Clinical course and outcome of antenatally detected atrioventricular block: experience of a single tertiary centre and review of the literature.

OBJECTIVE: The objective is to study the course and outcome of fetuses with congenital atrioventricular block (AVB) in a single centre. METHODS: Retrospective analysis of cases diagnosed prenatally with second and third degree AVB. The clinical characteristics and outcome of fetal AVB were evaluated including in utero treatment. RESULTS: Sixty-two cases were studied. AVB was associated with a congenital heart defect (CHD-AVB) in 17 cases (27%), whereas it was isolated (i-AVB) in 45 (73%), 42 of which were associated with maternal antibodies. There were nine (52.9%) live births in the CHD-AVB group, five of which (55%) resulted in infant deaths. In the i-AVB group, there were 40/45 (88.9%) live births and 1/40 (2.5%) infant death; 36 (90%) babies required a permanent pacemaker. The only factor predictive of postnatal death was the presence of CHD (5/9 vs 1/39 or 48.7 [3.6; 1457.7], p < 0.001). Nineteen fetuses (40.5%) with i-AVB received steroids in utero. No difference in outcome was found between the AVB treated in utero versus the no-treatment group in terms of permanent pacemaker placement, postnatal death or development of dilated cardiomyopathy. CONCLUSION: The most important prognostic factor for congenital AVB is the association with CHD. In utero treatment remains questionable.

[Prognosis of isolated atrioventricular block in children. Single center study of 135 cases]. / Pronostic des blocs auriculo-ventriculaires isolés de l'enfant, étude monocentrique de 135 cas.

INTRODUCTION: Isolated complete atrio-ventricular (AV) block in children occurs in anatomically normal hearts in the absence of any known predisposing factor likely to have caused the block. This study aimed to define the current prognosis of these conduction disorders. METHODS: 135 patients with isolated complete AV block, diagnosed before the age of 15 years, were included in this monocentric, retrospective study. RESULTS: 52.6% of the cases were diagnosed in the antenatal or neonatal period, demonstrating their congenital nature. The disease was heralded by symptoms in only 6.7% of cases. A search for maternal anti-Ro/La antibodies was performed in 111 of the patients. It was positive in 56 cases, and was associated with an early diagnosis, a short delay in cardiac pacemaker implantation, and the occurrence of cardiomyopathy (16 cases), the latter representing a critical point in the progression of the disease (37.5% mortality at 6 years). Among the 55 cases not associated with antibodies, 2 cases were familial and no etiology was discovered in the others. The diagnosis was therefore made later, and no progression to cardiomyopathy was noted. A cardiac pacemaker was implanted in 122 patients (66 epicardial, 56 endocardial). CONCLUSIONS: Isolated complete AV block in children is not a homogenous entity: 'immunological' blocks are genuinely congenital and their prognosis remains grave, owing to the risk of cardiomyopathy, which is sometimes diagnosed late. The other types of block are diagnosed much later in childhood, and their mechanism is still unknown. They have a good prognosis as long as the indications for pacing the child are respected.

[The Jervell and Lange-Nielsen syndrome. Natural history, molecular basis and clinical outcome]. / Syndrome de Jervell et Lange-Nielsen. Histoire naturelle, bases moléculaires et devenir clinique.

UNLABELLED: Data on the Jervell and Lange-Nielsen syndrome (JLN), the long QT syndrome (LQTS) variant associated with deafness and caused by homozygous or compound heterozygous mutations on the KCNQ1 or on the KCNE1 genes encoding the IKs current, are still largely based on case reports. We analyzed data from 186 JLN patients obtained from the literature (31%) and from individual physicians (69%). Most patients (86%) had cardiac events and 50% were symptomatic already by age 3. Their QTc was markedly prolonged (557 +/- 65 ms). Most of the arrhythmic events (95%) were triggered by emotions or exercise. Females are at lower risk for cardiac arrest and sudden death (CA/SD). A QTc>550 ms and history of syncope during the first year of life are independent predictors of subsequent CA/SD. Most mutations (90.5%) are on the KCNQ1 gene; mutations on the KCNE1 gene are associated with a more benign course. beta-blockers have only partial efficacy as 51% of the patients had events despite therapy and 29% had CA/SD. CONCLUSIONS: JLN syndrome is a most severe variant of LQTS, with a very early onset, major QTc prolongation, and is not well responsive to beta-blockers. Subgroups at relatively lower risk for CA/SD are identifiable and include females, patients with a QTc pound550 ms, without events in the first year of life, and with mutations on KCNE1. Early therapy with ICDs has to be considered.

