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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↗

Long-term psychologic implications of congenital heart disease: a 25-year follow-up.

Patients with various types of congenital heart disease were contacted 25 years after their original examination at the Mayo Clinic. In addition to providing their current health status, level of education achieved, and current occupation, they were asked to complete a detailed standardized questionnaire to assess their degree of psychologic stress. Of the original 463 patients, 168 completed and returned the psychologic questionnaires. These patients had evidence of psychologic stress in excess of that expected on the basis of normative data. Furthermore, the degree of stress was unrelated to the clinical severity of the original cardiac defect. In addition, the psychologic stress occurred despite "success" as defined by educational achievement and occupational level. One can speculate that as children these patients were exposed to environmental stresses that may well have been colored by parental attitudes and perceptions.

Adult↗

[The localization of parvovirus B19 in cardiac biopsy tissue of congenital heart disease].

OBJECTIVE: To explore the relationship between parvovirus B19 infection and congenital heart disease (CHD), and distribution of parvovirus B 19 gene in cardiac tissue. METHODS: We conducted a case controls study investigating the presence of B19 gene in cardiac biopsy tissue of 37 cases of CHD, 8 cases of rheumatic heart disease (RHD) and 20 cases of non-CHD with nested polymerase chain reaction (Nested PCR) and in situ hybridization (ISH). RESULTS: Among 37 CHD patients, 6 were B19 DNA positive in cardiac tissue (18.92%), while in control group, including 8 RHD patients (autopsy specimens) and 20 non-CHD patients, the B19 gene were all negative, there was a significant difference of B19 gene presence between CHD group and control group (P = 0.0421). ISH of B19 gene in cardiac tissue were positive in 4 CHD patients and all were negative in control group. The B19 gene was found locating in the nucleus of cardiac cell by ISH. CONCLUSIONS: Parvovirus B19 infection correlated with CHD. Since B19 gene was mainly found in the cardiac cell nucleus, maybe it influenced the regulation of gene expression and affected the development of heart with the result of CHD.

Adolescent↗

Magnetic resonance imaging in congenital heart disease of newborns: preliminary results in 23 patients.

Until now little attention has been paid to the potential of MR imaging in congenital heart disease of the newborn. ECG-gated MRI was therefore performed at 0.5 tesla in 23 newborns (mean age 7.5 days) with suspected congenital heart disease. Two newborns were controlled after surgery (switch, Blalock-Taussig procedure). All had undergone prior evaluation by two-dimensional Doppler echocardiography (2-D DE). MR imaging was of a satisfying quality in all but one newborn. The aim of this study was to assess complementary information provided by MRI in comparison to 2-D DE. Pre-operatively MRI missed some abnormalities shown by 2-D DE: one coartaction, one ductus arteriosus and one pulmonary atresia. MRI demonstrated lesions that echocardiography had either failed to visualize or found inconclusive, including double aortic arch (one patient), muscular ventricular septum defect (two patients) and severe ductus arteriosus (one patient). In one of the two patients with a ventricular septum defect, angiography was avoided and in the other patient it merely confirmed the MRI results. Post-operatively, MRI demonstrated information complementary to that obtained from to 2-D DE: (1) clearly visualizing the reinsertion of the coronary arteries in the 'switched' transposition of the great vessels, (2) appreciating the diameter and patency of the palliative shunt in the Blalock-Taussig procedure.

Angiography↗

[Anatomo-vectorcardiographic correlation in ventricular growth in congenital heart diseases].

Anatomo-vectorcardiographic correlations were studied in 59 cases of congenital heart disease. In the group with interventricular septal defect and pulmonary hypertensión, a statistically significant correlation between the thickness of the free right ventricular wall, at its outflow tract, and the inscription time of the vertex of the S loop, was found. In the group of Fallot's tetralogy, the segmentary hypertrophy of the right ventricle, which is the most frequent, explains very well the terminal portion of frontal and sagittal vectorcardiograms located below the 0 point. In the group with interatrial septal defect and pulmonary hypertension, a statistically significant correlation between the thickness of the antero-lateral superior third of the free right ventricular wall and the inscription time of the vertex of the S loop, was shown. In the group of congenital malformations with repercussion on the left ventricle (interventricular septal defect, patent ductus arteriosus and coarctation of aorta), a statistically significant correlation between the anatomical findings of a hypertrophied superior third of this ventricle and the inscription time of the vertex of the R loop, was observed.

