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[Quantification of right ventricular function in congenital heart disease: correlation of 3D echocardiography and MRI as complementary methods].

PURPOSE: In congenital heart disease, the exact determination of the right ventricular function is of high importance for therapeutic and especially surgical planning. The aim of this study was to correlate the parameters of the right ventricular function in MRI and 3D echocardiography to determine the agreement of both modalities. MATERIALS AND METHODS: In 18 patients suffering from congenital heart disease, 3D echocardiography was performed using a Philips Sonos 7500 system. In MRI short axis slices with a 4-mm distance were acquired using an SSFP sequence on a Siemens Sonata or Symphony System. Volumetry for both modalities was performed on an external workstation (Tomtec) using the EchoView software. RESULTS: Enddiastolic and endsystolic volumes showed a highly significant correlation with coefficients of 0.996 and 0.990, respectively. In echocardiography there was a systematic slight underestimation of enddiastolic volumes and overestimation of endsystolic volumes. The Wilcoxon test did not show significant differences between the volumes and ejection fractions assessed by both modalities. CONCLUSION: There is an excellent correlation in the quantification of right ventricular volumes in MRI and 3D echocardiography, which allows a comparison of acquired volumes in clinical follow-up.

Adolescent↗

Factor analysis of gated cardiac blood-pool data: application to patients with congenital heart disease.

Ventricular emptying was evaluated in patients with congenital heart disease (CHD) with left-to-right (L-R) shunt by factor analysis of gated equilibrium radionuclide angiography. In 36 (95%) of 38 ventricular septal defect patients and 20 (95%) of 21 atrial septal defect patients with small L-R shunt (pulmonary to systemic blood flow, Qp/Qs less than or equal to 2.5), as well as all patent ductus arteriosus patients, two significant cardiac factors corresponding to the ventricles (ventricular factor) and the atria plus large vessels (atrial factor) were extracted. However, in all of nine ventricular septal defect patients with large L-R shunt (Qp/Qs greater than 2.5), two different ventricular factors were determined which corresponded to the right and left ventricles (RV and LV). The RV factor showed a delay of ejection phase compared with the LV factor, and the delay was correlated with the value of Qp/Qs (r = 0.82, P less than 0.01). In eight (80%) of 10 ASD patients with large L-R shunt (Qp/Qs greater than 2.5), RV was described by the two different ventricular factors located in the septal and free-wall regions. The LV was extracted in the same factor as that located in the septal region of RV. This study demonstrates the capability of factor analysis in the pathophysiological investigation of CDH with L-R shunt.

Adolescent↗

The effects of congenital heart disease and cardiac surgery on liver blood flow in children.

Children with congenital heart disease may have some degree of hepatic impairment, with further impairment developing shortly after surgical correction of the cardiac defect. The redistribution of organ blood flow that occurs during cardiopulmonary bypass implicates ischaemia as one of the principal causes of injury. The aim of this study was to measure liver blood flow in children with congenital heart disease and to determine both the effects of cardiopulmonary bypass and the consequences of corrective surgery. Indocyanine green clearance and auricular densitometry, were used in 31 children. In 83% we demonstrated a reduced liver blood flow, with a mean percentage disappearance rate (PDR) of 12.9% (SEM +/- 1.2). This finding was unrelated to the patient's age, the type of congenital heart defect or the presence or absence of cyanosis. During cardiopulmonary bypass, hepatic perfusion was further reduced in 77% of children, by an average of 67%, out of proportion with the iatrogenic reduction in total body flow. Six hours after surgery, liver blood flow had increased significantly above preoperative levels (p < 0.001; t-test) to approximately normal values with a mean PDR of 20.4% (SEM +/- 1.5).

Cardiopulmonary Bypass↗

Noncardiac malformations in congenital heart disease: a retrospective analysis of 305 pediatric autopsies.

