Compared with transmitral velocities of flow, myocardial wall velocities obtained by tissue Doppler imaging are less influenced by left atrial pressure. The data supporting this assumption, however, are limited in patients with congenital cardiac disease. The aim of this study was to compare the effects of left ventricular preload on transmitral inflow and velocities assessed by tissue Doppler imaging. Tissue Doppler imaging, and conventional Doppler echocardiography with simultaneous invasive hemodynamic studies, were performed in 33 patients with a simple ventricular septal defect or patency of the arterial duct. Transmitral velocities (E, A) and mitral annular velocities (Ea, Aa) were measured, permitting calculation of the ratio of E to Ea. The ratio of pulmonary to systemic flows, and mean left atrial pressure, were also measured. In 10 of 33 patients, echocardiographic and hemodynamic studies were performed 4 to 5 months after surgery.

The E and A values in the patients were greater than those in the controls (p < 0.01). In contrast, neither Ea nor Aa differed between the two group. The ratio of E to Ea in the patients increased significantly compared with that in the controls (8.9 ± 2.1 vs 7.3 ± 1.3, p < 0.01). The E value was directly related to mean left atrial pressure and the ratio of pulmonary to systemic flows. The velocities measured by Tissue Doppler imaging, however, had no significant relationship to either of these measurements. The ratio of E to Ea correlated well with mean left atrial pressure (r = 0.75, p < 0.01). In 10 post-operative patients, the values for E and A decreased from 119 ± 14 to 89 ± 10 cm/sec (p < 0.01) and from 91 ± 22 to 61 ± 9 cm/sec (p < 0.01), respectively. No significant changes were noted in the values of Ea and Aa. The ratio of E to Ea, and mean left atrial pressure, decreased from 10.3 ± 1.9 to 8.2 ± 1.5 (p < 0.01) and from 11.0 ± 1.8 to 7.4 ± 1.0 mmHg (p < 0.01), respectively. The percentage change in left atrial pressure correlated with the percent change in the ratio of E to Ea (r = 0.64, p < 0.05).

The present study showed that the velocities obtained with tissue Doppler imaging are less dependent on mean left atrial pressure in children with elevated left ventricular preload caused by a left-to-right shunt.

Background: Acute renal failure is an occasional complication after cardiopulmonary bypass in infants. Whereas it is well known that postoperative hemodynamics inflict acute renal failure, the influence of extra-corporeal circulation on the kidney is less clear. Moreover, changes in blood viscosity occur during and after surgery, which may influence renal dysfunction. For this reason, we investigated the impact of blood viscosity on renal function during cardiopulmonary bypass. Methods: In 34 patients weighting less than 10 kg, we performed repeated analysis of urine, blood, and plasma viscosity. Results: Polyuria and proteinuria that appeared during cardiopulmonary bypass indicated an elevated transglomerular filtration gradient, which recovered within 24 hours. The appearance of N-acetyl-β-D-glucosaminidase in the urine, and elevated excretion of sodium, were additionally indicative of mild tubular damage. Elevation of blood viscosity during hypothermic perfusion showed a statistical correlation with proteinuria and N-acetyl-β-D-glucosaminidaseuria. With hypothermia, the relation of blood viscosity to plasma viscosity became stronger, while the relation to the hematocrit decreased compared to normothermia. Conclusions: During cardiopulmonary bypass perfusion, the kidney can be stressed by proteinuria and mild tubular damage. Our data provide evidence that the kidneys can be protected by improved blood viscosity during cardioplegia, but this needs confirmation in a prospective interventional study.

