Long-term neurodevelopmental outcome of children from euploid pregnancies with increased nuchal translucency in the first trimester screening.

OBJECTIVE: To assess the long-term neurodevelopmental outcome of children born from singleton euploid pregnancies with increased fetal nuchal translucency (NT) in the first trimester ultrasound screening and without structural anomalies in the second trimester ultrasound screening. STUDY DESIGN: This is a register-based retrospective cohort study carried out at a tertiary referral centre from 2002 to 2007. Children were followed up until 2012. All fetuses had increased NT (>95th percentile) at the first trimester ultrasound screening and normal findings in the second trimester ultrasound screening. Data about the neurodevelopmental outcome was retrieved from the hospital databases, The National Institute for Health and Welfare, and the Finnish Causes of Death Statistics Database. Information about received disability allowances was gathered from the Social Insurance Institute of Finland. RESULTS: The study population consists of 691 children. The mean follow-up time was 6.5 years. Neurodevelopmental disorders occurred in 29 children (4.2%). Twelve of these 29 children (1.7%) had severe neurodevelopmental impairment. CONCLUSIONS: The long-term neurodevelopmental outcome of children after increased fetal NT is reassuring. This information should be added to the parental counselling of such cases. © 2014 John Wiley & Sons, Ltd.

Impact of in vitro fertilization of bovine oocytes with sex-sorted frozen-thawed spermatozoa on developmental kinetics, quality and sex ratio of developing embryos.

We studied whether bovine embryos developing after in vitro fertilization (IVF) with sex-sorted spermatozoa differed in developmental kinetics, quality and sex ratio from embryos produced with unsorted spermatozoa. Abattoir-derived oocytes were fertilized with X-sorted, Y-sorted or unsorted spermatozoa from a single bull. To evaluate economical use of the sex-sorted spermatozoa, washed spermatozoa from a single straw (2 million spermatozoa) were used to fertilize each batch of collected oocytes without any further isolation steps. Concentration of the unsorted spermatozoa was adjusted accordingly. Fertilizations were assessed by staining sperm asters at 10 hpi and pronuclei at 20 hpi. Embryo development and morphological quality were monitored on days 2, 7, 8 and 9 of the development (IVF = day 0). All embryos were sexed using PCR. Following fertilization, penetration and subsequent cleavage rates were compromised in the X-sorted group compared with the Y-sorted and unsorted groups (penetration: 58.0% vs. 89.8% and 90.0%, cleavage: 65.3% vs. 81.5% and 75.0%). The use of the sex-sorted spermatozoa did not, however, reduce the proportion of transferable embryos (sex-sorted 29.6% vs. unsorted 27.7%) or their quality (quality 1: sex-sorted 36.0% vs. unsorted 19.9%). The Y-sorted spermatozoa produced more transferable embryos of better quality than the X-sorted spermatozoa (days 7-8: 31.9% vs. 26.4%, quality 1: 38.9% vs. 30.6%). On average, out of 10 transferable embryos, nine were of the predicted sex in the X- and Y-sorted spermatozoa groups. These results indicate that low numbers of X- and Y-sorted spermatozoa can be used successfully for female and male embryo production in vitro.

Neonaticides may be more preventable and heterogeneous than previously thought - neonaticides in Finland 1980-2000.

Neonaticide is a sad and infrequent crime with possibly a high level of underreporting. The aim of this study was to examine the circumstances of neonaticide, and whether there are subtypes of offenders, or suggestions for prevention. The study was retrospective and register-based using comprehensive nation-wide material of all cases of suspected neonaticide during 1980-2000 in Finland. Out of the 50 suspected cases, 32 women were included in the final analyses as neonaticide offenders. Most women (91%) had concealed their pregnancy, which was not the first for 66%. Most (66%) were not quite sure why they had offended, and the most frequent (63%) method of operation was neglect. Four women were diagnosed psychotic and formed a specific group. We concluded that there might be specific subgroups of offenders - even though our small population limited conclusions. Furthermore, prevention might be heightened. We call for international joint projects for enlarged material to enable grouping, as well as education and discussion among the public and the professionals to prevent neonaticide, unify its jurisprudence and improve the treatment of the offenders.

