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Biomedical subjects

A Bozio

Publications and source records attributed to A Bozio.

At least 73 records · Page 4Linked to original sources

[Cardiopathies in Di George's syndrome].

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.

DiGeorge Syndrome↗

[Echocardiography in children in 1991].

We review advances in ultrasound technology relative to the diagnosis of heart disease in infants and children and advances that have modified the practice of pediatric cardiology. Accurate non invasive diagnosis is now possible and cardiac surgery, based on clinical and ultrasonic information is routinely performed. We also review the contribution of antenatal echocardiography on the development of fetal cardiology.

Child↗

[Surgery without catheterization of aortic coarctation in newborn infants and infants under 3 years of age].

A retrospective study of 150 children under 3 months of age who underwent repair of coarctation of the aorta in the same center (between 1972 and 1987) was undertaken to assess the problems posed by surgery without cardiac catheterisation or angiography. The patients were divided into two groups: Group A, comprising 104 children operated after invasive investigations, and Group B, comprising 46 children operated on Doppler echocardiographic data alone. The two populations were comparable and "hypoplastic aortic arch" type coarctation was present in over 60% of cases. However, there were more ventricular septal defects in Group B (67.5%) than in Group A (49%). There were no significant diagnostic errors in Group B (one case of interrupted aortic arch diagnosed at surgery). The 1 month survival was the same in the two groups (82%). The indications of pulmonary artery banding were less frequent in Group B although there were more ventricular septal defects in these patients. These results confirm the value of Doppler echocardiography in the context of urgent surgery of congenital heart disease.

Angiocardiography↗

[Idiopathic dilated cardiomyopathies in children. Development and prognostic factors].

This review is a retrospective study of the cases of 103 patients under 20 years of age with idiopathic dilated cardiomyopathy and represents the whole experience of the Department of Paediatric Cardiology of the Hospital of Cardiovascular Disease, Lyon, between January 1970 and December 1988. The aetiological investigations were constantly negative: hypertrophic, restrictive and secondary cardiomyopathies were excluded. The clinical, radiological, electrocardiographic and echocardiographic data of each patient was noted at the time of admission and at the end of the clinical course. There were many more infants in this series. The onset of the disease was usually sudden. All patients were given medical treatment. The study period ranged over 19 years. Forty one patients (39.8%) died mainly of terminal cardiac failure. Sixty two patients are still alive, of whom 39 are completely cured, 2 have undergone cardiac transplantation, and 21 have a variable degree of chronic invalidity. The six months survival was 77.5%; the 1 year survival was 70% and the 5 year survival was 60%. Over one third of deaths (36.6%) occurred during the first month, 56% during the first 3 months and 70% during the first year. A statistical analysis comparing the initial criteria to death by the chi 2 test revealed the following prognostic factors: age at presentation (32.9% of deaths in patients under 2 years of age compared to 56.6% in the group over 2 years of age, p less than 0.025) and a family history of myocardial disease. The other clinical, radiological and echocardiographic parameters had no predictive value in this series and it was not possible to identify potential candidates for cardiac transplantation.

Adolescent↗

[Total anomalous pulmonary venous connection. Results of repair in 50 infants].

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.

Age Factors↗

[Treatment of ventriculo-pulmonary disconnections with prosthetic conduits. Long-term results].

Between 1973 and 1989, 81 consecutive patients aged 2 to 42 years old, with ventriculo-pulmonary discontinuity, were treated by implantation of prosthetic conduits. The initial pathology was Tetralogy of Fallot (33%), complete transposition of the great arteries (20%), truncus arteriosus (17%), double outlet right ventricle (17%) and atrioventricular discordance with L malposition of the great arteries (10%). The overall early mortality was 22% (18 cases) and 14% (5 cases) in the 36 patients operated after 1982. Sixty three patients were followed up for 3 months to 16 years; there were 8 late deaths which occurred spontaneously or at reoperation. Postoperative catheterisation was carried out in 33 cases; the average ventriculopulmonary systolic pressure gradient was 40 +/- 26 mmHg. Six patients were reoperated to change the conduit, on average 6 years +/- 23 months after the first operation. Five other patients underwent endoluminal dilatation of a stenosed conduit which delayed reoperation to change the conduit in 3 cases. Prosthetic conduits have been extensively used in patients with ventriculo-pulmonary discontinuity because they are readily available. However, because of progressive degradation of the prostheses between the 5th and 10th postoperative years, other therapeutic solutions should be considered, i.e. endoventricular repair when possible and, in other cases, the use of aortic homografts.

