Postnatal outcome following fetal aortic valvuloplasty for critical aortic stenosis.

OBJECTIVE: To report our experience of fetal aortic valvuloplasty (FAV) for critical aortic stenosis (AS), with a focus on the postnatal evolution of the patients. METHODS: This was a retrospective study including all fetuses with critical AS which underwent FAV in a single center between January 2011 and June 2022. FAV was performed under ultrasound guidance. Technical success was based upon balloon inflation across the aortic valve and improvement of the antegrade aortic flow across the aortic valve. At birth, a biventricular circulation (BVC) strategy was decided assuming the left ventricular (LV) systolic and diastolic function would ensure the systemic circulation. RESULTS: Sixty-three FAV procedures were performed in 58 fetuses, at a median (range) gestational age of 26.2 (20.3-32.2) weeks. The procedure was technically successful in 50/58 (86.2%) fetuses. There were 11/58 (19.0%) cases of in-utero demise and 9/58 (15.5%) terminations of pregnancy. No patient was liveborn after an unsuccessful procedure. Thirty-eight (65.5%) infants were liveborn, at a median (range) gestational age of 38.1 (29.0-40.6) weeks, of whom 21 (55.3%) required prostaglandin treatment. Twenty-eight of the 38 (73.7%) liveborn children (48.3% of the study population) entered the BVC pathway at birth. Among them, 20 (71.4%) required an aortic valvuloplasty procedure at birth (11 (55.0%) percutaneous balloon, nine (45.0%) surgical) and eight (28.6%) did not require any treatment at birth, but, of these, five (62.5%) underwent surgical valvuloplasty between day 26 and day 1200 of age. Eleven (39.3%) of the infants with BVC at birth required a second intervention and four (14.3%) of them required a third intervention. Two (7.1%) infants who entered the BVC pathway at birth underwent conversion to univentricular circulation (UVC). None of the surviving children with BVC developed pulmonary hypertension. The overall survival rate in those with BVC at birth was 22/28 (78.6%) at a median (range) follow-up of 23.3 (2.0-112.6) months. Ten of the 58 (17.2%) patients had UVC at birth. Among these, six (60.0%) received compassionate care from birth and four (40.0%) underwent surgery. Three of the 10 patients who had UVC at birth were still alive at the latest follow-up assessment, at a median (range) gestational age of 24.3 (8.3-48.7) months. CONCLUSIONS: FAV for critical AS led to increase of antegrade aortic flow in 86.2% of fetuses, with BVC being achieved in 48.3% (73.7% of the liveborn cases). Among patients with BVC at birth, the rate of reintervention was high, but 78.6% of these children were alive at the latest evaluation. © 2024 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

Pulmonary stenosis in complicated monochorionic twin pregnancies: prevalence, management and outcome.

OBJECTIVE: To review prevalence, management and prognostic factors of pulmonary stenosis (PS) in monochorionic diamniotic (MCDA) pregnancies complicated by twin-to-twin transfusion syndrome (TTTS). METHODS: Retrospective study over the last 10 years in a single referral center. We reviewed fetal echocardiography data of all MC twin cases with diagnosis of isolated PS. We assessed fetoscopy characteristics of those that underwent laser coagulation. We collected data regarding perinatal outcome, neonatal echocardiography and cardiac management. RESULTS: We found 24 cases of isolated PS among 2091 MCDA pregnancies. Among 1052 complicated MCDA that underwent fetal laser surgery, 22 (2.09%) developed PS of which 20 were diagnosed prenatally. Two cases were diagnosed in uncomplicated MCDA pregnancies (0.2%). Four of 22 (18.18%) cases with TTTS showed in utero regression after laser treatment. Thirteen newborns (65%) required valvular dilatation. Peak systolic velocities in the pulmonary artery trunk (PSV-PA) at diagnosis and the interval between the diagnosis of TTTS and that of PS were significantly different (p < 0.001 and p = 0.05 respectively) between PS requiring cardiac intervention and those who did not. CONCLUSION: An elevated PSV-PA at the time of PS diagnosis and a short time-interval between fetoscopic laser surgery and PS diagnosis are predictive of the need for interventional treatment after birth.