Congenital heart disease (CHD) is one of the important groups of birth defects and contributes significantly to infant mortality. Extracardiac anomalies occur in 15-45% of cases with CHD. In this retrospective study, autopsies of cases born alive and diagnosed as CHD between 1977-2002 at Hacettepe University Ihsan Doğramaci Children's Hospital, Pediatric Pathology Department, were investigated. In this period, a total of 3320 autopsies were performed and the incidence of CHD was 9.1%. The most commonly encountered CHD was ventricular septal defect (VSD) (15.3%). In 45.9% of cases, one or more extracardiac malformations were present. The most commonly encountered extracardiac malformation was craniofacial malformations. Less commonly seen were malformations of genitourinary, musculoskeletal, respiratory, gastrointestinal, central nervous systems and spleen anomalies. Ventricular septal defect, atrial septal defect (ASD), aortic coarctation, single ventricle, pulmonary stenosis, hypoplastic right heart syndrome, double outlet right ventricle, ASD+VSD, aortic arcus anomalies, and right and left atrial isomerism cases were often (>50%) accompanied by extracardiac malformations. No extracardiac malformations were detected accompanying pulmonary atresia with intact interventricular septum, Ebstein malformation, and mitral stenosis (MS). Spleen malformation was significantly high in cases with single ventricle (p<0.002). The anomalies of the gastrointestinal and genitourinary systems were found to be frequently associated with conotruncal heart defects (p<0.001). In the group with transposition of the great arteries, noncardiac malformations were present in only three cases (10%), differing from the rest of the material (p<0.001). In conclusion, when a heart malformation is detected in a patient, a detailed investigation should be done on extracardiac malformations or vice versa. Proper identification and treatment of CHD early in the prenatal period will save the family from the economic and emotional burden caused by having such a child with CHD.

Abnormalities, Multiple↗

Parameters of iron deficiency in children with cyanotic congenital heart disease.

A group of 67 children with cyanotic congenital heart disease (CCHD) were studied, and 35 were given iron treatment according to a regimen that gives iron to patients with a hematocrit (Hct) below 60%. The patients were categorized as iron-deficient and iron-sufficient according to their transferrin saturation and ferritin values. The pretreatment hemoglobin (Hb) and Hct values of the groups were similar. The mean Hct was nearly three times as much as the mean Hb in the iron-sufficient group and more than three times as much as the Hb in the iron-deficient group. Excessive erythrocytosis in the iron-deficient group was impressive. Mean corpuscular volume (MCV) values were below 72.7 fl in all of the iron-deficient patients. After treatment the Hb, Hct, transferrin saturation, and ferritin increased significantly in both groups, with the increments greater in the iron-deficient group. Increments in the erythrocyte (RBC) count were significant in the iron-sufficient group but insignificant in the iron-deficient one. Increments of MCV in the iron-deficient group were significant but insignificant in the iron-sufficient group. Our study demonstrated that prediction of Hb, RBC count, and MCV, measurements of which are easy and inexpensive and require little blood, can suffice for the diagnosis of iron deficiency in patients with CCHD without altering systemic perfusion.

Anemia, Hypochromic↗

Is noninvasive determination of pulmonary artery pressure feasible using deceleration phase Doppler flow velocity characteristics in mechanically ventilated children with congenital heart disease?

Noninvasive determination of pulmonary hemodynamics is important for the management of congenital heart disease complicated by pulmonary hypertension. Flow deceleration is less influenced by right ventricular function and would allow more accurate estimation of pulmonary hemodynamics than acceleration. Respiratory influences on pulmonary blood flow are exaggerated by mechanical ventilation. Doppler-derived pulmonary artery (PA) blood flow velocity characteristics were therefore compared with pulmonary hemodynamic parameters in 42 mechanically ventilated children, aged 0.2 to 14.8 years (mean +/- SD 6.7 +/- 4.9). Mean PA pressure ranged from 11 to 47 mm Hg (21 +/- 9 mm Hg). Pulmonary hypertension was present in 14 patients. Significant differences were found between patients with and without pulmonary hypertension in maximal velocity (1.03 +/- 0.22 vs 0.88 +/- 0.18 m/s), acceleration time (119 +/- 39 vs 136 +/- 29 ms), maximal acceleration (17.6 +/- 6.4 vs 13.1 +/- 4.0 m/s2), mean acceleration (9.3 +/- 2.6 vs 6.7 +/- 2.0 m/s2), and mean deceleration (4.5 +/- 1.0 vs 3.8 +/- 0.8 m/s2). In contrast to our hypothesis of the deceleration phase-derived parameters, only maximal deceleration correlated with PA pressure. Acceleration parameters showed closer relations with PA pressures, but correlations were generally low and did not permit accurate prediction of PA pressure (SEE 5 to 11 mm Hg), PA resistance (SEE 1.14 U. m2) or PA driving force (SEE 7 mm Hg). An analysis that took respiratory phase into account did not improve correlations. Measurement of mean acceleration, maximal deceleration, and rate-corrected preejection period permitted for accurate discrimination between the presence or absence of pulmonary hypertension, with positive and negative predictive values being 92% and 90%. In mechanically ventilated children with congenital heart disease, accurate noninvasive PA pressure assessment is not possible. Accurate predictions for the presence of pulmonary hypertension can be made by measurement of both acceleration and deceleration parameters.

