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In emergency cases of babies with a single ventricle and an obstruction to aortic outflow, low pulmonary flow or, on the contrary, high pulmonary flow, only palliative surgical procedures can be proposed. The authors set out to determine the prognosis of a population with this type of lesion having undergone one of the three following procedures: systemic pulmonary shunt, pulmonary artery banding, repair of the aortic arch (usually associated with pulmonary banding). One hundred and nineteen (63%) of the 185 patients hospitalised between 1/01/1970 and 31/12/1991 in the paediatric cardiology unit of the Cardiac Hospital of Lyon with a diagnosis of single ventricle, underwent one of these three procedures as a treatment of first intention. The survival of the 22 patients who underwent pulmonary artery banding (90 +/- 6%, 85 +/- 8%, 85 +/- 8% at 1.5 and 10 years respectively) was significantly better than that of the patients undergoing systemico-pulmonary shunt (63 +/- 6%, 53 +/- 6% and 49 +/- 6% at 1.5 and 10 years respectively). On the other hand, repair of an obstacle of the aortic arch was a precarious procedure as the survival was only 23 +/- 11%, 16 +/- 11% and 16 +/- 11% and 1.5 and 10 years respectively). These results suggest, with the reserve inherent to the methodology of retrospective studies of small populations, that it is not illogical to continue to propose pulmonary artery banding for babies with single ventricle associated with high pulmonary flow. This procedure should only be envisaged after strict selection of candidates and providing there are facilities for intensive postoperative care.(ABSTRACT TRUNCATED AT 250 WORDS)

The authors analysed the anatomical and clinical prognostic factors of single or common ventricle. This retrospective study was based on a series of 185 patients hospitalised between 1/2/70 and 31/12/91 in the paediatric cardiological unit of the Cardiological Hospital of Lyon with this condition. A number of anatomical and clinical parameters were identified in this population. For each parameter, a given patient could only relate to single modality and a survival graph determined by Kaplan-Meier analysis was established for each modality. For each variable, the survival curve of the most frequently encountered modality served as a reference and the other modalities were compared with it using a logrank test. The different modalities potentially related to patient survival were then entered into a multivariable model using logistic regression. The results of this study of multivariable analysis using the Odds-Ratio (OR) independently suggest that four variables may influence negatively survival of patients with a single ventricle: pH < or = 7.3 on admission (OR = 3.55), a non-left ventricular morphology of the main ventricular chamber (OR = 3.11), the presence of an obstacle on the aortic outflow (OR = 5.58) and a total anomalous pulmonary venous drainage (OR = 26.88).

The authors undertook a retrospective study of 69 cases of infective endocarditis (IE) in 68 children treated from 1971 to 1992. The comparison between two groups (Group I comprising 34 patients treated between 1971 and 1981; Group II comprising 34 patients treated between 1982 and 1992) based on a review of the literature showed that the natural history of paediatric IE has changed during these two decades: a slight increase in the incidence in young children. The sequellae of rheumatic heart disease play no role in determining IE in France. Congenital heart disease plays a major role (72% of cases) with increasing numbers having undergone surgical treatment for more complex lesions. Mitral valve prolapse has become a more common cause with multiple portals of entry, predominantly buccal and oto-rhino-laryngeal. Blood cultures are positive in 75% of cases, the commonest organisms being Streptococci and Staphylococci, but the frequency of uncommon pathogens is increasing. Echocardiography plays a major role in the diagnosis and inventory of IE (vegetations demonstrated in 64% of cases in Group II). Although mortality is progressively decreasing (3% in Group II) because of more frequent surgical indications (32% in Group II) and more severe sequellae: only 27% of children in Group II were cured without sequellae or aggravation of their previous cardiac lesion.

The poor results of palliative surgery in the hypoplastic left heart syndrome has led Bailey to propose neonatal cardiac transplantation for this condition. The authors report the case of a neonate who underwent transplantation 2 days after birth for hypoplastic left heart with mitro-aortic atresia, diagnosed in utero. The child was prescribed triple immunosuppressive therapy (steroids, azathioprine, ciclosporine) and is now 24 months old. An episode of acute rejection during the first month was treated with a short intensive course of intravenous steroids. At the fourth month, the child underwent aortic angioplasty for an isthmic stenosis. Growth is retarded (- 2SD) and he has mild renal failure but psychomotor development has been normal. The number of neonatal cardiac transplantations remains limited by parental refusal and the lack of donor organs. Despite encouraging medium term results, questions remain as to the long-term viability of the graft and the patient's renal function.

Children with complex cardiac anomalies do not always receive corrective surgery and remain susceptible to serious events, especially infectious endocarditis, brain abscesses, anoxic spells and ischaemic cerebral accidents. Many of these complications could be avoided by careful management.

Based on 11 personal cases a large review of the literature (188 cases), the authors studied the frequency and types of cardiac anomalies in Di George syndrome. A cardiac anomaly was found in 86% of published cases. Interrupted aortic arch (27%), truncus arteriosus (27%) and tetralogy of Fallot (22%) are the most frequent anomalies. Interrupted aortic arch and truncus arteriosus, being rare congenital heart diseases, their discovery in a neonate must lead to a systematic search for Di George syndrome.

