[Pediatric cardiology is a form of preventive medicine].
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Cardiovascular Diseases↗
Biomedical subjects
J Kachaner
Publications and source records attributed to J Kachaner.
[Anatomic evaluation of ostium secundum atrial septal defects by tridimensional echocardiography].
The decision to close an ostium secundum atrial septal defect by interventional catheterisation implies knowing its size, form and the relationship of its borders to neighbouring structures as accurately as possible. Three-dimensional echocardiography provides unique views of the interatrial septum and the authors set out to assess its performance. Ten patients, aged 8 to 20 years, included in a multicenter European clinical trial of closure of atrial septal defects with the CardioSEAL prostheses, were examined by transoesophageal echocardiography with three-dimensional reconstruction of the interatrial septum viewed from the left or right atrium. The septal defect had a very variable morphology, round, oval raquet-shaped and occasionally multiple. The surface area of these defects varied by about 70% during the cardiac cycle, maximal during ventricular systole and minimal during atrial systole. The maximal diameter measured by two-dimensional transoesophageal echocardiography underestimated that measured by three-dimensional echocardiography by about 30%. Two patients had a juxta-aortic caudal border or a juxta-superior vena caval cephalic border making the defect unsuitable for catheter insertion of a CardioSEAL occluder. On the other hand, another patient had an adequate juxta-aortic border although it seemed too narrow with conventional imaging techniques. The authors conclude that three-dimensional reconstruction of transoesophageal echocardiography is the best method of selecting candidates for closure of ostium septum atrial septal defect by intervantional catheterisation.
Adolescent↗
[Can partial cavo-pulmonary connection be considered an alternative to the Fontan procedure?].
The disappointing long-term results of the Fontan procedure led the authors to assess substitution with partial cavo-pulmonary connections for definitive palliative treatment of single ventricle malformations. One hundred and fifteen patients with a mean age of 4.3 +/- 4.5 years (1 month-22 years) were treated by termino-lateral anastomosis between the superior vena cava and corresponding pulmonary artery, either of necessity because of a contraindication to total cavo-pulmonary connections (31 cases) or electively (84 cases). Another source of pulmonary flow was preserved or added in 76% of children, the operative mortality was 4% and the secondary mortality 3.5%. Significant complications were observed in 15% of cases with a secondary morbidity of 13%. Reoperation was required in 18 cases (16%). In fact, the death rate was higher in indications of necessity (19 versus 3.6% in elective procedures). Similarly, the number of serious complications (veno-venous or pulmonary arterio-venous fistulae, ventricular dysfunction) was higher in this group than in patients undergoing an elective procedure (23 versus 2.4%). After 4.8 +/- 3.2 years' follow-up, 73% of children treated of necessity and 95% of children treated electively were well despite mild cyanosis (average saturation of 86 +/- 6%). On condition that these results are confirmed at long-term as present follow-up is relatively short, this strategy would seem to be justified, providing mild cyanosis with little functional impairment is accepted, so avoiding the serious complications of Fontan-like circulations.
Adolescent↗
[Aortocoronary bypass in children. Apropos of 6 cases].
The improvement in diagnostic techniques for myocardial ischaemia and in imaging of coronary anomalies in children has increased the number of coronary revascularisation procedures. Moreover, the indications are more diverse with the emergence of acquired coronary disease which was unknown until recently, related to operations comprising manipulation of the coronary arteries. The authors report their experience of 7 internal mammary artery bypass grafts in 6 children aged 3 days to 11.5 years (average 2 years): 3 left main coronary stenosis after arterial switch for transposition of the great arteries, 1 peroperative lesion of an intramural left coronary in a case of simple transposition of the great arteries, 1 congenital atresia of the right ostium associated with Tetralogy of Fallot, and 1 right coronary occlusion with giant aneurysms of the left coronary in a case of Kawasaki's disease. There were no operative fatalities. All the coronary grafts were patent at early postoperative control. These results indicate clearly that the method is very feasible from a very early age. Long-term patency, the growth of the anastomosis and of the distal vessels are questions which await a reply in the future.
