Spectrum and workload of consultation activities in a Belgian tertiary paediatric cardiac centre.

BACKGROUND: Few data are available on the spectrum and frequency of issues addressed in the paediatric cardiology consultation service of tertiary academic hospitals. METHODS: Those activities were collected prospectively during 6 months. RESULTS: A total of 967 consultations were performed. The origin was mostly the medical ward (n = 535), the intensive care unit (n = 195), the neonatal unit (n = 97), the operating room (n = 84) and the nursery (n = 44). In 553 cases, a heart disease was previously known and the most common reasons of consultation were postoperative (n = 279) and preoperative evaluations (n = 129). Thirteen newborns had a prenatal suspicion of congenital heart disease, which was confirmed in 11 cases. For the other 401 consultations, the most common clinical concerns included cardiac function in oncological disease (n = 60), murmur (n = 48), syncope (n = 33), diabetes mellitus (n = 28), prematurity (n = 27), syndrome (n = 19), unexplained stridor or respiratory distress (n = 19) and unexplained fever (n = 15). There were new diagnoses of congenital heart disease, acquired heart disease and arrhythmias in 35, 17 and 5 cases, respectively. CONCLUSIONS: The workload of the paediatric cardiology consultation service is increasing alarmingly. These data may be helpful in future planning of consultant manpower and in curriculum development in cardiac training of students and residents.

Association of a Cor Triatriatum Sinister and a right partial anomalous pulmonary venous return. A case report.

A 56-day old baby girl referred for recurrent respiratory tract infections and stridor, showed a rare association of a Cor Triatriatum Sinister (CTS) and a right Partial Anomalous Pulmonary Venous Return (PAPVR), in the form of a stenosed Scimitar vein, draining the right pulmonary veins to the subdiaphragmatic inferior vena cava (IVC), on echography and angiography. The baby underwent successful surgical repair of the CTS, by resection of the obstructive false membrane and reconnection of the Scimitar vein to the left atrium. At the age of 5 years, the child is doing well without any evidence of obstruction on the right or the left pulmonary venous drainage.

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 (< or = 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.

Short-term outcome of isolated and associated congenital heart defects in relation to antenatal ultrasound screening.

OBJECTIVE: To investigate the outcome of fetuses affected by congenital heart defects (CHD), either detected or undetected at ultrasound screening, according to their complexity and severity. DESIGN: The study group comprised 3633 malformed fetuses entered into the Eurofetus database of which 798 had CHD. We compared the short-term outcome in cases where a CHD was detected by ultrasound screening with that in cases where a CHD was not detected. Isolated and associated CHD (ICHD and ACHD) and the degree of severity of defects were considered separately. Outcome data included termination of pregnancy (TOP), intrauterine fetal death, neonatal death (< 6 days after birth), gestational age at diagnosis and at delivery, mode of delivery and birth weight. RESULTS: Of the 798 fetuses with CHD, 595 had ICHD and 203 had ACHD. The diagnosis of an anomaly was made significantly earlier in ACHD cases. TOP was chosen in 28% of cases with a prenatal diagnosis of CHD, 20% for ICHD and 37% for ACHD (P < 0.001). The survival rate of antenatally diagnosed fetuses was lower in those with ACHD than in those with ICHD (P < 0.001) and lower for fetuses with antenatal diagnosis than with postnatal diagnosis (P < 0.001); this was due to significant differences in the complexity and severity of the defect. Premature delivery (< 32 weeks) was more frequent in fetuses in which an antenatal diagnosis of CHD had been made. Severe CHD were diagnosed earlier and were associated with a higher rate of TOP and spontaneous loss. CONCLUSIONS: The severity of CHD has a positive influence on the sensitivity of screening but a negative influence on the outcome. CHD that are not diagnosed antenatally include a high proportion of benign malformations. This explains the apparent paradox of a poorer outcome in fetuses in which a CHD was detected prenatally compared to those fetuses in which the defect was undetected prenatally. However, prenatal diagnosis provides parents with the option of TOP or of preventive care in specialized centers.

Sensitivity of ultrasound screening for congenital anomalies in unselected pregnancies.