Objective: To assess the incidence of depression, and the ability to interact socially, in adult patients with chronic cyanosis and congenital cardiac malformations. Design: Prospective study of consecutive patients. Setting: Single institution, tertiary referral centre. Patients: Between 1993 and 2000, we assessed 76 patients with congenital cardiac malformations and persistent cyanosis, having a median age of 36.5 years, with a range from 19 to 64 years, at the time of referral. Female patients accounted for just under half (48.6%) of the sample. Just under two-fifths of the cohort (39.5%) had functionally univentricular cardiac anatomy, while 14.8% had tetralogy of Fallot with pulmonary atresia and aorto-pulmonary collateral arteries, and 17% had the Eisenmenger syndrome. During the period of follow-up, 17 (22.4%) of the patients died. Assessment: We used clinical interviews and non-invasive assessment, employing Zung's questionnaire which provides a scale for the self-rating of depression. On this scale, a score above 50 points is indicative of depression. Results: Of the survivors, 32 (54%) completed the self-rating questionnaires. Of these, 20 responders (63%) considered that they lead full lives, including sexual activities, while 26 (81%) had never harboured suicidal thoughts. Depression was diagnosed in 11 responders (34%), with a mean score of 66.9, standard deviation of 8.7, and a range from 53 to 89. The remaining 21 patients (66%) were without signs of depression, scoring a mean of 41.5, with standard deviation of 5.5, and a range from 35 to 46. Depression was associated with older age (40.5 years versus 33.5 years, p =0.01), worse functional state in the classification of the New York Heart Association (2.95 versus 2.48, p =0.03), and unemployment (p < 0.0001), but independent from the severity of cyanosis, the level of the haematocrit, the saturation of oxygen, or previous surgical treatment. Conclusions: To our knowledge, this is the first evidence suggesting a relatively high incidence of depression in adults with congenital cardiac malformations and persistent cyanosis. Larger, multi-centric studies will be needed to confirm or refute these findings.

Background: At present, a considerable number of patients survive who underwent an atrial switch operation for correction of complete transposition. Our study aimed to assess their long-term exercise performance and the serial evolution of cardiac function. Methods: We studied 22 patients 5 to 17 years after an atrial switch operation, and followed them serially for 3.5 ± 2 years after the first evaluation. Cardiorespiratory exercise function was assessed by analysis of gas exhange and by determination of the ventilatory anaerobic threshold. Echocardiography was performed on all evaluations. Results: All patients were in Class I of the classification of the New York Heart Association at all assessments. Ventilatory anaerobic threshold, however, was significantly lower than normal. It averaged 77.9% ± 13.7 of the normal mean value at the initial evaluation, and remained stable when re-evaluated later (76.2 ± 13.7%). At the initial study, the increase in oxygen uptake during graded exercise was below the 95% confidence limit in 6 of the patients, and was below this level in 10 patients at re-assessment. The subnormal values for oxygen uptake during submaximal exercise were associated with moderate to severe haemodynamic dysfunction. At echocardiography, 15 of 17 patients studied twice had mild to moderate right ventricular dilation and tricuspidregurgiation, which remained virtually the same at reasssesment. A stable sinus rhythm was initially present in 17 patients, and persisted in 15 patients during follow-up. Conclusion: At medium term follow-up, cardiorespiratory exercise performance remains stable in patients after atrial switch repair. Serial exercise testing appears useful, because in individual patients in the present study, a decreasing exercise tolerance correlated with development of haemodynamic sequels.

Aims: To assess the cognitive, and behavioural and emotional functioning of children aged 3 months to 7 years shortly before elective cardiac surgery or elective interventional catheterisation. Methods: We used the Bayley Scales of Infant Development, and the McCarthy Scales of Children's Abilities, to measure cognitive functioning. The Child Behavior Checklist was used to assess behavioural and emotional problems. Results: We found no significant differences in mean cognitive scores for children scheduled for cardiac surgery or interventional catheterisation when compared with reference groups. This was also the case for children awaiting cardiac surgery as opposed to those awaiting interventional catheterisation, and for those below as compared to those above the age of 2.5 years. Overall, our results regarding behavioural and emotional functioning were comparable to those of normative reference groups. The only difference found was that the children scheduled for cardiac surgery and aged from 2 to 3 years had significantly higher scores on the Child Behavior Checklist than did peers from normative groups. Conclusion: Cognitive, and behavioural and emotional functioning, both for young children awaiting elective cardiac surgery and interventional catheterisation, can be considered as quite favourable.