Adult↗

Atrioventricular pacing in congenital heart disease.

We have attempted to optimize cardiac performance in patients with congenital heart disease requiring artificial pacing by using pacemakers capable of both sensing and pacing both the atrium and the ventricle (DDD). We reviewed our results with 88 patients receiving DDD devices to determine the safety and dependability of these devices in children. Age ranged from 1 hour to 25 years. Endocardial leads were used in 68 patients, whereas epicardial leads were used in 20 patients. Previous cardiac procedures had been done in 30 patients. There were nine deaths but none due to pacemaker malfunction. Endocardial leads functioned better than epicardial leads. Ninety-eight percent of patients with endocardial leads and 62% of patients with epicardial leads were maintained in the DDD mode. Complications were infrequent and all were corrected without long-term sequelae. The DDD mode may offer considerable benefits to children who require artificial pacing. Our data allow us to conclude that most children can be paced safely and dependably in the DDD mode.

Adolescent↗

Fetal haemoglobin variations following hydroxyurea treatment in patients with cyanotic congenital heart disease.

Haematological features of 64 patients suffering from non operable cyanotic congenital heart disease (CCHD) treated with hydroxyurea (HU) were compared with those of 43 patients suffering from the same disorder who had not yet received this drug. Patients with subclinical renal dysfunction were excluded by measuring plasma creatinine levels. MCV and HbF were higher among patients receiving HU, the increase in MCV being cumulative with HU dosage but the rise in HbF dose independent. HbF response to HU was found to be due to the coordinated increase in F-cell and F-reticulocyte production rather than to a selective survival of F-cells. Absence of a relationship between plasma erythropoietin and HbF levels excluded a dominant role of the former in increasing F-cell production and results determined after doubling the HU dosage or immediately after initiating therapy suggested genetic differences to be responsible for the individual variations in Hb F response. No irreversible toxic effects or malignancies were noted in this series of patients. HU was administered for a relatively long period of time, the mean duration of treatment exceeding 5 years, while the study also included patients below the age of 10 years.

Adolescent↗

Quality of life in families of children with congenital heart disease.

Within a family perspective on quality of life (QL) with congenital heart disease, the study investigates parental QL, and patients' health-related QL as reported by themselves and by their parents. We examined the hypotheses that parental QL moderates the parental proxy reports. Sixty-nine patients (7-20 years, 61% male) and their caregivers participated in a computer-assisted QL-assessment. Children's self-rated and proxy-rated QL correlated moderately, with the highest intra-class correlation on the subscale psychological well-being/functioning (r = 0.61; p < 0.001), less convergence in physical well-being/functioning (r = 0.49; p < 0.001) and absent correlation in the evaluation of intra-family relationships. Parental QL was correlated both with the children's self-rated QL (r = 0.42; p < 0.05) and children's parent-rated QL (r = 0.60; p < 0.001). Support for the moderator hypotheses is indicated by the results of regression analyses demonstrating a significant interaction effect of parental QL and patients' self-reported QL in predicting parental proxy reports on their children's QL. Post-hoc tests reveal that parents with low own QL agree significantly more with their children than parents with high QL. Parent-child agreement on the children's QL is limited and reflects complementary subjective viewpoints. Psychosocial interventions should be family-focused and provide support for patients' and their caregivers' QL.

Adolescent↗

Monitoring of cerebral oxygenation during hypoxic gas management in congenital heart disease with increased pulmonary blood flow.

