Biomedical subjects
Publications and source records attributed to S Magnier.
Q fever is a zoonosis caused by Coxiella burnetii. The most frequent clinical expression of the chronic form is a bacterial culture negative aortic or mitral endocarditis. A case of tricuspid valve endocarditis due to C burnetii is described, with a favourable outcome after treatment with doxycycline and hydroxychloroquine.
Neonatal anatomical correction of transposition of the great arteries (TGA) has transformed the prognosis of this condition but the diagnosis must be made rapidly. The aim of this retrospective study was to evaluate the benefits of antenatal diagnosis on the outcome of TGA. The cases of 50 consecutive neonates with TGA with or without ventricular septal defect hospitalised between 1989 and 1996 were reviewed. All these children underwent anatomical correction of their malformation in the neonatal period. In seventeen of the children the diagnosis was made in the antenatal period at a gestational age of 28.7 +/- 5 weeks of amenorrhea and the other 33 had a postnatal diagnosis at 6.2 +/- 13 days. The clinical and echographic features were identical in the two groups. The risk factors of mortality for the whole population were a Yacoub type B or C coronary disposition, an intramural coronary course, difficulties in reimplantation of the coronary arteries and/or peroperative haemodynamic failure. In the authors' experience, the time of diagnosis (antenatal or postnatal) did not had on the management and prognosis of TGA. Studies with larger population groups are probably necessary to demonstrate the possible benefits of antenatal diagnosis.
Surgery for hereditary cardiopathies has made much progress over the last 25 years. For left/right ventricular shunts, mortality is 5% and quality of life is satisfactory in 85% at five years. All interventricular communications with major pulmonary hypertension should be operated during the first year of life. When surgery is indicated for atrial left/right shunt the operation should be done before the age of 4 or 5 years. Results for Fallot's tetralogy are now excellent with an actuarial survival of more than 30 years in 86% of simple forms after curative surgery. Complete repair should be done early (12 to 18 months) to reduce dysrhythmia and right ventricular hypertrophy. Corrective atrial surgery for transposition of the great vessels, a totally physiological operation, has been replaced by anatomic correction, improving prognosis to more than 90% survival and only 10% reoperation rate. Surgery for coarctation of the aorta gives very good results modulated by the risk of recurrence, especially when performed early (before 6 months). Medical-surgical management of hereditary cardiopathies begins at birth. Surgery for all operable malformations should be performed before the end of the second year of life, allowing for nearly normal life, both at home and at school.
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Diffuse supravalvular aortic stenosis can be treated by a variety of surgical approaches. In this case of severe diffuse supravalvular aortic stenosis in a child, we used the combination of an apicoaortic conduit followed 6 years later by aortic valve replacement, replacement of the ascending aorta and aortic arch, and an ascending to thoracic descending aorta bypass graft.
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The authors report a rare case which is interesting from three points of view: tricuspid valve endocarditis in a child complicating a fistula between the right coronary artery and right ventricle with negative blood cultures but a positive serology for Coxiella burnetii. This mutilating tricuspid endocarditis was complicated by multiple pulmonary embolism of the right lung. After two months of antibiotic therapy the coronaro-cardiac fistula was closed and the tricuspid valve replaced with a mitral homograft. All cases of blood culture negative endocarditis require serological investigation to detect intracellular organisms which are difficult to diagnose and justify specific prolonged antibiotic therapy. Control serological tests are essential in the long-term because of the risk of chronic infection, especially in cases with prosthetic intracardiac material. Treatment, based mainly on tetracyclines, should be continued for at least two years.
For over 20 years, different methods of interventional catheterisation have partially replaced surgical closure of patent ductus arteriosus (PDA). The authors report the results of a new operative technique, video-thoracoscopy, derived from endoscopic surgery. Under general anaesthesia and after tracheal intubation, two trocarts of 5 mm diameter are introduced into the thorax for the passage of the instruments required for dissection and closure of the PDA. Two hooks are also introduced to retract the lung and dissect the ductal region. Two 9 mm titanium clips are positioned under videoscopic control. Forty-five children underwent this procedure between February 1992 and July 1994. The average age at the time of operation was 13.8 months (range: 3 to 32 months) with an average weight of 14.5 kg (range: 2 to 48 kg) including 10 (22%) with a body weight of less than 6 kg. The surgical indications were haemodynamic in 27% of cases (large shunts with pulmonary hypertension) and prophylactic against endocarditis in 73% of cases. There were no operative fatalities. The immediate complications included: chylothorax (1 case) and left recurrent nerve paralysis (2 cases). A residual shunt was observed in 3 of the 45 cases (6%). In one of these cases, a supplementary clip was effective in suppressing the residual shunt. In the other 2 cases, the residual shunt was respected after a second failure of clipping the duct in one of the cases. The final closure rate was 95.6%. Closure of PDA by video-thoracoscopy is a rapid and safe technique.(ABSTRACT TRUNCATED AT 250 WORDS)
The authors report 5 cases of the long QT syndrome. The patients had malaises and syncopes triggered by exercise or emotional stress, occurring before 10 years of age. The diagnosis was delayed by an average of 38 months (range: 2 to 72 months) from the first symptom and systematic familial study which was positive in 4 of the 5 cases. The corrected QT intervals varied from 0.46 to 0.56 second. All patients were prescribed the betablocker nadolol, resulting in total regression of symptoms with no deaths during an average follow-up period of 40 months (range: 26 to 64 months). The long QT syndrome is a rare hereditary disorder comprising several entities. Recent genetic advances have improved our understanding of the condition. All have an idiopathic prolongation of the QT interval (> 0.44 second) in common with a predisposition to syncope due to torsades de pointes which may cause sudden death. The spontaneous arrhythmic mortality, which was early and high, has been remarkably decreased by betablocker therapy, from 73% to 6% at 10 years. The authors find nadolol to be extremely reliable and well tolerated, and recommend it as the drug of choice in this indication.