Introduction: The frequent occurrence of prenatal hypertrophy of the muscular ventricular septum has been widely reported in fetuses of diabetic mothers. Objectives: This experimental study was carried out to test the hypothesis that the weight of the heart, the ratio of the cardiac weight to that of the body, the thickness of the muscular ventricular septum, and the myocytic profile within the ventricular septum are all increased in fetuses of diabetic rats in comparison to fetuses of normal rats. Methods: Diabetes was induced in 5 pregnant Wistar rats, bearing 30 fetuses, on the eighth day after conception, by intraperitoneal injection of 50mg/kg of streptozotocin. Five normal pregnant Wistar rats, bearing 20 fetuses, made up the control group. Morphometric data were obtained by a computer-assisted method applied to the measurements of the thickness of the ventricular septum, and myocytic nuclear area. Statistical analysis utilized Student's t-test and Kruskal-Wallis test. Results: The mean thickness of the septum was 675.56 μm (± 159) in the control fetuses, and 904.39 μm (± 262) in the fetuses carried by diabetic mothers (p < 0.001). The cardiac weight was 0.016 g (± 0.004) in the control group, and 0.023 g (± 0.005) in the group of diabetic fetuses (p < 0.001). The ratio of cardiac to body weight was 0.294% (± 0.079) in the control group, and 0.514% (± 0.073) in the diabetic group (p < 0.001). The myocytic nuclear area was 14.70 μm2 in the control group, and 21.43 μm2 in the diabetic group (p < 0.001). Conclusions: The presence of cellular and morphologic cardiac hypertrophy in fetuses of diabetic rats was demonstrated by the significant difference between the two groups for each analyzed feature.

Background: The clinical symptoms of heart failure in infants with left-to-right shunts are thought to be explained by well-known hemodynamic disturbances such as pulmonary hypertension and overcirculation, but previous studies have not, thus far, found the expected correlations with hemodynamic and clinical parameters. Based on the neurohormonal model of heart failure, we hypothesised that the clinical symptoms of infants with left-to-right shunts are also related to neurohormonal disorders. Methods: We compared various neurohormonal and hemodynamic parameters measured invasively in 70 infants with left-to-right shunts to the respiratory rate and gain in weight over a corresponding period of time. Heart rate correlated significantly with respiratory rate (r = 0.62***, p < 0.001) and gain in weight (r =−0.31*, p = 0.015), but more conventional measures of severity, such as the ratio of pulmonary to systemic flows, failed to show comparable correlations with clinical symptoms. Respiratory rate was related to levels of norepinephrine (r = 0.47***, p < 0.001) and plasma renin activity (r = 0.65***, p < 0.001). The important impact of autonomic imbalance on respiratory rate was underlined by an analysis of variability of heart rate in 26 infants that showed significantly reduced values for the domains of time and frequency. We were not able to find a conclusive multiple regression model with which to explain the symptom "failure to thrive". Conclusions: A increased heart rate, reduced variability in heart rate, and elevated levels of norepinephrine and renin are significant predictors of clinical symptoms such as tachypnea in infants with congenital cardiac malformations. The neurohormonal hypothesis, in which heart failure is interpreted not only as a hemodynamic derangement but also as a neurohormonal disorder, may be valid for infants with congenital cardiac malformations.