Child↗
[Neonatal ventricular tachycardia].
Ventricular tachycardia is rare and poorly understood in the neonate. The authors undertook a retrospective study in 2 foetus and 8 neonates aged 1 to 20 days at the time of diagnosis. The tachycardia was permanent in 2 cases, observed in runs of variable variation in the other 8, incessant in 7 of these cases. Only two cases were symptomatic: cardiac failure with shock 16 hours after birth and hydramnios at 16 weeks gestation. The electrocardiographic criteria of ventricular tachycardia (wide QRS complexes of different morphology to the sinus QRS complexes, atrioventricular dissociation) were fulfilled in all patients. The arrhythmia was monomorphic 9 times out of 10 with a fixed (3 cases) or variable (7 cases) rate which was always > 150/min. Intravenous magnesium sulphate in the severe and permanent forms, oral betablockers in forms triggered by acceleration of the sinus rhythm, oral amiodarone alone or associated in one case with propranolol were prescribed but three neonates were not treated, either from the outset or after inefficacy of amiodarone: nine of the patients were cured and are treatment-free 12 to 24 months later: the other patient has a slow, well tolerated ventricular tachycardia. No aetiology was detected in 9 cases; the other had a metabolic disease of B-oxidation of long chain fatty acids. The authors conclude that isolated, idiopathic ventricular tachycardia of the neonate usually carry a good prognosis which is not dependent on the tachycardia of the permanence of the arrhythmia. Simple treatment (betablocker or amiodarone) is usually associate with restoration of sinus rhythm and definitive cure during the first year of life.
Adrenergic beta-Antagonists↗
[Long-term outcome and prospects in managing transposition of great vessels].
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Adolescent↗
Atrio-ventricular valve dysplasia in 22 newborn infants.
We retrospectively studied the experience of our institution with isolated dysplasia of one or both atrio-ventricular valves in 22 newborn infants. All patients with associated cardiac malformations were excluded. Ten patients exhibited isolated tricuspid valve dysplasia. One patient had tricuspid valve dysplasia and a dysplastic pulmonary valve. In 10 patients, both atrio-ventricular valves were affected. Finally, mitral valve dysplasia was associated with pulmonary valve stenosis in 1 case. Associated syndromes and/or chromosomal anomalies were: Down syndrome (n=2), trisomy 18 (n=1), Noonan syndrome (n=1), Marfan syndrome (n=3), Ehlers-Danlos and Cutis laxa (n=2). Mortality was 27.2% during follow-up (mean 51 months): 3 patients with chromosomal aneuploidies, 2 patients with severe neonatal Marfan syndrome and 1 with Ehlers-Danlos. Complications were: sustained supra-ventricular tachycardia in 3, neonatal staphylococcal tricuspid valve endocarditis in 1, persistent significant valvular disease in 8. In the remaining 9 survivors, the dysplasia of the atrio-ventricular valves persists with absent or mild incompetence. Beside obvious chromosomal anomalies, newborn infants with dysplastic valves should be investigated for manifestations of connective tissue disorders. This may help to identify new pleiotropic syndromes which include valvular dysplasia as one manifestation.
Aneuploidy↗
Microsatellite DNA markers detects 95% of chromosome 22q11 deletions.
Cono-truncal cardiac malformations account for some 50% of congenital heart defects in newborn infants. Recently, hemizygosity for chromosome 22q11.2 was reported in patients with the DiGeorge/Velo-cardio-facial syndromes (DGS/VCFS) and causally related disorders. We have explored the potential use of microsatellite DNA markers for rapid detection of 22q11 deletions in 19 newborn infants referred for cono-truncal heart malformations with associated DGS/VCFS anomalies. A failure of parental inheritance was documented in 84.2% of cases (16/19). PCR-based genotyping using microsatellite DNA markers located within the commonly deleted region allowed us either to confirm or reject a 22q11 microdeletion in 94.3% of cases (18/19) within 24 hours. This test is now currently performed in the infants referred to us for a cono-truncal heart malformation as a first intention screening for 22q11 microdeletion.
