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 were 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.

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.

[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.

[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.

[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.

Perinatal management of fetal cardiac anomalies in a specialized obstetric-pediatrics center.

Perinatal teams dealing with fetal heart disease frequently wonder which pregnancies might be terminated, and when delivery should take place in a specialized surrounding. We present a retrospective study of 229 fetuses, in which prenatal ultrasound showed a cardiac anomaly not compatible with a standard maternity ward delivery. One hundred nineteen pregnancies were terminated (group I) while 110 pregnancies led to the birth of a live baby (group II). Pathology in group I was discovered earlier than in group II (24 vs. 29.3 weeks' gestation; p <0.01), and associated malformations or chromosomal anomalies were much more frequent in group I (80/119 vs. 9/110; p <0.001). Among live born babies, three infants with transposition of the great arteries underwent Rashkind atrioseptostomy in the delivery room. With a minimum follow-up of 12 months, 69 children (63%) have undergone surgery. Among 92 survivors (1 child is lost to follow-up), 78 (71%) are asymptomatic and 14 symptomatic. Early prenatal diagnosis of fetal heart anomalies significantly facilitates prenatal work-up and perinatal care. We present the types of pathology having led to termination and define the situations in which children are at risk of perinatal hemodynamic compromise.

Prevalence of 22q11 deletion in fetuses with conotruncal cardiac defects: a 6-year prospective study.

OBJECTIVES: Conotruncal malformations (CTMs) are a major feature of 22q11 microdeletion (22qdel). The prevalence of 22qdel in fetuses harboring these defects is unknown. We assessed the prevalence of 22qdel in a population of fetuses with conotruncal cardiac defects. STUDY DESIGN: Consecutive fetuses (n = 261) with a CTM and a normal karyotype were included in the study. All fetuses were screened for 22qdel by means of fluorescent in situ hybridization. RESULTS: A 22qdel was found in 54 fetuses (20.7%). The proportion of 22qdel for each CTM was: tetralogy of Fallot (14/100), pulmonary atresia with ventricular septal defect (11/61), tetralogy of Fallot with absent pulmonary valves (6/16), interrupted aortic arch (10/22), truncus arteriosus (9/29), and complex transpositions of the great arteries (4/33). Additional vascular anomalies were present in 75%. Typical abnormal facial appearance at birth or at autopsy was observed in 80%, and thymus hypoplasia, in 76%. The pregnancy was terminated in 41 of 54 cases, including an intrauterine death in one case. The 22qdel was inherited in 7.7%. CONCLUSION: Prevalence of the 22qdel is high in fetuses with CTMs. The risk of mental retardation associated with the respective risk of cardiac surgery for each type of CTM may strongly influence prenatal counseling.

Percutaneous replacement of pulmonary valve in a right-ventricle to pulmonary-artery prosthetic conduit with valve dysfunction.

BACKGROUND: Valved conduits from the right ventricle to the pulmonary artery are frequently used in paediatric cardiac surgery. However, stenosis and insufficiency of the conduit usually occur in the follow-up and lead to reoperations. Conduit stenting can delay surgical replacement, but it aggravates pulmonary insufficiency. We developed an innovative system for percutaneous stent implantation combined with valve replacement. METHODS: A 12-year-old boy with stenosis and insufficiency of a prosthetic conduit from the right ventricle to the pulmonary artery underwent percutaneous implantation of a bovine jugular valve in the conduit. FINDINGS: Angiography, haemodynamic assessment, and echocardiography after the procedure showed no insufficiency of the implanted valve, and partial relief of the conduit stenosis. There were no complications after 1 month of follow-up, and the patient is presently in good physical condition. INTERPRETATION: We have shown that percutaneous valve replacement in the pulmonary position is possible. With further technical improvements, this new technique might also be used for valve replacement in other cardiac and non-cardiac positions.

[Prenatal diagnosis of transposition of great vessels reduces neonatal morbidity and mortality]. / Le dépistage anténatal de la transposition des gros vaisseaux diminue la mortalité et la morbidité néonatales.