Background: The hemodynamic status after a Fontan type procedure for definitive palliation of functionally univentricular hearts is dominated by a high central venous pressure, which seems to be one of several factors responsible for venous congestion appearing as a frequent complication in the early and late postoperative course. The purpose of our study was to find other hemodynamic parameters correlating with the presence of venous congestion and effusions in these patients. Methods: We compared the hemodynamic data of 18 patients who had an uneventful long-term course after a Fontan type procedure with the respective data of 10 patients who developed symptoms of venous congestion in the immediate postoperative period. Based on a theoretical model, we developed an algorithm to calculate mean hydrostatic capillary pressure from mean arterial pressure, systemic vascular resistance index and central venous pressure. Results: Pulmonary vascular resistance index (2.1 ± 1.0 mmHg L-1 min m2), mean left atrial pressure (9.7 ± 4.0 mmHg) and cardiac index (3.6 ± 0.6 1/min/m2) are mainly normal in patients with venous congestion in the immediate postoperative period, but mean hydrostatic capillary pressure is significantly higher compared to patients without venous congestion (24.3 ± 3.1 vs 18.3 ± 4.0 mmHg). Lower mean hydrostatic capillary pressures in these patients are due to a highly significant increase of systemic vascular resistance index (18.6 ± 4.2 versus 33.6 ± 6.6 mmHg L-1 min m2) and a concomitant decrease of cardiac index to 2.4 ± 0.3 1/min/m2. Conclusions: The increase of mean hydrostatic capillary pressure, caused by high central venous pressures but also by relatively low systemic vascular resistance indexes, seems to be the hemodynamic key parameter responsible for venous congestion and effusions in patients after a Fontan type procedure in the immediate postoperative period.

Background: This is a review of the experience over 26 year in a single institution with surgical repair of aortopulmonary window. Methods: Between July 1973 and March 1999, 38 patients underwent surgery for aortopulmonary window at a median age of 5 weeks, and with a median weight of 3.9 kg. Median follow-up was 6.6 years, with a range from 0.8 to 26 years. Additional defects were present in 25 (65%) patients, including interruption of the aortic arch in 7, tetralogy of Fallot in 7, ventricular septal defect in 5, functionally univentricular anatomy in 3, aortic coarctation in 2, and anomalous origin of a coronary artery in 1. We approached via an aortotomy in 45%, an incision through the defect in 31%, and using a pulmonary arteriotomy in 24% of patients. Closure was achieved using a single patch in 30 patients (79%). Results: There were 3 (7.9%) in-hospital deaths. Actuarial patient survival was 88% at 10 years. Three patients required reinterventions for stenoses of the great arteries. Freedom from any reintervention was 70% at 10 years. By multivariate analysis, the approach through a pulmonary arteriotomy was shown to result in a higher need for reintervention (p = 0.01). Conclusions: Repair of aortopulmonary window can be done with excellent results. A pulmonary arteriotomy should be avoided.

Objective: The concept of self – management of oral anticoagulation has been shown to entail better quality of treatment than conventional management when assessed in selected adults. We have extended the concept of self – management to include children with congenital cardiac disease, hypothesizing self-management of oral anticoagulation is also possible in this subset of patients. Our aim was to assess the quality of self-management. Methods: We trained 14 children aged from 2.2 to 15.6 years, with a mean age of 9.7 years, and their parents, in domiciliary analysis of the International Normalized Ratio and necessary adjustment of dosage of coumarin. The curriculum for training lasted for 27 weeks, and the patients and their parents were followed for a period of up to 31 months by weekly measurement of the values obtained for the International Normalized Ratio. Results: The patients were observed over a mean of 547 days, with a range from 214 to 953 days. The patients were within the therapeutic targetted range of the International Normalized Ratio for a median of 65.5% of the time, with a range from 17.6 % to 90.4 %. None of the patients experienced thromboembolic or bleeding complications requiring doctoral intervention. All the patients and their parents expressed full satisfaction with the treatment. Conclusion: Selfmanagement of oral anticoagulation provides a good quality of treatment, which is feasible and safe in selected children with congenital cardiac disease.

