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Biomedical subjects

A M Worms

Publications and source records attributed to A M Worms.

At least 37 records · Page 2Linked to original sources
PubMed1993

The Noonan syndrome. The Nancy experience revisited.

67 patients with Noonan syndrome seen over the last 29 years were selected preferentially on cardiac involvement. The cardiac anomalies consisted in the association of dysplastic pulmonary stenosis with asymmetric cardiomyopathy. In one patient, a translocation (3;22) was found. The relationship with cardio-facio-cutaneous syndrome and with the group of phacomatoses is discussed. The familial occurrence (10 families) seems compatible with autosomal dominant inheritance. A gene location on chromosome 22 cannot be excluded.

Adolescent↗
PubMed1992

Right ventricular overload and induced sustained ventricular tachycardia in operatively "repaired" tetralogy of Fallot.

The aim of the study was to evaluate the main predictors of the inducibility of sustained ventricular tachycardia (VT) in patients with repaired tetralogy of Fallot. Thirty-five patients (age 12 +/- 6 years) underwent right-sided cardiac catheterization, echocardiography, radionuclide angiography and ventricular stimulation; 10 had (group 1) and 25 had no (group 2) sustained VT. Group 1 patients were significantly older at the time of surgery and had longer follow-up periods (7 +/- 3 vs 4 +/- 4 years, p less than 0.02; and 12 +/- 4 vs 5 +/- 2 years, p less than 0.001, respectively). Right ventricular (RV) systolic pressure, end-systolic and end-diastolic normalized RV volumes were higher in group 1 (48 +/- 14 vs 38 +/- 11 mm Hg, p less than 0.05; 1.23 +/- 0.2 vs 0.86 +/- 0.17, p less than 0.001; and 2.35 +/- 0.37 vs 1.70 +/- 0.22, p less than 0.001, respectively). RV end-diastolic pressure, left ventricular and RV ejection fractions were similar in the 2 groups. A stepwise discriminant analysis was made to predict patients with inducible sustained VT (group 1): Time period from surgery to follow-up (p less than 0.001), normalized RV end-systolic volume (p less than 0.002) and RV systolic pressure (p = 0.01) were higher in group 1 and allowed classification of 90% of patients in group 1 and 96% in group 2.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1992

Bicaval pulmonary connection in tricuspid atresia using an extracardiac tube of autologous pediculated pericardium to bridge inferior vena cava.

From August 1989 to April 1991, four children, 1.5 to 4 years old with type IIb tricuspid atresia underwent total right heart bypass by means of a bicaval pulmonary connection using an extracardiac conduit of pediculated pericardium between the inferior vena cava and the main pulmonary artery. The tube was made from a large rectangular flap of the patient's own pericardium, pediculated along the right border, ensuring a vascular supply. The diameter of the tube, calibrated on a Hegar probe, equals that of the inferior vena cava, and the length is adapted to bridge the gap between the inferior vena cava and the main pulmonary artery. All four patients had an uneventful postoperative course. Assisted ventilation was stopped on the next morning and the chest tubes removed on the 2nd or 3rd day. The liver was only moderately enlarged and no pleural effusions developed. Sinus rhythm was permanent. Echocardiographic monitoring 6-10 months after the hospital discharge showed patent tubes, no collapse during the cardiac cycle, and no wall thickening and a laminar flow. The advantages of the pediculated pericardial tube are that no prosthetic material is used. There is no thrombogenicity or antigenicity. These tubes retain a growth potential, and we believe that this material is suitable for use in young patients.

Child, Preschool↗
PubMed1992

[Percutaneous angioplasty of branch pulmonary artery stenosis. A cooperative study].

Fifty balloon angioplasties of branch pulmonary artery stenosis in 34 patients aged 4 months to 20 years, performed in 7 French Centres, were included in this study: they were performed from 1984 to 1991 and concerned severe stenoses which were congenital in 36 cases and secondary to surgery in 14 cases. The criteria of inclusion were: diameter of stenosis less than or equal to 8 mm, right ventricular systolic pressure (RVP) greater than or equal to 50 mmHg, RVP/aortic pressure ratio (RVP/AO) greater than or equal to 50%, and a significant perfusion defect on radionuclide angioscintigraphy. There were no operative complications. The diameter of the stenosis increased by greater than 40% in 23 cases (46%); in only 7 of these cases (14%) did the RVP and RVP/AO ratio decrease by more than 20%, the RVP being less than 50 mmHg, or did the perfusion scintigraphy improve. No cases of restenosis were observed. The reasons for failure are discussed together with the limitations of this study. The respective indications of surgery and angioplasty are not easy to determine for these complicated lesions. New techniques such as the use of stents should improve results.

Adolescent↗
PubMed1992

[Results of the surgical repair of branch pulmonary artery stenosis in tetralogy of Fallot].

This study was based on the follow-up of 55 patients with tetralogy of Fallot and operated for branch pulmonary artery stenosis. Twenty two patients were followed up clinically (Group I): 91% of the patients were asymptomatic at the end of the first operative year. Thirty three patients (Group II) underwent control catheter studies after an average of 2.5 years after complete repair: only 70% of these were asymptomatic at 1 year; 8 were reoperated after an average of 3.6 years. The surgical result could only be evaluated by control catheter studies. The results were therefore based on the data obtained from Group II: this selection resulted in a pessimistic bias as the outcome in the two groups was significantly different. The stenoses were congenital and/or iatrogenic. They were located on the left branch (55%), the right branch (9%), the bifurcation (24%), separately on the two branches (12%). The diversity of the operative techniques and materials used reflects the difficulty of this surgery. The cumulative results were good in 33% of cases, average in 28% and poor in 39%. The results were better in isolated branch stenosis than in stenosis of the bifurcation, though not statistically significant. The results were related to age, body weight, the type, the site, the length, the association of stenoses and the material used. Significant predictive factors for a good result were: Gore-Tex (p less than 0.05) and pericardial pedicle (p less than 0.005). The average follow-up of patients with the latter type of patch was only 2 years. These results require confirmation by studies of larger numbers of patients followed up for a longer period.

