[Heart transplantation in children: today and tomorrow].
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Child↗
Biomedical subjects
J Kachaner
Publications and source records attributed to J Kachaner.
[Artificial cardiac stimulation in the newborn infant with complete congenital atrioventricular block. Study of 16 cases].
This is a retrospective study of 16 children with congenital complete atrioventricular block (CAVB) who were fitted with a pacemaker in infancy. All were neonates admitted at the age of 1 to 9 days for bradycardia; 3 had a cardiopathy. In 8 children a permanent pacemaker was implanted in the first two days of life on account of a heart rate slower than 50 beats/min, accompanied with threatening symptoms (heart failure or syncopes) in 4 cases. In 6 children the pacemaker was implanted at the age of 2 to 3 months; in spite of reassuring electrocardiograms, 5 of them were readmitted in an emergency for heart failure or syncope with slow heart rate; the 6th patient had disorders of ventricular excitability. Finally, 2 asymptomatic infants underwent pacing: one at 20 days for bundle branch block, the other at 6 months for slow phase abnormalities. Pacing was epicardial in all patients, the chamber being positioned in the space that separates the kidney from the parietal peritoneum. Ventricular synchronous pacing (VVI) was applied in 14 cases and atrioventricular pacing (DDD) in the two most recent cases. Two children died post-operatively, due to inadequate attachment of the electrode resulting in loss of ventricular capture in one case, and to extensive left atrial thrombosis in the other case. Two children died at a later stage of severe respiratory pathology. The 12 survivors were followed up for a mean period of 3.7 +/- 3.1 years. Three pacemakers were replaced: one at 28 months for infection, the others at 3 and 6 years respectively for running down of the batteries.2 +
Cardiac Pacing, Artificial↗
[Aptitude for sports in children operated on for aortic coarctation. Contribution of the exercise test].
Pronouncing on the fitness of children operated upon for coarctation of the aorta to practise sports, as well as on the nature and level of this activity, depends, to a great extent, upon their blood pressure profile at exercise (BPE) as measured by an exercise test on an ergometric bicycle. We studied thi profile in 43 children aged 11.7 +/- 2.5 years who had undergone surgery for coarctation of the aorta (isolated in 93 p. 100 of the cases) at the age of 3.8 +/- 3.2 years Systolic arterial pressure (SAP) in the upper limbs was normal at rest in 80p. 100 of the children but reached abnormal values at exercise in 81 p. 100 and even exceeded 200 mmHg in 16 p. 100. Diastolic arterial pressure (DAP) in the upper limbs was sometimes slightly elevated at rest, but it clearly decreased at exercise and became normal in all cases. This suggested that systolic hypertension at exercise was due to low peripheral resistance and either to an exercise-induced isthmic gradient or to a poor vascular impedance. As a result of this study, we warn such children against playing sports with a strong static stress (e.g. martial sports) or with a risk of thoracic trauma (e.g. rugby). For the other sports, we make no reservation when the BPE is normal, and we forbid competitions when the BPE is abnormal but the SAP at exercise remains below 200 mmHg. Beyond this value, children must be investigated for residual coarctation of the aorta which may be amenable to a specific treatment.
Adolescent↗
[Atresia of the left coronary ostium. Repair in a 2-month-old infant].
A case of atresia of the left coronary ostium revealed by neonatal heart failure is reported. The initial diagnosis was anomalous origin of the left coronary artery from the pulmonary artery. At surgery performed in this 6-week old infant the diagnosis was amended and the malformation was repaired. Soon after the operation the child rapidly developed hypertrophic "myocardiopathy" of the left ventricle. Seven and a half months later, he is asymptomatic and the echocardiographic parameters of left ventricular systolic function are gradually returning to normality. Atresia of the left coronary ostium is an exceptional anomaly which must be considered, together with the other anomalous origins of the left coronary artery, when confronted with a case of severe heart failure caused by coronary ischaemia during the first months of life. The diagnosis rests on opacification of the coronary network during cardiac catheterization. Coronary "revascularization" may be performed either by aortocoronary bypass or by anatomical repair of the malformation.
Angiocardiography↗
Anomalous left coronary artery arising from the pulmonary artery in infancy: is early operation better?