In the preoperative management of congenital heart disease (CHD) with increased pulmonary blood flow, hypoxic gas management to control pulmonary blood flow is useful. However, the cerebral oxygenation state has rarely been studied, and there is concern about neurologic development. In eight infants with CHD accompanied by increased pulmonary blood flow, hypoxia was induced after a 1-h baseline period in room air (FiO2, 0.21). The infants were simultaneously monitored in both the front-temporal region and the right-brachial region for 90 min using near-infrared spectroscopy (NIRS). The minimum SaO2 (pulse oximetry) after hypoxic gas administration was 80.8 +/- 2.9% when the minimum FiO2 was 16.2 +/- 1.1%. With a decrease in SaO2, oxy-Hb (O2Hb) decreased and total Hb [cHb: O2Hb + deoxy-Hb (HHb)] increased in both regions in the majority of infants. HHb increased in both regions with a decrease in SaO2. The maximum change in the tissue oxygenation index (TOI: O2Hb/cHb x 100) was -8.3 +/- 2.6% in the front-temporal region and -3.6 +/- 2.3% in the right-brachial region. Cerebral oxygenation decreased despite an increase in cerebral blood flow during hypoxic gas management. The change in TOI was < or =10% when the SaO2 was > or =80%. Safer control of SaO2 should be maintained over 80% for hypoxia management in CHD based on the results of the present study.

Brain↗

Screening for duct-dependant congenital heart disease with pulse oximetry: a critical evaluation of strategies to maximize sensitivity.

AIM: To evaluate the feasibility of detecting duct-dependent congenital heart disease before hospital discharge by using pulse oximetry. DESIGN: Case-control study. SETTING: A supra-regional referral centre for paediatric cardiac surgery in Sweden. PATIENTS: 200 normal term newborns with echocardiographically normal hearts (median age 1.0 d) and 66 infants with critical congenital heart disease (CCHD; median age 3 d). METHODS: Pulse oximetry was performed in the right hand and one foot using a new-generation pulse oximeter (NGoxi) and a conventional-technology oximeter (CToxi). RESULTS: With the NGoxi, normal newborns showed a median postductal saturation of 99% (range 94-100%); intra-observer variability showed a mean difference of 0% (SD 1.3%), and inter-observer variability was 0% (SD 1.5%). The CToxi recorded a significantly greater proportion of postductal values below 95% (41% vs 1%) in the normal newborns compared with NGoxi (p<0.0001). The CCHD group showed a median postductal saturation of 90% (45-99%) with the NGoxi. Analysis of distributions suggested a screening cut-off of <95%; however, this still gave 7/66 false-negative patients, all with aortic arch obstruction. Best sensitivity was obtained by adding one further criterion: saturation of <95% in both hand and foot or a difference of >+/-3% between hand and foot. These combined criteria gave a sensitivity of 98.5%, specificity of 96.0%, positive predictive value of 89.0% and negative predictive value of 99.5%. CONCLUSION: Systematic screening for CCHD with high accuracy requires a new-generation oximeter, and comparison of saturation values from the right hand and one foot substantially improves the detection of CCHD.

Case-Control Studies↗

Survival after surgery or therapeutic catheterisation for congenital heart disease in children in the United Kingdom: analysis of the central cardiac audit database for 2000-1.

OBJECTIVES: To analyse simple national statistics and survival data collected in the central cardiac audit database after treatment for congenital heart disease and to provide long term comparative statistics for each contributing centre. DESIGN: Prospective, longitudinal, observational, national cohort survival study. SETTING: UK central cardiac audit database. MAIN OUTCOME MEASURES: Survival at 30 days and one year after treatment in the year April 2000-March 2001, assessed by using both volunteered life status and independently validated life status through the Office for National Statistics, using the patient's unique NHS number, or the general register offices of Scotland and Northern Ireland. Institutional results following a group of six benchmark operations and three benchmark catheterisation procedures. RESULTS: Since April 2000 data have been received from all 13 UK tertiary centres performing cardiac surgery or therapeutic cardiac catheterisation in children with congenital heart disease. Altogether 3666 surgical procedures and 1828 therapeutic catheterisations were performed. Central tracking of mortality identified 469 deaths, 194 occurring within 30 days and 275 later. Forty two of the 194 deaths within 30 days were detected by central tracking but not by volunteered data. For surgery overall, survival at 30 days was 94.9%, falling to 91.2% at one year; this effect was most marked for infants. For therapeutic catheterisation survival at 30 days was 99.1%, falling to 98.1% at one year. Survival of individual centres or individual operators did not differ from the national average after benchmark procedures. CONCLUSIONS: Independent data validation is essential for accurate survival analysis. One year survival gives a more realistic view of outcome than traditional perioperative mortality. Currently no detectable difference exists in survival between any of the 13 UK tertiary congenital heart disease centres, but confidence intervals for small centres are wide, limiting our power to detect underperformance from analysis of a single year's data. Appropriately resourced, focused national audit is capable of accurate data collection on which nationwide, long term quality control can be based.