OBJECTIVE: To assess the influence of heart defect frequency and severity on screening sensitivity of the entire spectrum of congenital anomalies (CA) and on detection rate of congenital heart defect (CHD) when performing routine ultrasound screening in unselected pregnant women. STUDY DESIGN: Secondary analysis was performed on data from Eurofetus, a large international collaborative study of ultrasound screening for CA in unselected populations. The present study addressed the relations (i) between the frequency of CHD in the screened population and the global sensitivity of ultrasound in detecting CA; (ii) between frequency of ventricular septum defect (VSD) and detection rate of CA and CHD; and (iii) between seriousness of CHD and CHD sensitivity. RESULTS: A negative correlation (p<0.001) between CHD frequency and CA sensitivity was observed in this study. Likewise, a negative correlation was also found between frequency of VSD and sensitivity for CA or CHD detection (p<0.001). In addition, a positive significant relationship (p<0.001) was observed between sensitivity and severity of CHD. CONCLUSION: A low CHD frequency introduces bias in CA and CHD sensitivity evaluation. Incomplete assessment of fetal and neonatal malformations brings on systematic error, leading to the pre-eminence of severe defects in the studied population sample and artificially improving sensitivity figures. On the other hand, from a clinician's viewpoint, the higher sensitivity for severe defect is welcomed, since their detection is most crucial for appropriate management.

Tracheobronchial compression of vascular origin. Review of experience in infants and children.

BACKGROUND: Tracheobronchial compression of vascular origin is an uncommon but important cause of respiratory distress in infants and children. We reviewed our surgical experience with 31 children, presenting vascular tracheobronchial compression. METHODS: Thirty-one children, with a median age of seven months, were operated on for airway and/or oesophageal compression secondary to vascular anomalies. Diagnostic findings, mainly established by bronchoscopy and angiography, revealed several pathologic disorders such as right aortic arch with aortic diverticulum in 10, double aortic arch in five, innominate artery compression in six, arteria lusoria in four, and pulmonary artery sling in one patient. Five children developed tracheobronchial compression after previous repair of a complex congenital heart disease. Clinical symptoms on admission were dominantly stridor, recurrent airway infection, dyspnea, respiratory distress and upper digestive complaints such as dysphagia and gastroesophageal reflux. RESULTS: Surgical relief was successful in 30 patients. Only one child with palliated tetralogy of Fallot and pulmonary artery sling died because of intractable extensive tracheobronchial stenosis. Late follow-up over three to 90 months showed a symptom-free evolution in most patients, including one reoperation for recurrent compression and one late death. CONCLUSIONS: Tracheobronchial compression by vascular structures in childhood is uncommon and may be masked by nonspecific respiratory symptoms, resulting in an often delayed diagnosis. Once imaging studies have clearly delineated the causal pathologic vascular structures, surgical correction is often straightforward and effective, in spite of the common presence of tracheobronchial malacia.

Cavopulmonary connection after repair of pulmonary vein stenoses.

We report a case of heterotaxia with single-ventricle physiology undergoing successively pulmonary artery banding (PAB) at 8 months of age, repair of pulmonary vein (PV) stenoses at 15 months of age, and a fenestrated total cavopulmonary connection (TCPC) combined with repair of a regurgitant common atrioventricular (AV) valve and a Damus-Stansel operation for a restrictive subaortic ventricle at 5 years of age, followed by percutaneous closure of the fenestration. The child is now 10 years old and is, to the best of our knowledge, the only reported long-term survivor of a Fontan type procedure done after repair of PV stenoses.

European Study on Maternal and Fetal Management of Fetal Supraventricular Tachyarrhythmia:Proposed Protocol for an International Project

> Objectives: This European protocol is designed to estimate the efficacy of transplacental digoxin administered intravenously to the mother in sustained fetal supraventricular tachyarrhythmias (FSVT) with or without cardiac failure and in intermittent runs of FSVT with cardiac failure. The natural history of FSVT is evaluated in self-limited runs of FSVT without cardiac failure. Study structure: The fetal criteria for inclusion in the study are: gestational age <35 weeks, a normal cardiac anatomy, no severe extracardiac anomalies and a heart rate of the FSVT >200 beats/min. The maternal criteria include no prior treatment with digoxin or drugs effecting fetal heart rate and hemodynamics, and no contraindication to digoxin. Doppler fetal echocardiography is utilized for the diagnosis of the type of FSVT, sequential evaluation, and assessment of fetal hemodynamics. Fetuses are assigned two groups: Group I includes all fetuses with sustained FSVT and those with intermittent runs and cardiac failure; Group II comprises fetuses with intermittent runs and no cardiac failure. Fetuses in Group I will be treated. Group II will receive no treatment initially and will be observed. Treatment is based on a acute loading phase with digoxin administered intravenously to the mother for a period of 7 days. Doses are adjusted to through levels of maternal digoxin, maternal clinical condition, and electrocardiogram. If sinus rhythm is obtained, a maintenance phase follows with oral digoxin. Another drug, according to local experience and preference, is added to oral digoxin if digoxin intravenously fails to restore sinus rhythm and gestational age is <36 weeks. Post natal outcome will be followed by sequential clinical and Holter examinations. The data collected will be reviewed in a blinded fashion. The collection of the data for the protocol requires extensive information on the maternal, fetal, and long-term follow-up studies. They are detailed in the Appendix section. Expected results: We propose to obstetricians and pediatric cardiologists involved in fetal cardiology a rationale for the management and treatment of FSVT. Our expectation is to obtain a controlled study on a large number of cases at an international scale and thus provide a homogeneous maternal and fetal therapeutic strategy for FSVT.