Fifty infants with isolated total anomalous pulmonary venous connection (TAPVC) were operated between 1/01/73 and 31/12/87. The average weight at surgery, which was performed under hypothermia with circulatory arrest in 92 per cent of cases, was 4.5 Kg. The preoperative pulmonary to systemic pressure ratios (PAP/PS) enabled identification of two groups of patients: Group I: TAPVC without severe pulmonary hypertension (PAP/PS less than 0.85) (n = 35), and Group II: TAPVC with severe pulmonary hypertension (PAP/PS greater than 0.55) (n = 15). The hospital mortality was 22 per cent (8 cases) in Group I compared with 73 per cent (11 cases) in Group II (p less than 0.05). Patients in Group II were younger (64 days compared with 137 days, p less than 0.02), lighter (p less than 0.05) and had preoperative mean pulmonary artery systolic pressures of 83 mmHg (p greater than 0.001). Three patients in Group I required early reoperation for stenosis of the pulmonary veins at the site of repair resulting in pulmonary hypertension, and all died. The global survival was 28 patients with an average follow-up of 7 years (range 1 to 15 years). Six of these patients were reoperated (2 phrenoplications, 4 atrial shunts). All survivors are asymptomatic and have no conduction defects. Control echocardiography in 15 of the 28 survivors was judged to be normal. These results show that obstructive forms of TAPVC (Group II) carry a very poor prognosis: immediate results in this group could only be improved by earlier surgery. The clinical long-term results in those who survive surgery are very satisfactory.

Prostaglandin E1 (PGE1) was administered to increase ductus patency in 26 neonates who present a cyanotic heart disease with right ventricular obstruction (1st group, 13 cases) or congestive heart failure with left ventricular outflow obstruction (2nd group, 13 cases). Clinical improvement occurred in all but the oldest infant in the first group. The efficacy of PGE1 is inconstant in the second group, but medical status was very poor before treatment. We could usually reduce the dose of PGE1 but in no case we could stop the drug before surgery. Surgery could be delayed several hours or days to get an hemodynamic and biologic satisfactory condition and even a few weeks until the infants and their pulmonary arteries had grown (1st group). Side effects are common but relatively minor.

Forty five patients, 2 months to 42 years of age, had valved tubes implanted for ventriculo-pulmonary discontinuity. The underlying malformations were: certain forms of Fallot's tetralogy (16 cases), transposition of the great arteries with ventricular septal defect and pulmonary stenosis (8 cases), truncus arteriosus (9 cases), double outlet right ventricle (6 cases), other malformations (4 cases). Hospital mortality (at 30 days) was 31% (14 cases). This was mainly due to technical difficulties related to the large number of palliative procedures and to irreversible pulmonary hypertension, present in 43% of cases with fatal outcomes. Thirty one patients were followed up for 6 months to 7 years (mean 3 +/- 1,6 years). There were 3 late deaths, one during reoperation for a residual shunt. All survivors underwent clinical assessment with hemodynamic control in 17 cases (15 routine control catheterisations). To date it has only been necessary to change one valved tube: a good result was obtained.

Approximately 50% of patients with Trisomy 21 have congenital heart lesions and they account for 2.7% of the admissions to the Pediatric Cardiac Unit of the Cardiac Hospital of Lyons. In a series of 91 cases observed over a 10 year period (1070-1980) undergoing hemodynamic and angiographic investigation, 9 out of 10 cases - as previously reported - had the following abnormalities: endocardial cushion defect, 42.7% (39% of which were complete atrioventricular canals), ventricular septal defect, 33%, and Fallot's tetralogy, 12%. These lesions were characterised by the high incidence and precocity of pulmonary hypertension (69 out of 72 cases with a left-to-right shunt . A comparison of the hemodynamic data of children with Trisomy 21 with ventricular septal defects, and children without Trisomy 21 of the same age with ventricular septal defect showed the pulmonary arterial resistances to be significantly higher in the group with Trisomy 21, suggesting that Trisomy 21 plays a role in the pathogenesis of severe early pulmonary hypertension. The operative risk is much higher in these patients, especially with openheart surgery (4 operative deaths out of 12 cases of closure of VSD). The surgical indications are difficult to define because of the context of malformation and mental retardation, the high incidence of pulmonary hypertension and the high opertive risk requiring a large and detailed dialogue with the family.

The findings on echocardiography and their anatomical correlations are reported in a group of 15 neonates with the syndrome of hypoplasia of the left side of the heart. The lesions which make up this syndrome could be defined precisely using echocardiography. In the major forms of this syndrome (10 cases), the correlation between the clinical and the echocardiographical findings was sufficient to establish the diagnosis, and to avoid the necessity for cardiac catheterisation without prejudice to the treatment plan. Characteristic findings in the major types were: --an aortic diameter less than or equal to 5 mm; --a left ventricle which was absent or had a diameter of less than 11 mm with an LV/RV ratio of less than 0.6; --a mitral valve which was absent or had a very abnormal form with multiple echoes. The differential diagnosis on echocardiography and the limitations of the method are discussed.