Clinical course and outcome of antenatally detected atrioventricular block: experience of a single tertiary centre and review of the literature.

OBJECTIVE: The objective is to study the course and outcome of fetuses with congenital atrioventricular block (AVB) in a single centre. METHODS: Retrospective analysis of cases diagnosed prenatally with second and third degree AVB. The clinical characteristics and outcome of fetal AVB were evaluated including in utero treatment. RESULTS: Sixty-two cases were studied. AVB was associated with a congenital heart defect (CHD-AVB) in 17 cases (27%), whereas it was isolated (i-AVB) in 45 (73%), 42 of which were associated with maternal antibodies. There were nine (52.9%) live births in the CHD-AVB group, five of which (55%) resulted in infant deaths. In the i-AVB group, there were 40/45 (88.9%) live births and 1/40 (2.5%) infant death; 36 (90%) babies required a permanent pacemaker. The only factor predictive of postnatal death was the presence of CHD (5/9 vs 1/39 or 48.7 [3.6; 1457.7], p < 0.001). Nineteen fetuses (40.5%) with i-AVB received steroids in utero. No difference in outcome was found between the AVB treated in utero versus the no-treatment group in terms of permanent pacemaker placement, postnatal death or development of dilated cardiomyopathy. CONCLUSION: The most important prognostic factor for congenital AVB is the association with CHD. In utero treatment remains questionable.

Prenatal diagnosis of cardiac rhabdomyomas: incidence of associated cerebral lesions of tuberous sclerosis complex.

OBJECTIVES: To determine the prevalence of specific cerebral lesions of tuberous sclerosis complex (TSC) and neurological outcome in cases diagnosed prenatally with cardiac rhabdomyomas. METHODS: We reviewed all fetuses diagnosed prenatally with cardiac rhabdomyomas which had undergone detailed ultrasound evaluation and cerebral magnetic resonance imaging (MRI) and which were recorded in the database of a single institution covering the period January 1992 to December 2005. RESULTS: Fifty-one fetuses were included in the study. MRI was performed at a mean +/- SD gestational age of 30 +/- 3 gestational weeks and showed specific lesions of TSC in 49% of cases. Termination of pregnancy was chosen by the parents in 26 cases. Neurological development was studied in 20 cases, follow-up lasting 4.8 +/- 2.9 years. Neurodevelopmental events occurred during the follow-up period in 45% of cases. Neurological complications occurred in 67% of patients who had cerebral lesions at MRI and in 33% of patients with normal MRI results. There was no significant difference between the two groups of patients (P = 0.2). CONCLUSION: In fetuses with cardiac rhabdomyomas detailed ultrasound examination and third-trimester cerebral MRI are able to diagnose most TSC cerebral lesions, but fail to determine neurological outcome.

[Is antenatal diagnosis of coarctation of the aorta possible?]. / Le diagnostic anténatal de la coarctation de l'aorte est-il possible?

Antenatal diagnosis of coarctation of the aorta is difficult but primordial because it reduces the mortality due to this malformation by early treatment of the neonate. Echocardiography allows identification of groups at high risk but does not predict with certainty the constitution of a coarctation after birth. The authors review their experience of 202 foetus at risk of coarctation. Of the 167 known live births, 19% finally developed a coarctation. The predictive factors of coarctation were early diagnosis in the second trimester of pregnancy, a ratio of pulmonary artery diameter to aortic diameter greater than 1.6, the presence of a left superior vena cava and bicuspid aortic valve, the diagnosis of which is more often postnatal but which enables prediction of coarctation with nearly 90% accuracy when ventricular asymmetry has been identified at an early stage. It is therefore important to look for these echocardiographic signs which are an aid to antenatal diagnosis of coarctation and better identify subjects at risk. In fact, the finding of ventriculo-arterial asymmetry alone leads to the taking of unnecessary precautions in 80% of cases and anguish to parents who end up with a baby with a normal heart.