[Postoperative prognosis of congenital cardiac anomalies]. / Pronostic des cardiopathies congénitales opérées.

Congenital cardiac anomalies cannot be merely classified as "less", "more", and "very" complex. However, postoperative prognosis is also determined by: the wide variability in severity of certain anomalies; their frequent association with other cardiac or non-cardiac malformations; age at the time of surgery; the era, mode and quality of initial and subsequent treatment. Chosen examples will illustrate how recent progress has altered prognosis. The role of early, specific and multidisciplinary care in an appropriate center is emphasized.

Cardiac surgery with extracorporeal circulation in 23 infants weighing 2500 g or less: short and intermediate term outcome.

OBJECTIVE AND METHODS: From September 1990 to February 1997, 23 consecutive critically ill infants (12 males, 11 females) weighing 2500 g or less underwent cardiac surgery necessitating extracorporeal circulation (ECC). A retrospective study was carried out to evaluate short- and intermediate-term outcome. Mean weight at operation was 2265 g (range 1750-2500 g). Mean age at operation was 24 days. The indications for surgery were transposition of the great arteries (TGA; 7), ventricular septal defect (VSD; 4), aortic stenosis (AS; 3), univentricular heart (UVH; 2), tetralogy of Fallot (TOF; 2), interrupted aortic arch (IAA; 2), atrial septal defect (ASD; 1), atrioventicular septal defect (AVSD; 1) and total abnormal pulmonary venous return (TAPVR; 1). All patients were in NYHA class IV; 17 patients (74%) were intubated pre-operatively. RESULTS: The mean aortic cross-clamping time was 40 min. Twelve patients required deep hypothermia (<20 degrees C) with total circulatory arrest (mean duration 19 min). All patients were successfully weaned from extracorporeal circulation (ECC). Five patients left the operating room with an open sternum (mean duration before closure: 3.5 days). Mean duration of artificial ventilation was 10.6 days; of inotropic support 6.7 days and of intensive care stay 17.8 days. Severe complications were observed in 19 patients (83%): cardiac failure requiring high inotropic support (13), sepsis (7), and acute renal insufficiency (5). One patient needed a ventricular assist device. Five patients (22%) died in the intensive care unit (ICU): 2 AS with fibroelastosis, 2 IAA with VSD. and 1 UVH with pulmonary atresia. At discharge from the ICU, 7 patients were receiving no treatment. Mean duration of follow-up was 32 months (range 2-80 months). We had 2 reoperations: 1 for right ventricular outflow tract obstruction 1 year after a switch operation and 1 for mitral valve replacement 1 year after total abnormal pulmonary venous return repair (death 30 days post mitral valve replacement). Survival at I year was 73%. At the last clinical examination 16 patients were in NYHA class I. CONCLUSION: Despite the severity of pre-operative cardiac disease, early surgical repair with ECC in infants weighing 2500 g or less is feasible with tolerable mortality yet with significant early morbidity.

Combined repair of upper sternal cleft and tetralogy of Fallot in an infant.

We report on a 2-month-old infant with the unusual association of an upper sternal defect and tetralogy of Fallot. Surgical correction of the cardiac disease consisted of closing the ventricular septal defect and relief of the right ventricular outflow tract by infundibuloplasty. The already less compliant bony thorax of the infant made direct approximation of the upper sternal defect only possible with adjuvant bilateral chondrotomies. Sternal cleft repair is advised during the very first weeks of life, during which complete correction of the rarely associated tetralogy of Fallot can be successfully performed.

Nonvalved homografts of thoracic aorta in operation for complex congenital cardiac disease.