High amniotic fluid erythropoietin levels are associated with an increased frequency of fetal and neonatal morbidity in type 1 diabetic pregnancies.

AIMS/HYPOTHESIS: In this study we investigated whether chronic fetal hypoxia, as indicated by amniotic fluid erythropoietin levels, is associated with perinatal morbidity in type 1 diabetic pregnancies. METHODS: A total of 331 women with type 1 diabetes had at least one childbirth between 1995 and 2000. The amniotic fluid erythropoietin concentration was measured in 156 diabetic singleton pregnancies at a median time of 1 day before Caesarean section without labour contractions and in 19 healthy control subjects at Caesarean section. RESULTS: The median amniotic fluid erythropoietin level was 14.0 mU/ml (range 2.0-1975.0) in diabetic pregnancies and 6.3 mU/ml (range 1.7-13.7) in controls (p<0.0001). Of the 156 diabetic patients, 21 (13.5%) had amniotic fluid erythropoietin levels higher than 63.0 mU/ml. Amniotic fluid erythropoietin levels correlated negatively with umbilical artery pH (r=-0.49, p<0.0001) and pO2 (r=-0.62, p<0.0001) at birth and neonatal lowest blood glucose level (r=-0.47, p<0.0001). Positive correlations were found between amniotic fluid erythropoietin levels and umbilical artery pCO2 (r=0.49, p<0.0001) and last maternal HbA1c (r=0.43, p<0.0001). Furthermore, a U-shaped correlation was demonstrated between amniotic fluid erythropoietin levels and birthweight z score (z score below -0.6 SD units: r=-0.63, p=0.0007; z score above +1.0 SD units: r=0.32, p=0.0014). Neonatal hypoglycaemia, hypertrophic cardiomyopathy and admission to the neonatal intensive care unit occurred significantly more often in cases with high amniotic fluid erythropoietin levels (>63.0 mU/ml) than in those with normal levels. Multivariate logistic regression analysis revealed that amniotic fluid erythropoietin was the only variable independently related to low umbilical artery pH (<7.21; p<0.0001) and neonatal hypoglycaemia (p=0.002). Low umbilical artery pO2 (<15.0 mm Hg) was explained by amniotic fluid erythropoietin (p<0.0001) and birthweight z score (p=0.004). CONCLUSIONS/INTERPRETATION: Antenatal high amniotic fluid erythropoietin levels can identify type 1 diabetic pregnancies at increased risk of severe perinatal complications.

Relationship of maternal autoimmune response to clinical manifestations in children with congenital complete heart block.

OBJECTIVE: To study the autoimmune response in mothers of children with congenital heart block (CHB) diagnosed at different ages and with different clinical manifestations. PATIENTS AND METHODS: Clinical data and sera for the determination of immunological tests were available from 104 mothers of 113 children born between 1950 and 2000 and diagnosed with CHB before the age of 16 y. Prenatal diagnosis was performed in 74 (65%) children of 65 mothers, and 39 (35%) children had postnatal diagnosis of CHB. Maternal antibodies to 52 kd and 60 kd SS-A, and to 48 kd SS-B were determined by time-resolved fluoroimmunoassay (TR-FIA) and to antinuclear antibodies (ANA) by immunoflurescense (IF). RESULTS: Out of the 65 mothers of children with in utero diagnosed CHB, 88% had antibodies to 52 kd SS-A and 83% had ANA. Antibodies to 60 kd SS-A and 48 kd SS-B were less frequently present, in 48% and in 54% of the mothers, respectively. Seven (11%) of the mothers were negative by all immunoassays. Of the 13 mothers of children with in-infancy diagnosed CHB, one mother had high-titer ANA. After 1 y of age, CHB was diagnosed in 26 children; at 1 to 6 y in 16 and after 7 y in 10 children; 1/16 and 1/10 patients had positive antibodies. In all twin pregnancies (n = 4) and in all families with recurring cases of CHB (n = 5), maternal antibodies were positive in at least one assay. The titer of 48 kd anti-SS-B antibodies was significantly higher in children with cutaneous neonatal lupus (98.1 vs 41.0; p = 0.02). All mothers whose children died before the age of 4 y (n = 8) and 85% (11/13) of mothers whose children developed cardiomyopathy had elevated antibody titers in at least one assay. However, we could not find any prognostic value of maternal antibody levels or specificities on the clinical outcome of the children with CHB. CONCLUSIONS: Although rare, late detection or postnatal progression of CHB in antibody-mediated CHB should be taken into consideration. Maternal antibody levels or specificities have prognostic effect neither on the clinical outcome of the child with CHB nor on the risk of reappearance in the same family.

