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PubMed1991

Effect of fathers' age and birth order on occurrence of congenital heart disease.

STUDY OBJECTIVE: The aim was to examine if there is an effect of fathers' age and of birth order on the occurrence of congenital heart disease. DESIGN: This was a hospital based case-referent study including use of birth defects surveillance data. SUBJECTS: Subjects were 497 cases of congenital heart disease aged between 3 months and 5 years, born in Beijing and Hebei Province, China; 6222 children without congenital heart disease serve as reference baseline. MEASUREMENTS AND MAIN RESULTS: With stratified analysis and logistic regression analyses, congenital heart disease was found to be associated with fathers' age less than 25 years (odds ratio 2.63), independent of mothers' age and of birth order. There was also evidence to show a higher birth order effect on the occurrence of congenital heart disease independent of parental ages. CONCLUSION: Higher birth order and fathers aged less than 25 years were both independently associated with some categories of congenital heart disease and with congenital heart disease overall.

Birth Order↗
PubMed2005

Hypoxic-ischemic brain injury in infants with congenital heart disease dying after cardiac surgery.

Cardiac surgery for congenital heart disease is performed increasingly earlier in infancy, including in the neonatal period. With increased survival of infants, there is growing concern about the long-term neurological sequelae of hypoxic-ischemic injury due to congenital heart disease itself prior to surgery, corrective surgery with the use of low-flow cardiopulmonary bypass (CPB) and/or deep hypothermic circulatory arrest (DHCA), and/or unstable hemodynamic factors postoperatively. In analyzing the neuropathology of 38 infants dying after cardiac surgery, we tested a set of questions related to the severity and patterns of brain injury, CPB, DHCA, and age of the infants at the time of surgery. In all infants dying after cardiac surgery, irrespective of the modality, cerebral white matter damage [periventricular leukomalacia (PVL) or diffuse white matter gliosis] was the most significant lesion in terms of severity and incidence, followed by a spectrum of gray matter lesions. There was no significant association between the duration of deep hypothermic circulatory arrest and the degree of severity of overall brain injury, and the pattern of brain injury was similar irrespective of the modality of cardiac surgery. There was no significant association between the age at the time of surgery (neonatal versus postneonatal) and the severity of overall brain injury. The patterns of brain injury were not age-related in the limited time-frame analyzed, except that infants who developed acute PVL after both closed and DHCA/CPB surgery (14/38 infants, 34%) were significantly younger at death (median age 13.0 days) compared to unaffected infants (median age at death 42.5 days) (P=0.031). This observation suggests that neonatal (<30 postnatal days), but not postneonatal (>30 postnatal days), brains are at risk for acute PVL, and likely reflects the vulnerability of immature (pre-myelinating) white matter to hypoxia-ischemia.

Age Factors↗
PubMed2003

MR imaging of congenital heart disease.

MR examination of patients with congenital heart disease is a useful means of explicitly demonstrating chamber morphology and, in particular, morphologic changes caused by physiologic changes brought on by particular defects. Use of MR techniques allows characterization of chamber morphology for determination of cardiac connections and great artery relationships. The high-contrast resolution of spin echo acquisition provides important morphologic detail. Cine gradient echo techniques complement spin echo acquisition by providing functional and flow information. Although MR examination complements echocardiographic investigation in pediatric and adult patients, is may be useful for replacing angiocardiography, shortening examination time, and decreasing morbidity in diagnostic workups of these patients.

Heart Defects, Congenital↗
PubMed2006

The team approach to pregnancy and congenital heart disease.

The successful pediatric management of congenital heart disease has resulted in increasing numbers of these patients in the reproductive age group and increasing clinical challenges for their physicians. These challenges can be met successfully, with improved results for mother and child, through a concerted comprehensive team approach that relies on a thorough understanding of the patient's underlying cardiac pathology and its anticipated interaction with the pregnancy, and ongoing close evaluation and communication with a team of trained and experienced specialist, including (but not limited to) cardiologist, obstetricians, anesthetists, pediatricians, clinical nurse specialists, and clinical geneticists. Such teams are not always available locally and it will be necessary to refer medium- and high-risk patients to a specialized tertiary care center.

