Paediatric on-call consultants' learning within and beyond the objectives of a coherent CPD program.

BACKGROUND: Evaluations, using questionnaires, of a two-year long CPD program for on-call consultant paediatricians, showed that the overall objective of the program was largely met. We stipulate that the coherency of the CPD program contributed to the learning. To gains a deeper understanding of the participants learning within and beyond the overall objectives of the program, we decided to conduct an interview study enrolling participants from the first two CPD courses. METHODS: Nine experienced paediatric consultants were interviewed 1-4 years after completing a coherent two-year long CPD program, focusing on what and how they learned. The interviews were audio-recorded and transcribed as text, analysed, and categorised using qualitative content analysis. RESULTS: What the participants learned: improved medical competences, greater confidence in the role of an on-call consultant, better understanding of the role of an on-call consultant and importance of professional networks. Several categories were outside the overall objective, at personal level: an understanding of one's own and other's competences, taking responsibility for one's own CPD and managing things one does not know. At professional level: more secure as an individual and with colleagues. How it was learned: relevant objectives, preparatory material and case discussions were important. Participants learned by preparing, repeating, reflecting, and participating actively, and by applying what they learned in clinical practice. The participants learned from one other over a period of two years, when they also got to know one another and created networks. A safe learning environment imposed demands and enabled participants to define their competence and learn accordingly. CONCLUSIONS: This study describes what and how on-call consultant paediatricians learned during a coherent two-year long CPD program. The learning took place within and beyond the framework of the overall objectives. The study suggests that evaluation methods based on objectives may be blind to important areas of learning and need to be combined with qualitative methods that examine a broad impact of learning. Taken together, the analysis of what and how the participants learned shows that they were better equipped to work as consultant on call and deal with the things they did not know.

Strengthening paediatric competencies and educational skills among general paediatricians.

AIM: To develop a coherent programme that addresses the need for continuing professional development in general paediatrics and educational skills for senior paediatricians at outpatient clinics in Sweden today. METHODS: Educational needs in the target group were investigated using a questionnaire. Themes and sub-themes extracted from responses informed the curriculum formulation of the continuing professional development (CPD) programme, which was completed using a variety of learning, assessment and evaluation methods. RESULTS: Forty-six paediatricians identified 355 clinical situations. Competencies in general paediatrics and educational skills were incorporated in a CPD programme, implemented in western Sweden between 13 October 2016 and 23 May 2019, with 23 learning modules and 18 participants (male/female 3/15, median age 55 years). The participants' evaluation emphasised the importance of adult learning principles. Their responses to open reflective questions on 23 May 2109 suggested that the programme offered a learning environment in which they could develop their paediatric and educational practices and improve their mentorship, networks and work-based learning environment. They also described an enhanced feeling of joy at work. CONCLUSION: The involvement of experienced paediatricians in the programme formulation may be inspirational to clinicians and contribute to the definition, revitalisation and prioritisation of general paediatrics in Sweden in the future.

Integrating a pedagogic course in a CPD programme for paediatricians at out-patient clinics.

Equipping paediatricians for the challenges of the explosive development of knowledge and specialised health care calls for a well-planned continuing professional development (CPD) strategy which updates paediatric competencies and the pedagogic skills among paediatricians. The purpose of the study was to evaluate the effects of a pedagogic course, integrated into a CPD programme for paediatricians at out-patient clinics. The pedagogic course comprised three learning components, participation in a CPD programme, during two and a half years, a pedagogic learning module and a pedagogic assignment. The objectives of all the learning activities, including the pedagogic course, were developed according to adult learning theories. Evaluations were made using questionnaires. Seventeen paediatricians participated in the CPD programme; 13 of them completed the pedagogic learning module and six the full pedagogic course, including the pedagogic assignment - teaching at one's own clinic. Evaluation of the pedagogic assignment at the participants' own clinics by 64 co-workers revealed that the co-workers appreciated the training activities and would recommend them to a colleague. We conclude that it is possible to combine medical and pedagogic education in a CPD programme for paediatricians and that the participants were able to digest and apply the pedagogic principles used in the course.