Cardiac Catheterization↗
PubMed1992

[Extracardiac pulmonary bypass in the treatment of tricuspid atresia].

Six patients aged 1.5 to 4 years with type 13 tricuspid atresia underwent extracardiac bicavopulmonary repair. This corrective procedure comprises control of palliative aorto-pulmonary anastomosis, the construction of an anastomosis between the superior vena cava and right pulmonary artery and the interposition of an extracardiac tube of autologous pediculated pericardium between the inferior vena cava and the main pulmonary artery. This tube, fashioned from a rectangular flap, remains attached along its right border, conserving its vascular pedicle with the pericardium. Its diameter is calibrated to that of the inferior vena cava. The postoperative course was uncomplicated: all children survived. Assisted respiration was discontinued 24 to 36 hours after surgery, and the pleural drains withdrawn after 48 to 72 hours. The rhythm remained sinusal. Central venous pressure ranged from 8 to 12 mmHg. Peripheral arterial situation was over 95%. At follow-up at 3 to 15 months, the children were very active, reflecting good exercise capacity. Holter monitoring was normal. Saturation was 94 to 97%. Doppler echocardiography showed laminar blood flow. This technique insures good venous drainage without any prosthetic thrombogenic material. In addition, the pediculated pericardium conserves a potential for growth, justifying its use in small children. Long-term evaluation is essential but the initial results are encouraging and encourage perseverance with this method.

Anastomosis, Surgical↗
PubMed1992

Diffuse pulmonary lymphangiectasis with heart defect discovered 4 months post-natally.

Congenital pulmonary lymphangiectasia is a rare abnormality with dilatation of pulmonary lymphatics and the radiological pattern of a pulmonary interstitial syndrome. It is usually symptomatic at birth and is almost always lethal. We report the case of an infant with congenital pulmonary lymphangiectasis and congenital heart disease who is still alive at 11 years. This case is interesting from the pathogenic, nosologic and prognostic point of view.

Child↗
PubMed1992

Persistence of a single pulmonary vein in a child.

Persistence of a single pulmonary vein with normal pulmonary venous return is a rape and unusual radiographic finding. We report such a case with the venous opacity partially visible on the right border of the heart on a plain frontal chest film. MRI showed beautifully the pulmonary vein and can now replace angiocardiography.

Angiocardiography↗
PubMed1991

Systemic artery-pulmonary vein fistula without sequestration.

Fistulae between an artery arising from the aorta and a pulmonary vein associated with absence of a branch of the pulmonary artery, but without anomalies of the bronchial tree, are exceedingly rare. We report a case in a child who presented with a continuous vascular murmur and developed heart failure due to the left-left shunt. Left lobectomy was performed and showed that the entire left lower lobe was occupied by a huge arteriovenous fistula between an artery stemming from the aorta and the pulmonary veins.

Aorta↗
PubMed1991

[Congenital long QT syndrome. Mid-term prognosis].

Twenty-five cases of the congenital, idiopathic long QT syndrome, occasionally diagnosed at birth and persisting after the 4th day of life, were analysed retrospectively to determine the medium-term prognosis and to identify risk factors. The age of the patients varied: 13 neonates including 10 of less than 5 days of age, and 12 children 1 to 12 years old. Eleven patients belonged to families with long QT syndromes. The recruitment also varied: syncope or near syncope (9 cases), bradycardias or tachyarrhythmias (9 cases) or by systematic familial enquiry (7 cases). The corrected QT interval (QTc) was greater than 0.44 s in all patients, and 17 patients had conduction (atrioventricular or intraventricular) defects and/or ventricular arrhythmias (tachycardia, torsades de pointe or fibrillation). All except two older children were prescribed betablocker therapy and in 6 cases of resistant arrhythmias a pacemaker was implanted. There were 6 deaths: 4 deaths occurred in neonates who had the longest QT intervals (greater than 0.65 s) complicated by conduction defects and tachyarrhythmias. The other 2 fatalities were in older children who both died during syncope. The 19 survivors were followed up for 3 months to 16 years: 4 children with the shortest QTc values (0.44 to 0.48 s) have completely recovered; 2 neonates treated for 9 months and then weaned off therapy without any complications and 2 older children who were not treated. The other 15 cases are all under treatment but do well and have had no syncopal episodes during follow-up.(ABSTRACT TRUNCATED AT 250 WORDS)

Adrenergic beta-Antagonists↗
PubMed1991

[Non-surgical repair of atrial septal defect by "buttoned" double disk device in children. First results in 3 children].

Transcatheter closure of ostium secundum atrial septal defect (ASD) with a "buttoned" double-disk device was attempted in 5 children aged 14 months to 12 years, with success in 3 cases. The diameter of the ASD was 15 to 20 mm. The occluding devices were chosen with sizes 12-15 mm greater than those of the ASDs. There were no operative complications: the ASD was practically completely occluded in 2 children and left a very small shunt in a third case. Those three patients are doing well, 8 to 9 months after the procedure. In the other two cases, the device fell into the right atrium and had to be removed surgically without any complications. The "buttoned" double-disk device seems to be relatively simple and easy to insert through an 8F catheter. Additional technical improvements should enable more complete occlusion of ASD and easier retrieval by the transvenous route when incorrectly inserted. Further experimental and clinical studies are required to assess the results and to compare them with those of the other teams.

Cardiac Catheterization↗