Fetal aortic valvuloplasty for critical aortic stenosis: single-center retrospective study focusing on postnatal outcome.

OBJECTIVE: We aimed to report our experience on fetal aortic valvuloplasty (FAV) for critical aortic stenosis (AS) focusing on the postnatal evolution of the patients. METHODS: This retrospective study was approved by our local Institutional Review Board (n°2002-0128143827). All fetuses with critical AS who underwent FAV in a single center between 01/2011 and 06/2022 were included. FAV were performed under ultrasound guidance. Technical success was based upon balloon inflation across the aortic valve and improvement of the anterograde aortic flow across the aortic valve. At birth, biventricular circulation (BVC) strategy was decided assuming the left ventricle (LV) systolic and diastolic functions would ensure the systemic circulation. RESULTS: Sixty-three FAV were performed on 58 fetuses at 24.6[21.4-32.4] weeks of gestation. The procedure was successful in 52/58(89.6%) fetuses. There were 11/58(19%) in utero demises and 9/58(15.5%) terminations of pregnancy. There were no liveborn patients after the unsuccessful procedures. 38/58(65.5%) infants were delivered at a median gestational age of 38.1[29-40.6] weeks and 21/38(55.3%) of them required prostaglandin. 28/38(73.7%) [28/58(48.3%)] children entered the BVC path at birth. Among them, 20 required an aortic valvuloplasty at birth (11 percutaneous, 9 surgical) and 8 did not require any treatment at birth but of those, 5/8 underwent a surgical valvuloplasty between day 26 and day 1200 of life. 11/28(39.3%) infants with BVC at birth required a second intervention and four of them required a third intervention. Two infants who entered the BVC at birth underwent a conversion to UVC. None of the surviving children with BVC developed pulmonary hypertension. The global survival rate in case of BVC was 22/28(78.6%) at 23.3[8-112] months of life. 10 patients had UVC at birth. Among them, 6 received comfort care from birth and only 4 underwent surgery. 3/10 patients were still alive at the latest assessment (48[22-102] months). CONCLUSION: FAV for critical aortic stenosis led to anterograde aortic flow in 89.6% of the fetuses, with BVC being achieved in 48.3% (73.7% of the live born). Among patients with BVC at birth, the rate of reintervention is high but long-term survival is satisfactory. This article is protected by copyright. All rights reserved.

Accuracy and impact of prenatal diagnosis of common arterial trunk.

OBJECTIVES: Outcome of common arterial trunk (CAT) depends mainly on truncal valve function, presence of coronary artery abnormalities and presence of interrupted aortic arch. The main objective of this study was to evaluate the accuracy of prenatal diagnosis of CAT by analyzing prenatal vs postnatal assessment of: (1) anatomic subtypes and (2) truncal valve function. The secondary objective was to assess the potential impact of prenatal diagnosis of CAT on postnatal mortality and morbidity by comparing prenatally vs postnatally diagnosed patients. METHODS: This was a retrospective analysis of all CAT patients diagnosed either prenatally, with postnatal or fetopsy confirmation, or postnatally, from 2011 to 2019 in a single tertiary center. Cohen's kappa statistic was used to evaluate agreement between pre- and postnatal assessment of anatomic subtypes according to Van Praagh and of truncal valve function. Mortality and morbidity variables were compared between prenatally vs postnatally diagnosed CAT patients. RESULTS: A total of 84 patients (62 liveborn with prenatal diagnosis, 16 liveborn with postnatal diagnosis and six terminations of pregnancy with fetopsy) met the inclusion criteria. The accuracy of prenatal diagnosis of CAT anatomic subtype was 80.3%, and prenatal and postnatal concordance for subtype diagnosis was only moderate (κ = 0.43), with no patient with CAT Type A3 (0/4) and only half of patients with CAT Type A4 (8/17) being diagnosed prenatally. Fetal evaluation of truncal valve function underestimated the presence (no agreement; κ = 0.09) and severity (slight agreement; κ = 0.19) of insufficiency. However, four of five cases of postnatally confirmed significant truncal valve stenosis were diagnosed prenatally, with fair agreement for both presence and severity of stenosis (κ = 0.38 and 0.24, respectively). Mortality was comparable in patients with and those without prenatal diagnosis (log-rank P = 0.87). CAT patients with fetal diagnosis underwent earlier intervention (P < 0.001), had shorter intubation time (P = 0.047) and shorter global hospital stay (P = 0.01). CONCLUSIONS: The accuracy of prenatal diagnosis of CAT is insufficient to tailor neonatal management and to predict outcome. Fetal assessment of truncal valve dysfunction appears unreliable due to perinatal transition. Improvement is necessary in the fetal diagnosis of anatomic subtypes of CAT requiring postnatal prostaglandin infusion. © 2022 International Society of Ultrasound in Obstetrics and Gynecology.

[Prenatal diagnosis of aorta coarctations]. / Diagnostic prénatal des coarctations de l'aorte.

Coarctations of the aorta can be associated with severe neonatal consequences. Screening for and diagnosis of this prenatal malformation remain difficult. We review the various tools currently available to us, and their respective limits, to minimize the rate of false negatives and false positives associated with prenatal screening for this situation.

[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.

[Transcatheter exclusion of a giant aneurysm of the left pulmonary artery using covered stents]. / Exclusion d'un anévrisme géant de l'artère pulmonaire gauche par cathétérisme interventionnel: utilisation de stents couverts.