[Transcatheter exclusion of a giant aneurysm of the left pulmonary artery using covered stents]. / Exclusion d'un anévrisme géant de l'artère pulmonaire gauche par cathétérisme interventionnel: utilisation de stents couverts.

An 11 year-old boy with an Alagille syndrome was referred for an urgent echocardiography because of new onset of febrile hemoptysis. Follow-up in our institution to that point had shown severe hypoplastic pulmonary arteries. The diagnosis of tricuspid endocarditis complicated by pulmonary embolisms and aneurysm of the left pulmonary artery was done. Despite effective antiobiotics, the evolution was marked by rapid progression in size of the left pulmonary aneurysm. Since the surgery was not an option because of high risk of death, this aneurysm was treated by a trans-catheter technique using covered stents (CP stents, Numed Inc). Its exclusion allowed to stop its progression in size and to avoid its rupture.

[Obstetrical management of patients at risk of neonatal lupus syndrome: review of the literature]. / Prise en charge obstétricale des patientes à risque de "lupus néonatal"

Fetuses and infants of women with anti-SSA/Ro and anti-SSB/La antibodies are at risk of neonatal lupus syndrome, featuring skin lesions, hematological and hepatic disorders, and congenital heart block (CHB) in the absence of severe cardiac malformation. The prevalence of CHB in newborns of anti-SSA/Ro positive women with known connective tissue disease is 1 to 2% and the risk of recurrence ranges from 10 to 17%. CHB is definitive and is associated with significant morbidity (pacemaker must be implanted in 2/3 of cases) and mortality (16 to 19%). Myocardial involvement may either be associated or appear subsequently. Other manifestations are discussed. For anti-SSA/Ro positive pregnant women, echocardiograms should be performed every 2 weeks from 16 to 24 weeks of gestation, and every week in case of past history of CHB. Electrocardiogram should be performed in the first days of life for all children to detect incomplete CHB. Therapy for CHB detected in utero is based on fluorinated steroids, especially betamethasone. Its efficiency is variable.

Arrhythmias and conduction defects as presenting symptoms of fatty acid oxidation disorders in children.

BACKGROUND: The clinical manifestations of inherited disorders of fatty acid oxidation vary according to the enzymatic defect. They may present as isolated cardiomyopathy, sudden death, progressive skeletal myopathy, or hepatic failure. Arrhythmia is an unusual presenting symptom of fatty acid oxidation deficiencies. METHODS AND RESULTS: Over a period of 25 years, 107 patients were diagnosed with an inherited fatty acid oxidation disorder. Arrhythmia was the predominant presenting symptom in 24 cases. These 24 cases included 15 ventricular tachycardias, 4 atrial tachycardias, 4 sinus node dysfunctions with episodes of atrial tachycardia, 6 atrioventricular blocks, and 4 left bundle-branch blocks in newborn infants. Conduction disorders and atrial tachycardias were observed in patients with defects of long-chain fatty acid transport across the inner mitochondrial membrane (carnitine palmitoyl transferase type II deficiency and carnitine acylcarnitine translocase deficiency) and in patients with trifunctional protein deficiency. Ventricular tachycardias were observed in patients with any type of fatty acid oxidation deficiency. Arrhythmias were absent in patients with primary carnitine carrier, carnitine palmitoyl transferase I, and medium chain acyl coenzyme A dehydrogenase deficiencies. CONCLUSIONS: The accumulation of arrhythmogenic intermediary metabolites of fatty acids, such as long-chain acylcarnitines, may be responsible for arrhythmias. Inborn errors of fatty acid oxidation should be considered in unexplained sudden death or near-miss in infants and in infants with conduction defects or ventricular tachycardia. Diagnosis can be easily ascertained by an acylcarnitine profile from blood spots on filter paper.

