspid aortic valves in 1022 heart specimens belonging to the anatomical collection of the Institute of Pathological Anatomy of the University of Padua. A bicuspid aortic valve was observed in 95 specimens. It occurred as an isolated congenital cardiac defect in 28 cases, seven of which had spontaneous laceration of the aortic valve (aortic dissection). It was associated with other congenital cardiac malformations in 67 out of the remaining 994 specimens (6.7%), 41 of which (61.2%) showed obstruction of the aortic arch. The frequency of bicuspid aortic valve in specimens with complete transposition of great arteries was only 1%. Bicuspid aortic valve was particularly frequent in association with ventricular septal defect and was significantly more frequent in cases with (51.1%) than in cases without (20.5%) aortic arch obstruction (p < 0.001). There was no significant relationship between the occurrence of bicuspid aortic valves and left ventricular outflow tract obstructions or mitral valve malformations. The morphology of the pulmonary valve was also examined. Concurrence of a bicuspid aortic and pulmonary valve was detected in 11 specimens, five of these had trisomy-18. Our findings cast doubt on the assumption that altered fetal blood flow through the aortic valve may be the main factor producing the bicuspid condition. Indeed, they rather support the hypothesis that most bicuspid aortic valves are expressions of a developmental complex that affects the aortic arch and the wall of the ascending aorta as well as the aorta valve.

Adult↗
PubMed1998

[Total cavo-pulmonary connection for complex congenital heart disease].

Between April 1994 and January 1997, 10 patients with complex congenital heart disease were treated with total cavo-pulmonary connection(TCPC). They included 7 males and 3 females, aged 2-17 years(mean 10.1 years). These cases were single ventricle in 5, tricuspid atresia in 4, severe Ebstein anomaly in 1. For construction of the right atrial lateral tunnel, Gore-Tex patch was used in 9 cases and autologous right atrial wall in another, 4 of the 10 patients underwent TCPC without aortic crossclamping. There were no operative deaths. The follow-up is 2-36 months(mean 12.3 months). All of these patients did well and were in NYHA class I. The advantages, operative indication and technique of TCPC and the experience of improvement of surgical results are briefly discussed in this paper.

Adolescent↗
PubMed1982

Reactions of families to the death of a child with congenital heart disease.

We interviewed 57 parents of 33 children who died of congenital heart disease to obtain their descriptions and perceptions of their own grief. Irrespective of the child's age or length of illness, the parents consistently described a grief reaction characterized by an initial period of shock lasting hours to several weeks which evolved into a four- to six-month period of sadness/depression with somatic complaints, which slowly progressed to resolution through acceptance. No parent described symptoms of pathologic grief. Only four of 33 surviving siblings demonstrated recognizable problems at the time of follow-up. There was physician follow-up of 15 families; only two families received follow-up from local physicians, even though nine children died at home or in community hospitals. All parents expected follow-up and accepted it when offered. Specific follow-up recommendations are made.

Adolescent↗