Martial M. Massin

Long term outcome up to 30 years after the Mustard or Senning operation: a nationwide multicentre study in Belgium

Objective: To assess long term outcome of patients who underwent Mustard or Senning repair for transposition of the great arteries up to 30 years earlier. Design: Retrospective review of medical records. Setting: The six university hospitals in Belgium with paediatric cardiology departments. Patients: 339 patients were reviewed, of whom 124 underwent the Mustard procedure and 215 the Senning procedure. This represents almost the entire population of patients in Belgium with either simple or complex transposition. Main outcome measures: Mortality, morbidity, functional abilities, social integration. Results: Overall mortality was 24.2%. Early mortality (⩽ 30 days after surgery) accounted for 16.5%, late mortality for 7.7%. Actuarial survival of early survivors at 10, 20, and 30 years after surgery was 91.7%, 88.6%, and 79.3%, respectively. Patients in the Senning cohort had a slightly better survival rate than those in the Mustard cohort (NS). Baffle obstruction occurred more often after Mustard repair (15.3%) than after the Senning procedure (1.4%). Arrhythmia-free survival did not differ between the two cohorts, but was determined by the complexity of the transposition. Survivors of the Senning cohort had better functional status, and tended to engage in more sports activities. Conclusions: The long term outcome for patients surviving the Mustard or Senning operation was favourable in terms of late mortality, morbidity, functional, and social status. Overall mortality in the Senning cohort did not differ from the Mustard group, but Senning patients had better functional status, greater participation in sports activities, and fewer baffle related problems.

Normal Ranges of Heart Rate Variability During Infancy and Childhood

Abstract. Heart rate variability is a noninvasive index of the neural activity of the heart. The present study examined heart rate variability indices in 210 infants and children aged 3 days to 14 years to obtain normal ranges for all age classes. Heart rate variability was measured by calculating mean RR interval over the length of the analysis, mean RR interval during quiet sleep, 5 time-domain (SDNN, SDNN-i, SDANN-i, r-MSSD, pNN50), and 4 frequency-domain (VLF, LF, HF, LF/HF ratio) indices. Our data show a significant positive correlation between all indices and the mean RR interval over the length of the analysis, except for the LF/HF ratio for which the correlation was binomial. A positive power correlation was also found between all parameters and age. The multiple correlation analysis confirmed the independent effect of age and mean RR interval on the heart rate variability. These data in a healthy pediatric population confirm a progressive maturation of the autonomic nervous system during childhood and may be utilized to examine the effects of underlying disease processes or therapeutic interventions on cardiac autonomic tone during infancy and childhood.

Circadian rhythm of heart rate and heart rate variability

BACKGROUND Measurements of heart rate variability (HRV) are increasingly used as markers of cardiac autonomic activity. AIM To examine circadian variation in heart rate and HRV in children. SUBJECTS A total of 57 healthy infants and children, aged 2 months to 15 years, underwent ambulatory 24 hour Holter recording. Monitoring was also performed on five teenagers with diabetes mellitus and subclinical vagal neuropathy in order to identify the origin of the circadian variation in HRV. METHODS The following variables were determined hourly: mean RR interval, four time domain (SDNN, SDNNi, rMSSD, and pNN50) and four frequency domain indices (very low, low and high frequency indices, low to high frequency ratio). A chronobiological analysis was made by cosinor method for each variable. RESULTS A significant circadian variation in heart rate and HRV was present from late infancy or early childhood, characterised by a rise during sleep, except for the low to high frequency ratio that increased during daytime. The appearance of these circadian rhythms was associated with sleep maturation. Time of peak variability did not depend on age. Circadian variation was normal in patients with diabetes mellitus. CONCLUSION We have identified a circadian rhythm of heart rate and HRV in infants and children. Our data confirm a progressive maturation of the autonomic nervous system and support the hypothesis that the organisation of sleep, associated with sympathetic withdrawal, is responsible for these rhythms.

Congenital heart disease in 111 225 births in Belgium: Birth prevalence, treatment and survival in the 21st century

Aim: To investigate the birth prevalence, treatment modalities and short‐term survival of children with congenital heart disease who were born in 2002.

Chest Pain in Pediatric Patients Presenting to an Emergency Department or to a Cardiac Clinic

The aim of this study was to assess the epidemiology of chest pain among unselected Belgian children referred to an emergency department or to a cardiology clinic. Material and methods - We analyzed the etiology to chest pain and the diagnostic workup of 168 consecutive pediatric patients, seen in our emergency department with the primary diagnosis of chest pain over a 5-year period (group A). Simultaneously a sample of 69 consecutive pediatric patients referred to the cardiology clinic by primary care physicians with the same chief complaint was prospectively included in the study (group B). Results - Chest wall pain was the most common diagnosis in the group A (64%). Other causes included pulmonary (13%), psychological (9%), cardiac (5%), traumatic (5%), and gastrointestinal problems (4%). The organic causes were easily identified or suspected by history and physical examination. Chest radiography, electrocardiography, and blood analysis were performed in most patients with suspected nonorganic chest pain but in no case were organic diseases diagnosed by those ancillary studies. In group B, chest wall pain was also the most common diagnosis (89%). Supraventricular tachyarrhythmia and exercise-induced asthma were demonstrated in 5 (7%) and 3 patients (4%), respectively. The most important tools in assessing a child with acute chest pain in an emergency department are thorough history and physical examination. Assessment of recurrent chest pain is more difficult; arrhythmia, and allergic and exercise-induced asthma may be underestimated when investigations are not performed.