Coronary artery obstruction after the arterial switch operation for transposition of the great arteries in newborns.

OBJECTIVES: We sought to describe a large series of coronary artery obstructions after the arterial switch operation for transposition of the great arteries and to discuss their clinical implications. BACKGROUND: Aortic root angiography and myocardial perfusion imaging yield ambiguous results regarding the fate of the coronary artery anastomoses after the arterial switch operation. Late death related to coronary artery obstruction and growth of the translocated coronary arteries are of major concern in these patients. METHODS: Selective coronary artery angiography was performed prospectively in a total of 165 children. RESULTS: A total of 12 coronary occlusions, 8 major stenoses, 6 minor stenoses of the left ostium and 4 stretchings of one coronary artery were identified. Obstructions were more frequent in types D and E (p < 0.001) of the Yacoub and Radley-Smith classification. Coronary obstruction was documented in all patients with electrocardiographic and ultrasound evidence of myocardial ischemia at time of study. Early postoperative ischemia did not predict coronary artery lesion if the patient had fully recovered. Persistent or delayed myocardial ischemia was highly predictive of coronary artery lesions. The incidence of coronary artery obstruction was very high (11 of 35) in patients operated on by a rapidly abandoned technique of single-orifice reimplantation of both coronary artery ostia. CONCLUSIONS: Selective coronary angiography is the most accurate means to assess coronary artery obstruction after the arterial switch operation. Precise diagnosis of coronary artery lesions after this operation will help to elucidate the pathogenesis, develop adequate therapeutic strategies and might indicate how to prevent coronary complications after operation.

beta(1)-adrenergic antagonists improve sleep and behavioural disturbances in a circadian disorder, Smith-Magenis syndrome.

Smith-Magenis syndrome (SMS) is a clinically recognisable contiguous gene syndrome ascribed to interstitial deletions of chromosome 17p11.2. Patients have a phase shift of their circadian rhythm of melatonin with a paradoxical diurnal secretion of the hormone. Serum melatonin levels and day-night behaviour were studied in nine SMS children (aged 4 to 17 years) given acebutolol, a selective beta(1)-adrenergic antagonist (10 mg/kg early in the morning). Cardiac examination, serum melatonin, motor activity recordings, and sleep diaries were monitored before and after drug administration. The present study shows that a single morning dose of acebutolol suppressed the inappropriate secretion of melatonin in SMS. A significant improvement of inappropriate behaviour with increased concentration, delayed sleep onset, increased hours of sleep, and delayed waking were also noted. These results suggest that beta(1)-adrenergic antagonists help to manage hyperactivity, enhance cognitive performance, and reduce sleep disorders in SMS.

[Pediatric cardiac pacing: indications, implant techniques, pacing mode]. / Stimulation cardiaque chez l'enfant: indications, voies d'abord, et modes de stimulation.

Over the last years, pacing leads design and pacemaker (PM) generator size, reliability, and longevity have markedly improved, so that reliable paediatric implant can now be performed at any age with a low complication rate. Main indications include congenital and postoperative atrioventricular block (AVB) and postoperative sick sinus syndrome. Implantation of a PM is mandatory for children who are symptomatic from syncope or congestive heart failure and for those who have advanced block persisting more than 10 days after cardiac surgery. Criteria for pacing have been established in relation with the bradycardia and prophylactic pacing is recommended in children with congenital AVB and a mean heart rate <50 beats/minute. The majority of paediatric cardiologists recommend epicardial pacing in children less than 10 kg and when venous access to the heart is limited by congenital anomalies or prior operation; for older children, transvenous implantation has become the technique of choice. As heart rate is the main determinant of cardiac output at exercise in children with normal heart structures, the VVI-R mode is an alternative to dual chamber transvenous pacing in young patients. Patients with isolated sinus failure are paced in the atrium. Although the majority of patients are doing well, late complications within the paediatric population include venous thrombosis and difficulties in lead extraction. Myocardial dysfunction in children with congenital AVB is increasingly reported, but it is not determined whether it is due to the underlying disease or to right ventricular apical pacing and adverse remodelling.

[Neonatal lupus syndrome: Literature review]. / « Lupus néonatal ¼ : revue de la littérature.