Adolescent↗

Venous Doppler ultrasound in 146 fetuses with congenital heart disease.

OBJECTIVE: To assess the influence of isolated congenital heart disease (CHD) on fetal venous Doppler blood flow velocity waveforms. METHODS: Doppler flow velocimetry was performed in the inferior vena cava and ductus venosus in 146 consecutive fetuses with antenatally diagnosed CHD. Gestational age ranged between 19 and 39 weeks. Fetuses with isolated CHD without non-immune hydrops fetalis (NIHF) (Group A, n = 89) were separated from seven fetuses showing isolated CHD with NIHF (Group B) and 50 cases complicated by chromosomal or other extracardiac malformations, intrauterine growth restriction or non-cardiogenic NIHF (Group C). The control group comprised 109 healthy fetuses of uncomplicated pregnancies. Individual peak velocity index for veins (PVIV) measurements were converted into their Z-scores (delta values) for statistical analysis. RESULTS: There was no statistical difference between fetuses with isolated CHD (Group A) and controls, for the delta PVIV of neither the ductus venosus nor the inferior vena cava. Statistical analysis revealed significant differences between non-isolated CHD fetuses (Group C) and controls for both vessels. However, in a separate analysis of isolated right heart malformations compared with the remaining isolated heart malformations (Groups A and B), a significant difference was observed for the ductus venosus, but not the inferior vena cava. There was an overall survival of 62%. In Group A, 58% of fetuses survived despite increased PVIV and 22% of fetuses with normal venous Doppler had an adverse outcome. All fetuses with cardiogenic NIHF (Group B) died. CONCLUSION: Doppler studies of the ductus venosus and inferior vena cava in fetuses with isolated CHD do not present sufficient alterations to be a reliable marker for screening purposes for CHD in mid-second- and third-trimester fetuses. Furthermore, venous Doppler did not predict fetal outcome in cases of isolated CHD. Abnormal venous Doppler results were mainly attributable to myocardial dysfunction and also to severe right heart obstruction even in the absence of congestive heart failure. Therefore, venous Doppler studies are clinically helpful in indirectly monitoring cardiac function in fetuses with cardiac malformations.

Blood Flow Velocity↗

Gingival colonization with selective HACEK microbes in children with congenital heart disease.

It was previously shown that children with congenital heart disease (CHD) harbored Hemophilus, Actinobacillus, Cardiobacter, Eikenella, and Kingella (HACEK) microbes to a greater extent and had more severe gingival inflammation than a normal group of children. The purpose of this study was to determine if HACEK microbes are more prevalent in children with CHD than in normal children when there is no difference in gingival inflammation. Two groups of 12 children were matched with respect to gingival inflammation. Each child had a gingival index recorded as described by Massler. The experimental group consisted of 12 children with CHD 2.5-10 years old (average 5.5) and the control group consisted of 12 healthy children 2-13 years old (average 5.6). Subgingival samples were obtained and cultured for HACEK microbes. Fischer's exact test was performed with the significance level defined at P<0.05. The average gingival indices for the experimental and control groups were 6.5 and 6.4, respectively (N.S.). Nine of 12 children with CHD had Eikenella corrodens (E.c.) compared to 3/12 control patients ( P<0.05). Three of 12 CHD patients but no control patient had Actinobacillus actinomycetemcomitans (A.a.) (N.S.). There were no significant differences in E.c. or A.a. presence between cyanotic and acyanotic CHD patients. This study found that the greater extent of specific HACEK microbes harbored by children with CHD is not associated with cyanosis or the degree of gingival inflammation. Further study is needed to delineate fully the medical significance of this observation.

Aggregatibacter actinomycetemcomitans↗

Impact of cardiac rehabilitation on the exercise function of children with serious congenital heart disease.