From January 1975 to January 1987, 21 consecutive infants aged less than six months (mean (SD) 2.6 (1.2] were admitted with anomalous origin of the left coronary artery from the pulmonary artery. In the first 12 patients, who were seen up to 1982, operation was performed after the age of one year (mean (SD) 29 (29) months) (group 1). The next nine infants, seen from 1983 to 1987, had their operations within a few weeks of the onset of symptoms (mean (SD) age 4.8 (1.4) months) (group 2). In group 2 the left coronary artery was relocated into the aorta, whereas in group 1 there was additional resection of the left ventricular wall or mitral valvoplasty or both. At presentation there were no differences in age, clinical condition, heart enlargement, and echocardiographic left ventricular dysfunction between groups 1 and 2. Seven of the 12 patients in group 1 died, five while they were awaiting operation (three died suddenly at home) and two at operation. The five survivors are doing well 6.4 (3.1) years after operation with normal left ventricular function which improved slowly over several months after operation. Two of the nine patients in group 2 died; both deaths occurred at or soon after operation. The seven survivors are doing well 1.8 (0.9) years after operation. In three, left ventricular function recovered within three weeks; and there was even partial or total regression of the Q waves in the supposedly necrotic areas. In the remaining four the pattern of improvement in left ventricular function resembled that in group 1. Operation should be undertaken early in infants with anomalous left coronary artery arising from the pulmonary artery because the procedure is relatively safe, prevents a high natural mortality, and offers a better chance of a faster recovery of left ventricular function.
Age Factors↗
Fate of pulmonary artery after anatomic correction of simple transposition of great arteries in newborn infants.
From April 1984 to April 1987, surgical anatomic correction was performed in 86 newborn infants, 2-23 days old (6.8 +/- 3.6 days, mean +/- SD) with simple transposition of the great arteries. In all patients, the pulmonary artery was reconstructed by end-to-end anastomosis according to the Lecompte maneuver, including eight patients with side-by-side position of the great arteries. Three different approaches were used. In the first 10 patients (group 1, six survivors), two separate patches of preserved tanned pericardium were used to reconstruct the pulmonary artery, whereas in the next 15 patients (group 2, 13 survivors), a single patch of the same material was used, and in the last 61 patients (group 3, 56 survivors), surgery was performed with a single patch of fresh autologous pericardium. Among the 75 survivors, 68 (including six in group 1, 12 in group 2, and 50 in group 3) were followed serially for at least 6 months (6-48 months, 26 +/- 9 months) with sequential noninvasive evaluations. At follow-up, all were asymptomatic with normal growth. Two patients with severe pulmonary artery stenosis (group 1) were successfully reoperated on. Four infants with moderate pulmonary artery stenosis have been followed medically and have had stable right ventricular pressures. The last 62 patients have normal or near-normal right ventricular pressures. The spatial relation of the great arteries did not affect the quality of the results. Group 1 had clearly the worst results.(ABSTRACT TRUNCATED AT 250 WORDS)
Anastomosis, Surgical↗
[Organ transplantation in children].
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Child↗
[Cardiac transplantation in the infant and young child. Preliminary results].
Between January and December, 1987, a programme of heart transplantation in paediatrics was designed and carried out in 9 children by the medical and surgical teams of the Necker/Enfants Malades-Laënnec hospitals group, Paris. Six of the patients were infants of less than 2 years (4 were under one year), and the oldest child was 10 years old. All patients seemed to be condemned to an early death either because their congenital heart disease was beyond the resources of conventional surgery (6 cases) or because their dilated cardiomyopathy was refractory to all medical treatments. Three children died at the end of the operation or a few days afterwards, due to poor quality graft (1 case), fulminating bacterial superinfection (1 case) or intractable pulmonary hypertension (1 case). The remaining 6 children are now living as normally as possible in their respective families. The long-term immunosuppressive treatment consists of cyclosporine and azathrioprine; corticosteroids are only used at the very beginning of treatment or in case of graft rejection. Only two episodes of rejection, confirmed by endomyocardial biopsy, were observed in the same patient during the first postoperative month. Biopsy was never performed systematically in order to spare the patient's vein, and the diagnosis of rejection was suspected on clinical grounds.(ABSTRACT TRUNCATED AT 250 WORDS)
Biopsy↗
[Development of aortic valve stenosis in infants and children. Apropos of 66 cases hospitalized from 1973 to 1984].