[Prognosis of atrioventricular canal in euploid foetus without abnormality of atrial situs]. / Pronostic des canaux atrioventriculaires chez les foetus euploïdes sans anomalie du situs atrial.

Atrioventricular septal defects are commonly diagnosed during fetal life. Postnatal prognosis of atrioventricular septal defects associated with trisomy 21 and with heterotaxia sequences are relatively well known. However, predicting postnatal outcome in fetus with atrioventricular septal defects and normal chromosome and normal atrial situs remains a challenge. In a series of 141 fetal atrioventricular septal defects, we analyzed 80 fetuses with normal karyotype. Twenty-seven had an abnormal atrial situs. One fetus was lost for follow-up. Finally, 52 fetuses were included in the study. Termination of pregnancy was performed in 18 cases (34%). Six fetuses died in utero (18% of ongoing pregnancies). Twenty eight infants were born alive, 2 of them were lost for follow-up right after birth and 3 live born infants died postanatally (11%). Postoperative mortality was 3/15 (20%). Complete repair was proceed for 13 infants, palliative repair for 2; and 8 infants didn't have surgery at the end of follow-up because of partial or intermediate atrioventricular septal defect. The only factor significantly associated with poor outcome was the small size of the left ventricle. Isolated atrioventricular septal defects are of poor cardiac prognosis particularly when associated with left heart obstructions.

[Neonatal lupus syndrome: Literature review]. / « Lupus néonatal ¼ : revue de la littérature.

Neonatal lupus syndrome is associated with transplacental passage of maternal anti-SSA/Ro and anti-SSB/La antibodies. Children display cutaneous, hematological, liver or cardiac features. Cardiac manifestations include congenital heart block (CHB); endocardial fibroelastosis and dilated cardiomyopathy. The prevalence of CHB in newborns of anti-Ro/SSA positive women with known connective tissue disease is between 1 and 2% and the risk of recurrence is around 19%. Skin and systemic lesions are transient, whereas CHB is definitive and associated with significant morbidity and a mortality of 18%. A pacemaker must be implanted in 2/3 of cases. Myocarditis may be associated or appear secondly. Mothers of children with CHB are usually asymptomatic or display Sjogren's syndrome or undifferentiated connective tissue disease. In anti-Ro/SSA positive pregnant women, fetal echocardiography should be performed at least every 2 weeks from the 16th to 24th week gestation. An electrocardiogram should be performed for all newborn babies. The benefit of fluorinated corticosteroid therapy for CHB detected in utero remains unclear. Maternal use of hydroxychloroquine may be associated with a decreased recurrent CHB risk in a subsequent offspring. A prospective study is actually ongoing to confirm these findings.

Treatment of central nervous system B lymphoproliferative syndrome by local infusion of a B cell-specific monoclonal antibody.

A 9-month-old infant developed Epstein-Barr virus-induced lymphoproliferative syndrome with mediastinal and central nervous system localizations, associated with mediastinal tuberculosis, 5 months after heart transplantation. As a combination of anti-B cell antibodies (CD21- and CD24-specific) and recombinant interferon alpha 2b, given intravenously, was not effective on the central nervous system disease, the anti-CD21 antibody was infused intrathecally via an Ommaya reservoir. High local concentrations of monoclonal antibodies were achieved, with no adverse effects. A dramatic clinical response was obtained, with clearance of abnormal cells from the cerebrospinal fluid and a clear reduction in the abnormalities on the brain images. The patient is well 7 months later. This observation indicates that treatment of B lymphoproliferative syndrome with central nervous system localization is feasible using a nontoxic, local B cell-specific approach.

Donor-recipient size matching in pediatric heart transplantation: a word of caution about small grafts.