Right atrial isomerism in four siblings.

Heterotaxy syndromes, right or left atrial isomerism, result from disruption of left-right axis determination and their manifestations include complicated heart defects. Recent studies in model organisms have revealed complex genetic pathways and several genes involved in this process. In affected humans, however, molecular studies have identified mutations in a small number of individuals, while in most the cause remains unknown. Furthermore, although family data suggest, autosomal recessive inheritance, such genes have not yet been identified. We have studied six members of a family, four children affected with right atrial isomerism (RAI) and their healthy parents, for disturbances of left-right axis development. The children, one female and three males who all had complicated heart defects, succumbed and had an autopsy. Their nonconsanguineous parents were examined by cardiac and abdominal ultrasound or MRI. In all four children the heart defects included single ventricle with dysplastic atrioventricular (AV) valve, total anomalous pulmonary venous drainage (TAPVD), and malposition of great arteries (MGA) with pulmonary stenosis (PS). All had asplenia; two also had dextrocardia and abdominal situs inversus. The diagnosis of RAI was made postnatally in the first child and prenatally in others. Two siblings had no surgery and died as a newborn, one with obstructed supracardiac TAPVD and the other with regurgitating AV valve. Two children underwent heart surgery. One had repair of obstructive infracardiac TAPVD but died in infancy. The other underwent both hemi-Fontan operation and heart transplantation but died at the age of 2 years. This is the first report describing four children with RAI in the same family. The occurrence of RAI in male and female siblings without any indication of left-right axis abnormalities in their parents suggests autosomal recessive inheritance of human isomerism.

Controlled trial of dexamethasone in neonatal chronic lung disease: an 8-year follow-up of cardiopulmonary function and growth.

AIM: To evaluate the long-term effects of postnatal dexamethasone treatment in high-risk infants of very low birthweight. METHODS: The study included 16 children aged 7.8 to 9.2 y who had been born very prematurely at gestational ages of 24-29 wk and with birthweights of < 1500 g and who had participated in a randomized study of dexamethasone or placebo treatment in ventilator-dependent infants at 10 d of age. Flow-volume spirometry, impulse oscillometry, skin-prick tests and Doppler echocardiography were carried out at school age, and respiratory morbidity and overall neurological outcome evaluated. Controls were 18 non-atopic children born at term, tested for lung function. RESULTS: No significant differences were found in respiratory morbidity at school age between the dexamethasone (n = 8) and placebo (n = 8) groups. Six of the 16 children had moderate to severe neurosensory impairments, but all were able to walk without support and attended primary school. In prematurely born children, standardized height was significantly less than that in controls, but between the two study groups, no significant differences existed in somatic growth. Atopy was uncommon: skin-prick tests were positive in only one child in the placebo group. In the dexamethasone group, forced vital capacity adjusted to height was significantly higher than that in the placebo group, but impairment of basic lung function and bronchial obstruction was evident in both study groups. No hypertrophic cardiomyopathy was apparent, and non-invasive measurements of pulmonary arterial pressure did not reveal any significant difference between the study groups. CONCLUSION: In very low-birthweight children, high respiratory morbidity and neurological impairment continued until school age. Neonatal dexamethasone treatment at school age was not associated with any detectable adverse effects on somatic growth, or pulmonary or cardiac function.

