Do not forget the parents-Parents' concerns during transition to adult care for adolescents with congenital heart disease.

BACKGROUND: Growing up with congenital heart disease (CHD) often means transfer to adult care and lifelong medical follow-up. An optimal transition process usually involves a multipart collaboration between the patient, their parents and other family members, and the healthcare providers. Taking an active role while knowing when it is time to step aside can be difficult for all the concerned parties, even the healthcare professionals. The aim of the present study therefore, was to explore parents' expectations and needs during their adolescent's transition to adult care. METHOD: Semi-structured interviews were conducted with 18 parents of 16 adolescents (aged 13-18 years) with CHD in 4 pediatric cardiology settings in Sweden. The interviews were analysed with qualitative content analysis. RESULTS: The analysis resulted in 2 main themes: (a) Feeling secure-the importance of being prepared and informed. This theme focused on the need to be prepared and informed about transition and future transfer to adult care. (b) Recognizing when to hand over at the right time. This theme addressed the process of handing over the responsibility from the parent to the adolescents and contained handing over from pediatric care to adult care. CONCLUSION: Being prepared and informed about the upcoming transition process was essential. The parents underlined the importance of being involved in the transition planning for gradually handing over responsibility to the adolescent. They also considered establishing contact with the adult healthcare team before transfer as important and needed to be assured that CHD-related information of importance for the young person's daily life would be given.

Pulmonary vein stenosis: the UK, Ireland and Sweden collaborative study.

OBJECTIVE: To describe clinical features, morphology, management and outcome of pulmonary vein stenosis (PVS) in childhood. DESIGN AND SETTING: Retrospective international collaborative study involving 19 paediatric cardiology centres in the UK, Ireland and Sweden. PATIENTS: Cases of PVS presenting between 1 January 1995 and 31 December 2004 were identified. Cases where pulmonary veins connected to a morphological left atrium were included. Functionally univentricular hearts and total anomalous pulmonary venous connection were excluded. All available data and imaging were reviewed. RESULTS: 58 cases were identified. In 22 cases (38%) there was premature delivery. 46 (79%) had associated cardiac lesions; 16 (28%) had undergone previous cardiac surgery before PVS diagnosis. 16 children (28%) had a syndrome or significant extracardiac abnormality. 36 presented with unilateral disease of which 86% was on the left. Where there was adequate sequential imaging, disease progression was shown with discrete stenosis leading to diffusely small pulmonary veins. Collateral vessels often developed. 13 patients had no intervention. Initial intervention was by catheter in 17 and surgery in 28. Overall 3-year survival was 49% (95% CI 35% to 63%) with patients undergoing initial surgical intervention having greater freedom from death or re-intervention (hazard ratio 0.44, 95% CI 0.2 to 0.99, p = 0.023). CONCLUSIONS: PVS is a complex disease of uncertain cause and frequently associated with prematurity. Early intervention may be indicated to deter irreversible secondary changes.

Analysis of atrioventricular plane movements by Doppler tissue imaging and M-mode in children with atrial septal defects before and after surgical and device closure.

Our objective was to compare the effects of surgical and device closure of atrial septal defects (ASDs) on atrioventricular plane function. In healthy individuals, both short- and long-axis motion contribute to ventricular pump function. Short-axis function (i.e., the amplitude and velocity of atrioventricular plane movements) may be evaluated by M-mode and Doppler tissue imaging. The study group consisted of 19 children with ASD of the secundum type before and after surgical (n = 12) or device (n = 7) closure and 10 healthy controls. Surgical and device closures were uncomplicated and all defects were completely closed. Registrations of atrioventricular plane systolic and diastolic amplitude and velocity were made from the tricuspid and the mitral annulus and from the septum in the apical four-chamber view. Comparisons were made between examinations before and after closure, between the two subgroups of patients treated by surgical and device closure, respectively, and between the patient group and the control groups. Before ASD closure, all measurements were normal or near normal. After surgery, systolic amplitudes and velocities of the tricuspid annulus and in the septum decreased significantly, whereas no changes were seen in the device group. Less marked changes were seen in diastolic measurements. However, in the surgical group significant decrease to subnormal values were found in the tricuspid annulus and in the septum, which may indicate a decreased diastolic function postoperatively. Mitral valve annulus amplitude and velocity were not affected by the treatment. Atrioventricular function is normal in children with right ventricular volume overload. The decrease to subnormal values after open-heart surgery is not seen after device closure of the ASD, indicating that surgery affects right ventricular function.