BACKGROUND: The increased shortage of donor organs led centers to extend the conventional donor criteria, particularly regarding donor-recipient size mismatching. Little information is available in the pediatric age group. METHODS: Between December 1987 and May 1994, 73 pediatric orthotopic heart transplantations were performed. Indications for heart transplantation included congenital heart defect (29 patients), cardiomyopathy (40 patients), valvular disease (1 patient), and retransplantation (3 patients). Patients ranged in age from 9 days to 18 years (mean: 5.7 +/- 5 years). The following factors were evaluated: cardiomyopathy, congenital heart disease, age, pretransplantation pulmonary hypertension, previous sternotomy, status at transplantation, donor to recipient weight ratio, graft ischemic time, degree of donor inotropic support, ABO compatibility, gender mismatch. RESULTS: The overall mortality rate before discharge (7 days to 4.5 months) was 27.4%. Donor heart failure occurred in 31.5%. Donor heart failure and early mortality were strongly correlated (p = 0.0002). Risk factors for donor heart failure were pretransplantation pulmonary hypertension (p = 0.024), donor/recipient ratio (p = 0.033), and major donor inotropic support (p = 0.034). Donor heart failure rate was 50% in donor/recipient ratio less than 1, 33% in donor/recipient ratio between 1 and 1.6, and 7% in donor/recipient ratio more than 1.6. Donor/recipient ratio less than 1 was the only significant risk of postoperative death by univariate (p = 0.0045) and multivariate (p < 0.01) analysis. CONCLUSIONS: Donor heart failure remains the main cause of early mortality in pediatric heart transplantation. The use of oversized donor may be beneficial, particularly in patients with pretransplantation pulmonary hypertension. The use of undersized donor grafts should be strongly discouraged.

Pediatric cardiac transplantation for congenital heart defects: surgical considerations and results.

Among 54 children who underwent 55 heart transplantations, 24 (44%) (mean age, 4.9 +/- 4.8 years; range, 9 days to 18 years) had congenital defects with the following diagnoses: single-ventricle variants (6), hypoplastic left heart syndrome variants (5), transposition complex (6), and miscellaneous defects (7). Twenty patients (83%) had undergone 43 prior operations. Additional surgical procedures included repositioning of transposed great arteries (11), reconstruction of the aortic pathway (4), reconstruction of the pulmonary pathway (8), correction of situs inversus (1), and correction of anomalous pulmonary (1) or systemic (1) venous drainage. Reconstructive procedures were performed using donor or recipient tissue or both. There were six early deaths (hyperacute rejection, 1 patient; pulmonary hypertension, 1; graft failure, 2 patients; infection, 2) and six late deaths (sudden death, 2; chronic rejection, 2; nonspecific graft dysfunction, 1; lymphoproliferative disease, 1). The survival rate was 43% +/- 12% at 3 years. No deaths were related to surgical technique. Survival was not significantly different in pediatric recipients with cardiomyopathy (67% +/- 9%; p = 0.22). Accelerated coronary artery disease was noted in 4 operative survivors (22%; 70% confidence limits, 12% to 36%). All late survivors were free from cardiac symptoms after a mean follow-up of 34 +/- 24 months (range, 6 to 71 months). Based on this study, we reached three conclusions. (1) Careful planning of both harvesting and transplantation procedures allows heart transplantation in recipients with congenital heart diseases. (2) The surgical technique may be demanding, but the early risk is not increased.(ABSTRACT TRUNCATED AT 250 WORDS)

Heart- and heart-lung transplantation in children.