Absent aortic and dysplastic pulmonary valves associated with ventricular septal defect in fetal hydrops.

We describe worsening cardiac failure in a hydropic male fetus with a large ventricular septal defect associated with severe pulmonary and mild aortic regurgitation detected prenatally by echocardiography. The hemodynamic transition after emergency cesarean section at 31 weeks of gestation resulted in amelioration of the pulmonary incompetence but exacerbation of aortic insufficiency causing coronary steal. Death followed withdrawal of support 4 hours after delivery. Autopsy revealed absent aortic valve cusps, dysplastic pulmonary valve, and a large ventricular septal defect.

The circumboreal tundra-taiga interface: late Pleistocene and Holocene changes.

Creating a global perspective on past treeline changes is problematic due to the varying methods and definitions used. A general lack of a detailed description of the modern treeline position and vegetation complicates any comparative analysis of the magnitude of the most important changes. However, one seemingly common factor in most regions was an extremely rapid dispersal of trees when climate warmed drastically from full glacial conditions. Most Arctic treelines reached their northernmost positions in the early Holocene and receded to present positions starting at about 5.8 ka. The early occupation of the northernmost sites in ice-free and early deglaciated areas was possible because of the close proximity of invading trees in nearby glacial refugia, particularly in Fennoscandia and northern Russia. In Canada, the Northwest Territories and Quebec-Labrador were out of phase with this general trend due to their late deglaciation. However, even here colonization was rapid, indicating that the tree species were present adjacent to the glaciers. Following this trend and based on the present evidence, we propose a scenario of a continuous but modest occupation of eastern Beringia by spruce during the late-Pleistocene instead of an exceptionally rapid spread of conifers from the glacial refugium south of the Laurentide ice sheet (2000 to 3000 km in about 200 years), which typically has been assumed. Macrofossil evidence of scattered occurrences of "exotic species" (for instance Siberian larch in central Sweden) far from their natural range limits in the early Holocene highlight the disparity between pollen and macrofossil analyses. It questions the validity of assigned pollen percentages to indicate the presence of a species within a region as these species were not observed in the pollen record. Thus, it is likely that trees were present at any given site well before the rise in pollen abundance. There is still a large potential to improve our knowledge about the environmental history of the circumboreal treeline areas. In particular, future research should concentrate not only on patterns of species displacement, but on finding the factors, apart from climate, which cause treeline shifts.

Cardiovascular manifestations in 75 patients with Williams syndrome.

OBJECTIVE: The prevalence and types of various cardiovascular diseases in different age groups as well as the outcomes of cardiac surgery and other interventions were assessed in a population of 75 Williams syndrome (WS) patients aged 4 months to 76 years (median 22.7 years). STUDY DESIGN: The diagnosis of WS was in each case confirmed by the clinical phenotype and by a FISH test showing elastin hemizygosity. Clinical and operative data were collected from all hospitals where the patients had been treated. RESULTS: Cardiovascular symptoms were evident in 35 of 75 (47%) WS children at birth. During follow up, 44 of 75 (53%) WS patients were found to have cardiovascular defects. Among them, the definitive diagnosis was made before 1 year of age in 23 (52%) infants, between 1 year and 15 years of age in 14 (32%) children, and older than 15 years of age in 7 (16%) adults. Multiple obstructive cardiovascular diseases were found in six infants. Supravalvular aortic stenosis (SVAS) was diagnosed in 32/44 (73%), pulmonary arterial stenosis (PAS) in 18/44 (41%), aortic or mitral valve defect in 5/44 (11 %) of cases, and tetralogy of Fallot in one (2%) case. Altogether, 17/44 (39 %) underwent surgery or intervention. Surgery was most frequently performed in the infant group (6% v 21% v 0%, p=0.004). After 1 year of age, seven patients underwent SVAS relief and two cases PAS relief. Postoperatively there was no mortality (median follow up time 6.9 years). Arterial hypertension was found in 55% of adults. In three adults, arterial vasculopathy was not diagnosed until necropsy. CONCLUSIONS: Our data indicate the following in WS. Cardiac symptoms are common in neonates. Heart disease diagnosed in infancy frequently requires operation. After 1 year of age, PAS tends to improve and SVAS to progress. Life long cardiac follow up is necessary because of the risks of developing vasculopathy or arterial hypertension.