Long-distance transports of newborn infants with congenital heart disease.

Little has been published about specific problems that may occur during long-distance transports of newborn cardiac patients. During a 4-year period after centralization of pediatric heart surgery in Sweden, 286 transports were prospectively investigated. A majority (77.3%) of the transports were carried out by nonspecialized teams. Ten severe adverse events, including the death of 1 infant, occurred during the 286 transports (3.5%). Another infant died later of cerebral complications from hypoxia, rendering a transport-related mortality of 0.7%. Twenty-two infants (7.7%) were severely hypoxic (oxygen saturation < or =65%) at arrival, and 12 of these infants suffered from transposition of the great arteries. During the second 2-year period increased use of intravenous prostaglandin E1 and transportation from tertiary-level units was associated with better transport outcome. During the same time period, overall 30-day postoperative mortality for pediatric cardiac surgery decreased from 4.0% to 1.2% in our hospital. When highly specialized treatment is centralized for quality reasons it is also important that risks associated with transport are considered and that the quality of transport is high. For some cardiac malformations antenatal diagnosis and referral of the mother for delivery to a center with pediatric cardiac surgery would probably further increase the chance of healthy survival in some infants.

The outcome of children born with pulmonary atresia and intact ventricular septum in Sweden from 1980 to 1999.

OBJECTIVE: To study the outcome of all children born with pulmonary atresia and intact ventricular septum in Sweden between 1980 and 1999. DESIGN: Retrospective study of medical records with review of the initial, preoperative angiocardiographic and echocardiographic examinations. RESULTS: A total of 84 children were born with pulmonary atresia and intact ventricular septum, giving an incidence of 4.2 per 100,000 live births. In all, 77 were operated on with a 1-year survival rate of 75%. Thirty-six children had ventriculocoronary communications, with a 1-year survival rate of 50%. At the end of the study period, 52 children were alive, 32 with biventricular repair, and 19 with univentricular repair. Follow-up time was 14 days-20 years (median, 6 years). Statistical analysis of incremental risk factors for death showed statistical significance for low birth weight, male sex, muscular pulmonary atresia, and having a systemic-to-pulmonary shunt as the sole initial intervention. CONCLUSION: Complete national data of all patients born with pulmonary atresia and intact ventricular septum during 1980-1999 in Sweden revealed a total mortality in accordance with previous reports for results for surgery. Further improvements demand a thorough preoperative investigation of the cardiac anatomy, particularly of the ventriculocoronary communications, to enable right ventricular decompression whenever possible.

Emergency pacing and subsequent permanent pacemaker implantation in a premature infant of 1770 g with a follow-up of 6 years.

We report successful emergency pacing followed by permanent pacemaker implantation due to complete block in an otherwise healthy premature infant of 1,770 g. Via the umbilical vein a temporary bipolar pacing lead was placed in the right ventricle. The lack of spontaneous improvement warranted implantation of a permanent pacemaker system at the age of 2 weeks. Via a transatrial approach an endocardial unipolar screw-in lead was placed in the right ventricle and connected to a pulse generator implanted subcutaneously. During the follow-up period of 6 years the child has been doing well with his VVI-R pacemaker operating at rates between 80 and 160 pulses/minute at a chronic stimulation threshold below 0.9 V at 0.37 msec.

Left-to-right cardiac shunts: comparison of measurements obtained with MR velocity mapping and with radionuclide angiography.