Cardiac Catheterization↗

Joint outpatient clinics for the adult with congenital heart disease at the district general hospital: an alternative model of care.

Congenital heart disease (CHD) affects approximately 250,000 adults in the UK. Most of these patients would benefit from specialized follow-up. However, there is at present a significant shortfall of specialized tertiary care expertise and facilities for this growing cardiovascular field in the UK and around the world. We aimed to report our experience with a joint adult CHD clinic run in a district general hospital with regular input from the local cardiology team and a visiting adult CHD specialist. In total, 148 patients aged 33.6+/-14.1 years were seen once or more in 12 clinics over the study period (September 1999 to January 2003). Diagnostic case mix consisted of 2.9% complex, 67.9% moderate and 29.2% minor cases of CHD. Twenty percent of patients visited the counterpart tertiary center for additional investigations (mostly MRI) and 8% for intervention (with no operative mortality). There was one death during the study period giving an overall mortality of 0.2%/year. Patients were referred to the clinic from tertiary centres, the local cardiology and paediatric clinics and with time from obstetric and community sources. Nonattendance rates were relatively low, comparing favourably with tertiary care. This model of joint care for the adult CHD patient at a general district hospital with regular onsite specialized input appears to be effective and highlights the need for additional resource allocation to provide optimal care for these patients. Our data may be useful in future planning for CHD services.

Adult↗

Pulmonary-to-systemic blood flow ratio effects of sevoflurane, isoflurane, halothane, and fentanyl/midazolam with 100% oxygen in children with congenital heart disease.

UNLABELLED: The cardiovascular effects of volatile anesthetics in children with congenital heart disease have been studied, but there are limited data on the effects of anesthetics on pulmonary-to-systemic blood flow ratio (Qp:Qs) in patients with intracardiac shunting. In this study, we compared the effects of halothane, isoflurane, sevoflurane, and fentanyl/midazolam on Qp:Qs and myocardial contractility in patients with atrial (ASD) or ventricular (VSD) septal defects. Forty patients younger than 14 yr old scheduled to undergo repair of ASD or VSD were randomized to receive halothane, sevoflurane, isoflurane, or fentanyl/midazolam. Cardiovascular and echocardiographic data were recorded at baseline, randomly ordered 1 and 1.5 mean alveolar anesthetic concentration (MAC) levels, or predicted equivalent fentanyl/midazolam plasma levels. Ejection fraction (using the modified Simpson's rule) was calculated. Systemic (Qs) and pulmonary (Qp) blood flow was echocardiographically assessed by the velocity-time integral method. Qp:Qs was not significantly affected by any of the four regimens at either anesthetic level. Left ventricular systolic function was mildly depressed by isoflurane and sevoflurane at 1.5 MAC and depressed by halothane at 1 and 1.5 MAC. Sevoflurane, halothane, isoflurane, or fentanyl/midazolam in 1 or 1.5 MAC concentrations or their equivalent do not change Qp:Qs in patients with isolated ASD or VSD. IMPLICATIONS: Sevoflurane, halothane, isoflurane, and fentanyl/midazolam do not change pulmonary-to-systemic blood flow ratio in children with atrial and ventricular septal defects when administered at standard anesthetic doses with 100% oxygen.