The quantitative risk of violent crime and criminal offending: a case-control study among the offspring of recidivistic Finnish homicide offenders.

OBJECTIVE: To study if later risk of violent offending and criminality among high-risk children can be estimated quantitatively on the basis of parental crimes. METHOD: The criminal and prison records of the offspring (N=11) of homicide recidivists (N=36) were compared with data from controls (N=220) who were matched for sex, domicile of birth and date of birth and death. RESULTS: The risk (odds ratio) was increased up to 24-fold for violent crimes (P=0.01), and up to 17-fold for criminality (P=0.0008) among the offspring of homicide recidivists. CONCLUSION: The quantitative risk of a child for later violent offending and criminality can be estimated on the basis of parental homicide recidivism. This kind of method could be used to choose target groups for early preventive interventions, and to study the effectiveness of prevention.

Violent women, blame attribution, crime, and personality.

BACKGROUND: The attribution of blame for criminal act has an impact, both on the way the offender must come to terms with the offence and on the risk of recidivism. Blame attribution has been found to be related to the type of crime and personality. AIMS: This study examined the relationship of blame attribution and feelings of guilt in violent female offenders, according to factors related to crime, background and personality. METHODS: The Gudjonsson Blame Attribution Inventory (BAI) and the Defense Style Questionnaire (DSQ) were administered to 58 violent female offenders. The Hare Psychopathy Checklist-Revised (PCL-R), Structured Clinical Interview II for DSM-IV (SCID II) and clinical interviews were used for assessment. RESULTS: Lower scores of guilt feelings were found in women than has been reported for men. The feelings of guilt were higher in homicide than in other violent crimes, but lower in the cases where women had committed prior violent crimes. No differences were found between psychiatric patients and prisoners. The duration of hospitalisation and incarceration did not result in differences in blame attribution and guilt, but when the victims survived, guilt feelings seemed to increase with the passing of the time. External attribution was related to motives of crime, but traumatic experiences did not have a crucial impact upon blame attribution. The attribution of blame and the amount of guilt feelings were found to be related to personality. Women scoring high on psychopathy reported less guilt than women with low scores. The corresponding result was not obtained in the group of women with antisocial personality disorder, who, however, showed an unexpectedly low use of external attribution. CONCLUSION: The results suggest that it might be difficult for women to cope with the violent offence and that the characteristics of psychopathy seems to have a crucial impact on the guilt feelings and consequently on coping with the crime in female offenders.

Congenital complete heart block in the fetus: hemodynamic features, antenatal treatment, and outcome in six cases.