OBJECTIVES: The exercise capacity of children with congenital heart disease (CHD) is often depressed. This depression is thought to be attributable to (1) residual hemodynamic defects and (2) deconditioning secondary to physical inactivity. We hypothesized that this latter component would be ameliorated by a formal cardiac rehabilitation program designed specifically for children. The objective of this study was to characterize the effect of a cardiac rehabilitation program on the exercise performance of children with CHD and to define the physiologic mechanisms that might account for any improvements that are observed. METHODS: Nineteen patients with CHD who were referred for exercise testing and found to have a peak oxygen consumption (VO2) and/or peak work rate <80% of predicted were enrolled in the study. Sixteen patients (11 Fontan patients, 5 with other CHD) completed the program and had postrehabilitation exercise tests, results of which were compared with the prerehabilitation studies. RESULTS: Improvements were found in 15 of 16 patients. Peak VO2 rose from 26.4 +/- 9.1 to 30.7 +/- 9.2 mL/kg per min; peak work rate from 93 +/- 32 to 106 +/- 34 W, and the ventilatory anaerobic threshold from 14.2 +/- 4.8 to 17.4 +/- 4.5 mL/kg per min. The peak heart rate and peak respiratory exchange ratio did not change, suggesting that the improvements were not attributable merely to an increased effort. In contrast, the peak oxygen pulse rose significantly, from 7.6 +/- 2.8 to 9.7 +/- 4.1 mL/beat, an improvement that can be attributed only to an increase in stroke volume and/or oxygen extraction at peak exercise. No patient experienced rehabilitation-related complications. CONCLUSION: Cardiac rehabilitation can improve the exercise performance of children with CHD. This improvement is mediated by an increase in stroke volume and/or oxygen extraction during exercise. Routine use of formal cardiac rehabilitation may greatly reduce the morbidity of complex CHD.

Adolescent↗

Myocardial beta-adrenoceptor density and the distribution of beta 1- and beta 2-adrenoceptor subpopulations in children with congenital heart disease.

Twenty-six infants and children with congenital heart disease (CHD) undergoing cardiac surgery were investigated for alterations in myocardial beta-adrenoceptor density. The patients were divided into three groups according to type and severity of CHD: group I consisted of 6 patients with acyanotic shunt lesions of moderate severity; group II comprised 13 children with severe acyanotic shunt and valve lesions and group III included 7 children with cyanotic CHD. The myocardial beta-adrenoceptor density was determined using (-)3-[125I]Iodocyanopindolol [( 125I]ICYP) and was reduced by approximately 50% in severe acyanotic CHD (33.6 fmol/mg protein) and cyanotic CHD (35.3 fmol/mg protein) in comparison with the group with less severe acyanotic shunt defects (64.4 fmol/mg protein). The affinity dissociation constant (Kd.ICYP) did not differ statistically between the groups. The proportion of beta 1- and beta 2-subpopulations was evaluated by ICI 118,551-[125I]ICYP competition studies. In group II (61.5%) and group III (69.1%) significant lower portions of beta 1-adrenoceptors were found compared with group I (78.2%). This shift of subpopulations was due to a decreased beta 1-receptor density while beta 2-receptor density was unchanged in all groups. While the plasma noradrenaline levels of group I were similar to those of a control group of 13 healthy children, respective values of group II and III were significantly elevated. A significant negative correlation was found between plasma noradrenaline levels and myocardial beta-adrenoceptor density. It is concluded that exposure of these receptors to increased circulating catecholamines, due to an enhanced sympathetic tone, leads to a reduction of their density.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

[Hematic and plasmatic viscosity in cyanotic congenital heart diseases (author's transl)].