Adult↗
PubMed2000

Influence of congenital heart disease on mortality after noncardiac surgery in hospitalized children.

OBJECTIVE: To evaluate the incremental risk of congenital heart disease on mortality after noncardiac surgery in children. DESIGN: We reviewed the clinical information network database of the University Hospital Consortium for the period January 1, 1993, through December 31, 1996, and identified 191 261 patients <18 years old having 1 or more noncardiovascular surgical procedures. Of these patients, 6.5% had a diagnosis of congenital heart disease. Patients having ambulatory surgery are excluded from this database. Short-term (1-, 2-, and 3-day) and 30-day mortality were compared, as well as mortality for neonates, infants (31 days to 1 year), and older children (1-17 years); mortality for the 100 most common surgical procedures, mortality for 10 relatively minor surgical procedures, and mortality in subgroups of patients with minor versus severe cardiac diagnoses. RESULTS: Short-term and 30-day mortality was increased in the patients with congenital heart disease patients (30-day mortality odds ratio 3.5; 95% confidence limit, 3.2-3.9). Mortality was also increased in patients with congenital heart disease in the 2 youngest age groups, for the 100 most common operations, and for 10 relatively minor operations. Children with more severe heart disease diagnoses had higher mortality than did children carrying less serious cardiac diagnoses. CONCLUSION: A diagnosis of congenital heart disease adds significant incremental risk of mortality in children requiring inpatient noncardiovascular surgery. This outcome difference is present for both minor and major surgical procedures, and regardless of whether mortality is measured at 1, 3, or 30 days. The incremental risk is greatest in neonates and infants where the presence of congenital heart disease is associated with a 2-fold increase in mortality from noncardiac surgery.

Adolescent↗
PubMed1976

Sequential chamber localization--logical approach to diagnosis in congenital heart disease.

A nomenclature is described for congenital heart disease employing sequential chamber localization. It is an eclectic system based in part upon the previous classifications of Van Praagh and Kirklin. It links together the atrial, ventricular, and arterial segments of the heart and then permits tabulation of associated anomalies. The atrial segment of the heart can exist as situs solitus, situs inversus, or situs ambiguus. Atrioventricular connexions can be concordant or discordant. In certain circumstances the terms concordant and discordant cannot be used. These are in the presence of primitive ventricle and in the presence of situs ambiguus. Alternative terms are described for these contingencies. Ventriculo-arterial connexions can be (a) normal; (b) transposition; (c) double outlet ventricle; or (d) single arterial trunk. These are defined as connexions; relations are relegated to secondary position. Associated anomalies are categorized in terms of venous return, atria, atrioventricular junction, ventricles, and great arteries. Controversial topics are discussed with regard to previous definitions.

Angiocardiography↗
PubMed1976

Postoperative pathology of congenital heart disease.

Complete and unselected data concerning the postoperative pathology of congenital heart disease are presented for the first time. This study was based on 2,365 autopsies performed at the Children's Hospital Medical Center, Boston, in the 9 years from 1966 through 1974. Of these, 586 autopsies (25 percent) revealed congenital heart disease--238 performed in medically treated patients (41 percent) and 348 in surgically treated patients (59 percent). Tetralogy of Fallot, including cases with pulmonary outflow tract atresia and other associated malformations, was the congenital heart disease most often encountered in the postoperative autopsy series (88 cases, 25 percent of that series). D-transposition of the great arteries, including cases with other associated anomalies, was second (54 cases, 15.5 percent). Early death (hospital mortality) accounted for 320 (92 percent) of the 348 surgical cases; late death occurred in 28 patients (8 percent). Causes of late postoperative death included arrhythmias, excessively small ventricular septal defect with tricuspid atresia, massive hemoptysis, rupture of the pulmonary artery, cyanotic spell, congestive heart failure and infection. Prophylactic penicillin is recommended for patients with the asplenia syndrome because of their probably enhanced vulnerability to fulmfulminating septicemia by encapsulated bacteria such as the pneumococcus. Completeness and lack of selection in reporting data are essential in the interests of perspective and comparability of findings.