Congenital heart block (CHB) can result in intrauterine cardiac failure leading to fetal or neonatal loss. To establish perinatal hemodynamic factors which might predict adverse outcome, six fetuses with CHB diagnosed between 20 and 30 gestational weeks were examined by echocardiography at 2-week intervals. Neonatal morbidity and outcome in infancy are detailed. The fetuses showed a significant decrease in ventricular rate (VR) with advancing gestation (60 +/- 7 vs 51 +/- 4 beats/min, p = 0.03). Cardiac decompensation defined as hydrops or pericardial effusion was associated with VR of lower than 55 beats/min in two fetuses. Three mothers had a therapeutic trial with a sympathomimetic and digoxin. Salbutamol increased VR 10% in one of three fetuses treated. Digoxin decreased pericardial effusion in one hydropic fetus with autoimmune myocarditis. In this fetus, poor left ventricular fractional shortening (LVFS) was accompanied with high umbilical artery resistance index (RI). High amniotic fluid erythropoietin indicated severe hypoxia preceding death. Pacemaker was indicated in all the newborns. At the age of 2 weeks all the surviving infants had tricuspid regurgitation and a shunt through foramen ovale due to asynchronized atrioventricular contraction. During the 12-month follow-up two of five surviving infants had no symptoms. One had symptomatic neonatal lupus. Two infants had patent ductus arteriosus, one with dilated cardiomyopathy. In conclusion, poor fetal outcome was associated with low VR, low LVFS, and high RI. Despite early pacing, morbidity was high in infancy due to cardiomyopathy and associated heart defects. Regular echocardiographic monitoring during pregnancy and after delivery is required in order to optimize care and timing of any interventions.

Fetal nuchal translucency and normal chromosomes: a long-term follow-up study.

OBJECTIVE: To assess the impact of increased nuchal translucency observed during early pregnancy on the subsequent health of children with normal chromosomes, with special attention to cardiac anatomy and function. METHODS: Clinical examination and detailed cardiac evaluation were carried out in 50 chromosomally normal children at the age of 2.4-7.1 years who had had a nuchal translucency measurement of > or = 3 mm at 13-15 weeks' gestation. The data of two babies who died of heart defects were also included. RESULTS: Major cardiac defects were identified in four (8%) of the children. The growth of all children was within normal limits. One child had Noonan syndrome, one had a cleidocranial dysplasia and a third had a developmental delay together with an unrecognized syndrome. Webs in the neck region were noticed in two children, with no associated pathology. CONCLUSIONS: In chromosomally normal fetuses with increased nuchal translucency, fetal echocardiography is necessary to identify major cardiac defects. In general, the parents can be reassured that, in the great majority, postnatal development is normal.

Long-term outcome of mothers of children with isolated heart block in Finland.

OBJECTIVE: To study the long-term outcome of mothers of children with isolated heart block in a defined population. METHODS: We reviewed the Finnish hospital registries for patients born between 1950 and 1999 who had been diagnosed as having isolated heart block before the age of 15 years. We identified 101 children with isolated congenital heart block (CHB) and 55 with isolated heart block detected after the newborn period. Eighty-three (91%) of the 91 mothers of children with CHB and 48 (87%) of the 55 mothers of children with heart block detected after the newborn period were studied according to a protocol defining clinical characteristics (mean 9.9 years, range 0-49 years, and mean 22.9 years, range 4-47 years after the index delivery, respectively). Maternal survival was compared with survival in an age-matched population of normal Finnish women. RESULTS: Before the index delivery, 29 (37%) of the 78 surviving mothers of children with CHB had a self-reported clinical diagnosis of a chronic autoimmune disease, and 55 (71%) had had symptoms, signs, or abnormal laboratory findings suggesting an underlying subclinical disease. Of the 23 mothers who were completely asymptomatic before the index delivery, 10 (13% of the surviving mothers) remained so after a mean followup of 9.6 years (range 0-21 years). In mothers of children with CHB, clinical characteristics different from those of healthy mothers were photosensitivity, fatigue, dry eyes, and dry mouth. Forty-eight (58%) of these 83 mothers developed an autoimmune disease during followup. The most common diagnosis was primary Sjögren's syndrome (22 definite, 11 probable), followed by systemic lupus erythematosus (SLE). The standardized mortality ratio of mothers of children with CHB was 5.1, and 3 of the 5 deaths were associated with SLE. Mothers of children with heart block detected after the newborn period had similar symptoms and signs of autoimmune diseases as the healthy controls, and their standardized mortality ratio was 1.9. CONCLUSION: Primary Sjögren's syndrome, either definite or subclinical, is the predominant autoimmune disorder in mothers of children with CHB. Mothers of children with isolated heart block detected after the newborn period do not, as a group, have clinical features suggestive of autoimmune diseases.