Transposition of the great arteries (TGA) is a common malformation which sometimes has a dramatic presentation at birth but which is completely curable with early and appropriate initial management. Antenatal diagnosis of this condition may change the neonatal prognosis. The authors compared morbidity and mortality in the pre- and postoperative periods of 68 neonates with an antenatal diagnosis of TGA (foetal diagnosis) with that of 250 neonates in whom the diagnosis was made after birth (neonatal diagnosis). The delay before admission to the department was 2 +/- 2.8 hours in the foetal group and 73 +/- 210 hours in the neonatal group (p < 0.01). Severe haemodynamic distress (metabolic acidosis, multi-organ failure) were more common in the neonatal group (p < 0.01). Management on admission was identical in the two groups (p > 0.05). The preoperative mortality was 15/250 in the neonatal group (6%, 95% CI = 3-9%) compared with 0/68 in the foetal group (p < 0.05). The postoperative morbidity was comparable in the two groups (25/235 and 6/68) but the hospital stay was longer in the neonatal group (30 +/- 17 versus 24 +/- 11 days, p < 0.01). Finally, postoperative mortality was significantly higher in the neonatal group (20/235 compared with 0/68, p < 0.01) although the risk factors of death at arterial switch surgery were identical in the two groups. Therefore, antenatal diagnosis of TGA reduces neonatal morbidity and mortality in this condition. Antenatal diagnosis must be developed by the education of obstetricians. The transfer of mothers with a foetus affected by TGA to centres capable of assuming the initial management, sometimes during labour, is essential.

Anti-B-cell monoclonal antibody treatment of severe posttransplant B-lymphoproliferative disorder: prognostic factors and long-term outcome.

B-lymphoproliferative disorder (BLPD) is a rare but severe complication of organ and bone marrow transplantation (BMT). Profound cytotoxic T-cell deficiency is thought to allow the outgrowth of Epstein-Barr virus-transformed B cells. When possible, reduction of immunosuppressive treatment or surgery for localized disease may cure BLPD. Therapeutic approaches using chemotherapy or antiviral drugs have limited effects on survival. Adoptive immunotherapy with donor T-cell infusions has given promising results in BMT recipients. We previously reported that administration of two monoclonal anti-B-cell antibodies (anti-CD21 and anti-CD24) could contribute to the control of oligoclonal BLPD. Here we report the long-term results of treatment with these monoclonal anti-B-cell antibodies for cases of severe BLPD. In an open multicenter trial, 58 patients in whom aggressive B-cell lymphoproliferative disorder developed after BMT (n = 27) or organ (n = 31) transplantation received 0.2 mg/kg/d of specific anti-CD21 and anti-CD24 murine monoclonal antibodies (MoAbs) for 10 days. The treatment was well tolerated. Thirty-six of the 59 episodes of BLPD in the 58 patients presented complete remission (61%). The relapse rate was low (3 of 36, 8%). Multivariate analysis identified the following risk factors for partial or no response to anti-B-cell MoAb therapy: multivisceral disease (P

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)

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.

[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)

[Right pulmonary artery arising from the proximal ascending aorta. A model of reflex pulmonary hypertension of the left lung?]. / Artère pulmonaire droite naissant de l'aorte ascendante proximale. Un modèle d'hypertension artérielle pulmonaire réflexe du poumon gauche?

The right pulmonary artery arising from the proximal ascending aorta is a rare and severe malformation. This retrospective study of 11 children with this condition was undertaken to determine the conditions of diagnosis, to analyse the results of surgery, and, above all, to clarify the mechanism of the left pulmonary arterial hypertension which was always present. Ten of these patients were 4 to 90 days old. All had severe congestive cardiac failure with iso- or suprasystemic left pulmonary arterial hypertension. The only associated lesions were ventricular septal defect (1 case) and patent ductus arteriosus (7 cases). None of the patients had significant left-to-right shunts and only one had left atrial hypertension: this patient died before surgery could be performed. The other 9 patients underwent surgical correction and the pulmonary pressures immediately fell to normal or almost normal values. The child with the ventricular septal defect died of infection 6 weeks after surgery. The 8 survivors are doing well 1 month to 12 years later and left pulmonary pressures are normal in all, including those (5 cases) with a stenosed (4 cases) or completely occluded right pulmonary arterial circulation (1 case) and in 1 patient with obstructive vascular disease. The eleventh patient was very different: she had no signs or symptoms until 2 years of age, when a right pulmonary obstructive arterial disease but with normal left pulmonary pressures was documented. She was not operated on and remains well nine years later.(ABSTRACT TRUNCATED AT 250 WORDS)

