Henri Verhaaren

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.

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.

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.

Low-Dose Dobutamine Stress Echocardiography in Children and Young Adults

Dobutamine stress echocardiography has become widely accepted in the evaluation of adult patients with coronary heart disease. We wanted to assess the feasibility and the physiologic responses of stress echocardiography at low doses of dobutamine in a population of normal children and adults. Once achieved, we submitted a group of post-anthracycline patients to the test to assess the sensitivity of low-dose dobutamine stress echocardiography in the detection of cardiac dysfunction. Thirty-two healthy children and young adults (19 male and 13 female subjects, median age 15 years [range 6 to 26]) were studied. After the initial study of normal subjects, we submitted a cohort of 39 patients (18 female and 21 male, aged 6 to 25 years), who completed anthracycline chemotherapy, to the same protocol. Dobutamine was infused at rates of 0.5 to 2.5 and 5 microg/kg/min and echocardiographic measurements were obtained at rest and at the end of each stage. The test could be completed in 100% of the subjects without major complications. Statistically significant differences between resting echocardiographic values of systolic and diastolic function and values at 2.5 and 5 microg/kg/min of dobutamine were found. Moreover, dobutamine revealed or enhanced differences between normal subjects and the post-anthracycline patients. Thus, low-dose dobutamine stress echocardiography is feasible and safe in older children. The test is very sensitive for the detection of subclinical cardiac dysfunction in post-anthracycline patients and could possibly assess functional myocardial reserve.

Limitations of Doppler echocardiography for the post-operative evaluation of aortic coarctation

Doppler blood flow measurements and derived pressure differences, through the Bernoulli equation, are used in the diagnosis of aortic coarctation, a congenital stenosis distal to the left subclavian artery. Doppler velocities remain elevated at the coarctation site after successful repair of coarctation, leading to high Doppler derived pressure differences without significant arm-leg pressure differences. We studied this apparent contradiction of two diagnostic methods, in vivo using patient and control data, and in vitro using a hydraulic model. Clinical and echocardiographic data from 31 patients, aged 13.0 +/- 4.0, 10.5 +/- 4.7 yr after coarctectomy by end-to-end anastomosis, and 18 age-matched healthy subjects were reviewed. Doppler peak velocities at the aortic isthmus were elevated in patients (2.2 +/- 0.4 vs. 1.2 +/- 0.2m/s, P < 0.001), corresponding to significant Doppler differences (20 +/- 7 mmHg), however, without significant arm-leg pressure differences. In all patients, a mild anatomic stenosis could still be observed. Local stiffness was increased. The hypothesis that the less distensible surgical scar in post-coarctectomy patients leads to a significant dynamic obstruction in systole was validated in a latex model of the aorta. Rigid rings (0.5-1.5 cm), matching the unloaded aortic diameter, were mounted around the aorta. Under loading conditions, Doppler peak velocities increased by 40 +/-7%, yielding Doppler differences of 21 +/- 3 mmHg, without a significant pressure drop. An alternative expression to calculate pressure differences, using both velocity and geometric information, was validated in the model. In conclusion, post-operatively, Doppler velocities remain elevated due to a mild anatomical and significant dynamic narrowing, but the specific geometry, resembling a tubular hypoplasia rather than an abrupt stenosis, permits an almost complete pressure recovery explaining the occurrence of Doppler differences in disagreement with the negligible arm-leg pressure difference.

Lack of increase in stroke volume during exercise in asymptomatic adolescents in sinus rhythm after intraatrial repair for simple transposition of the great arteries

We studied stroke volume during exercise in 12 male asymptomatic patients in sinus rhythm after intraatrial repair for d-transposition of the great arteries. Data indicate that during exercise, stroke volume did not increase in the patient group, while in control subjects stroke volume increased.

Endocarditis from a dental focus. Importance of oral hygiene in valvar heart disease

Fastidiously growing bacteria more and more are recognised as a source of infectious endocarditis. Over recent years, three new cases of endocarditis caused by Actinobacillus actinomycetemcomitans were diagnosed in our institution. The rise in frequency is possibly secondary to better laboratory skills. Two patients with Actinobacillus (Haemophilus) actinomycetemcomitans endocarditis presented the classical history of preexisting valvar disease together with poor dental hygiene. The third patient had no congenital or rheumatic preexisting lesion to the valves. The distal part of a ventriculo-atrial drainage device had caused microtrauma to the tricuspid valve. The right-sided endocarditis in this patient was complicated by pulmonary septic emboli. Dental origin of the infection was very likely in this patient too. No dental procedure had been performed in the months preceding the endocarditis of our three patients. They presented endocarditis with an oral microorganism in the absence of any dental manipulation. All three had very poor dental hygiene. Better dental care could possibly have prevented this serious complication.

Survey of attitudes towards curriculum reforms among medical teachers in different socio-economic and cultural environments

Background: Curriculum reforms in medical schools require cultural and conceptual changes from the faculty. Aims and Methods: We assessed attitudes towards curriculum reforms in different academic, economic, and social environments among 776 teachers from 2 Western European medical schools (Belgium and Denmark) and 7 medical schools in 3 countries in post-communist transition (Croatia, Slovenia, Bosnia and Herzegovina). The survey included a 5-point Likert-type scale on attitudes towards reforms in general and towards reforms of medical curriculum (10 items each). Results: Teaching staff from medical schools in Bosnia and Herzegovina had a more positive attitude towards reforms of medical curriculum (mean score 36.8 out of maximum 50 [95% CI 36.1 to 37.3]) than those from medical schools in Croatia or Slovenia (30.7 [29.8 to 31.6]) or Western Europe (27.7 [27.1 to 28.3]) (P < 0.001, ANOVA). Significant predictors of positive attitudes towards medical curriculum reform in post-communist transition countries, but not in Western European schools, was younger age, as well as female gender in Bosnia and Herzegovina. Conclusions: Factors influencing faculty attitudes may not be easy to identify and may be specific for different settings. Their identification and management is necessary for producing sustainable curriculum reform.

Adenosine-tri-phosphate treatment for supraventricular tachycardia in infants.

Adenosine is an endogenous nucleoside acting on coronary perfusion and myocardial conduction. Although the anti-arrhythmic effects of adenosine have been known for decades, interest in the use of adenosine or adenosine-tri-phosphate (ATP) (a precursor of adenosine) in termination of supraventricular tachycardia (SVT) has been renewed. We studied the use of striadyne (ATP and a mixture of other nucleosides including adenosine) in 22 infants younger than 6 months in order to evaluate efficiency and safety of the drug in this particular age group. Striadyne stopped SVT in 17 cases and was diagnostic in another 4 cases. Ten out of 17 successfully converted infants showed one or more reinitiations of SVT, which were easily controlled. The results support the efficiency of ATP for the termination of re-entry types of tachycardia, as well as its diagnostic value and its lack of serious side-effects.There could be a role for adenosine or ATP for safe termination of most cases of SVT in infancy before transferring these infants to a specialised paediatric cardiology department.