Clinical and haemodynamic correlates of heart rate variability in children with congenital heart disease.

Abstract Heart rate variability (HRV) represents a noninvasive parameter for studying the autonomic control of the heart. Cardiac patients have a complex autonomic disturbance. The relation of HRV to this abnormality in children with congenital heart disease (CHD) has not yet been examined. The present study examined HRV indices from 24 h Holter recordings in 258 children with an operated or non-operated CHD, to determine their differences as an indicator of the severity of heart disease. The latter was defined clinically as New York Heart Association (NYHA) functional class I to IV and haemodynamically by invasive parameters. Five time-domain measures (SDNN, SDNNi, SDANNi, rMSSD and pNN50) and three frequency-domain measures (LF, HF and balance LF/HF) were compared with normal ranges. HRV was reduced in children with CHD, except in patients of NYHA class I. The level of reduction depended on the NYHA functional class. None of the measures was significantly related to haemodynamic data. Conclusion Heart rate variability is reduced in children with Congenital heart disease depending on the functional limitation but not on haemodynamic disturbances. Heart rate variability indices are sensitive markers of the clinical state.

Correlations between Indices of Heart Rate Variability in Healthy Children and Children with Congenital Heart Disease

Heart rate variability, as determined from 24-hour Holter recordings, represents a noninvasive parameter for studying the autonomic control of the heart. It decreases with certain disease states characterized by autonomic dysfunction such as congestive heart failure. No study in healthy or cardiac children has been performed to determine the correlations between and within time and frequency domain indices of heart rate variability. We examined five time domain (SDNN, SDNNi, SDANNi, rMSSD and pNN50) and five frequency domain measures (ULF, VLF, LF, HF and balance LF/HF) in 200 healthy children and 200 children with congenital heart disease, aged 3 days to 14 years. All measures were significantly correlated with each other. However, the strength of correlation varied greatly. Our data show that variables strongly dependent on vagal tone (rMSSD, pNN50 and HF) were highly correlated (r value > 0.90), as well as SDNN and SDANNi. We conclude that certain time and frequency domain indices correlate so strongly with each other that they can act as surrogates for each other.

Changes in Perceived Health of Children with Congenital Heart Disease After Attending a Special Sports Camp

Sports camps for children with cardiac anomalies have existed for many years. However, no formal evaluation of the benefits of attending such camps has been undertaken heretofore. We assessed potential changes in the self-perceived health of children with congenital heart disease who attended a special sports camp. Thirty-one children with cardiac anomalies attended a 3-day multisports camp. Sixteen children, all of whom were 10 years or older, literate, and Dutch- or French-speaking, completed the Child Health Questionnaire (CHQ-CF87) before and after attending the camp. The scores of the children were compared with those of healthy peers by calculating mean standardized differences. After attendance at the sports camp, the children achieved significant improvements in the self-perception of their physical functioning, role functioning due to emotional problems, role functioning due to behavioral problems, mental health, and general behavior. The children’s self-esteem and general behavior after the camp were significantly better than that of their healthy counterparts. We conclude that children with congenital heart disease who participate in activities at special sports camps may reap benefits in terms of their subjective health status. Although further research is needed, we recommend the participation in sport activities by children with heart defects, and more specifically their participation in sports camps.

Epidemiology of heart failure in a tertiary pediatric center

In contrast to the adult age group, epidemiologic studies on heart failure (HF) in the pediatric population are lacking. The aim of this prospective study was to analyze the epidemiology of HF during infancy and childhood.

Spectrum and frequency of illness presenting to a pediatric emergency department.

Abstract Background - Knowledge of the spectrum and relative frequencies of pediatric emergencies is an important factor in developing appropriate training curricula for pediatric residents. Material and methods - To provide these data, we indexed the 11,483 consecutive patients seen in our pediatric emergency department (PED) during the year 2003. Results - Age ranged from 1 week to 27 years, with a mean age of 3.9 ± 4.3 years. 52.7% of the visits were by children younger than 3 years, 9.5% by adolescents, and 0,1% by young adults with chronic conditions. 55.1% of the patients arrived on day shift, 32.5% on evening shift and 12.4% on night shift. 61.8% of the patients were seen during the evening/nighttime or on the weekends. 25.2%, 22.1% and 28.6% of the patients seen on night, day and evening shifts respectively were hospitalized. The most common chief complaints were fever (22.1%), upper respiratory tract infection (13.2%) and diarrhea (10%). The most common final diagnoses were upper respiratory tract infection (26.7%), viral syndrome (13.1%) and gastroenteritis (10.7%). The majority of chief complaints and final diagnoses were related to infection (63.9%). Conclusion - These data may contribute to curriculum development in training of PED physicians. We especially recommend an emphasis on management of fever and infections to optimize the quality of care delivered in the pediatric emergency department.