Autopsy↗
PubMed1988

Isolated infundibuloarterial inversion (S,D,I): a newly recognized form of congenital heart disease.

A newly recognized form of congenital heart disease is presented that is characterized by viscero-atrial situs solitus (S), D-loop ventricles (D), and inverted normally related great arteries (I), the segmental combination being (S,D,I). This anomaly may be called isolated infundibuloarterial inversion because only the subsemilunar infundibulum and the great arteries are inverted, whereas the atrial and the ventricles are not. All three patients had atrioventricular concordance, ventriculoatrial concordance, dextrocardia, superoinferior ventricles, crisscross atrioventricular relations, underdevelopment of the right ventricle, a large ventricular septal defect, and an inverted tetralogy of Fallot type of malformation of the infundibulum and great arteries. The condition known as crisscross atrioventricular relations was found in these three patients to be a major ventricular malposition characterized by marked clockwise rotation of the ventricles, as seen from the front. Two of these three cases were diagnosed accurately and repaired successfully.

Angiocardiography↗
PubMed2001

Arterial Doppler ultrasound in 115 second- and third-trimester fetuses with congenital heart disease.

OBJECTIVE: To assess the influence of isolated congenital heart disease (CHD) on fetal arterial Doppler blood flow velocity waveforms. METHODS: Doppler flow velocimetry was performed in the umbilical artery and middle cerebral artery in 115 consecutive fetuses with antenatally diagnosed CHD. Gestational age ranged between 19 and 41 weeks. Fetuses with isolated CHD were defined as group A (n = 55), showing cardiogenic hydrops fetalis in six cases; group B included 60 cases complicated by chromosomal or non-chromosomal extracardiac malformation, uteroplacental dysfunction or non-cardiogenic non-immune hydrops fetalis. The control group comprised 100 healthy fetuses of uncomplicated pregnancies. Individual pulsatility index measurements were converted into their Z-scores (delta values) for statistical analysis. RESULTS: In regard to the umbilical artery pulsatility index, 115 fetuses with CHD showed a significantly greater (P < 0.001) difference from the normal mean for gestation (delta values) than the control group. However, 29 of the 33 cases with indices above the 95% reference interval were additionally associated with extracardiac malformations, uteroplacental dysfunction or non-cardiogenic non-immune hydrops fetalis. While fetuses with isolated CHD still showed significantly higher values than healthy fetuses (P < 0.01), only in 4 of 55 (7%) fetuses did the measured umbilical artery pulsatility index exceed the 95% reference interval. There was no significant difference from the control group, in which 4 of 100 cases showed an umbilical artery pulsatility index above the 95% reference interval. Elevated umbilical artery pulsatility indices were seen in only four cases of severe obstruction of the outflow tracts leading to reverse perfusion of the affected great artery and in one case of Ebstein's anomaly with pulmonary insufficiency. Although all four fetuses with isolated CHD and elevated umbilical artery pulsatility index died, 14 of 18 fetuses with lethal outcome had normal pulsatility index values in the umbilical artery. Investigations of the middle cerebral artery blood flow revealed no significant difference between fetuses with and without CHD or any subgroups. CONCLUSIONS: This study shows that arterial blood flow velocity waveforms in fetuses with isolated CHD do not show sufficient alterations to be of diagnostic value. Only in severe outflow tract obstructions due to a 'steal effect' or in significant insufficiencies of semilunar valves leading to an impaired 'wind-kessel function' may the special hemodynamic changes induced by CHD result in a significant increase of pulsatility index in the umbilical artery. In the majority of cases with CHD the increase of pulsatility index of umbilical arterial blood flow velocity waveforms, however, results from extracardiac anomalies, especially uteroplacental dysfunction and chromosomal abnormalities. Furthermore, umbilical artery Doppler sonography is not clinically helpful in predicting fetal outcome.