An overview of how on-call consultant paediatricians can recognise and manage severe primary immunodeficiencies.

Severe primary paediatric immunodeficiency syndromes are rare and potentially fatal unless suspected, diagnosed and treated early. We provide clinical guidance and support for on-call consultant paediatricians working in secondary level hospitals on how to recognise and manage children with these conditions. Our paper addresses four conditions that risk the most severe outcomes if they are not adequately cared for during on-call periods, such as weekends: severe combined immunodeficiency, haemophagocytic lymphohistiocytosis, severe congenital neutropaenia and chronic granulomatous disease. CONCLUSION: On-call paediatricians are provided with advice on handling the most severe primary immunodeficiencies.

Fifteen-minute consultation: Recognising primary immune deficiencies in children.

Children with primary immunodeficiency syndromes present with broad variation of clinical features and the consequences are often severe if not promptly recognised. Here, support is provided for the general paediatrician to recognise primary immunodeficiencies among the many children they meet in their clinical practice.

Combining learning for educators and participants in a paediatric CPD programme.

BACKGROUND: Most continuing professional development (CPD) programmes do not include an educational training module. In our country, educational practice in the areas of CPD and continuing medical education relies traditionally on conventional lectures. This is in sharp contrast to the educational research that clearly demonstrates that educational programmes emphasising adult learning methods have greater potential to change physicians' clinical practice. To investigate whether lecture-oriented educators were prepared to change their educational practice towards principles of adult learning, we decided to combine learning for educators and participants in a paediatric CPD programme. The aim of the study was to investigate educators' reflections on their learning and educational practice after they have undergone an educational skills component integrated in the implementation of a CPD learning module for paediatricians and evaluate the results from the participants' perspective. METHODS: The objectives of the educational skills component of the learning module were developed according to adult learning theories. The learning objectives for the CPD learning module were based on a pre-course needs assessment. Evaluations were made using questionnaires. RESULTS: Seven of 10 participants in the educational skills component of the learning module and all the participants, 13 paediatricians and 14 nurses, who participated in the learning module, answered the questionnaires. The results of this pilot study show that educators whose main experience of teaching was based on lectures were strengthened in their practice; they defined their competence and were prepared to move towards adult learning principles. The participants in the learning module expressed a high degree of satisfaction. CONCLUSIONS: We conclude that it is feasible to combine learning for educators and participants in a paediatric CPD programme and that lecture-oriented educators are prepared to change their educational practice towards principles of adult learning.

Using scenario-based training to promote information literacy among on-call consultant pediatricians.

BACKGROUND: Traditionally, teaching hospital staff to search for medical information relies heavily on educator-defined search methods. In contrast, the authors describe our experiences using real-time scenarios to teach on-call consultant pediatricians information literacy skills as part of a two-year continuing professional development program. CASE PRESENTATION: Two information-searching workshops were held at Sahlgrenska University Hospital in Gothenburg, Sweden. During the workshops, pediatricians were presented with medical scenarios that were closely related to their clinical practice. Participants were initially encouraged to solve the problems using their own preferred search methods, followed by group discussions led by clinical educators and a medical librarian in which search problems were identified and overcome. The workshops were evaluated using questionnaires to assess participant satisfaction and the extent to which participants intended to implement changes in their clinical practice and reported actual change. CONCLUSIONS: A scenario-based approach to teaching clinicians how to search for medical information is an attractive alternative to traditional lectures. The relevance of such an approach was supported by a high level of participant engagement during the workshops and high scores for participant satisfaction, intended changes to clinical practice, and reported benefits in actual clinical practice.

Blood pressure and anthropometry in children treated with stimulants: a longitudinal cohort study with an individual approach.