Alleles↗
Iopentol (Imagopaque 300) compared with iopromide (Ultravist 300) in pediatric angiocardiography. A clinical trial assessing adverse events, ECG and diagnostic information.
Iopentol (Imagopaque, Nycomed Imaging AS, Oslo, Norway) 300 mg.l/ml was compared with iopromide (Ultravist, Schering AG. Berlin, Germany) 300 mg I/ml in pediatric angiocardiography in 97 children (48 and 49 patients, mean age 2.8 and 4.1 years in the respective contrast medium groups). The volume injected was usually 4-6 ml/kg b.w. ECG, blood pressure, heart rate, adverse events and efficacy were evaluated. Five patients in the iopentol group and four in the iopromide group reported adverse events. This difference was not statistically significant. One adverse event in each of the two groups was considered as possibly related to the contrast medium. Only small and transient changes in heart rate, blood pressure and ECG parameters were observed. No difference between the two contrast media was found for efficacy. It can be concluded that iopentol is well suited for angiocardiographic examinations in children.
Angiocardiography↗
Progressive left main coronary artery obstruction leading to myocardial infarction in a child with Williams syndrome.
UNLABELLED: We report a 3-year-old child with Williams syndrome in whom the first vascular feature of the syndrome was a myocardial infarction related to the occlusion of the left main coronary artery trunk. This coronary artery occlusion was not associated with supravalvular aortic stenosis. CONCLUSION: This report emphazises that acute vascular events related to systemic artery anomalies may reveal Williams syndrome.
Child, Preschool↗
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.
Child, Preschool↗
Thrombus in coarctation of the aorta masquerading as an interrupted aortic arch.
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Aorta, Thoracic↗
[Congenital coronary-cardiac fistula in children. Effects of surgical occlusion and percutaneous embolization].
Twenty-four children aged 2 months to 8 years (average: 3 years) with congenital coronary artery fistulae were studied. In 20 cases, the fistula presented with a continuous murmur: in 4 cases, pulmonary flow was increased to such an extent that it led to cardiac failure. Echocardiography and coronary angiography showed that the fistula originated from the left coronary artery or one of its branches in 13 cases and from the right coronary artery in 11 cases. All but one fistula, which drained into the left atrial appendage, drained into the right heart chambers (ventricle: 14 cases; atrium: 9 cases). Spontaneous regression after 9 years was observed in 1 case. The other children were treated: by surgery in 20 cases (1 external ligature and 19 open heart occlusions) with 2 residual shunts (including the case ligated) which had to be reoperated. Three children underwent percutaneous embolisation resulting in 1 failure and 2 successes. After an average follow-up of 6.5 years (5 months to 15.5 years), all patients were alive and doing well with normal resting and exercise ECGs, normal Thallium scintigraphy (5 cases) and normal left ventricular function on echocardiography. Selective control coronary angiography (14 cases) showed a reduction in fistula diameter from 10 +/- 3.7 to 4.5 +/- 1.2 mm (p < 0.0001). The authors conclude that all congenital coronary fistulae should be occluded, by percutaneous embolisation when the anatomical features are favourable. The other cases require surgical occlusion, the long-term results of which are very good.
Cardiac Surgical Procedures↗
[Double discordances with ventricular septal defect and pulmonary obstruction. A study of 72 cases].