Since January 1987, 16 prepubertal children have undergone heart (13) or heart-lung (3) transplantation. Immunosuppression included cyclosporine and azathioprine and excluded steroids except in case of rejection. The indications for heart transplantation were hypoplastic left heart syndrome (4 infants, mean age = 2 months), congenital heart disease (4 patients, mean age = 5.7 years) and cardiomyopathy (5 patients, mean age = 2.8 years). There were 4 early deaths (acute graft failure in 2, pulmonary hypertension in 1, infection in 1) and 1 late death (heart failure at 3 months). The 8 survivors had a mean follow-up of 12 months (range 1-19 months). Late complications were minimal. There were 4 episodes of rejection in 2 patients. There was no infection, normal somatic growth and no systemic hypertension. Renal function remained within normal limits although mild-to-moderate tubulointerstitial lesions were found in 4 renal biopsies. Three children (9-11 years old) underwent heart-lung transplantation. The early postoperative course was difficult with 6 episodes of rejection and 5 infections. One patient died at 3 months from infectious complications. One child has a complete rehabilitation 8 months posttransplantation. The last patient is clinically well at 7 months but has a residual tracheal stenosis. The long-term fate of these children, and particularly the long-term effects of cyclosporine therapy are unknown. Heart and heart-lung transplantation remain under investigation but may be reasonable approaches for infants and children with end-stage cardiac and/or pulmonary disease.

Heart transplantation in children: risk factors of early and late mortality.

In order to identify predictive risk factors of poor outcome following heart transplantation in children, we performed a retrospective analysis of our pediatric recipient population: 31 children, aged 15 days to 15 years (mean = 5.2 +/- 4.9 years). The preoperative diagnosis was cardiomyopathy in 17 (55%), congenital heart disease in 13 (42%) and end-stage valvular disease in 1 (3%). There were 5 operative deaths: hyperacute rejection (2), low cardiac output syndrome (3); 4 in-hospital deaths: infection (2), multiorgan failure (2) and 4 late deaths: acute rejection (1), chronic rejection (1), lymphoma (1), unknown (1). The actuarial probability of survival (+/- SE) was 62% +/- 10% at 1 year and 53% +/- 12% at 2 years. Univariate analysis was used to evaluate the following risk factors: age, diagnosis, hemodynamic decompensation, previous cardiac surgery, ischemic time of the graft, technique of graft preservation, preoperative pulmonary artery pressure, occurrence of postoperative low cardiac output syndrome (LCOS) with pulmonary hypertension (PHT). The occurrence of early LCOS with PHT significantly increased both early and late mortality (78% early mortality, 100% overall mortality). This syndrome occurred in 9 patients (29%) and was attributed to primary graft failure in 2, increased pulmonary vascular resistances in 6 and multiple factors in 1. Although not significant, two factors may increase early survival: young age (less than or equal to 1 year) at operation and improved technique of graft preservation.

[Cardiac retransplantation in childhood. Questions and answers]. / Retransplantation cardiaque en pédiatrie. Interrogations et réponses.

Cardiac transplantation in childhood remains a long-term palliative procedure and it is probable that an increasing number of patients undergoing this procedure in childhood will require a new transplant one day. Coronary artery disease of the transplanted heart is the main indication. The results (in terms of survival and morbidity) in the literature and in the authors' experience are encouraging and justify the indication for retransplantation in cases of severe coronary disease of the transplanted heart. The indication is not as clear-cut in transplantation performed early or as an emergency for primary graft dysfunction, which carries a high mortality. Renal failure and allo-immunisation are not contra-indications to this procedure. Occasionally, procedures of coronary revascularisation provide time to wait for a new transplant. In the absence of more effective preventive or curative treatment of coronary disease of the transplant, the good results of retransplantation justify the procedure for this indication and reinscription on the waiting list for cardiac transplantation.

[Artificial cardiac stimulation in the newborn infant with complete congenital atrioventricular block. Study of 16 cases]. / Stimulation cardiaque artificielle chez le nouveau-né atteint de bloc auriculo-ventriculaire complet congénital. Etude de 16 cas.

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 +

[Surgical treatment of complete atrioventricular canal in early infancy]. / Traitement chirurgical du canal atrioventriculaire complet dans la première enfance.