Psychopathy and the posterior hippocampus.

Neurobiology of psychopathy is of interest, not only because neural underpinnings of psychopathy remain obscure, but also because psychopaths may provide a model to study violent behavior, neurology of morals and impaired decision-making. Medial temporal lobe pathology has been suggested to be a part of the neural systems dysfunction which manifests as violent and psychopathic behavior. Yet, so far no sound evidence of neuroanatomical correlates for psychopathic behavior has been found. In this study regional hippocampal volumes were measured using magnetic resonance imaging in 18 habitually violent offenders with antisocial personality disorder and type 2 alcoholism (derived from forensic psychiatric evaluation). The regional volumes along the anteroposterior axis of the hippocampus were correlated with the subjects' degree of psychopathy as evaluated by the Psychopathy Checklist-Revised. Strong negative correlations, up to -0.79, were observed, among the study subjects, between the psychopathy scores and the posterior half of the hippocampi bilaterally. These data are in accordance with experimental studies proposing that lesions of the dorsal hippocampus impair acquisition of conditioned fear, and with theories on psychopathology according to which one of the central features in the birth of psychopathy is a deficit in acquisition of conditioned fear.

Long-term outcome of children with complete heart block diagnosed after the newborn period.

The aims of this study were to assess morbidity, mortality, and long-term cardiac outcome of children with congenital complete heart block (CHB) diagnosed between the ages of 3 months and 15 years. The study population consists of 61 children with CHB diagnosed in five tertiary referral centers in Finland. There were two study groups: CHB diagnosed 3 months to 2 years of age (group 1; n = 29) and CHB diagnosed between the age of 2 and 15 years (group 2; n = 32). Neonatal morbidity was significantly higher in group 1 than in group 2 (p = 0.047). In the whole study population, permanent pacemaker implantation with the main indications of low ventricular rate and syncope (52%) was performed in 80% of cases, with a median age of 11.2 years. Structural heart defect not causally associated with CHB was evident in 9 of 61 patients (15%). Of 8 patients with atrial septal defect (ASD) secundum, 6 (75%) were operated on and 7 (88%) were paced. One patient had small ventricular septal defect. The incidence of dilated cardiomyopathy was 7%. The mortality with cardiomyopathy was very high (75%). Of the 61 children, 3 (5%) died due to cardiomyopathy at the ages of 2, 26, and 31 years. In the long-term follow-up of 17 years (median, range 2.9-46 years) 17% of patients who survived had cardiac problems: arrhythmias in 5, mitral valve insufficiency in 3, ASD secundum in 1, and cardiomyopathy in 1. The long-term outcome did not depend on the age at which CHB was diagnosed. In conclusion, CHB diagnosed after the newborn period carries relatively low mortality and morbidity. Pacemaker application was indicated in most children. The possibility of an associated heart defect or dilated cardiomyopathy indicates regular echocardiographic monitoring in all children and young adults with CHB.

Fatal outcome of two siblings with idiopathic arterial calcification of infancy diagnosed in utero.

Idiopathic arterial calcification of infancy (IACI) is a rare condition characterized by extensive arterial calcification and stenoses of large and medium-sized arteries. Its complications include severe cardiac failure diagnosed in utero as hydrops fetalis or postnatally as respiratory failure combined with cardiomegaly. Two newborn male siblings with IACI are described. In utero, echocardiography revealed poor ventricular function and hyperechogenic foci in arterial walls. Both had fatal outcome during the newborn period. At autopsy, medial calcifications in the walls of great arteries, in coronary arteries, in glomeruli, and in subendocardium were detected. In addition, an inflammatory process in the shoulder joint was determined to be large periarticular tissue calcifications. Because of an autosomal recessive inheritance pattern of IACI, fetal echocardiography is recommended in future pregnancies of all affected families.