BACKGROUND: Knowledge about the long-term effects on blood pressure (BP) and body mass index (BMI) when treating young patients for attention-deficit/hyperactivity disorder (AD/HD) with stimulants is limited. Most of the studies have reported mean and not individual values for anthropometrics and BP in treatment with stimulants. This seems to be the first study of changes based on the analyses of individual data measured over time. PATIENTS AND METHODS: Seventy young patients (aged 8-18 years) diagnosed with AD/HD and responding well to treatment with stimulants were followed for a mean period of 3 years and 3 months. BP, heart rate, height, weight, and BMI were transformed to standard deviations or z-scores from before treatment to the last registered visit. RESULTS: The mean dose of methylphenidate was 0.95 mg/kg. The mean increase of systolic and diastolic BP was 0.4 z-score and 0.1 z-score, respectively. The systolic BP was associated with BMI; a higher BMI at baseline increased the risk for an increase in systolic BP. Ten percent of the total group had a weight at follow-up of <-1.5 standard deviation (SD) and 12% had a height of <-1.5 SD. Mean height at follow-up was -0.2 SD, but 40% had a reduced height of at least 0.5 SD during the treatment period. BMI on a group level was reduced from +0.8 SD to +0.3 SD. Of the 19 patients with a BMI >+1.5 SD at baseline, 50% had a significantly reduced BMI. CONCLUSION: Consequences of stimulant treatment must be evaluated individually. Besides significant effects on core AD/HD symptoms, some patients have lower BMI and BP and some increase/maintain their BMI and/or increase their systolic BP. The risk of reduced height trajectory needs further research.

[On call education for paediatricians may improve patient safety. Continuing professional development project in western Sweden evaluated]. / Bakjoursutbildning för barnläkare kan stärka patientsäkerheten - Fortbildningsprojekt utvärderas i Västra Götalandsregionen.

As part of a mandate to provide continuing professional development (CPD) for paediatricians in western Sweden and to support patient safety, we have addressed the professional demands which are imposed on today's consultant paediatricians on call, by designing and implementing a CPD programme for this group. Learning objectives for specific learning modules were developed from a pre-course needs assessment among consultants. The implemented programme included a variety of learning, assessment and evaluation methods. Each specific learning module began with a reading assignment exploring the key concept. This prepared the participants for the case discussion, in which they would analyse, reflect and achieve a deeper level of understanding. We believe that the educational methods used and the approach of the programme are applicable to specialist training as well as to CPD programmes in general, as part of a lifelong learning process.

Devising a competency-based continuing professional development programme to meet the needs of on-call consultant paediatricians.

AIM: To define the clinical competencies currently required by on-call consultant paediatricians in Sweden and to describe how these competencies can be acquired by using an outcome-based continuing professional development (CPD) programme. METHODS: The overall aims and objectives of the programme were formulated to meet the current responsibilities of an on-call consultant. It was delineated during a precourse needs assessment of 26 consultants, before being implemented with 16 participants, using a variety of learning, assessment and evaluation methods. RESULTS: The precourse needs assessment identified 217 clinical situations and 17 themes, and 139 subthemes were developed. During the programme evaluation, carried out using a reflective questionnaire, participants stated that they had improved their understanding of the role of, and demands on, the on-call consultant. They had also felt more confident on-call, had built a network of colleagues and updated their knowledge in relevant fields. CONCLUSION: We defined and implemented a competency-based CPD programme to meet the changing demands faced by on-call consultant paediatricians. The aims and objectives of the programme were formulated according to a needs assessment among consultant paediatricians active in on-call services. Evaluation of the programme indicates that participants moved in the direction of the overall objectives.

Clinical manifestation and a new ISCU mutation in iron-sulphur cluster deficiency myopathy.