PURPOSE: To investigate the agreement between two noninvasive methods, magnetic resonance (MR) velocity mapping and first-pass radionuclide angiography, to quantify the pulmonary-to-systemic blood flow ratio (QP/QS) in adults, adolescents, and children with left-to-right cardiac shunts. MATERIALS AND METHODS: The accuracy and precision of MR velocity mapping were studied in 12 control subjects (six men, six women) and in a phantom. MR velocity mapping and radionuclide angiography were performed on the same day in 24 patients (16 adults, two adolescents, six children; five male patients, 19 female patients). RESULTS: The mean error in QP/QS at MR velocity mapping in phantom experiments was -1% +/- 1 (mean +/- SD). In control subjects, QP/QS at MR velocity mapping was 1.03 +/- 0.03, and the cardiac index was 3.1 L/min/m2 +/- 0.2 and 3.2 L/min/m2 +/- 0.3 for women and men, respectively. In patients, QP/QS at radionuclide angiography was 14% +/- 13, higher than at MR velocity mapping. Interobserver variability was four times higher for radionuclide angiography compared with MR velocity mapping, 0% + 16 versus 0% +/- 4 (n = 12). The difference between repeated MR flow measurements in the same vessel was -1% +/- 5 (n = 36). CONCLUSION: The data suggest that MR velocity mapping is accurate and precise for measurements of shunt size over the whole range of possible QP/QS values.

Parental reactions to fetal echocardiography.

The impact of fetal echocardiography (FE) was studied in 65 families where a previous child had been born with heart disease and where FE showed normal cardiac anatomy in the fetus. Forty-six of the families had lost their child with heart disease (Group 1), while the child was alive in 19 of the families (Group 2). A questionnaire concerning the attitude of the parents toward FE was sent to all families. The possibility of having FE had influenced the decision to have another child in 64% in Group 1 and in 26% in Group 2 (P < 0.01). If a serious cardiac malformation had been found at FE, 69% in Group 1 and 29% in Group 2 would have chosen abortion (P < 0.01). The anxiety decreased significantly in both groups after FE. Of the participating mothers, 96% wishes FE at the next pregnancy. The study indicates that FE probably is very important to families who had previously had a child with a serious malformation but also that many families intend to continue the pregnancy even if a malformation is diagnosed.

Clinical and immunohistochemical findings in a case of neonatal Marfan syndrome.

A newborn girl with extreme cardiomegaly discovered by fetal ultrasound after 34 gestational weeks is presented. The girl was delivered through a Caesarean section. After birth, multiple skeletal stigmata and generalized cardiac involvement with abnormal valves and dilated great arteries suggested Marfan syndrome. The girl died at the age of 10 h. The postmortem examinations showed cardiovascular lesions typical of Marfan syndrome. Immunofluorescence studies from cultured fibroblasts of the patient showed decreased amounts of immunostained fibrous material, supporting the clinical diagnosis of a severe Marfan syndrome.

Left ventricular thrombosis during infancy: report of two cases.

Two patients with left ventricular thrombosis diagnosed by echocardiography are presented. The first patient was a 6-week-old girl with supraventricular tachycardia. Cross-sectional echocardiography showed a rounded and mobile structure protruding from the left ventricular wall. The girl was in heart failure and had signs of peripheral embolization. After sinus rhythm had been restored the thrombus diminished gradually and the girl recovered. The second patient was a newborn boy with severe aortic stenosis and a large massive thrombus along the left side of the interventricular septum. The boy died after valvotomy, the post-mortem examination confirmed the diagnosis of a large thrombus. Left ventricular thrombosis is uncommon in neonates and infants but may appear secondary to abnormal hemodynamics.

Cardiac function in healthy infants and children: Doppler echocardiographic evaluation.

To establish normal values for Doppler-derived parameters of cardiac function, pulsed-wave Doppler recordings from the ascending aorta were obtained in 80 healthy infants and children. Stroke and minute distance, peak velocity, mean acceleration, acceleration and ejection time intervals, and the acceleration/ejection time ratios were measured or calculated from the Doppler recordings. The relations between the Doppler parameters and heart rate, age, and body surface area were analyzed separately for the children below and above 6 months of age. The normal values for the two groups are given as the median and range and as the mean and standard deviations, respectively. For the older age group, strong negative correlations with heart rate were found for stroke distance and ejection time, suggesting that these parameters should be evaluated in relation to heart rate. Intraobserver and interobserver reproducibility were studied in 10 children. Good reproducibility was found for stroke and minute distance, peak velocity, and left ventricular ejection time. For measurements related to acceleration, the reproducibility was less good.