We compared the levels of lipoprotein (a) in 48 Caucasian patients with pulmonary arterial hypertension, comprising 32 females and 16 males, aged 28.0 ± 12.0 years, with a range from 4 through 52 years, with 48 normal Caucasian subjects matched for age and sex. Pulmonary hypertension was secondary in 41 patients with Eisenmenger's syndrome, these comprising 27 females and 14 males aged 27.0 ± 12.0 years, with a range from 4 through 51 years, and primary in the other 7 patients, 5 females and 2 males, whose age was 30.0 ± 14.0 years, with a range from 9 through 52 years. Lipoprotein (a) was measured using an immunoprecipitation and turbidimetric assay after a 12 hour fast. Levels of the protein, expressed as the median (% 25; % 75), were higher in those with Eisenmenger's syndrome than in normal controls (p = 0.003). In addition, there was a greater prevalence of levels of lipoprotein greater than 30.0 mg/dl in those with secondary pulmonary arterial hypertension patients than in our normal population (p = 0.03). We have found no differences, however, in the levels of lipoprotein(a) in those who had primary pulmonary arterial hypertension when compared with their matched controls, albeit that the number of patients studied was small. We conclude that increased levels of lipoprotein (a) may be secondary to pulmonary arterial hypertension as a marker of tissue damage or may be genetically determined. In either way, the increase in lipoprotein (a) could be an additional factor predisposing to the vascular alterations known to occur in this disease.

Aims: Coronary arterial abnormalities, congenital and acquired, may co-exist with structural congenital malformations of the heart. This study analyses information obtained from coronary angiography in grown-up patients with congenital cardiac disease undergoing cardiac catheterisation. Methods and Results: We studied 448 patients, of whom 181 (40%) had selective coronary angiography. This was undertaken as a routine measure in 165 (37%), and because of clinical indications in 16 (4%). Aortic root angiography was carried out in 107 (24%). Coronary arterial abnormalities were demonstrated in one-third of those undergoing selective angiography, and in 69% of those with a clinical indication for angiography, compared with 30% who had a routine procedure. In those patients undergoing aortography alone, abnormalities were recognized in 17%, while the angiographic images were inconclusive in 24%. In the remaining 59%, no abnormality was apparent, but only the proximal coronary arteries could be seen clearly. In total, congenital abnormalities of the coronary arteries were found in 11% of the patients, acquired lesions were demonstrated in 5%, and surgically acquired lesions in 2%. Conclusion: Routine coronary angiography during catheterization of grown-up patients with congenital heart disease revealed a high incidence of abnormalities of the coronary arteries. Selective injection into the arteries had a higher sensitivity than aortography. Consideration should be given to performing routine coronary arteriography during invasive investigation of patients with congenital cardiac disease, as it provides useful and often unexpected information, which may be valuable for the interpretation of symptoms and the planning of subsequent surgery.

The development of pulmonary arteriovenous malformations is a well-known complication after Fontan operations, and may result in significant morbidity due to increasing arterial desaturation. We compared the use of bubble contrast echocardiography and pulmonary angiography in detecting such malformations. We also examined which anatomical and haemodynamic variables were associated with their development. Our study includes 20 patients who had undergone modified Fontan procedures, 10 with atriopulmonary and 10 with total cavopulmonary connections, in Gothenburg between 1980 and 1991. All patients underwent cardiac catheterisation and pulmonary angiography. Bubble contrast echocardiography was performed at the same time, with injection of agitated polygelin colloid solution (Haemaccel, Hoechst) into the right and left pulmonary arteries, respectively. Transoesophageal echocardiography was used to detect the appearance of bubble contrast in the pulmonary venous atrium. The aim was also to evaluate the role of hepatic venous blood. Of the 20 patients, 9 (45%) had a positive contrast echocardiography study, compared with only 2 (10%) detected by pulmonary angiography. Patients with positive contrast echocardiography had a significantly lower arterial oxygen saturation than those with negative studies, both at rest (88% vs 95%, p < 0.01) and during exercise testing (78% vs 89%, p = 0.01). Bubble contrast echocardiography is much more sensitive in detecting pulmonary arteriovenous malformations than pulmonary angiography. By injecting echo contrast into the right and left pulmonary arteries, the method can be made highly selective. Pulmonary arteriovenous malformations develop much more frequently in patients with the Fontan circulation than previously reported.