Sixty-six children aged over 6 months hospitalized for isolated aortic valve stenosis without cardiac failure but with a transvalvar systolic pressure gradient of more than 40 mmHg were followed up and re-evaluated for a mean period of 5.4 +/- 3.5 years. Twenty-two children who were asymptomatic, had no ST-T changes and had a less than 60 mmHg gradient were not operated upon; they remained stable over a mean period of 5.4 +/- 3.4 years; their gradient was not significantly modified (53 instead of 51 mmHg) and none of them underwent surgery. Forty-four children with symptoms and/or a more than 60 mmHg gradient underwent valvotomy under extracorporeal circulation without mortality. Surgery was effective against the major symptoms in all cases, and it reduced the gradient from 75 +/- 25 to 36 +/- 18 mmHg at the expense of aortic regurgitation in 7 cases, 2 of them with significant haemodynamic repercussions. During the follow-up period (5.8 +/- 3.7 years) 1 patient died of bacterial endocarditis, 5 were reoperated upon (with prosthesis in 4) without mortality and with 4 good results, and 1 patient underwent valvoplasty which proved partially effective but resulted in moderate aortic regurgitation. All other children are now doing well; 29 are completely asymptomatic and without ST-T changes at rest or during exercise. We conclude that aortic valve stenoses with moderate gradient are very stable at mid-term and that surgical valvotomy is a generally effective and low-risk procedure.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
[Heart and heart-lung transplantation in the child. Indications, methods and results].
Over a 20 month period, 19 children aged 13 days to 15 years (10 under 3 years), received a heart transplantation (isolated: 16 cases, heart-lungs: 3 cases). There were 5 early deaths and 2 during the 3rd postoperative month (total: 44%). Postoperative period is currently over for 8 of the 9 survivors, who lead a normal life, with rather simple constraints: oral cyclosporin-azathioprine immunosuppressive therapy, monthly evaluation in the out-patient department. The method is relatively simple when it concerns heart transplantation. It is quite heavier and complex when it concerns heart-lung transplantation. On the whole, subject to a rigorous selection of the transplant (whose later dysfunction was the major cause of failure, with bacterial or viral infections), these methods find reasonable indications in a number of heart and/or lung malformations or dysfunctions inaccessible to conventional treatments or which used up their resources, making short-term death likely.
Adolescent↗
[Practical aspects in the surveillance of heart and heart-lung grafts in children].
The short and middle term survivors of the 19 transplanted children (heart and heart-lungs) of our program are treated with an association of cyclosporine and azathioprine. The detection of rejection is the main worry and relies on endomyocardial biopsy, but this investigation may be dangerous and lowers the available veins of smaller children. Thus, we use it only for the situations of clinical suspicion (18 times in 20 months in 8 children, which proved rejection 6 times in 3 patients). Renal function supervision is careful and comprises systematic renal biopsy; tubulo-interstitial lesions were constant: minimal 3 times, moderate 3 times. This prompts to prescribe the lowest possible cyclosporine dosages which ensure an effective residual blood level (100-300 ng/ml). In case of heart-lung transplantation (3 cases), pulmonary rejection is difficult to prove, opportunistic infections more frequent and severe, and the tracheal suture is responsible for complications (stenosis in 2 cases). By and large, the constraints of supervision are reasonable and allow an almost normal life.
Child↗
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.
Coronary Vessels↗
Restrictive cardiomyopathy in children. Ultrastructural findings.
Restrictive cardiomyopathy is usually related to fibrosis of the endocardium or to an infiltrative disorder. However, in few cases, it can be due to isolated pathology of the myocytes but such alterations are not well characterized. This paper reports the disease in two 7 year old patients. There was severe venous congestion and catheterisation revealed increased end diastolic pressure in the ventricles. Both pericardial and myocardial biopsies were performed, as the clinical and haemodynamic data were indistinguishable from constrictive pericarditis. The structure of the pericardium was normal. The endocardium was not thickened. The interstitium of the myocardial tissue was not increased. Electron microscopic examination revealed intracellular masses of disorganized myofilaments. These large deposits may have produced decrease compliance of the myocytes and of the ventricular walls.
Biopsy↗
Pulmonary artery "stop-flow" angiography to visualize the anomalous origin of the left coronary artery from the pulmonary artery in infants.
In five infants, anomalous left coronary from the pulmonary artery (ALCA) was diagnosed by selective pulmonary artery angiography using the "stop flow" technique: the left pulmonary artery was occluded by inflating a balloon and a large bolus of dye was injected proximal to the balloon at a high flow velocity. These conditions, by increasing pulmonary artery pressure, increase the chances of imaging directly an ALCA. It also leaves the left pulmonary artery branches free of contrast medium and allows easier visualization of ALCA, mainly in the antero-posterior view. When an ALCA is injected from the pulmonary artery, left heart catheterization can be avoided. However, a false-negative result is to be expected in specific conditions and was observed in one of our patients. If pulmonary angiography fails to prove a ALCA, left heart catheterization remains mandatory to exclude the diagnosis.
Angiography↗
Anatomic correction of simple transposition of the great arteries in 50 neonates.