Myopathy with deficiency of succinate dehydrogenase and aconitase is a recessively inherited disorder characterized by childhood-onset early fatigue, dyspnoea and palpitations on trivial exercise. The disease is non-progressive, but life-threatening episodes of widespread weakness, severe metabolic acidosis and rhabdomyolysis may occur. The disease has so far only been identified in northern Sweden. The clinical, histochemical and biochemical phenotype is very homogenous and the patients are homozygous for a deep intronic IVS5 + 382G>C splicing affecting mutation in ISCU, which encodes the differently spliced cytosolic and mitochondrial iron-sulphur cluster assembly protein IscU. Iron-sulphur cluster containing proteins are essential for iron homeostasis and respiratory chain function, with IscU being among the most conserved proteins in evolution. We identified a shared homozygous segment of only 405,000 base pair with the deep intronic mutation in eight patients with a phenotype consistent with the original description of the disease. Two other patients, two brothers, had an identical biochemical and histochemical phenotype which is probably pathognomonic for muscle iron-sulphur cluster deficiency, but they presented with a disease where the clinical phenotype was characterized by early onset of a slowly progressive severe muscle weakness, severe exercise intolerance and cardiomyopathy. The brothers were compound heterozygous for the deep intronic mutation and had a c.149 G>A missense mutation in exon 3 changing a completely conserved glycine residue to a glutamate. The missense mutation was inherited from their mother who was of Finnish descent. The intronic mutation affects mRNA splicing and results in inclusion of pseudoexons in most transcripts in muscle. The pseudoexon inclusion results in a change in the reading frame and appearance of a premature stop codon. In western blot analysis of protein extracts from fibroblasts, there was no pronounced reduction of IscU in any of the patients, but the analysis revealed that the species corresponding to mitochondrial IscU migrates slower than a species present only in whole cells. In protein extracted from isolated skeletal muscle mitochondria the western blot analysis revealed a severe deficiency of IscU in the homozygous patients and appearance of a faint new fraction that could represent a truncated protein. There was only a slight reduction of mitochondrial IscU in the compound heterozygotes, despite their severe phenotype, indicating that the IscU expressed in these patients is non-functional.

Plasma levels of natriuretic peptide type B and A in children with heart disease with different types of cardiac load or systolic dysfunction.

Natriuretic peptide levels B (BNP) and A (ANP) have been described in children with different diagnose of congenital heart defects (CHD). However, the impact of the type of cardiac load per se on natriuretic peptide levels, irrespective of diagnosis, has not been reported. The aim of the present study was to evaluate the levels of BNP and ANP in children with congenital and acquired heart disease according to different types of cardiac load. Plasma BNP and ANP were analysed in 137 children with CHD/heart disease, median age 2.9 (0.3-16.7) years. Haemodynamic load was classified as: no overload, pressure overload, volume overload of right and/or left ventricle and systolic ventricular dysfunction. Twenty-three children without heart disease served as controls for the natriuretic peptide measurements. The highest BNP and ANP values were observed in the systolic dysfunction, 613 ng l(-1) (81.8-3910) and 431 (43.8-1990), and volume groups, 29.8 (5.5-352) and 93.0 (15.9-346), respectively, whereas the values in the pressure, 17.9 (0.7-315) and 51.9 (8.7-210), and no overload groups, 10.3 (0.2-28.1) and 28.6 (8.6-105), respectively, were only slightly higher than those in the controls 4.7 (0.0-17.7) and 32.9 (11.7-212.2), respectively. The highest BNP and ANP values were seen in children with systolic dysfunction, while volume overload in the absence of heart failure resulted in higher levels than pressure overload.

Age- and gender-specific mortality rates in childhood hypertrophic cardiomyopathy.

AIMS: Hypertrophic cardiomyopathy (HCM) is the commonest inherited cause of sudden cardiac death in children; current guidelines suggest HCM screening after 12-15 years of age. The study aims to establish the age range at highest risk. METHODS AND RESULTS: Cohort study from six regional centres of paediatric cardiology, including children presenting with sudden death; n = 150 (59% = male; 39% familial HCM). Age- and gender-specific mortality was calculated, and compared with rates calculated from the Swedish National Cause of Death Registry. There were 56 deaths within the cohort, 39 were sudden arrhythmia deaths, with 31 at <19 years of age. Between 9-13.9 years of age annual sudden death mortality averages 7.2%, vs. 1.7% after 16 years of age; P = 0.025, odds ratio for proportions 3.75 [95% confidence intervals (CI) 1.18-11.91], similar in both familial and idiopathic HCM. The risk for sudden death peaks earlier in girls (10-11 years), with male preponderance after the age of 15. National cause of death statistics confirm that the mortality rate from HCM is significantly higher in the 8-16 year olds (0.112 per 100,000 age-specific population) than in the 17-30 year olds (0.055 per 100,000; 95% CI 0.011-0.099). CONCLUSION: In families with HCM, children should be screened at an early age.