57 patients with cyanotic congenital heart diseases were studied. 39 patients presented t. of Fallot, 18 patients had patent ductus arterious with pulmonary hypertension and right to left shunt. The average whole blood viscosity in patients with t. of Fallot was 6.86+/-0.32 cps. This parameter was correlated with hematocrit, fibrinogen, euglobulin lysis time, pulmonary output, systemic output, and O2 saturation. The value of "r" resulted as statistically significant only for the correlation between viscosity and hematocrit and between viscosity and fibrinogen (t=13.22, p less than 0.0005; t=3.35, p less than 0.001 respectively). The average value of plasma viscosity in patients with t. of Fallot was 1.61+/-0.024 cps. The correlations of this parameter with fibrinogen, euglobulin lysis time, pulmonary output and systemic output did not result as statistically significant. The average fibrinogen value was 256.10+/-20.63 mg%, whereas that of euglobulin lysis time was 12,67+/-1.50 hours. In patients with patent ductus arteriosus the average blood viscosity was 6.65+/-0.56 cps. Such parameter was correlated with hematocrit, fibrinogen, euglobulin lysis time, pulmonary output, systemic output and O2 saturation. The value of "r" resulted as statistically significant only between blood viscosity and hematocrit and between blood viscosity and O2 saturation (t=9.30, p less than 0.0005; t=5.800, p less than 0.001 respectively). The average value of plasma viscosity was 1.61+/-0.04 cps. This parameter was correlated with fibrinogen, euglobulin lysis time, pulmonary output and systemic output. The correlation index "r" never resulted as statistically significant. The average fibrinogen value was 299.20+/-20.30 mg. The average euglobulin lysis time was 16.02+/-2.73 hours.

Adolescent↗

Further delineation of the Opitz G/BBB syndrome: report of an infant with complex congenital heart disease and bladder exstrophy, and review of the literature.

The combination of complex congenital heart disease (double outlet right ventricle with pulmonary atresia, malalignment ventriculoseptal defect, right-sided aortic arch with left ductus arteriosus) and bladder exstrophy occurred in an infant with Opitz syndrome. Neither of these defects has previously been reported in association with Opitz syndrome. These malformations, which are midline defects, further characterize this syndrome as an impairment in midline development. The spectrum of congenital heart disease and genitourinary anomalies seen in Opitz syndrome is reviewed.

Abnormalities, Multiple↗

Use of the Internet by parents of children with congenital heart disease.

OBJECTIVE: We sought to document the prevalence of Internet access and usage patterns among families with children diagnosed with congenital heart disease. MATERIAL AND METHODS: A questionnaire was administered to the parents of 509 unselected children with congenital heart disease attending the outpatient cardiac clinic. RESULTS: 389 questionnaires were completed. The most common source for acquiring medical information was via the paediatric cardiologist (83%), the family doctor (53%), the general paediatrician (40%) and the Internet (15%). Among the 238 families with Internet access (62%), the most common locations for Internet access were home (74%) and work (33%), and 84 used the Internet to obtain information related to their child's cardiac diagnosis, mostly at the time of diagnosis. Two-thirds of these families considered finding information related to the cardiac diagnosis as easy or very easy, and found this information helpful or very helpful. CONCLUSION: A quarter of the families uses the Internet to find information about their child's congenital heart disease. Internet use in this patient population is expected to increase and our vigilance in providing accurate references and in identifying inaccurate information is of paramount importance.

Adolescent↗

Central nervous system outcomes in children with complex congenital heart disease.

PURPOSE OF REVIEW: To provide a brief overview of our current understanding of the types of neurodevelopmental sequelae in congenital heart disease survivors and to review the most recent studies from the past year, which have focused on 4 interrelated issues: (1) outcome studies, (2) the mechanism and etiology of central nervous system injury in children with CHD, (3) perioperative monitoring for brain injury, and (4) strategies for neuroprotection during cardiac surgery. RECENT FINDINGS: As the number of survivors of surgery for complex congenital heart disease continues to rise, it is recognized that there is an increased incidence of adverse neurological outcomes in the survivors. In particular, a pattern similar to that seen in premature infants is emerging, including learning disabilities, behavioral abnormalities, inattention and hyperactivity. Imaging studies have revealed a high prevalence of structural brain abnormalities and periventricular leukomalacia, fetal and postnatal cerebral blood flow is abnormal, postnatal oxygen delivery is decreased, and intraoperative support techniques and postoperative low cardiac output are associated with cerebral hypoperfusion. SUMMARY: The causes of these late developmental abnormalities are most likely sequential, cumulative and multifactorial.

Central Nervous System Diseases↗

Spectrum of congenital heart disease in Malta. An excess of lesions causing right ventricular outflow tract obstruction in a population-base study.