Ninety children with complete atrioventricular canal without any significant associated abnormality underwent surgery between 1973 and 1982. The first group of 26 infants, including 7 with trisomy 21 (average age 5.3 +/- 3.8 months) had severe pulmonary hypertension and underwent pulmonary artery banding: 10 died in the immediate or early postoperative period (38 p. 100). The mortality was 50 p. 100 in children under 6 months of age. In two cases, the banding was a failure because it was ineffective or "vicious" and both patients had to undergo reoperation 2 to 6 months later for total correction which resulted in 1 death and 1 poor result; the surviving 14 patients were improved: 3 are waiting for total correction under good conditions and 11 underwent open heart surgery 36 +/- 16 months after banding with 7 good results and 4 average results because of residual significant mitral regurgitation associated in one case with atrioventricular block requiring pacing. The overall results of this group were 42 p. 100 mortality, 19 p. 100 average results and 27 p. 100 good results; 12 p. 100 are waiting for surgery. The second group included 64 children, with 34 with trisomy 21 (average age 17.4 +/- 16.7 months) who underwent complete correction straight away (before 2 years in 50 cases, before 1 year in 30 cases). The operative mortality was 28 p. 100, but only 7 p. 100 in the 15 cases in which pulmonary atrial pressure did not exceed 2/3 of the systemic pressure and 35 p. 100 in the other 49 cases with severe pulmonary hypertension.(ABSTRACT TRUNCATED AT 250 WORDS)

[Spectrum analysis of heart rate and arterial systolic pressure after heart transplantation in children]. / Analyse spectrale de la fréquence cardiaque et de la pression artérielle systolique de l'enfant transplanté cardiaque.

The aim was to examine the short-term variability of blood pressure (BP) and heart rate (HR) in 19 children who had received heart transplants and in 8 normal control children. BP was determined by a finger arterial pressure device. We examined the power spectra for HR and systolic blood pressure (SBP) in the supine and standing posture. In the transplanted children we could distinguish two groups (groups A and B) in whom HR variability differed, though in both it was greatly reduced compared to controls (group C). In group A there were no significant fluctuations in the mid frequency (MF) range for HF. Gain of the relationship between SBP and HR was very low and there were virtually no HR changes associated with passive tilting. By contrast, in group B transplant patients the HR variability, as assessed by standard deviation was about half that of normal controls. The power spectra attenuation was greater in the high frequency (HF) than in the MF bands. On standing the latter became enhanced, but not the HF variability. The findings suggest some reinnervation involving cardiac sympathetic fibres to a greater degree than the fast-responding vagal fibres. Time since operation was a critical factor for reinnervation since all subjects from group B were transplanted more than 44 months prior to the recording. We conclude that in a proportion of children who have received heart transplantation there is a delayed reinnervation of the heart, which probably involves sympathetic effectors rather than the vagus.

[Double discordance with ventricular septal defect and pulmonary artery hypertension. A study of 21 cases]. / Doubles discordances avec communication interventriculaire et hypertension artérielle pulmonaire. Etude de 21 cas.

This paper reports a retrospective study of 21 children with atrioventricular and ventriculo-arterial discordance, or double discordance, associated with a large ventricular septal defect responsible for pulmonary hypertension. Other associated congenital defects were: atrioventricular block (5 cases), coarctation of the aorta with neonatal cardiac failure (6 cases), tricuspid valve malformations responsible for significant tricuspid regurgitation (11 cases) and right ventricular hypoplasia (1 case). Two children died before any therapeutic intervention, one from syncope related to atrioventricular block and the other after a decision of therapeutic abstention. Three children underwent total correction with one good result (the only case of situs inversus), one late death and one lost to follow-up. The majority of patients (n = 16) underwent initial palliative surgery consisting in pulmonary artery banding occasionally associated with reconstruction of the aortic arch: there was no early mortality but there were 2 late deaths. Of the 14 survivors, 6 are well after a mean follow-up period of 31 months. Eight underwent open heart surgery with 1 operative death, 6 post-operative complete atrioventricular blocks requiring cardiac pacing and 5 poor results due to aggravation or secondary tricuspid regurgitation leading to 1 cardiac transplantation (death) and 2 reoperations for valvular surgery (1 plasty and 1 tricuspid valve replacement). The overall results of this series are poor: high mortality (33 per cent) and equally high morbidity when direct surgery is undertaken. Two major complications are observed: complete atrioventricular block (55 per cent) and regurgitation of the systemic atrioventricular valve (45 per cent), both of which often necessitate invalidating complementary procedures.(ABSTRACT TRUNCATED AT 250 WORDS)