Neonatal lupus syndrome is associated with transplacental passage of maternal anti-SSA/Ro and anti-SSB/La antibodies. Children display cutaneous, hematological, liver or cardiac features. Cardiac manifestations include congenital heart block (CHB); endocardial fibroelastosis and dilated cardiomyopathy. The prevalence of CHB in newborns of anti-Ro/SSA positive women with known connective tissue disease is between 1 and 2% and the risk of recurrence is around 19%. Skin and systemic lesions are transient, whereas CHB is definitive and associated with significant morbidity and a mortality of 18%. A pacemaker must be implanted in 2/3 of cases. Myocarditis may be associated or appear secondly. Mothers of children with CHB are usually asymptomatic or display Sjogren's syndrome or undifferentiated connective tissue disease. In anti-Ro/SSA positive pregnant women, fetal echocardiography should be performed at least every 2 weeks from the 16th to 24th week gestation. An electrocardiogram should be performed for all newborn babies. The benefit of fluorinated corticosteroid therapy for CHB detected in utero remains unclear. Maternal use of hydroxychloroquine may be associated with a decreased recurrent CHB risk in a subsequent offspring. A prospective study is actually ongoing to confirm these findings.

Heterozygous mutation in the pore of potassium channel gene KvLQT1 causes an apparently normal phenotype in long QT syndrome.

Mutations in KvLQT1, a gene encoding a potassium channel, cause both the recessive Jervell and Lange-Nielsen (JLN) syndrome and the dominant Romano-Ward (RW) syndrome. These diseases are characterised by a prolonged QT interval on the ECG, syncopes and sudden death due to cardiac arrhythmias. The JLN syndrome is also associated with a congenital bilateral deafness. We report here a novel missense mutation, W305S, in the pore region of KvLQT1 identified by PCR-SSCP analysis in two consanguineous JLN families. In contrast to several missense mutations found in the same region of KvLQT1 in RW patients which are associated with severe cardiac phenotypes, the W305S mutation is responsible for an apparently normal phenotype in heterozygous JLN carriers.

Switch operation for transposition of the great arteries in neonates. A study of 120 patients.

From March 1984 to January 1987, anatomic surgical correction was performed on 110 newborn infants (2 to 23 days old, mean 7.8 +/- 3.5, standard deviation) with simple transposition of the great arteries and 10 additional neonates (7 to 30 days old, mean 17.9 +/- 8.3, standard deviation) with transposition and a large ventricular septal defect. All had preoperative catheterization. Ninety-six percent of the patients underwent balloon atrial septostomy and 90% received prostaglandin E1 infusion until the time of the operation. The anatomy of the coronary arteries according to the Yacoub classification was as follows: type A, 82 patients; type B, 5 patients; type C, 4 patients; type D, 23 patients; and type E, 6 patients. Continuous hypothermic bypass with no circulatory arrest was used for all patients except two. Myocardial protection was ensured by crystalloid cardioplegia. Coronary artery relocation was performed according to the Yacoub technique with some modifications, and pulmonary artery reconstruction was done according to the Lecompte maneuver in all patients, even when the great vessels had a side-by-side relationship. The proximal pulmonary artery was reconstructed with two circular patches for the first 10 patients and with a single large posterior patch for the last 110 patients. Tanned heterologous pericardium was used for the first 25 patients and autologous native pericardium for the last 95 patients. The perioperative mortality rates were 8.3% for the entire series and 5.4% for the last 110 patients, with no deaths in the group having transposition plus ventricular septal defect. Late death from acute myocardial infarction occurred in two patients in the second month after operation. No patient was lost to follow-up, which ranged from 2 to 46 months (mean 16 +/- 11.2, standard deviation). The follow-up included sequential noninvasive evaluations and 32 catheterizations performed 10 to 18 months postoperatively. Two patients were reoperated on for pulmonary stenosis caused by retraction of the two heterologous pericardial patches, but neither died. Six others have mild to moderate pulmonary stenosis. Two patients have trivial aortic regurgitation. None have aortic dilatation or supravalvular aortic stenosis. The 108 survivors have no cardiovascular symptoms. They all are in sinus rhythm, have normal left ventricular function, have no ischemic problems, and receive no medication.