Actuarial Analysis↗

[Apico-aortic shunts. Indications, results].

Seven children or adolescents aged 8 to 20 years (average 13.5 years) underwent implantation of an apico-aortic conduit from 1980 to June 1989. Eleven previous operations under cardiopulmonary bypass had been performed. The indications were recurrence of muscular subaortic stenosis and/or congenital valvular stenosis in 3 cases, and stenotic, previously implanted aortic valve prosthesis of small calibre in 4 cases. The preoperative left ventricular-aortic systolic pressure gradient was between 70 and 130 mmHg. The first two patients had a bioprosthetic valvulation and the 5 succeeding patients a St Jude Medical mechanical prosthesis. The early and late mortality was nil. The average follow-up period is 5, 6 years at present (range 6 months to 9 years). One patient had to undergo repeat valve replacement after 5 years for degenerescence of the porcine bioprosthesis implanted in the conduit. No other complications related to the conduit or valve were observed. At the endpoint of the study all patients were asymptomatic without treatment. Control echocardiographic data showed normalisation of the indices of left ventricular function. Apico-aortic conduits would seem to be a safe and effective technique for the treatment of recurrent severe obstruction of the left ventricular outflow tract.

Adolescent↗

[Surgical treatment of complete atrioventricular canal. Value of the "composite double patch" technic].

Between January 1, 1982 and January 1, 1988, 49 complete corrections of complete atrioventricular canal were performed in children aged from 4 months to 8 years. 41 were infants less than 2 years' old and 31 were less than one year old. In the last 35 patients the "composite double patch" technique was used, consisting of closure of the interventricular septal defect with a dacron patch, followed by closure of the ostium primum with a pericardial patch. The mitral cleft was left intact in the last 6 operations. The overall mortality rate was 35 p. 100 (17 patients). It was 23 p. 100 in infants under 1 year and 17 p. 100 in infants aged from 6 to 12 months at the time of surgery (p less than 0.01). Seven of the 35 children in whom the "composite double patch" technique was used died (20 p. 100). Only one early death was recorded among the last 15 children operated upon. Two reoperations were performed: one within one month of the first operation, the other 4 months later for residual mitral regurgitation with haemolysis. 32 children were followed up for periods of 2 months to 6 years, 10 of them for more than 3 years. Two late deaths occurred during the follow-up. Grade 2 or 3/4 residual regurgitation was found in 14 patients who have regular clinical and echocardiographic examinations.(ABSTRACT TRUNCATED AT 250 WORDS)

Age Factors↗

[Current results of the treatment of transposition of the great vessels. Apropos of a series of 168 cases including 138 physiologic corrections].

This study takes stock of the current results of physiological correction (Mustard's or Senning's operation) in simple transposition of the great vessels, at a time when anatomical corrections, or detranspositions, are developing. Between January 1, 1974 and December 31, 1984, 168 neonates with simple transposition of the great vessels were operated upon and followed up for a mean period of 3.67 years (up to 11 years and 10 months). Thirty of them died before correction (12.7% mortality rate with palliative surgery) and 15 immediately after corrective surgery (11.2%). Among the 111 children who survived corrective surgery, the results were satisfactory in 62.7%, fair in 17.8% and poor in 10.2%; the late mortality rate was 9.3%. Post-correction morbidity mainly consisted of mechanical complications suspected in one-third of the patients (with 3 consecutive deaths) and heart rhythm disorders (50% of patients in this series had abnormal Holter recordings), with predominance of atrial rhythm disorders (regression of sinus rhythm was 3.82% per annum). This, after correction the survival curve underwent an actuarial regression of 1.31% per annum. This primary and secondary morbidity and mortality justifies a switch to anatomical corrections the results of which remain to be fully evaluated.