Seventy-seven live born children (September 1988 to May 1993) had prenatally detected morphological cardiac malformations (61 cases) and/or arrhythmias (17 cases). The outcome of these children was analysed with an average follow-up of 21.4 months (range: 1 day to 11 years). The parameters studied were age and condition of prenatal diagnosis, the term of pregnancy, the extracardiac malformations associated and the outcome of the live born children. Fifty-two children survived with a mean follow-up of 28.2 months (range: 5 months to 11 years) and 25 children died at a mean age of 3.3 months (range: 1 day to 32 months). The cardiac malformations correlated with the postnatal diagnosis in 59 children (60 cardiac malformations). They included left or right outflow tract obstruction in 22%, septal defects or Tetralogy of Fallot with or without pulmonary atresia in 18.4%, arterial malposition in 12.9% and arrhythmias or conduction defects in 20.7% of cases. The main reason for the high mortality was the complexity of the cardiac malformations with, in particular, ventricular hypoplasia with right or left outflow tract obstruction (41% of mortality). The functional consequences of these abnormalities were only partially appreciated at prenatal diagnosis. The extracardiac malformations, more easily diagnosed, contributed less to this mortality rate. The favourable prognosis of the arrhythmias or conduction defects diagnosed prenatally was confirmed but was, however, associated with a mortality rate of 12%.(ABSTRACT TRUNCATED AT 250 WORDS)
Surgical indications of isolated ventricular septal defects (VSD) with large shunts are a common problem in paediatric cardiology. The present study was undertaken retrospectively and continuously over 5 years in 50 patients in whom the age of diagnosis varied from birth to 1 year. The surgical results are presented and the clinical and paraclinical parameters used for determining the surgical indications are reviewed. Only 16 out of 50 children (32%) were operated without catheter study. However, since February 1992, when colour Doppler echocardiography became a routine investigation in the department, 75% of children have been operated on echocardiographic data alone. Knowing that the risk of pulmonary vascular disease is practically nil in this condition at this age, the most important problem lies in distinguishing between single and multiple VSDs. Until recently, angiography has always been the reference investigation. In this series, it did not appear to be superior to a good colour Doppler study. The surgical results showed a hospital mortality of 4%, the persistence of a well tolerated VSD in 16% of cases, none of which required reoperation. Two cases of complete atrioventricular block required permanent cardiac pacing. The authors conclude that when echocardiography provides all the necessary data concerning site, number, size and haemodynamic consequences of VSD, catheterisation may be dispensed with at this age. The surgical indication is, generally, closure of the VSD by a patch, usually possible by a right atrial approach; pulmonary artery banding is an exceptional necessity in infants with a precarious haemodynamic status and/or with multiple apical VSD.
BACKGROUND: Low osmolality contrast media are generally better tolerated than those with high osmolality. This report describes a case of severe collapse after injecting a low osmolality contrast medium. CASE REPORT: A 15 month-old girl with aortic arch abnormalities was admitted for investigation under general anesthesia. She had a history of allergy but had not previously been exposed to contrast medium. She was given ioxaglate (2 mg/kg) into an artery. The infusion immediately resulted in generalized erythema, tachycardia, hypotensive collapse and ECG abnormalities. The patient was immediately given adrenaline, vasopressive drugs, corticosteroids and plasma protein fraction, which led to a rapid recovery. The ductus arteriosus was ligated a few days later without complications despite the application of iodine to the skin and the use of anesthetic drugs. CONCLUSION: An unpredictable adverse reaction to low osmolality contrast medium can occur. Risk factors must be identified and immediate arrangements made for resuscitation.