From April 1984 to January 1986, anatomic surgical correction was performed in 50 newborn (2- to 23-day-old, mean 8 +/- 5 [SD]) infants with simple transposition of the great arteries. Before surgery, balloon atrial septostomy was performed in all patients, prostaglandin E1 was infused in 42, and left ventricular shape on a two-dimensional echocardiogram was considered "satisfactory" in 48. Surgery was performed in patients on cardiopulmonary bypass without cardiac arrest; the pulmonary artery was reconstructed by end-to-end anastomosis according to Lecompte's maneuver with a pericardial patch. In all but one patient coronary artery transfer was possible regardless of the distribution of these vessels. There were eight early deaths (16%), but only four (10%) of the last 41 patients treated died. There was one late death (2%) due to a secondary myocardial infarction caused by compression of the left coronary artery. Reoperation was successfully performed in two patients for supravalvar pulmonary artery stenosis. The only late medical complication was a transient episode of myocardial ischemia 6 months after surgery. The 41 late survivors were in excellent condition, were in sinus rhythm, and had a normal left ventricle 1 to 22 months after surgery (mean 7.2 +/- 5.4 [SD]). Aortic growth was normal; pulmonary artery supravalvar stenosis occurred in six patients (mild in four). We conclude that anatomic correction can be applied successfully in the first few days of life in newborns with simple transposition of the great arteries, regardless of coronary distribution.
Cardiopulmonary Bypass↗
[Problems posed by percutaneous pulmonary valvuloplasty in children].
Percutaneous valvuloplasty with a balloon catheter was attempted in 41 young patients with pulmonary valve stenosis. There were 9 neonates (1 to 7 days), 6 infants (1.5 to 7 months) and 26 children (16 months to 17 years). The attempt failed in 8 cases, either because the balloon could not be inserted due to intravenous progression problems or to quasi-atresia of the orifice, or because serious accidents (air embolism in one patient, perforation of the infundibulum in another) forced us to abandon the procedure before dilatation. Failures mostly occurred in very young children: 7 out of 15 aged less than 1 year, 1 out of 26 older than 1 year. Dilatation was effective in 33 cases. Among 13 patients who had moderate stenosis with infra- or iso-systemic right ventricular systolic pressure (RVP) (mean: 70 +/- 13 mmHg) and modest ventriculo-pulmonary gradient (49 +/- 12 mmHg), there was only 1 failure due to coexistent biventricular myocardiopathy; success was immediate and complete in 8 cases, partial in 4 cases. Among 20 children presenting with tight stenosis with supra-systemic RVP (164 +/- 39 mmHg) and high ventriculo-pulmonary gradient (140 +/- 43 mmHg), there were 5 failures; success was partial in 8 cases, complete and immediate in 7 cases. Accentuation of the infundibular stenosis after dilatation was a frequent cause of failure, as we have shown by calculating the infundibular "reactivity index" on pre- and post-operative angiocardiograms. In such cases one may hope for spontaneous improvement, as was indeed subsequently observed in 2 of our patients.(ABSTRACT TRUNCATED AT 250 WORDS)
Adolescent↗
[Left ventricular function and cardiovascular adaptation to exercise in young sportsmen].
In order to find out whether the expression "sports heart" can be used in children and if so, to give an objective definition of it, we studied a group of 40 young people (mean age 12.2 +/- 1.6 years) subjected to intensive training and practising sports regularly, i.e. about 12 hours of physical activity per weeks on average. The sports practised were mainly tennis among boys and ice-skating among girls. A group of 40 subjects with similar characteristics but no particular physical training was studied simultaneously and served as control. These 80 young people had normal heart structure. All underwent routine cardiovascular examinations (physical, radiological, electrocardiographic), complete one - and two - dimensional echocardiography to evaluate left ventricular function and an exercise-test on ergometric bicycle. No difference was found between the left ventricle of sportsmen and that of controls; in particular, there was neither dilatation nor parietal thickening in sportsmen, so that all functional values were the same. During exercise-tests sportsmen performed better than controls, with significantly longer duration of effort and greater maximal oxygen consumption. However, heart rate at rest, then during exercise and recovery, and variations in systolic blood pressure during exercise and in the 10 minutes which followed were identical in both groups. The only notable difference was a deeper fall in diastolic blood pressure in sportsmen, indicating a better opening of the peripheral vascular bed during exercise. It is concluded that there are few cardiovascular differences between trained sporting children and children with normal physical activity, probably because the relatively short training undergone does not leave sufficient time for most of the adaptation systems to become established.
Adolescent↗
[Non-surgical treatment of valvular stenoses in pediatric cardiology: percutaneous valvuloplasty].
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Aortic Valve Stenosis↗