Blood Flow Velocity↗
PubMed1979

The use of prostaglandin E1 and Blalock-Taussig shunts in neonates with cyanotic congenital heart disease.

Six unselected neonates with cyanotic congenital heart disease and life-threatening degrees of arterial oxygen desaturation have been managed by a protocol that includes administration of prostaglandin E1 (PGE1) and early Blalock-Taussig shunting. In 5 patients (seven paired observations) partial pressure of arterial oxygen (PaO2) rose from 19 mm Hg to a mean of 32.9 mm Hg within 20 minutes of initiation of PGE1 (0.1 to 0.2 microgram/kg/hr), infused intravenously or through an aortic catheter placed at ductal level or with both methods. The nonresponsive patient was older than the patients showing a positive response (1 month versus 24 to 96 hours). Following catheterization, immediate palliative operation including a Blalock-Taussig shunt was carried out. Although all had a satisfactory PaO2 (mean, 49 mm Hg) postoperatively, the PGE1-nonresponsive patient experienced serious intraoperative bradycardia, hypotension, and acidosis in contrast to the PGE1-responsive group. In this study, the use of PGE1 was not associated with any apparent serious side effects.

Drug Evaluation↗
PubMed1998

Respiratory syncytial virus immune globulin for prophylaxis against respiratory syncytial virus disease in infants and children with congenital heart disease. The Cardiac Study Group.

OBJECTIVE: To examine the effectiveness of respiratory syncytial virus immune globulin administered intravenously (RSV-IGIV) in reducing hospitalization for treatment of RSV in children with congenital heart disease (CHD). METHODS: Children younger than 4 years of age were randomly assigned to a treatment group receiving RSV-IGIV, 750 mg/kg, monthly or to a control group not receiving infusions. Surveillance for respiratory tract infections was carried out and management decisions were made by physicians blinded to treatment group. RESULTS: Hospitalization for treatment of an RSV infection occurred in 32 of 214 (15%) of control children and 21 of 202 (10%) of the children receiving RSV-IGIV, a 31% reduction (P = .16). However, in infants younger than 6 months of age at study entry, 20 of 82 (24%) in the control group and 10 of 96 (10%) in the RSV-IGIV group had RSV hospitalizations (58% reduction, P = .01). The incidence of hospitalization for any respiratory tract symptomatology was lower in the RSV-IGIV group (34 of 202, 17%) than in the control group (57 of 214, 27%; P = .02). There was a significantly higher frequency of unanticipated cyanotic episodes and of poor outcomes after surgery among children with cyanotic CHD in the RSV-IGIV group (22 of 78, 28%) than in the control group (4 of 47, 8.5%; P = .009). CONCLUSION: RSV-IGIV should not be used for prophylaxis of RSV disease in children with cyanotic CHD. RSV-IGIV did not reduce RSV hospitalization in all children with CHD, but it was effective in preventing RSV hospitalization in infants younger than 6 months of age. Further studies in these children are indicated.

Age Factors↗
PubMed1994

Thrombin regulation in congenital heart disease after cardiopulmonary bypass operations.

Children with cyanotic congenital heart disease who undergo operation with cardiopulmonary bypass are at increased risk of thromboembolic or hemorrhagic complications, or both. Regulation of thrombin, a key enzyme in coagulation, is essential in preventing these complications. We therefore examined the in vitro capacity of plasma from 15 children with cyanotic congenital heart disease to generate thrombin and to inhibit 125I-thrombin before and after cardiopulmonary bypass. We also assessed whether thrombin had been generated in vivo by assaying levels of fibrinogen, thrombin-antithrombin III complexes, and D-dimer. Plasma levels of the thrombin inhibitors, antithrombin III, alpha-2-macroglobulin, and heparin cofactor II were also measured. Thrombin regulation was normal before operation. After cardiopulmonary bypass, the in vitro capacity to generate thrombin decreased by 50%, and this was primarily a result of hemodilution (31%). Similar postoperative decreases were noted in the levels of antithrombin III, heparin cofactor II, and alpha-2-macroglobulin (26% to 45%). However, the total in vitro plasma thrombin inhibitory capacity decreased by only 13%. Levels of thrombin-antithrombin III and D-dimer increased after operation, indicating that thrombin had been generated and inhibited in vivo. Clinically, there were no thromboembolic complications although six patients required replacement therapy for excessive small-vessel bleeding. In conclusion, thrombin regulation is significantly altered after cardiopulmonary bypass. Although thrombin is generated in vivo, the total residual capacity to do so is impaired because of hemodilution. Despite a concomitant decrease in thrombin inhibitor levels, the total residual in vitro capacity of plasma to inhibit thrombin is relatively spared. This suggests that after cardiopulmonary bypass the risk of hemorrhagic complications after an additional hemostatic challenge is relatively greater than the risk of thrombotic complications. This might be reflected in the predominance of hemorrhagic complications in our patients.