Dilation of the ascending aorta is rare in childhood. When seen, it is usually associated with some form of connective tissue disease or predisposing cardiac malformations, especially an aortic valve with two leaflets. We describe four children in whom significant dilation of the ascending aorta was encountered as an incidental finding. No patient had any sign of an associated connective tissue disease, nor did we detect any predisposing cardiac anomalies. One patient had undergone surgical ligation of the arterial duct in infancy, whilst another had undergone repair of aortic coarctation, also in infancy. A third child has had repair of an atrioventricular septal defect with exclusively atrial shunting, whereas the fourth patient had a structurally normal heart. The aortic valve had three leaflets, and was functionally normal in all. The dilation of the ascending aorta was progressive in all patients, and finally surgical treatment was recommended, relying on the guidelines established for the management of patients affected with the Marfan syndrome.

Objectives: We undertook this study to assess the immediate and long-term outcome of balloon angioplasty performed for recurrent or residual coarctation of the aorta, and to assess the changes in the vessel wall caused by this procedure. Methods: Clinical, echocardiographic, angiographic and hemodynamic data from 71 patients who underwent balloon angioplasty for recoarctation between January 1987 and January 1998 were analysed retrospectively. Results: Angioplasty was performed after a median of 82.6 months (range 1.4 mo – 20.9 y, mean 88.5 mo) following surgery for coarctation. Mean systolic pressure gradients were reduced from 27±15 mmHg to 11 ± 11 m m Hg after angioplasty (p< 0.0001). The mean diameter at the site of recoarctation increased from 5.5±2.5 to 7.5±2.7 mm(p< 0.0001). Outpouchings of contrast agents, indicating the disruption of the inner layers of the vessel wall, were defined as extravasations. They were observed in one-quarter of the angiograms performed immediately after the intervention. Immediate success of angioplasty was achieved in 71%, and persisted in 69% of patients during long-term follow up. The main determinant for immediate success was the age at the time of the procedure (p<0.05), while the main determinant for long-term success was the increase achieved in diameter. Extravasations did not progress to aneurysms, neither acutely nor during echocardiographic follow-up studies. For further follow-up, more sensitive imaging techniques will be necessary to delineate the morphology of the site of extravasation observed immediately after angioplasty.

We sought to analyse the long-term follow-up after primary repair of tetralogy of Fallot in infancy in the first year of life, paying particular attention to growth of the pulmonary arteries and the need for reintervention. We performed a combined retro- and prospective echocardiographic study, including measurements of the pulmonary valve and right and left pulmonary arteries, indexed to the square root of body surface area, in 62 patients prior to primary repair, 18 to 24 months after this event, and at the most recent follow-up, with a mean of 80.4 ± 24 months. Of these, 38 patients, with an age at operation of 5.0 ± 3.4 months, had presented preoperatively with hypoxic spells or increasing cyanosis. The remaining 24 patients had been asymptomatic, with adequate flow of blood to the lungs. Their age at elective operation was 7.4 ± 3.0 months. A transannular patch was needed in 37 patients (63%). There were 3 early postoperative deaths (4.8%).