Increased natriuretic peptide type B level after the second palliative step in children with univentricular hearts with right ventricular morphology but not left ventricular morphology.

We analyzed the natriuretic peptide type B (BNP) and A (ANP) levels in the plasma of 38 children with stable functionally univentricular heart defects, 10 of whom had undergone the first palliative step (Shunt), 13 of whom had undergone the second palliative step (Glenn), and 15 of whom had completed total cavopulmonary connection (TCPC). The levels of BNP and ANP were significantly higher after the first palliative step, 31.6 ng/l (8.3-122) (median and range) and 101.2 ng/l (17.1-203), respectively, than after the Glenn procedure, 6.7 ng/l (0.0-16.0) and 23.6 ng/l (15.7-54.4), respectively, as well as after completed TCPC, 9.0 ng/l (0.0-39.1) and 20.9 ng/l (11.2-28.3), respectively, and, in a control group of children without heart defects, 5.8 ng/l (0.0-38.7) and 32.9 ng/l (11.7-212.1), respectively (p = 0.0003 and p = 0.0003, respectively). After the first palliative step, the BNP and ANP levels were the same in children with right and left ventricular morphology (p = 0.67 and p = 0.52, respectively). After unloading the ventricle (Glenn and TCPC together), BNP levels were higher in children with right ventricular morphology compared with those with left ventricular morphology and controls (p = 0.02). Children with functionally univentricular hearts in stable condition have increased BNP and ANP levels after the first palliative procedure. After the second and third palliative steps, the BNP and ANP levels were low and similar to those of children without heart defects. However, BNP levels in children with a systemic ventricle of right ventricular morphology were higher than those in children with left ventricular morphology.

Brain natriuretic peptide assessed at long-term follow-up before and after maximal exercise in surgically palliated patients with functionally univentricular hearts.

We evaluated the concentrations of brain natriuretic peptide in the plasma as a marker of systolic ventricular function before and after maximal exercise in 15 surgically palliated patients with functionally univentricular hearts, with apparently good ventricular function. Of the patients, 6 with median age of 14.6 years, and a range from 12.5 to 17.9 years, had been palliated by construction of a total cavopulmonary connection, while the other 9 patients, with a median age of 32.1 years, and a range from 15.6 to 54.2 years, had undergone the classical Fontan procedure. We used 8 healthy individuals, with a median age of 13.9 years, and a range from 12.8 to 14.2 years, as a control group for the measurements of brain natriuretic peptide. The values of the peptide were significantly higher in those with the classical Fontan procedure, both before, when the median value was 131.8 nanogram per litre, with a range from 0.5 to 296.4, and after maximal exercise, when the median value was 108.1, with a range from 0.1 to 235.9. The comparable values in those with a total cavopulmonary connection were a median of 12.8, and a range from 0.5 to 39.1 before, and a median of 9.7, with a range from 2.7 to 26.2 after maximal exercise. The median value for the control group was 13.1, with a range from 2.6 to 38.7 before exercise (p = 0.016), and a median of 24.1, with a range from 5.8 to 66.7 after maximal exercise (p = 0.03), respectively. In the control subjects, the level of the peptide increased by a median of 9.7 nanograms per litre, with a range from 1.2 to 28.0 after maximal exercise (p = 0.008). The level was unchanged after maximal exercise in those with classical Fontan procedures and total cavopulmonary connections, with a difference between levels before and after exercise of a median of 5.9 nanogram per litre, and a range from -23.7 to 31.0 (p = 0.96), and a median of -1.0 nanogram per litre, with a range from -12.0 to 3.9 (p > 0.99), respectively. We conclude that maximal exercise did not increase the level of brain natriuretic peptide level in those patients with the classical Fontan procedure, nor those with a total cavopulmonary connection, findings which may indicate that systolic ventricular dysfunction is not the major cause of the decreased working capacity observed in patients with well functioning palliated functionally univentricular hearts.