Adolescent↗
PubMed2006

A multicentre approach for the management of adults with congenital heart disease.

OBJECTIVE: At present, the level of care for adult congenital heart disease is not completely satisfactory in many European countries including Italy. The aim of this multicentre study was to evaluate the possibility of creating an active connection among different centres involved in the care of this patient population. METHODS: This study lasted two years, from December 2002 to December 2004. It involved six clinical centres located in the north, centre and south of Italy; a research centre (ISBEM) affiliated to the National Research Centre was also involved. Each centre was supposed to contribute with written information (about their specific subject) on the web site; our target readers were physicians, nurses and patients. The intranet part of the web connection was used for a registry to be filled with very simple information (demographic data, main diagnosis, reason for hospitalisation, type of treatment) about patients hospitalised after January 2000. The aim of the registry was to get a snapshot of the composition of the treated population, and the spectrum of the lesions. RESULTS: The main results are the following: (i) the creation of the web site www.guch-italia.it which can be accessed without a specific password; (ii) the creation of a registry in which we entered the clinical information of 1,231 patients; (iii) the presentation of guidelines on the web site, extrapolated from the recommendations for the management of adults with congenital heart disease from the Canadian Cardiovascular Society Consensus Conference. CONCLUSIONS: With this study we tried to create a national network of centres that have as an objective to optimise the assistance to patients with adult congenital heart disease, trying to make the approach as much homogeneous as possible.

Adolescent↗
PubMed1996

Cerebrovascular events in adult patients with cyanotic congenital heart disease.

OBJECTIVES: We sought to determine the frequency of spontaneous cerebrovascular events in adult patients with cyanotic congenital heart disease and to evaluate any contributing factors. BACKGROUND: Cerebrovascular events are a serious complication of cyanotic congenital heart disease in infants and children but are said to be uncommon in adults. METHODS: Between 1988 and 1995, 162 patients with cyanotic congenital heart disease (mean age 37 years, range 19 to 70) were retrospectively evaluated for any well documented cerebrovascular events that occurred at > or = 18 years of age. Events related to procedures, endocarditis or brain abscess were excluded. RESULTS: Twenty-two patients (13.6%) had 29 cerebrovascular events (1/100 patient-years). There was no significant difference between those with and without a cerebrovascular event in terms of age, smoking history, degree of erythrocytosis, ejection fraction or use of aspirin or warfarin (Coumadin). Patients who had a cerebrovascular event had a significantly increased tendency to develop hypertension, atrial fibrillation, microcytosis (mean corpuscular volume < 82) and history of phlebotomy (p < 0.05). Even when patients with hypertension or atrial fibrillation were excluded, there was an increased risk of cerebrovascular events associated with microcytosis (p < 0.01). CONCLUSIONS: Adults with cyanotic congenital heart disease are at risk of having cerebrovascular events. This risk is increased in the presence of hypertension, atrial fibrillation, history of phlebotomy and microcytosis, the latter condition having the strongest significance (p < 0.005). This finding leads us to endorse a more conservative approach toward phlebotomy and a more aggressive approach toward treating microcytosis in adults with cyanotic congenital heart disease.

Adult↗