[Incessant idiopathic ventricular tachycardia in infants]. / Tachycardies ventriculaires incessantes idiopathiques du nourrisson.

Reduction of the arrhythmia was obtained in 1 to 5 days, spontaneously (2 cases) of after oral amiodarone (500 to 2000 mg/m2/24 hr) (5 cases) or intravenous flecainide or propafenone in the two most critically ill patients. With the exception of 1 patient who was successfully treated with propranolol for 1 year, amiodarone was prescribed in all cases for 7 to 36 months after reversion to sinus rhythm: 5 children have been taken off treatment and seem to be completely cured 4 to 9 years after withdrawal; the other 3 patients have not had recurrences but have only been treated for 6 to 15 months. One of them has neurological sequellae from a cerebral embolism originating from an atrial thrombus. These results show that incessant ventricular tachycardia in infancy is a serious condition but amenable to medical treatment with high dose amiodarone or, when poorly tolerated, intravenous Class 1c antiarrhythmics for reduction of the arrhythmia, and amiodarone at adequate doses for 2 years to prevent recurrence. With the exception of the rare arrhythmogenic right ventricular dysplasia, the long term prognosis would seem to be excellent and, in general, there would seem to be little need for surgery.

[Cardiac transplantation in the infant and young child. Preliminary results]. / Transplantation cardiaque chez le nourrisson et le jeune enfant. Résultats préliminaires.

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)

[Enzymatic activities of the mitochondrial respiratory chain in child cardiomyopathies. 34 cases prospectively studied by endomyocardial biopsy]. / Activités enzymatiques de la chaîne respiratoire mitochondriale dans les myocardiopathies de l'enfant. Etude prospective de 34 cas par biopsies endomyocardiques.

The arguments in favour of mitochondrial pathology of certain childhood cardiomyopathies (multi tissue involvement, lactic acidosis, histochemical abnormalities of skeletal muscle) are indirect and may be absent in isolated cardiomyopathy. The authors therefore set up a prospective study of enzyme activity of the mitochondrial respiratory chain directly by endomyocardial biopsy. Fifty children aged 2 months to 16 years were included. Thirty four had cardiomyopathy which was dilated and hypokinetic with thin walls in 21 cases, restrictive in 2 cases, and hypertrophic in 11 cases; the remaining 16 children had either normal hearts (13 catheterised for other reasons) or myocardial hypertrophy due to pulmonary or aortic stenosis (3 cases). Both ventricles were evaluated in 3 cases; macro-surgical biopsies were obtained in 6 cases and skeletal muscle biopsy was obtained in 9 cases. The results indicate the method to be reliable with no difference between the micro and macro biopsies. The absolute values of enzyme activity were too variable to serve as quantitative parameters but some ratios of activity were remarkably stable and allowed a qualitative assessment which was all the more accurate when identical values were obtained in the myocardium, skeletal muscle and liver. The mitochondrial respiration was independent of ventricular pressures and of the type (right or left) of ventricle. Enzyme activity was nearly always normal in dilated cardiomyopathy (20/21) which suggests that it was unaffected by dilatation of the heart and by abnormal myocardial contractility. The results could be normal in myocardial hypertrophy and in valvular stenosis and in over half the cases of hypertrophic cardiomyopathy.(ABSTRACT TRUNCATED AT 250 WORDS)