Cross-sectional echocardiography revealed a significantly smaller diameter for the pulmonary valve in patients who had been symptomatic preoperatively compared to the asymptomatic patients (1.09 versus 1.3 cm/BSA0.5; p = 0.019). The diameters of the right and left pulmonary arteries did not differ significantly between the groups. Examination of echocardiographic data obtained 18 to 24 months postoperatively in 43 patients revealed a significant increase in the diameter of the pulmonary arteries; 0.83 ± 0.17 cm/BSA0.5 versus 1.1 ± 0.26 cm/BSA0.5 for the diameter of the right pulmonary artery, 0.85 ± 0.2 cm/BSA0.5 versus 1.0 ± 0.25 cm/BSA0.5 for the left pulmonary artery. On comparison between individuals, 18.6% and 25.6% of the patients, respectively, did not show any change in the diameters of their right and left pulmonary arteries, whereas the increase in diameter reached or exceeded the measurements in normal controls in 55.8% and 46.5% of the patients, respectively. On recent follow-up, with a range from 56 to 147 months, no further increase in the indexed diameters of the pulmonaries could be documented in 21 of 33 patients. Of these, 9.1% and 18.2%, respectively, presented with small right and left pulmonary arteries compared with measurements obtained in normal controls. Moderate pulmonary incompetence was found on colour flow mapping in one-third. Of 56 longterm survivors, 8 (14%) had required reinterventions, which were surgical in 6 and achieved by transcatheter techniques in the other 2 patients. Thus, primary correction of tetralogy of Fallot in infancy, with restoration of normal pressures and flows, resulted in sustained increase in the diameters of the right and left pulmonary arteries. It allowed for early normal development of the proximal pulmonary arterial system in most patients regardless of their age and symptomatic status at operation. Patients with persistent subnormal diameter of the pulmonary arteries did not present with significantly elevated right ventricular pressure. Early one-stage repair of tetralogy of Fallot in infancy was associated with a low rate of reinterventions.

We studied 10 boys, and 15 girls, all below the age of 16, who had been referred to the National Hospital in Norway for evaluation for transplantation of either the heart, or the heart and lungs. These represent the complete cohort of patients being considered for transplantation between 1990 and 1997. Of the 25 children and their families, 24 sets underwent a comprehensive psychosocial assessment, including interviews with both parents and their children. The parents completed the Child Behavior Checklist and the General Health Questionnaire. We had accepted 15 children for transplantation and placed them on the waiting list. The others were rejected for medical reasons, and 3 died whilst waiting for an organ. One was reconsidered for conventional surgery and removed from the list. Transplantation was performed in 11 children, whilst one of the patients we had rejected underwent transplantation abroad, and was included in the study. This left 12 patients in the final sample, with a mean age of 8 years, and with a range from 11 months to 13.9 years. We reassessed their psychosocial and physical functioning two years after transplantation. Of those undergoing transplantation of the heart and lungs, two were severely affected by progressive obliterative bronchiolitis. The others were in good general physical condition. At the assessment prior to transplantation, three already fulfilled the criterions for diagnosis of an overanxious disorder. Two others had symptoms of anxiety and depression, but without fulfilling the accepted criterions. At follow- up, two patients retained this psychiatric diagnosis. Increased levels of stress were uncovered in the parents prior to surgery, but these had normalised at follow-up. The study shows that, in general, physical and psychological conditions improve in children undergoing transplantation, but they and their parents live in a stressful environment, and are in need of psychosocial support both before and after transplantation.

Objective: Measurements of the variability in heart rate are increasingly used as markers of cardiac autonomic activity. We sought to establish the development this variability in healthy young infants while sleeping. Patients: We carried out polygraphic studies with electrocardiographic recording in 587 healthy infants aged from 5 to 26 weeks. Methods: We determined several variables over a period of 400 minutes sleeping: mean RR interval, 5 time-domain (SDNN, SDNNi, SDANNi, RMSSD, and pNN50) and 5 frequency-domain indexes (spectral power over 3 regions of interest, total power and low-to-high frequency ratio). Frequency-domain indexes were also assessed separately for the periods of quiet sleep and those of rapid eye movement sleep. Results: Our data showed a significant correlation between the indexes of heart rate variability and the mean RR interval, the breathing rate, and the corrected age of the infants. We also demonstrated the importance of the maturation of the sleeping patterns. Conclusion: These data in a large cohort of healthy infants confirm a progressive maturation of the autonomic nervous system during sleep, and may be used to examine the influence of physiological and pathophysiological factors on autonomic control during polygraphic studies.