Anti-B-cell monoclonal antibodies in the treatment of severe B-cell lymphoproliferative syndrome following bone marrow and organ transplantation.

BACKGROUND: The B-cell lymphoproliferative syndrome is an infrequent life-threatening complication of marrow or organ transplantation that is the consequence of profound immunosuppression. The results of treatment have been disappointing, although a small number of patients have been cured by chemoradiotherapy or antiviral agents after a reduction in the dosage of immunosuppressive therapy. We report here the results of treating this disorder with anti-B-cell antibodies. METHODS: Twenty-six patients in whom aggressive B-cell lymphoproliferative syndrome developed after bone marrow (n = 14) or organ (n = 12) transplantation received 0.2 mg of CD21-specific and of CD24-specific antibodies per kilogram of body weight for 10 consecutive days in an open, prospective, multicenter trial. RESULTS: The treatment was well tolerated. All patients had transient neutropenia, apparently because the CD24 molecule is also expressed on granulocytes. The treatment was ineffective in seven patients with monoclonal B-cell proliferation. In contrast, 16 patients with oligoclonal B-cell proliferation had complete remission. Systemic remission also occurred in two other patients with oligoclonal proliferation who had central nervous system involvement, although they subsequently died because of progression of the central nervous system disease. In one patient who died early, clonality was not determined. Of the 16 patients who had complete remission, 2 with persistent immunodeficiency due to graft (marrow) rejection or acute graft-versus-host disease had a relapse, and the 1 with graft-versus-host disease subsequently died. Eleven patients were alive and disease-free after a median follow-up of 35 months (5 of 14 marrow recipients and 6 of 12 organ recipients). Four other patients in complete remission died of unrelated causes 4 to 12 months after treatment. CONCLUSIONS: Intravenous administration of anti-B-cell antibodies may be effective in controlling diffuse, severe, oligoclonal B-cell proliferation not involving the central nervous system.

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.

[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)

[Stenosis of the pulmonary veins in total anomalous pulmonary venous drainage. Associated or iatrogenic abnormality?]. / Sténose des veines pulmonaires dans les retours veineux pulmonaires anormaux totaux. Anomalie associée ou iatrogène?

Obstruction of total anomalous pulmonary venous drainage (TAPVD) is sometimes observed in neonates, usually at the site where the collector drains into the caval system. Stenosis of the pulmonary veins themselves before joining the collector is less common. This was observed in 6 cases of intra- and supracardiac TAPVD; the prognosis is usually very bad due to postoperative pulmonary hypertension (PHT) which is difficult to treat. Three of our cases had TAPVD into the coronary sinus with obstruction presenting at birth. Conventional surgery did not reduce the PHT of the first 2 children because the congenital stenosis of the pulmonary veins was not corrected. In the first case, the pulmonary veins resembled fibrous cords and in the second case, the collector was stenosed at its junction with the coronary sinus. These two children died despite surgery. The third child, however, was cured because the obstruction was diagnosed preoperatively and successfully treated when the TAPVD was corrected. In the other three cases, TAPVD to the superior vena caval system was not obstructed but the pulmonary veins retracted progressively after surgery causing PHT and right ventricular failure. After unsuccessful percutaneous dilatation, reoperation revealed obstruction due to exuberant scar tissue with retraction at the site of anastomosis and (or) reconstruction of the pulmonary veins by incision or enlargement with a pericardial patch. One of these children survived after pneumonectomy of the obstructed lung which relieved reflex PHT of the contralateral lung.(ABSTRACT TRUNCATED AT 250 WORDS)