Two brothers, aged 27 and 20 months, born from consanguineous healthy parents, presented with cardiomyopathy, lactic acidosis and carnitine abnormalities in serum and muscle, without clinical evidence of muscle involvement. The histochemical reaction for cytochrome c oxidase (COX) activity was negative in all muscle fibres, although the holoenzyme and subunits were present at a normal level, as shown by immunocytochemistry. The COX activity was, respectively, 5 and 25% of control values, in muscle biopsies. Partial deficiency of complex IV was confirmed in fresh isolated muscle mitochondria from patient 2 and was associated with a defect of complex I. Patient 1 died at age 3 yr 6 months. Partial improvement of cardiomyopathy in patient 2 was obtained under carnitine therapy, but seizures occurred and CT scan and magnetic resonance imaging (MRI) revealed thalamic hypodensity. Thus, the disorder appears to be progressive despite the clinical stabilization of the cardiomyopathy. This further demonstrates the complexity and clinical heterogeneity of combined respiratory chain complex deficiencies.
Cardiorespiratory adaptation of congenital atrioventricular block is not well known. The authors present the case of a 20 year old patient with excellent muscular force and maximal aerobic capacity despite severe chronotropic insufficiency on exercise, the heart rate not exceeding 80/mn. This condition may be explained by increased peripheral oxygen uptake and an increased oxygen transport due to high hemoglobin level.
A study was carried out to compare the haemodynamic and respiratory effects, as well as the quality of recovery, of anaesthesia with ketamine, sodium gamma-hydroxybutyrate (GOH) and etomidate in children undergoing cardiac catheterization. Thirty children, mean age 48 +/- 35 months, ranked ASA 2 or 3 on account of congenital heart disease, were assigned to one of three groups (n = 10). They were given: in group E1, a 0.3 mg.kg-1 bolus of etomidate, followed by 0.1 mg.kg-1.min-1 for 10 minutes, and 0.026 mg.kg-1.min-1 thereafter; in group G2, a 50 mg.kg-1 bolus of GOH, and in group K3, a 4 mg.kg-1 bolus of ketamine followed by a continuous infusion of 0.083 mg.kg-1.min-1. The patients breathed spontaneously. Monitoring included heart rate, systolic, diastolic and mean blood pressure, pulse oximetry, and capnography. Femoral venous or arterial catheterization was performed after local anaesthesia (with no more than 2 mg.kg-1 of lidocaine). Measurements were performed before induction, and then 1, 10, 30 and 60 minutes afterwards. The quality of anaesthesia was assessed according to Steward's scale. The investigation lasted between 50 and 100 min in all three groups. There were no significant differences in haemodynamic and respiratory parameters during the investigation between the groups. Recovery was shorter and of better quality in group E1. On the opposite, 30 minutes after the end of the catheterization, all the patients in group K3 were stuporous, with 5 of them displaying involuntary movements. The patients of the other two groups reacted correctly to stimuli, but those in group G2 went back to sleep very rapidly. There were no complications.(ABSTRACT TRUNCATED AT 250 WORDS)
Thirty-five patients aged 6 days to 18 years (average 7.5 +/- 5.2 years) were treated for an average period of 16 months (range 8 days to 50 months) with flecainide acetate at an average dose of 4.8 +/- 1.4 mg/kg (2.9 to 10 mg/kg) or 130 +/- 30.5 mg/m2 administered twice daily. The cardiac arrhythmia was a resistant paroxysmal junctional tachycardia due to a Wolff-Parkinson-White syndrome in 27 cases, intranodal reentry in 6 cases and a chronic reciprocating rhythm in 2 cases. Treatment was successful with complete suppression of the tachycardia in 24 cases. Partial success with a good clinical result was obtained in 4 cases and there were 7 failures, 6 due to inefficacy of the drug, and 1 because of an extracardiac secondary effect. One case of incessant junctional tachycardia was observed in a 9 month old child in whom the preexcitation disappeared. Atrioventricular preexcitation persisted in 20 out of 24 cases. The duration of the non-preexcited QRS complexes increased significantly from 73.6 +/- 13.8 to 82.2 +/- 15.2 ms; n = 14, p less than 0.01. The minimal effective plasma concentration was 347 +/- 147 ng/ml. The plasma concentration/dose ratio of children over 4 years of age was the same as in adults. It was significantly higher in babies and infants suggesting a progressive acquisition of the capacity to metabolise flecainide during the first year of life. In conclusion, flecainide acetate was easy to use with respect to administration and follow-up, and seems to be a drug of choice for the treatment of junctional tachycardia in children.
A case of symptomatic splenic infarct in an 11 year-old girl suffering from bacterial endocarditis is reported; this occurrence is a rare and serious condition in this disease. The diagnosis of haemorrhagic splenic infarct was suggested by ultrasonography and confirmed by MRI. No splenic abscess developed and this was correlated to a rapid improvement of the clinical state following an antibiotic treatment only. The authors discuss the role of sonography and MR in the etiologic diagnosis of splenic involvement in patients suffering from bacterial endocarditis.
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