Cross-sectional echocardiographic measurements of right ventricular size and growth in patients with pulmonary atresia and intact ventricular septum.

Fifteen patients with pulmonary atresia or critical pulmonary stenosis and intact ventricular septum were studied. All were operated on in the neonatal period, with valvotomy or a systemic to pulmonary arterial shunt, or both. In 12 patients, right ventricular to pulmonary arterial communication was established in the neonatal period. In three patients, only systemic to pulmonary arterial shunts were constructed. Six patients died. The median follow-up period for the surviving patients was 64 months (range, 12-87 months). Right and left atrial and ventricular dimensions and areas, the tricuspid annular diameter, and the cross-sectional area of the aortic root were measured in cross-sectional echocardiograms from the neonatal period, at the age of 1 year, and at the latest clinical follow-up. A classification of right ventricular morphology was made, based on identification of the inlet, the trabecular, and the outlet parts. Most of the patients had hypoplastic right ventricles at birth but at the latest follow-up, seven of nine surviving patients had right ventricles in the normal range. Right ventricular growth was better in patients who were given a right ventricular to pulmonary arterial communication in the neonatal period and those with complete right ventricular anatomy. The patients who died had severely hypoplastic right ventricles and small tricuspid valves.

Cross-sectional echocardiographic measurement of right atrial and right ventricular size in children with atrial septal defect before and after surgery.

A total of 25 children operated on for atrial septal defect were studied by cross-sectional echocardiography before and after operation. Right atrial and right ventricular dimensions and areas were measured preoperatively and 1-4 years (median, 32 months) postoperatively. The length, width, and area of the right atrium (RA) and the right ventricle (RV) were measured in the apical and the subcostal four-chamber views. The dimension of the RV outflow tract (RVOT) was measured in the parasternal short-axis view. Preoperatively, all group mean values except the subcostal four-chamber length of the RV were significantly larger than normal. After surgery, there was a significant decrease in all of the parameters studied, except for RV length. The mean postoperative values of RA measurements in the apical four-chamber view and of RVOT were still significantly larger than normal. The RV four-chamber measurements except the apical four-chamber length were not significantly enlarged. The greatest decrease in RA and RV size occurred in the first postoperative year. Longer follow-up periods did not change the measurements significantly.

Dimensions of cardiac chambers and great vessels by cross-sectional echocardiography in infants and children.

A total of 120 healthy infants, children, and teenagers were examined by cross-sectional echocardiography. Right and left atrial and ventricular dimensions and areas were measured in the parasternal, apical, and subcostal views. Dimensions of the inferior caval vein, the pulmonary artery and the aorta were obtained in the parasternal, suprasternal, and subcostal views. Reproducibility was studied in separate material consisting of 19 children with various forms of congenital heart disease examined consecutively by two different observers. Interobserver reproducibility was expressed as the 95% tolerance limit for the difference between two measurements. Good correlation with body surface area was demonstrated for all measurements, and the regression equations for the normal values of the parameters studied are given. Interobserver reproducibility was fairly good for measurements in the parasternal views, but moderate or low for measurements in the apical and the subcostal four-chamber views. Measurements in cross-sectional echocardiography are clinically useful, especially in the study of the right-sided cardiac structures that are difficult to evaluate with M-mode echocardiography, but the problems of reproducibility have to be taken into account.

Plasma and urinary beta-hexosaminidase in juvenile diabetes mellitus.

The activity of beta-hexosaminidase (E C 3.2.1.30) in plasma and urine was determined in 99 patients with juvenile diabetes mellitus and in 40 age-matched controls. Plasma enzyme activities were above normal in plasma from diabetics and showed significant correlations with blood glucose and glycosylated HbA1 (GHbA1) but not with the presence of retinopathy. The urinary excretion of beta-hexosaminidase, which is a sensitive indicator of renal injury, was also excessive in the diabetics and showed a significant correlation with blood GHbA1. The results show that plasma and urinary beta-hexosaminidase correlate with parameters of diabetic control and that urinary beta-hexosaminidase may be more sensitive than urinary albumin as an indicator of early renal damage.