Daniël De Wolf

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.

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.

Phosphorylcholine coating of extracorporeal circuits provides natural protection against blood activation by the material surface

OBJECTIVE The aim of this study is to evaluate the use of a new coating, mimicking the outer cell membrane, in paediatric cardiac surgery. METHODS Two groups of ten patients with a body weight below 8 kg, undergoing elective cardiac operations for different congenital anomalies, were prospectively enrolled in this study. In one group the whole extracorporeal circuit, including the cannulas, was coated with phosphorylcholine (PC). In the second group the same circuit was used without coating. Platelet activation (thromboxane B2 (TXB2), beta-thromboglobulin (betaTG)), activation of the coagulation system (F1+2), leukocyte activation (CD11b/CD18) and terminal complement activation (TCC) were analyzed pre-cardiopulmonary bypass (CPB), at 15, 60 min of CPB, at the end of CPB, 20 min post CPB and at postoperative day 1 and 6. RESULTS No statistical differences were found for F1+2 and CD11b/CD18. After onset of CPB mean levels of TCC remained stable in the PC group whereas an increase was observed in the control group. During CPB betaTG values in both groups increased to a maximum at the end of CPB. Within groups the increase in betaTG levels during CPB was statistically significant (P<0.05) from baseline in the control group starting from 60 min of CPB whereas no statistical difference was observed in the PC group. After the start of CPB TXB2 mean levels increased to 405+/-249 pg/ml in the PC group vs. 535+/-224 pg/ml in the control group. After this initial increase there was a small decline in the PC group with further increase. This was in contrast to the control group were TXB2 levels further increased up to a mean of 718+/-333 pg/ml at the end of CPB (P=0.016). CONCLUSIONS Phosphorylcholine coating had a favourable effect on blood platelets, which is most obvious after studying the changes during cardiopulmonary bypass. A steady increase of TXB2 and betaTG was observed in the control group, whereas plateau formation was observed in the phosphorylcholine group. Clinically, this effect may contribute to reduced blood loss and less thromboembolic complications. Complement activation is lower in the coated group.

Dobutamine stress echocardiography in the evaluation of late anthracycline cardiotoxicity in childhood cancer survivors

Late anthracycline cardiotoxicity has been of increasing concern to pediatric oncologists. An increasing number of patients with cardiac dysfunction has been reported without a good correlation between cardiac function or symptoms and routine echocardiographic follow-up. We studied dobutamine stress echocardiography in patients who had received moderate doses of anthracyclines years before. Twenty-three patients (14 male, 9 female; 7-25 y) who completed chemotherapy with moderate doses of anthracyclines (180-380 mg/m2) more than 2 y previously underwent dobutamine stress echocardiography and were compared with a control group of 26 healthy young people (15 male, 11 female; 6-26 y) matched for age and weight. Dobutamine was administered in three periods up to a rate of 5 μg/kg/min. Eighty-five percent of the patients showed an abnormal response to dobutamine. Both systolic and diastolic functions were affected. The systolic dysfunction was not related to diminished contractility but to an elevated systolic wall stress due to inadequate cardiac muscle thickening. The diminished wall thickening was related to the length of follow-up. Dobutamine proved to be a very sensitive method to detect clinical and subclinical cardiac dysfunction in patients post anthracycline chemotherapy and questions the concept of a safe dose.

Congenital Hypothyroid Goiter and Amiodarone

ABSTRACT. Amiodarone is an anti‐arrhythmic drug with a content of 39% Iodine. No adverse effects on fetal thyroid function have previously been observed with maternal ingestion of Amiodarone during pregnancy. A case of severe congenital hypothyroidism with goiter, associated with maternal ingestion of 200 mg Amiodarone daily from the 13th week of pregnancy, is described here. No other environmental causes of goiter, nor a congenital organic thyroid disorder could be demonstrated.