BACKGROUND: In contrast to the wide and successful use of valved aortic and pulmonary homografts, the non-valved prolongation of the thoracic aorta for the repair of some complex congenital heart diseases has rarely been described. We present here our experience with the use of descending aorta and aortic arch homografts as non-valved conduits for the surgical repair of complex cardiac malformations in 8 patients. METHODS: One atriopulmonary conduit replacement four extraatrial cavopulmonary connections, and one intraatrial cavopulmonary repair were achieved by means of a homograft of the descending aorta with a diameter of 15 to 17 mm. Three pulmonary unifocalization procedures were carried out in 2 more patients using a curved homograft of the aortic arch with a diameter of 18 mm. Except for 1 patient, whose incompetent common atrioventricular valve was replaced with a mechanical prosthesis, all other patients were managed without anticoagulation. RESULTS: No conduit-related complications were seen during a follow-up of 18 to 42 months. One patient died perioperatively during an emergency central repair of the right ventricular outflow tract after bilateral unifocalization with arch homografts. Another patient died suddenly 4 months after cavopulmonary connection. The remaining 6 patients are currently doing well. CONCLUSIONS: Regardless of the complexity of the underlying malformations, we are encouraged to use nonvalved thoracic aortic homografts in the repair of congenital cardiac diseases because of the reduction in thrombotic, hemorrhagic, and infectious complications associated with their use; convenience in handling; and their versatility in size and shape.

[Fetal cardiology: prenatal diagnosis of cardiac malformations]. / Cardiologie foetale: le diagnostic anténatal des malformations cardiaques.

Foetal cardiology is now a recognised chapter of both paediatric cardiology and foetal medicine. We describe here our experience with antenatal diagnosis of cardiac malformations and discuss both its genetic and epidemiological issues. Our experience confirms if need be that foetal medicine requires a well organised first-level foetal evaluation.

Total anomalous pulmonary venous connection. Results of a small consecutive series.

The surgical experience in 13 infants with total anomalous pulmonary venous connection (TAPVC) between 1987 and 1991 is reviewed. The age vary from 2 days to 35 months with a weight at intervention from 2.130 kg to 5.400 kg. The types of TAPVC were supracardiac in 4 patients, cardiac in 4, and infracardiac in 5. Seven patients (54%) were operated on in emergency. Cardiopulmonary bypass consisted of profound hypothermia and total circulatory arrest in 8 patients (60%) and continuous hypothermic bypass with low flow for the remaining 5 patients (40%). There was no operative death. The follow-up ranges from 21 to 58 months, mean 40 months. There was one reoperation. All the patients were asymptomatic and the height growth percentile is less than 5% in 20%, and the weight growth percentile is less than 5% in 30%. The early repair of infants born with TAPVC can be done with low morbidity with a good prospect on medium term follow-up.

Evaluation of membranous subaortic stenosis using biplane transesophageal echocardiography. Report of two cases.

A biplane transesophageal echocardiography was performed in two young adult patients with discrete subvalvular aortic stenosis. The combined information obtained in the transverse and in the longitudinal plane allows in these instances an accurate diagnosis and the choice of an optimal approach.

[Late results of surgical repairs of tetralogy of Fallot]. / Résultats tardifs des cures de tétralogie de Fallot.

The late results of 115 children with tetralogy of Fallot born and corrected between 1969 and 1988 are analyzed; the patients were distributed into 3 anatomical groups: normal pulmonary annulus (group A, 49 patients) annular stenosis (group B, 56 patients) and pulmonary atresia (group C, 10 patients). The probability of survival 15 years after correction is 97 p. 100 in cumulated groups A and B and 72 p. 100 in group C (p = 0.005). The probability of reoperation ten years after correction is 4 p. 100 in cumulated groups A and B without a previous shunt, 20 p. 100 in A and B with a previous shunt (p less than 0.05) and 55 p. 100 in group C (p = 0.0004). The proportion of serious cardiac sequellae amounts to 17 p. 100 in group A, 55 p. 100 in group B and 100 p. 100 in group C (p = 0.0005). As a whole, 55 p. 100 of the adolescents operated on for tetralogy of Fallot during infancy present a residual problem of some severity.

Neonatal tuberous sclerosis. US, CT, and MR diagnosis of brain and cardiac lesions.

Cortical, subcortical and subependymal tubers were more completely and more clearly depicted by MR than by CT and US in a 3-week-old infant. These lesions were best shown on T1-weighted images as areas of high signal intensity.

Unusual ventilation-perfusion mismatch in partial anomalous venous return.

A 3-year-old girl with a complicated partial anomalous pulmonary venous return presented a scintigraphic pattern characterized by an absence of perfusion of the right lung, contrasting with normal ventilation.

Lung function in children and adolescents with antecedents of acute rheumatic fever.

Static lung volumes, CO-lung transfer, airway resistance, maximal expiratory flows and lung elastic properties were studied in 29 children and adolescents 1-10 years after recovery from acute rheumatic fever. There were essentially no changes in lung function even in the subjects with a residual valvular disease. The only abnormality was a tendency for the elastic lung recoil at TLC to be low, which is interpreted as probably reflecting a decrease in inspiratory muscle force.