Arrhythmias, Cardiac↗

[Postmeningococcal pericarditis in children. A case favoring an immunoallergic process].

A case of pericarditis following meningococcal meningitis in a 19 month old boy is presented. First clinical signs were noted after 5 days of antibiotherapy (Cefotaxime); at that time the meningitis was cured and the bacteriologic studies yielded negative results. Complete clinical recovery was obtained within 24 hours of treatment with prednisone (2 mg/kg), antibiotherapy being stopped 4 days earlier. The pathogenesis of post meningococcal pericarditis is discussed based on the data from the literature. This observation adds support to an immuno-allergic origin of this pericarditis.

Humans↗

[Congenital aortic valve stenosis. Long-term results after valvulotomy].

The cases of 73 patients undergoing valvulotomy for congenital valvular aortic stenosis between 1957 and 1982 were reviewed. Data was updated after recalling patients to the outpatient clinic and/or analysis of the results of a questionnaire sent to the patient's family doctor or cardiologist. Babies less than 12 months old at the time of surgery were excluded from the study. Operation consisted of valvulotomy under direct control with few associated procedures as the valvular lesion was isolated in 89 p. 100 of cases. 5 patients died in the first 30 postoperative days, an operative mortality of 5,4 p. 100. The follow-up period ranged from 1 to 25 years, with 15 patients having been followed up for over 10 years. 6 patients were reoperated with no operative mortality. 2 of whom have since undergone a second reoperation. Of the 59 patients not re-operated, 54 were class I and 5 class II of the NYHA. Of the latter group, 4 are candidates for aortic valve replacement for significant aortic regurgitation. The actuarial survival graph shows a 92.82 p. 100 probability of survival at 5 years, and 86.83 p. 100 at 10 years. Aortic valvulotomy remains a palliative operation which does not protect the patient from subsequent sudden death.

Adolescent↗

[Coronary anomalies associated with the Williams-Beuren syndrome. Apropos of 2 cases].

Two coronary artery anomalies associated with the Williams-Beuren syndrome are reported. The first case was a 14 year old child with severe supra-aortic stenosis associated with severe hypoplasia of the ascending aorta; 2 D echocardiography and angiography showed a voluminous aneurysm of the left main coronary artery. Aortoplasty with a patch gave a good early result. The second case was a two and a half months old baby operated as an emergency after cardiac arrest and who died at the end of surgery. The baby had severe supra-aortic stenosis, occlusion of the left coronary ostium by the left anterior cusp which was abnormally adherent to the aortic wall with subendocardial infarction and reaction fibroelastosis. The frequency of coronary anomalies associated with the Williams-Beuren syndrome is probably underestimated. Because of their severity they must be looked for routinely, by echocardiography and angiography. Their tendency to progress is an indication for early surgical correction of severe supra-aortic stenosis.

Adolescent↗

[Acute endocarditis caused by Kingella kingae in an infant].

A case of bacterial endocarditis in a one year-old boy is reported. There was no underlying heart disease. The organism was a Kingella kingae, an aerobic Gram negative bacillus, a normal inhabitant of the upper respiratory tract. It has rarely been implicated as a pediatric pathogen. Occasionally it can cause bone and joint infections and exceptionally endocarditis.

Acute Disease↗

[Value of prostaglandin E1 in cardiac malformations in the newborn infant].

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.

Ductus Arteriosus↗

[Bacterial endocarditis due to Corynebacterium diphtheriae (author's transl)].

A case of bacterial endocarditis with unusual features in a 5 year-old boy is reported. There was no underlying heart disease or provocating factor. The organism was a non toxigenic Corynebacterium diphtheriae which was difficult to classify. It appeared to be close to the JK group of Corynebacterium described by the Center for Disease Control and considered as possibly responsible for severe infections. However, it was sensitive to penicillin. The course of the disease was complicated by the destruction of coronary valves and by splenic infarctus. Replacement of the aortic valve with a prosthesis was necessary. The boy was finally cured but the outcome remains uncertain.

Child, Preschool↗