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.

Blood volume (51Cr) in severe protein-calorie malnutrition.

The intravascular volumes were determined in 42 protein-calorie-depleted Shi children, and in 14 healthy patients. The red cell volume (RCV) was determined by a radiochromium technique; the total blood volume (TBV), the plasma volume (PV), and the total circulating albumin (TCA) were derived. The values were related to weight without edema, to expected weight for height, and to body surface area. Related values were averaged and were correlated with both the weight deviation and the plasma albumin level. Figures for healthy Shi children were comparable with American normal standards. Malnourished children were hypovolemic relative to their body size measurements; low TBV was the consequence of an absolute decrease in RCV and of a possible reduction of PV; TCA was lowered in the same proportion as albuminemia. RCV and TBV correlated well with albuminemia, but did not correlate with the weight deviation; the relationship between PV and albuminemia was not linear. Despite some quantitative discrepancies, similar results were obtained with each kind of related values. In a discussion of the results, stress is laid on risks of misinterpretation resulting from the use of anthropometric parameters as a basis of reference when studying intravascular volumes in marasmic kwashiorkor.

Hemodynamic findings during treatment of protein-calorie malnutrition.

Several hemodynamic parameters were sequentially determined at rest in 19 African children recovering from marasmic kwashiorkor. Before treatment, the red cell volume averaged 160 ml, the circulation time: 6.6 sec, the cardiac output: 1.00 liter/min, the stroke volume: 9.7 ml/beat, the systemic vascular resistances: 79 units and the arteriovenous differences in blood oxygen content 5.1 volume %. On the 20th realimentation day, red cell volume averaged 169 ml, the circulation time: 5.8 sec, the cardiac output: 1.38 liter/min, the stroke volume: 11.4 ml/beat, systemic vascular resistances: 62 units and arteriovenous difference: 4.2 volume %. On the 60th realimentation day, red cell volume averaged 208 ml, circulation time: 5.6 sec, cardiac output: 1.53 liter/min, stroke volume: 11.8 ml/beat, systemic vascular resistances: 56 units and arteriovenous differences: 4.1 volume%; both red cell volume and stroke volume were still significantly lower than in the control group. The children recovering from marasmic kwashiorkor showed a rapid transition from a hypocirculatory state into a near normal circulatory state with delayed increase in both red cell volume and stroke volume.

Hemodynamic findings in servere protein-calorie malnutrition.

This study was undertaken because circulatory distrubances had been advanced as a possible cause of death during initial renourishing of protein-calorie deprived children. Body weight, plasma albumin concentration, intravascular volumes (radiochromium), cardiac index (dye dilution technique), intravascular pressures (flow-guided catheterization), and related hemodynamic parameters were determined at rest in 43 infection-free African children with a form of protein-calorie malnutrition known as marasmic kwashiokor, and were compared with values observed in 24 convalescent children. The malnourished children showed a prolonged circulation time with a tendency to bradycardia and hypotension; cardiac index, stroke index, and heart work were significantly reduced, as were the intravascular volumes. Hemodynamic data correlated with either body weight or plasma albumin and cardiac index bore a direct relation to red cell volume. In the most severely malnourished subjects, ventricle filling pressures were low and vascular resistances were high. It is inferred that most patients were in an adaptive hypocirculatory state comparable to hypothroidism, while the most severely malnourished children showed frank peripheral circulatory failure comparable to hypovolemic shock. Circulatory failure on admission was associated with high death rate during treatment but the relation between cause and effect could not be clearly demonstrated.

Blood volume changes during treatment of protein-calorie malnutrition.

Intravascular volmes were sequentially determined in 13 African children recovering from protein-calorie malnutrition. The red cell volume was measured by a radiochromium technique; the total blood volume and the plasma volume were calculated. The absolute volumes were related to body size and were compared with the control values. Before treatment, the total blood volume was low per unit of body size, the red cell volume was more reduced than the plasma volume. On the 20th realimentation day, the absolute red cell volume had increased less than the absolute plasma volume, the total blood volume was almost normal per unti of body size. On the 60th realimentation day, near recovery, the absolute red cell volume had again increased, but again less than the absolute plasma volume, and the venous hematocrit was lower than on admission; the total blood volume was normalized per unit of body size, the red cell volume was low with a supranormal plasma volume. The rehabilitation period was characterized by an aggravation of the hemodilution already present before treatment. The significance of the changes in volemia per unit of body size was unclear because of the changing body composition.

Acquired pulmonary artery aneurysm in an infant.

An aneurysm of the left pulmonary artery developed in an infant during a staphylococcal generalized infection. The successful surgical treatment required a left pneumonectomy. In this report the causes of that rare lesion and the prognosis of pneumonectomy in infancy are reviewed.

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.

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.

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.

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.

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.

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.

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.

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.

[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.

[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.

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.

Incidence and prognosis of rheumatic carditis in a Belgian hospital. A 10-yr review (1965-1974).

106 cases of first attack of acute rheumatic fever were recorded during a decade (ending in 1974) in a Brussels pediatric clinic. 56 patients were Belgian and 50 were children of immigrants. The incidence of acute carditis was 23% among the former, 46% among the latter (P less than or equal to 0.01), and 34% among the whole group. The frequency of residual heart disease after a 3-yr antistreptococcal prophylaxis fell to 20% among the whole group. The difference in morbidity observed between Belgian children and children of immigrants calls for a prospective epidemiological study.