An 11 year-old boy with an Alagille syndrome was referred for an urgent echocardiography because of new onset of febrile hemoptysis. Follow-up in our institution to that point had shown severe hypoplastic pulmonary arteries. The diagnosis of tricuspid endocarditis complicated by pulmonary embolisms and aneurysm of the left pulmonary artery was done. Despite effective antiobiotics, the evolution was marked by rapid progression in size of the left pulmonary aneurysm. Since the surgery was not an option because of high risk of death, this aneurysm was treated by a trans-catheter technique using covered stents (CP stents, Numed Inc). Its exclusion allowed to stop its progression in size and to avoid its rupture.

[Coarctation of the aorta presenting as a pulsatile occipital mass]. / Coarctation de l'aorte révélée par une masse occipitale pulsatile.

A healthy 18 months old boy, is referred to our hospital for a thrilling pulsatile mass behind the left ear. Aortography in the ascending aorta shows a severe coarctation with almost interruption of the aorta between the left carotid and left subclavian artery. Both external carotid arteries provide an important collateral pathway through occipital arteries to two dilated vertebral arteries. Descending aorta is feeding by a reverse blood flow into vertebral and subclavian arteries. The child has been operated. This is an original presentation of severe coarctation of the aorta with development of an important and vital collateral pathway.

[Risks and benefits of cardiac surgery in Down's syndrome with congenital heart disease]. / Avantages et risques de la chirurgie cardiaque dans la trisomie 21.

UNLABELLED: Down's syndrome is the most frequent chromosomic anomaly. Fifty percent are associated with a congenital heart disease. Life expectancy and quality of life are increasing since 15 years with improving surgical techniques. PATIENTS AND METHODS: This study presents 73 patients with Down's syndrome who underwent a surgical repair of congenital heart disease between 1992 and 2002. Among them, 37 (50.6%) had a complete atrio-ventricular septal defect (AVSD) and 36 an other anomaly. RESULTS: Five children died in the post operative period: all had an AVSD. Two late deaths occurred due to extra cardiac causes during the follow up. Two children who underwent a repair of AVSD had to be reoperated. The majority of the children are doing well, without any cardiac symptom. Thirty-eight percent of patients with AVSD repair have a middle mitral or tricuspid regurgitation and three had a massive mitral or tricuspid regurgitation. One child presents neurological sequelae related to surgery. CONCLUSION: Congenital heart diseases in Down's syndrome can be repaired with a limited risk of death. Final results are good for many children with significant improvement of the quality of life and life expectancy.

[Retro-tracheal left pulmonary artery. Report of 2 cases]. / Artère pulmonaire gauche rétro-trachéale. A propos de 2 cas.

The authors report the cases of two neonates with retro-tracheal left pulmonary arteries (pulmonary artery sling). In the first case, there was rapid onset of cardiac failure with signs of airway compression. Echocardiography showed the vascular anomaly associated with a large ventricular septal defect (VSD). After standard radiological investigation and bronchoscopy to exclude an associated tracheo-bronchial malformation, the VSD was repaired surgically with reimplantation of the left pulmonary artery. Unfortunately, the patient died of major airways obstruction in the postoperative period. Autopsy showed tracheo-bronchial anomalies which had not been diagnosed preoperatively. The second patient presented with hypoventilation of the right lung. After echocardiographic diagnosis of the anomaly, a thorough investigation (thoracic CT, helicoidal scan, bronchoscopy) was carried out and no associated bronchial malformations were observed. Reimplantation of the left pulmonary artery was successful and the postoperative course was uneventful. Retro-tracheal left pulmonary artery is a rare malformation Which is difficult to diagnose. It requires extensive pulmonary investigations and a multi-disciplinary approach. The prognosis is poor when there are associated tracheo-bronchial malformations.

[Procalcitonin and viral meningitis: reduction of unnecessary antibiotics by measurement during an outbreak]. / Procalcitonine et méningites virales: réduction des traitements antibiotiques inutiles par le dosage en routine au cours d'une épidémie.

OBJECTIVE: Viral meningitis are often treated with antibiotics in emergency because routine analysis of CSF is not always efficient for distinguishing between viral and bacterial infection. The aim of the study was to evaluate the usefulness of procalcitonin (PCT) to reduce antibiotic treatments. METHODS AND RESULTS: A blood PCT level < 0.5 ng/mL was prospectively used for the diagnosis of viral origin of meningitis in 58 patients (two months-14 years), in which enterovirus was isolated by culture or PCR during an outbreak (May-June 2000). CSF cells range was 10 to 2800/mL (m: 244), PMN 5 to 2464/mL and CSF proteins range was 0.19 to 0.92 mg/dL (m: 0.37). Seventeen patients received antibiotic therapy in admission. In nine patients, PCT (dosage was routinely measured 3/week) result < 0.5 ng/mL was obtained in 24 h and in 48 h in six: treatment was then stopped and children led hospital. In two patients, PCT was > 1 ng/mL because of bacterial coinfection. CSF and PCT values were similar to those of an already published control group. CONCLUSION: PCT dosage allowed to shorten hospitalization of 4.47 (controls) to 2.06 (patients) days in patients receiving unnecessary antibiotic treatments. During this outbreak, PCT dosage allowed to reduce 40 days of hospitalization.

[Possibilities of combined maxillary-orthopaedic and prosthetic treatment of malocclusion in adult patients]. / Mozliwosci zespolowego szczekowo-ortopedycznego i protetycznego leczenia wad zgryzu u pacjentów doroslych.

The possibilities of an efficient and safe maxillary--orthopaedic intervention in adult patients in order to eliminate or diminish the intensification of some malocclusion have been discussed in the paper. It enables to achieve the full functional--esthetic effect of the prosthetic treatment. The results of the combined treatment of the patients with common defects, i.e. hypodontia of lateral incisors and deformities towards the front, that create disturbances in prosthetic treatment planning have been given as an example.