AIMS: To quantify birth prevalence and spectrum of congenital heart disease in the Island population of Malta, and compare these rates with previous studies. METHODS: All patients diagnosed as having congenital heart disease by echocardiography, cardiac catheterization, surgery or post-mortem by 1 year of age between 1990-1994 were included. There were 231 cases of live born congenital heart disease with a birth prevalence of 8.8/1000 live births. The commonest lesions were ventricular septal defect, pulmonary stenosis and tetralogy of Fallot. The rates of individual lesions were compared with two recent epidemiological studies with similar methodologies. RESULTS: Although the overall birth prevalence of congenital heart disease was similar in three studies, significantly higher rates of pulmonary stenosis, tetralogy of Fallot and double outlet right ventricle were found in Malta, all of which predispose to right ventricular outflow tract obstruction. In contrast, there were lower rates of lesions causing left ventricular outflow tract obstruction. A higher rate of ventricular septal defect was also found. The Maltese gene pool may contain an inherent predisposition towards lesions causing right ventricular outflow tract obstruction.

Double Outlet Right Ventricle↗

[Expression and pathological implication of transforming growth factor-beta1 mRNA and endothelin-1 mRNA in intraacinar pulmonary arterioles of congenital heart disease accompanied with pulmonary hypertension].

OBJECTIVE: To study the expression and pathological implication of transforming growth factor-1 (TGF-1) and endothelin-1 (ET-1) in intraacinar pulmonary arterioles of children with congenital heart disease and pulmonary hypertension (HP). METHODS: Forty-one children with left-to-right shunt congenital heart disease were studied including 25 cases of HP (group A), 16 cases without HP (group B) and 10 children without congenital heart disease as the contols (group C). Expression of TGF-beta1 mRNA and ET-1 mRNA in intraacinar pulmonary arteriolar (IAPA) was studied using in-situ hybridization and image pattern analysis of their absorption values (A value). Changes of the intraacinar arterioles and lung tissue were studied by elastic fiber staining and electronic microscopy respectively. RESULTS: (1) There was a significant difference in the amount of intraacinar pulmonary arterioles (partial-muscular and muscular) counted in either group A or B in comparing with that of group C (F values 149.96 and 142.01 respectively, P < 0.01); (2) Electronic microscopy demonstrated endothelial proliferation of the small arteries, thickening of arteriolar wall, increased density of collagen fibers at adventitia and increased thickness of the capillary basal membrane; (3) The A value of TGF-beta1 mRNA expressed in the pulmonary arterioles of groups A and B by in-situ hybridization were 0.1988 +/- 0.0498 and 0.1098 +/- 0.0428 respectively, however, the expression was weak in group C (A value: 0.0578 +/- 0.0096). There were all significant between each two groups (F = 45.95, P < 0.01). The expression of ET-1 mRNA was markedly increased as well in the endothelial cells of pulmonary arterioles in both groups A and B, with A values of 0.1692 +/- 0.0205 and 0.1004 +/- 0.0140 respectively, whereas the expression was weak in group C (A value of 0.0746 +/- 0.0119). There were all significant between each two groups (F = 139.996, P < 0.01). CONCLUSIONS: The number of intraacinar pulmonary partial-muscular and muscular arterioles in patients with left-to-right shunt congenital heart defect is drastically increased, along with marked restructuring of the pulmonary vasculatures. In addition, there seems a correlation present between the overexpression of TGF-beta1 mRNA and ET-1 mRNA in intraacinar pulmonary arterioles and the occurrence of pulmonary hypertension in patients with congenital heart disease.

Child↗

Psychosocial functioning of the adult with congenital heart disease: a 20-33 years follow-up.

AIMS: Since knowledge about the psychosocial function of adult patients with congenital heart disease is limited, we compared biographical characteristics, and emotional and social functioning of these patients with that of the reference groups. METHODS AND RESULTS: Patients with congenital heart disease (N=362, aged 20-46 years), belonging to five diagnostic groups, were subjected to extensive medical and psychological examination, 20-33 years after their first open heart surgery. All the patients were seen by the same psychologist, who examined their psychosocial functioning using a structured interview and questionnaires. The majority (78%)was living independently and showed favourable outcome regarding the marital status. Among married/cohabitant patients, 25-39-year-olds showed normal offspring rates. None of the 20-24-year-old patients had any children. The offspring rate dropped after the age of 40. The proportion of adult patients with a history of special education was high (27%). Accordingly, patients showed lower educational and occupational levels compared to reference groups. As regard to the emotional and social functioning (leisure-time activities), the sample showed favourable results. CONCLUSIONS: Overall, this sample of patients with congenital heart disease seemed capable of leading normal lives and seemed motivated to make good use of their abilities.

Adult↗