Surgical angioplasty of the main coronary arteries in children.

OBJECTIVE: To determine the safety and efficacy of surgical angioplasty of the coronary arteries in children. METHODS: We performed 9 surgical reconstructions of the left main coronary artery and 1 of the right coronary artery ostium in 10 children (mean age 5.7 years; range 2 months-15 years). The basic diseases included the following: congenital atresia of the left coronary artery (n = 2) and atresia of the right coronary artery in a patient with an aortoventricular tunnel (n = 1); stenosis of the left main coronary artery (1) in a patient with Williams syndrome (n = 1), (2) in a patient with familial hypercholesterolemia (n = 1), (3) after the arterial switch operation for transposition of the great arteries (n = 3), (4) after reimplantation of an anomalous left main coronary artery from the pulmonary artery (n = 1), and (5) by compression after a réparation à l'étage ventriculaire procedure (n = 1). Myocardial viability was assessed by single photon emission computed tomography (thallium 201; 7/10). The coronary artery stem was enlarged with a saphenous (n = 5), a pericardial (n = 4), or a polytetrafluoroethylene patch (n = 1). RESULTS: There was 1 hospital death and 9 patients are alive (mean follow-up 46 +/- 30 months; range 12 months to 10.5 years). Eight of 9 survivors had a selective coronary artery angiogram and had normal coronary artery ostia. Two patients had stenosis of the left anterior descending coronary artery, 1 of whom underwent successful internal thoracic artery grafting. CONCLUSIONS: Surgical angioplasty of the coronary stems restores physiologic coronary perfusion and conserves bypass material. It can be performed safely in children and provides encouraging midterm results.

[Congenital complete atrio-ventricular block]. / Bloc auriculo-ventriculaire complet congénital.

Congenital complete atrio-ventricular block is a rare condition, the result of an anatomical anomaly of the conduction pathways, or even the trans-placental passage of maternal antibodies causing fetal myocarditis with fibrosis of the conduction tissue. It is not clear whether AV block discovered later in childhood is really "congenital". Whatever the age of the child, the only treatment is pacemaker implantation. Cardiac failure and syncope are absolute indications for implantation. Children whose heart rate is below 50 beats per minute (bpm) represent a group at high risk of syncope or even sudden death and must be paced, even if asymptomatic. Moreover, certain patients with immunological complete AV block have cardiopathy and must also be paced. In our department, the route for pacing is epicardial in younger children, in practice weighing less than 10-15 kg, and endocavitary in older children. The choice is either double chamber pacing, which restores AV conduction initiated by the child's sinus, or ventricular pacing with activity-controlled heart rate. Although worrying complications have been described, such as venous thrombosis, infections related to repeated interventions, and delayed cardiomyopathy, the results of paediatric pacing are generally satisfactory and the great majority of children with congenital complete AV block lead a normal life.

[Outcome of Wolff-Parkinson-White syndrome in children. Transesophageal study of anterograde permeability of the accessory pathway and of atrial vulnerability]. / Evolution du syndrome de Wolff-Parkinson-White chez l'enfant. Etude transoesophagienne de la perméabilité antérograde de la voie accessoire et de la vulnérabilité atriale.

Twenty-nine children with the Wolff-Parkinson-White syndrome (WPW) were evaluated by transoesophageal electrophysiological studies to determine the quality of anterograde-conduction in the accessory pathway and the atrial vulnerability. The study group included 15 neonates, 1 to 30 days old, and 14 children from 5 to 15 years of age; Anterograde conduction through the bundle of Kent was tested by incremental transoesophageal atrial pacing and by the determination of the shortest conducted cycle with preexcited RR waves; bursts of atrial pacing were then used to try to trigger an atrial arrhythmia. In the group of the 15 neonates, 11 had accessory pathways capable of conduction to the ventricules at frequencies > 300/min (stimulation cycle < or = 2.00 ms) but no atrial arrhythmias could be induced. The older children had slower conduction in the accessory pathways with the shortest conducted cycle length > 200 ms in 11/14 cases; on the other hand, atrial fibrillation was easily induced in 4 children, all over 12 years of age. The risk of syncope by rapid conduction of an atrial arrhythmia through the accessory pathway is negligeable in young children, including those on digoxin. This study suggests that this low risk is explained more by the absence of atrial vulnerability than by the electrophysiological properties of the accessory pathways.