Seventy-two patients with corrected transposition of the great arteries with ventricular septal defect and pulmonary obstruction were studied. Four deaths occurred in the neonatal period and two were lost to follow-up. The remaining 66 were divided into three groups: 1) Eight patients were not operated on because the lesions were well compensated; they are all alive and doing well eight years later. 2) Thirty-eight patients were treated with palliative surgery: one or more systemico-pulmonary shunts (33 cases), total cavo-pulmonary connection (1 case) and partial cavo-pulmonary connection complementary to a shunt (4 cases). There were 3 deaths and 17 patients were lost to follow-up. However, the 18 survivors are all well seven years later. 3) The other 20 patients underwent surgery for severe hypoxia, after previous shunt in 18 cases. A conventional surgical protocol was respected in 17 cases (closure of ventricular septal defect with pulmonary disobstruction by a direct pulmonary plasty or by ventriculo-pulmonary conduit). There were 4 deaths, 2 tricuspid valve replacements, 5 complete atrioventricular blocks requiring permanent pacing, 5 lost to follow-up and 8 good results after 4 years follow-up. In the last 3 cases, "anatomical" correction was attempted by tunnelling of the left ventricle to the aorta, conduit from the right ventricule to the pulmonary artery and intra-atrial Mustard procedure: these 3 children are doing well after 1 year though one of them required permanent pacing. Therefore, there is no place for elective surgery in this malformation: when necessary, the best option is to remain palliative as long as possible: when correction is required, an anatomical correction is the best procedure.
Cardiac Surgical Procedures↗
[Non-surgical ventricular training before arterial switch. Focus on an animal model in lambs].
One of the conditions for successful anatomical correction of transposition of the great arteries (arterial switch) or of double discordance (double switch) is the ability of the subpulmonary left ventricle to adapt to the systemic circulation when restored to its subaortic position. A balloon catheter designed for training the subpulmonary ventricle by progressive occlusion of the main pulmonary artery (NuMed) was implanted in six lambs aged 45 days and inflated progressively to obtain the maximum tolerated right ventricular pressure. Six controls were instrumented without balloon inflation. The experiment lasted 5 days. Progressive inflation of the balloon to a right ventricular pressure > 70% of the systemic (carotid) pressure led to bradycardia with venous desaturation and acidosis which regressed when the balloon was deflated. In one animal, progressive adjustment enabled a right ventricular pressure of 75% of the systemic pressure to be obtained throughout the last day of the study. No significant right ventricular hypertrophy was obtained. The value of this technique is in assessing the afterload reserve of the tested ventricle before surgical banding of the pulmonary artery, the first step to anatomical correction for restoration of concordance of double discordance or of transposition of the great arteries previously treated by a Senning or Mustard procedure.
Animals↗
[Aortic-ventricular tunnel with right coronary artery atresia].
An aortico-left ventricular tunnel was diagnosed at echocardiography in a 6 week old baby after fortuitous detection of a systolic and diastolic murmur. Its association with atresia of the right coronary ostium, suspected at echocardiogoraphy, was confirmed at surgery which included occlusion of the tunnel orifices, aortic commissurotomy and reimplantation of the right coronary artery. The short-term postoperative result is excellent. This is an exceptional malformation, the clinical diagnosis of which is relatively easy. Its relationship with the right coronary artery conforms to an embryological logic and must be investigated thoroughly before surgery to avoid compromising myocardial revascularisation.
Aorta, Thoracic↗
[Marfan's syndrome. Cardiovascular manifestations and therapeutic indications].
Marfan's syndrome is a disease of collagen microfibrils, the essential composition of which, fibrillin, is defective. The coding gene of this large protein has been localised on chromosome 15 and partially cloned. Its mutation is transmitted in the autosomal dominant mode. It is common, affects many organs, especially those rich in collagen. They include the skeleton, the eye and cardiovascular system. The prognosis of these patients depends on the degree of cardiovascular involvement; the aorta is the principal site of the lesions, particularly the aortic root which tends to develop aneurysmal dilatation, then dissection, the main cause of premature death of these patients. Mitral valve prolapse giving rise to mitral regurgitation may also be observed. Much progress has been made is recent years in the management of Marfan's syndrome, especially in ultrasound follow-up of the aortic root and the definition of risk factors for dissection. In parallel, advances have been made in the treatment, both medical with the use of betablockers to try to slow progression to aneurysm, and surgical, in replacement of the aorta and aortic valve in time and under optimal conditions. Life expectancy is thereby significantly increased. Similar progress has been made to allow these patients to have a pregnancy, classically thought to carry a high risk of maternal death.
Adrenergic beta-Antagonists↗
[Long-term evaluation of heart transplantation in children].
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Adolescent↗