3-methylcrotonyl-CoA carboxylase deficiency and severe multiple sclerosis.

This report describes a female with isolated 3-methylcrotonyl-CoA carboxylase deficiency. She had a mild Reye-like episode, loss of scalp hair, psychomotor retardation, and an attention-deficit hyperactivity disorder. The diagnosis was made at 13 years of age when she developed relapsing remitting multiple sclerosis with a malignant course. Treatment with steroids had initially a good therapeutic effect on the relapses. The response to interferon beta-1a treatment was poor. On mitoxantrone treatment there was a considerable neurologic recovery.

Echocardiographic and electrocardiographic identification of those children with hypertrophic cardiomyopathy who should be considered at high-risk of dying suddenly.

BACKGROUND: Hypertrophic cardiomyopathy is a common cause of sudden death in children. In this study, we aimed to identify clinical measures for stratification of this risk in childhood. PATIENTS AND METHODS: By means of a retrospective cohort study from six regional centres of paediatric cardiology, we identified 128 patients with hypertrophic cardiomyopathy presenting below 19 years of age, with a mean follow-up of 10.8 years. Of the patients, 31 had died, 16 suddenly, with a median age at sudden death of 13.3 years. RESULTS: Cox regression shows that electrocardiographic voltages, analysed as the sum of the R and S waves in all six limb leads (p equal to 0.001), and septal thickness expressed as proportion of the 95th centile for age (p equal to 0.036), were independent predictors of sudden death. When the sum of the R and S waves is over 10 millivolts, the odds ratio for sudden death was 8.4, with 95% confidence intervals from 2.2 to 33.7 (p equal to 0.0012), and finding a septal thickness over 190% of 95th centile for age gives an odds ratio of 6.2, with confidence intervals from 1.5 to 25.1 (p equal to 0.011). Noonan's syndrome, with a p value equal to 0.043, and the ratio of the left ventricular wall to its cavity in diastole, with a p value equal to 0.005, were independent predictors of death in cardiac failure, with a ratio of the mural thickness to the dimension of the cavity over 0.30 giving an odds ratio of 36.0, with confidence limits from 4.2 to 311, and a p value equal to 0.00009. At follow-up, patients deemed to be at a high risk of dying suddenly were identified by the combination of the sum of the R and S waves greater than 10 millivolts and septal thickness over 190%, with a sensitivity of 91%, specificity of 78%, positive predictive value of 50%, and a negative predictive value of 97%. CONCLUSIONS: Children at high risk of dying suddenly with hypertrophic cardiomyopathy, with a subsequent annual mortality of 6.6%, can be distinguished at the time of diagnosis from those patients having a low risk of sudden death, the latter with an annual mortality of 0.27%.

Increased plasma levels of natriuretic peptide type B and A in children with congenital heart defects with left compared with right ventricular volume overload or pressure overload.

AIM: Natriuretic peptide levels B (BNP) and A (ANP) have been described in children with congenital heart defects (CHD) with pressure and volume overload. However, the impact of ventricular morphology per se on natriuretic peptide levels has not been reported. The aim of the present study was to evaluate plasma BNP and ANP in children with CHD with left or right ventricular volume or pressure overload. METHODS AND RESULTS: Plasma BNP and ANP were analysed in 61 children, median age 3.1 (0.3-16.2) years. Haemodynamic load was evaluated by echo-Doppler and/or catheterization measurements and classified as: pressure overload of the right (RV pressure) or left (LV pressure) ventricle, or volume overload of the right (RV volume) or left (LV volume) ventricle, of a sufficient degree to indicate surgery/catheter intervention. Twenty-three children, with a median age of 1.1 (0.1-8.3) years, without heart disease, served as controls for the natriuretic peptide measurements. Children in the LV volume group had significantly higher BNP and ANP values, 55.4 ng l-1 (10.7-352) and 164 (31.8-346), than children in the RV volume, 15.6 (0.0-105.1) and 57.2 (11.3-234.1), LV pressure, 6.8 (0.7-170) and 40.8 (12.6-210), and RV pressure, 18.0 (5.0-29.1) and 69.3 (8.7-182), groups respectively (P<0.0001). The values in the LV pressure group were close to the values in the Control group, 4.7 (0.0-17.7) and 32.9 (11.7-212.1), respectively (P=0.051 and P=0.378, respectively). CONCLUSIONS: Plasma concentrations of BNP and ANP were higher in children with CHD with left ventricular volume overload compared with right ventricular volume overload or pressure overload.