[Late supraventricular arrhythmia complicating Fontan or cavopulmonary type procedures. Apropos of 7 cases]. / Troubles du rythme auriculaire tardifs compliquant les opérations de type Fontan ou de type cavopulmonaire. A propos de 7 observations.

The authors report 7 cases of late arrhythmias after atriopulmonary (5 cases) or total cavopulmonary (2 cases) bypass procedures. There were 6 cases of atrial flutter and one case of atrial tachycardia. The condition presented with cardiac failure in 5 cases. In 2 patients, atrial flutter caused syncope or dizziness. The arrhythmia was reduced by atrial stimulation (3 cases) or by cardioversion (1 case). Prevention of recurrence with oral amiodarone was effective in all cases but was responsible for secondary effects in 4 cases. In one patient, recurrence of atrial flutter was complicated by right atrial thrombosis with cerebral embolism. Five patients were reoperated after cardiac catheterisation and angiography. Surgery consisted of resection of a stenosis of the anastomosis in one case, and the transformation of atriopulmonary anastomosis into a total cavopulmonary bypass because of a very dilated right atrium without stenosis in 4 patients. The immediate postoperative period was complicated by a recurrence of the arrhythmia in 3 children not treated by antiarrhythmic therapy. At long-term, one patient died 6 months after withdrawal of amiodarone therapy of recurrence of atrial flutter. Five of the 6 survivors are treated with amiodarone or a betablocker; 3 have had pacemaker implantation for severe bradycardia. Late atrial arrhythmias complicating atrio- and cavopulmonary bypass procedures carry a risk of cardiac failure and sudden death. When diagnosed, the patient should be investigated for stenosis of the anastomosis but severe dilatation of the right atrium is often the only finding. After restoration of sinus rhythm, maintenance antiarrhythmic therapy should be continued indefinitely.

[Predictive factors for late complete atrio-ventricular block after surgical treatment for congenital cardiopathy]. / Facteurs prédictifs de bloc auriculo-ventriculaire complet tardif après traitement chirurgical des cardiopathies congénitales.

The late occurrence of complete atrio-ventricular block (CAVB) after cardiac surgery is rare but potentially responsible for cases of late sudden death. We searched for factors allowing prediction of this complication, retrospectively reviewing the case notes of 11 patients in hospital with complete AVB, 2 months to 10 years after correction of a cardiac malformation. All had a normal pre-operative ECG. The diagnosis had been made based on symptoms in 8 patients: syncope or collapse (4 cases) symptoms on effort (3 cases) or fatigue (1 case). In the others the diagnosis had been made on ECG. The block was infra-His in 5 patients who had electrophysiology. ECG analysis showed that all the patients had CAVB immediately post-operatively lasting 3-14 days. After restoration of conduction the ECGs showed the following anomalies compared to the pre-operative ECGs: long PR (1 case), long PR + right bundle branch block (2 cases), long PR + left axis deviation (1 case), RBBB + left deviation or rotation of the QRS axis (3 cases), long PR + RBBB + left axis deviation (4 cases). All of these patients had been fitted with a cardiac stimulator. In conclusion, the children who had CAVB immediately post-operatively lasting more than 48 hours and who then had an ECG showing different QRS compared to the pre-operative QRS and/or long PR had a risk of late complete AVB. These patients should have electrophysiology and a stimulator must be implanted in those who have an infra-His block.

[Arrhythmia after atrial correction of transposition of the great vessels. Holter recording study of 123 surgically treated patients]. / Troubles du rythme après corrections atriales des transpositions des gros vaisseaux. Etude par enregistrement Holter de 123 opérés.