Adolescent↗

Risk of surgery for congenital heart disease in the adult: a multicentered European study.

BACKGROUND: Surgery for congenital heart disease (CHD) has changed considerably during the last three decades. The results of primary repair have steadily improved, to allow treating almost all patients within the pediatric age; nonetheless an increasing population of adult patients requires surgical treatment. The objective of this study is to present the early surgical results of patients who require surgery for CHD in the adult population within a multicentered European study population. METHODS: Data relative to the hospital course of 2,012 adult patients (age > or = 18 years) who required surgical treatment for CHD from January 1, 1997 through December 31, 2004 were reviewed. Nineteen cardiothoracic centers from 13 European countries contributed to the data collection. RESULTS: Mean age at surgery was 34.4 +/- 14.53 years. Most of the operations were corrective procedures (1,509 patients, 75%), followed by reoperations (464 patients, 23.1%) and palliative procedures (39 patients, 1.9%). Six hundred forty-nine patients (32.2%) required surgical closure of an isolated ostium secundum atrial septal defect. Overall hospital mortality was 2%. Preoperative cyanosis, arrhythmias, and NYHA class III-IV, proved significant risk factors for hospital mortality. Follow-up data were available in 1,342 of 1,972 patients (68%) who were discharged home. Late deaths occurred in 6 patients (0.5%). Overall survival probability was 97% at 60 months, which is higher for corrective procedures (98.2%) if compared with reoperations (94.1%) and palliations (86.1%). CONCLUSIONS: Surgical treatment of CHD in adult patients, in specialized cardiac units, proved quite safe, beneficial, and low-risk.

Adult↗

Current and potential impact of fetal diagnosis on prevalence and spectrum of serious congenital heart disease at term in the UK. British Paediatric Cardiac Association.

BACKGROUND: Assessment of the effect of fetal diagnosis on the prevalence of congenital heart disease at term requires national ascertainment because referral patterns are not rigorously structured. METHODS: Between 1993 and 1995, all 17 paediatric cardiac centres in the UK submitted to a database lists of all fetuses diagnosed, and all infants needing surgery or interventional catheterisation or dying in the first year of life because of structural heart disease; details included the postal area of residence. FINDINGS: There were 4799 affected pregnancies, 4165 babies born alive, 1124 fetal diagnoses, and 567 terminations of pregnancy because the fetus had structural heart disease. Thus, a fetal diagnosis was made in 23.4% of affected pregnancies (11.7% of all affected livebirths) with geographical variability in diagnostic rates. INTERPRETATION: Fetal cardiac screening has an effect on the prevalence and types of congenital heart disease seen at term because many affected pregnancies are terminated. If detection rates of affected fetuses rose nationally to those seen in the 15 postal areas where detection rates were significantly higher than the national average in 1993-95, we would expect about 218 fewer affected individuals to be born annually.

Abortion, Legal↗

Systemic endothelial dysfunction in adults with cyanotic congenital heart disease.