Pulmonary gas exchange during exercise in Fontan patients at a long-term follow-up.

UNLABELLED: Desaturation during exercise in the Fontan type of circulation may be due to known right-to-left shunts in some patients, while in others there are no obvious signs of shunts. AIM: To evaluate the gas exchange and exercise capacity in patients with Fontan circulation. MATERIAL AND METHODS: Twenty patients with the Fontan type of circulation, median age at operation 7.5 years (3.0-35 years), follow-up time 12.1 years (8.3-20.4 years), were included. The intrapulmonary gas mixing (N(2)-slope) and diffusion capacity for carbon monoxide (D(LCO)) was evaluated and determination of cardiac output was performed at rest and during exercise with measurements of ventilation, oxygen uptake, respiratory rate, carbon dioxide production and heart rate. Samples for blood gases (P(a)O(2), S(a)O(2)) were obtained at rest and at the end of each work load. RESULTS: The median exercise capacity was 1.47 l min(-1). During exercise, all the patients had an alveolar-arterial oxygen partial pressure difference (P(A-a)O(2)) above 2 SD. Patients with known right-to-left shunts had lower P(a)O(2) and S(a)O(2) values and higher P(A-a)O(2) values both at rest, median 7.5 kPa, 90%, and 7.2 kPa, respectively, and during exercise, median 5.8 kPa, 77% and 10.1 kPa, compared with those with no shunts, median 11.2 kPa, 97% and 4.1, respectively, at rest and 9.7 kPa, 94%, and 6.3 during exercise (P<0.01). No correlation was found between cardiac index, the N(2)-slope or the D(LCO) at rest and the P(a)O(2) and S(a)O(2) at rest or during exercise. CONCLUSION: In patients with the Fontan type of circulation, right-to-left shunts are the major cause of desaturation during exercise.

Increased levels of brain and atrial natriuretic peptides after the first palliative operation, but not after a bidirectional glenn anastomosis, in children with functionally univentricular hearts.

We evaluated the concentrations of the brain and atrial natriuretic peptides in the plasma as markers of ventricular function and volume load in children with functionally univentricular hearts. We studied 7 children aged from 0.5 to 0.7 years with functionally univentricular hearts who had undergone a first palliative operation, and 10 children aged from 1.8 to 3.7 years who had undergone a bidirectional Glenn anastomosis at ages ranging from 0.4 to 1.0 year. As a control group, we studied 14 children without heart defects aged from 0.1 to 4.5 years. Levels of the brain natriuretic peptide were measured at 8.3 to 122 ng/l, with a mean of 52.8 ng/l, after the first palliative operation, compared to 0 to 16 ng/l, with a mean of 7.3 ng/l, after a bidirectional Glenn anastomosis, and 0 to 13.8 ng/l, with a mean of 5.9 ng/l, in the children serving as controls. Corresponding values for atrial natriuretic peptide were 17 to 203 ng/l, with a mean of 103 ng/l, after the first palliative operation, compared to 16 to 54 ng/l, with a mean of 29 ng/l, after the bidirectional Glenn anastomosis, and 12 to 52 ng/l, with a mean of 32 ng/l in the controls. Echocardiography showed that all the children with functionally univentricular hearts had normal ventricular function. Blood presssure, pulmonary arterial pressure, and arterial saturations of oxygen did not differ between the groups. We conclude, that in children with functionally univentricular hearts, the volume overload imposed on the heart after the first palliative operation is associated with increased production of brain and atrial natriuretic peptides, while after ventricular unloading, levels of the natriuretic peptides return to control values.