Neurocognitive Consequences of Surgically Corrected Congenital Heart Defects: A Review

With advances in surgical procedures, neuropsychological assessment after congenital heart defects and pre, peri- and/or postoperative predictors of adverse outcome has become an important focus in research. We aim to summarize neuropsychological sequelae associated with different types of congenital heart defects, critically review the methodology used in more than 20 empirical studies that were retrieved from biomedical electronic search engines, and identify possible directions for future research. Despite the lack of adequate control groups and long-term studies, there seem to be some cognitive deficits. The largest group of children with isolated congenital heart defects present with normal intellectual capacities. However, they tend to show language deficits and motor dysfunction. Although performances on memory tasks are good, unambiguous conclusions concerning their attentional and executive functioning are still lacking. Serious behavioral problems are not an issue. In addition to a detailed description of the (neuro) psychological consequences of pediatric cardiac surgery, an overview of the predictors of the cognitive defects is provided.

Reference Values for Echocardiographic Assessment of the Diameter of the Aortic Root and Ascending Aorta Spanning All Age Categories

Thoracic aortic dilatation requires accurate and timely detection to prevent progression to thoracic aortic aneurysm and aortic dissection. The detection of thoracic aortic dilatation necessitates the availability of cut-off values for normal aortic diameters. Tools to evaluate aortic dimension above the root are scarce and inconsistent regarding age groups. The aim of this study was to provide reference values for aortic root and ascending aortic diameters on the basis of transthoracic echocardiographic measurements in a large cohort of children and adults. Diameters at the level of the sinuses of Valsalva (SoV) and ascending aorta (AA) were assessed with transthoracic echocardiography in 849 subjects (453 females, age range 1 to 85 years, mean 40.1 ± 21.3 years) and measured according to published guidelines. Linear regression analysis was applied to create nomograms, as well as equations for upper limits of normal and z-scores. SoV and AA diameters were strongly correlated with age, body surface area (BSA), and weight (r = 0.67 to 0.79, p <0.001 for all). Male subjects had significantly larger aortic dimensions at all levels in adulthood, even after BSA correction (p ≤0.004 for all age intervals). Gender-, age-, and BSA-specific upper limits of normal and z-score equations were developed from a multivariate regression model, which strongly predicts SoV and AA diameters (adjusted R(2) for SoV = 0.84 and 0.67 and for AA = 0.82 and 0.74, for male and female subjects, respectively). In conclusion, this study provides widely applicable reference values for thoracic aortic dilatation screening purposes. Age, BSA, and gender must be taken into account when assessing an individual patient.

Propofol versus remifentanil for monitored anaesthesia care during colonoscopy

Background and objective: We conducted an open, prospective, randomized study to compare the efficacy, safety and recovery characteristics of remifentanil or propofol during monitored anaesthesia care in patients undergoing colonoscopy. Methods: Forty patients were randomly assigned to receive either propofol (1 mg kg−1 followed by 10 mg kg−1h−1, n = 20) or remifentanil (0.5 μg kg−1 followed by 0.2 μg kg−1 min−1, n = 20). The infusion rate was subsequently adapted to clinical needs. Results: In the propofol group, arterial pressure and heart rate decreased significantly from the baseline. These variables remained unchanged in the remifentanil group, but hypoventilation occurred in 55% of patients. Early recovery was delayed in the propofol group (P < 0.002). Recovery of cognitive and psychomotor functions was faster in the remifentanil group. Fifteen minutes after anaesthesia, the Digit Symbol Substitution Test score was 28.6 ± 12.8 versus 36.2 ± 9.4 and the Trieger Dot Test score was 25.6 ± 8.1 versus 18.7 ± 4.1 in the propofol and remifentanil groups, respectively (both P < 0.05). Patient satisfaction, using a visual analogue scale, was higher in the propofol group (96 ± 7 versus 77 ± 21, P < 0.001). Conclusions: Remifentanil proved efficient in reducing pain during colonoscopy. Emergence times were shorter and the recovery of cognitive function was faster with remifentanil compared with propofol. Remifentanil provided a smoother haemodynamic profile than propofol; however, the frequent occurrence of remifentanil‐induced hypoventilation requires the cautious administration of this agent.

Left ventricular outflow obstruction in rhabdomyoma of infancy: meta-analysis of the literature.

Primary heart tumors are exceptional in infants and children. Most common is the rhabdomyoma, often associated with tuberous sclerosis (Bournevilles disease). This tumor is generally believed to have no hemodynamic effects in the majority of cases. Recently, severe obstruction of the left ventricular outflow tract by a solitary tumor was diagnosed during pregnancy and emergency surgery was needed soon after birth.

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.