Sinus node dysfunction after intra-atrial repair of transposition of the great arteries by a Mustard or Senning procedure is well known. We undertook this study to evaluate the frequency, the nature, the severity and evolution of these dysrhythmias by performing Holter monitoring in 123 children followed up over 5 years; 302 Holter recordings were reviewed. The patients were divided into 3 groups of increasing severity: I = no sinus node dysfunction, II = moderate sinus node dysfunction, III = severe sinus node dysfunction with bradycardia of less than 30/min and/or pauses of over 2000 ms. The association of atrial hyperexcitability was classified in 3 subgroups: A = no extrasystoles, B = at least 4 extrasystoles per 24 hours, C = atrial tachycardia (focal tachycardia or flutter) after the first six postoperative months. There were only 15% of normal recordings (IA) and the majority of children (58%) were classified in the intermediary groups (IB, IIA and IIB). Sinus node dysfunction tended to become more severe with time in nearly 30% of the 69 cases followed up sequentially. The bradycardia tended to become more severe and associated with episodes of atrial tachycardia: the frequency of type B and C increased to 30% in Group I, to 68% in Group II and to 91% in Group III. The attacks were severe, especially in patients with a mediocre postoperative haemodynamic result. This explains the global mortality of 3%, the morbidity of 15% and the pacemaker implantation rate of 12%.(ABSTRACT TRUNCATED AT 250 WORDS)

[Results of anatomical correction of simple transposition of the great vessels in 46 newborn infants]. / Résultats de la correction anatomique de la transposition simple des gros vaisseaux chez 46 nouveau-nés.

Between the 4th April 1984 and 6th December 1984, a strategy of anatomical correction of transposition of the great arteries was undertaken in the newborn if three criteria were respected: parental consent, patient's age of less than 15 days and satisfactory left ventricular geometry. We report our results in 47 consecutive cases operated by the same surgeon at an average age of 8.2 +/- 5.3 days. Atrioseptostomy was performed in all cases before surgery, with a ratio of left to right systolic ventricular pressures greater than 0.66 (m = 0.8) and an infusion of prostaglandin E1 was maintained until surgery in 40 cases. The geometry of the left ventricle assessed by echocardiography was considered to be normal in 23 cases, satisfactory in 22 cases and unfavourable in 2 cases. Except for one case with the great vessels situated in the frontal plane in which an atrial correction was decided upon during surgery, all the patients underwent detransposition under cardiopulmonary bypass without cardiac arrest, irrespective of the coronary anatomy. The pulmonary tract was reconstructed by direct anastomosis with a piece of tanned (first 25 cases) or autologous (following 21 cases) pericardium. There were 18 immediate deaths (17%) but only 4 out of 37 after September 1984 (11%); the causes of failure were: 5 technical errors, 1 irreducible postoperative pulmonary hypertension and 2 probable left ventricular failures. The 38 survivors were followed up for an average of 6.8 +/- 5.4 months (range 1 to 21 months; 28 cases were followed up for over 3 months).(ABSTRACT TRUNCATED AT 250 WORDS)

[Evaluation of the systolic pressure of the right ventricle by echocardiographic study of left ventricular geometry]. / Estimation de la pression systolique du ventricule droit par l'étude échocardiographique de la géométrie ventriculaire gauche.

Right ventricular systolic pressure is an important parameter in paediatric cardiology. A critical haemodynamic situation is attained if it exceeds the systemic pressure: strain proximal to severe pulmonary stenosis or the result of pulmonary hypertension. In these circumstances, the patient must be closely followed up at the least, and appropriate treatment, often surgical, has to be instituted. Ambulatory methods of assessing this parameter are either unreliable, like the surface electrocardiogram, or relatively complex, like certain ultrasonic methods. We therefore looked for a method relating the systolic pressures of the two ventricles and the end systolic geometry of the left ventricle as assessed simply from a short axis view of the left ventricle at the level of the junction between the chordae and papillary muscles. Two orthogonal diameters are measured and the ratio of the two dimensions calculated: this ratio which we call the "septal curvature" reflects left ventricular compression by the pressure that the right ventricle exerts through the interventricular septum. Seventy-two children aged 6 hours to 18 years (average 5.2 years) hospitalised for pre or post-operative investigation of congenital heart disease were evaluated by this method. The results were compared by statistical analysis with the ratio of ventricular pressures measured directly almost simultaneously during cardiac catheterisation. In general, a linear relation was observed between septal curvature (x) and ratio of pressures (y); y = 0.88, x - 0.63 and R = 0.92.(ABSTRACT TRUNCATED AT 250 WORDS)