BACKGROUND: Secondary erythrocytosis results in increased shear stress in cyanotic congenital heart disease (CCHD), which may modify the balance between vasodilators and vasoconstrictors and affect systemic endothelial function. Because no data are available on systemic vasomotion, systemic endothelial function and nitric oxide (NO) availability were investigated in CCHD patients. METHODS AND RESULTS: Responses to arterial endothelium-dependent (acetylcholine [Ach]) and -independent (sodium nitroprusside [SNP]) vasodilation, NO synthase blockade (NG-monomethyl-L-arginine [L-NMMA]), endothelin-1 (ET-1), and ET-1 receptor blockade by BQ-123 in 11 CCHD patients (O2 saturation <90%; mean+/-SD, 79+/-1%; mean+/-SD age, 39+/-2 years) were compared with those in 10 age-matched healthy referents by using forearm venous occlusion plethysmography. Resting forearm blood flow (FBF) was lower in CCHD patients than in referents (2.4+/-0.2 versus 3.5+/-0.4 mL.min(-1).100 mL(-1) of forearm volume [FAV], P<0.05). Although the response to SNP was similar in both groups (CCHD, 2.0+/-0.3 to 8.3+/-1.0; referents, 3.6+/-0.7 to 11.9+/-1.2 mL.min(-1).100 mL(-1) of FAV; P>0.1), the response to Ach was markedly reduced in CCHD (maximal increase in FBF, 2.8+/-0.8 versus 37.5+/-4.4 mL.min(-1).100 mL(-1) of FAV; P<0.0001). l-NMMA was less effective in CCHD (decrease in FBF, 25+/-6% versus 40+/-4%; P<0.05). ET-1 caused less vasoconstriction in the CCHD group (-25+/-9% versus -51+/-7%, P<0.05), but the response to BQ-123 was similar in both groups (32+/-9% versus 27+/-9%). CONCLUSIONS: Systemic endothelial dysfunction is evident in CCHD patients as shown by strikingly reduced endothelial vasodilation to Ach. The response to exogenous ET-1 is reduced, possibly because of elevated endogenous ET-1 levels, but the effects of endogenous ET-1 on arterial tone are not enhanced, as indicated by the similar response to ET-1 blockade.

Acetylcholine↗

Congenital heart disease: the impact of delivery in a tertiary care center on SNAP scores (scores for neonatal acute physiology).

OBJECTIVE: It has been hypothesized that delivery in a tertiary care center might improve the clinical condition and outcome of infants born with congenital heart disease. The purpose of this study was to determine the effect of delivery in a tertiary care center on SNAP scores (scores for neonatal acute physiology) of infants admitted to the neonatal intensive care unit with major structural cardiac defects. STUDY DESIGN: This retrospective cohort study included 195 infants with major congenital heart disease admitted to the neonatal intensive care unit at the New England Medical Center between July 1, 1992, and June 30, 1998. SNAP scores were abstracted from the medical record. The values of 97 neonates with major cardiac defects born at the New England Medical Center were compared with those of 98 neonates transferred to our center after delivery in a community setting. A 2-tailed Student t test for independent samples was used to compare the mean SNAP scores between the 2 cohorts. RESULTS: The SNAP scores for infants with major cardiac defects who were born at the New England Medical Center ranged from 0 to 41, with a mean of 10.6 +/- 8.8. The values for infants with congenital heart disease who were transferred to our center after birth in community-based hospitals ranged from 0 to 34, with a mean of 11.1 +/- 7.0. There was no significant difference between the 2 populations (P =.646). A comparison of the mean SNAP scores of infants with prenatally diagnosed disease who were delivered at our center versus infants with postnatally diagnosed disease who were delivered in community hospitals was also statistically not significant (P =.824). CONCLUSION: Delivery in a tertiary care center does not improve SNAP scores of infants with major structural cardiac defects.

Alprostadil↗

The evolution of diagnostic trends in congenital heart disease: A population-based study.

OBJECTIVE: To analyse historical trends in diagnosis of congenital heart disease (CHD) in all diagnosed cardiac malformations born between 1945 and 1994 in a population-based study, the first of its type. METHODOLOGY: Retrospective analysis of age and mode of diagnosis was carried out in 953 patients with CHD, in the setting of a regional hospital providing diagnostic and follow up services for all of Malta. Main outcome measures were age at diagnosis, cumulative percentage diagnosis by age and mode of diagnosis. RESULTS: A significant negative correlation of age at diagnosis with time was found for both lesions not requiring intervention, and requiring intervention (P < 0.0001). Cumulative percentage diagnosis by age increased progressively with time. Echocardiography increased the birth prevalence of definitively diagnosed defects, particularly of lesions not requiring intervention. The annual number of cardiac catheters for these conditions has decreased with an increased number and proportion of interventional catheters. The current catheterization rate for CHD is 6.1/100 000 population. CONCLUSION: Echocardiography has led to an increase in the diagnosis in the birth prevalence of CHD, and provided early diagnosis, supplanting cardiac catheterization as a primary diagnostic tool.

Age Factors↗