Myriam Bensemlali

Discordances Between Pre-Natal and Post-Natal Diagnoses of Congenital Heart Diseases and Impact on Care Strategies

BACKGROUND Pre-natal diagnosis of congenital heart disease (CHD) allows anticipation of urgent neonatal treatment and provides adequate information to the parents on cardiac outcomes. OBJECTIVES This study sought to analyze the discordances between expert fetal cardiac diagnosis and final diagnosis of CHD and their impact on neonatal and long-term care strategies. METHODS We included 1,258 neonates with a pre-natally diagnosed CHD and 189 fetopsies following termination of pregnancy at our tertiary center over a 10-year period. Pre-natal echocardiographic and final diagnoses were compared. RESULTS For live births, we identified 368 (29.3%) discordances between pre- and post-natal diagnoses. The pre-natal diagnosis was different from the post-natal diagnosis in 36 cases (2.9%) and partially different with a major impact on neonatal treatment of the CHD in 97 cases (7.7%). In 235 cases (18.7%), the diagnosis was partially different with no impact on neonatal planned treatment. The discordances had a negative impact on late care strategy in 62 cases (4.9%): more complex CHD that was unsuitable for biventricular repair, leading to unplanned compassionate care, additional surgery or increase of the complexity level of the Aristotle score. A positive impact was found in 31 cases (2.5%): less complex CHD that allowed biventricular repair, fewer surgical procedures, or decrease of the complexity of the Aristotle score. For 275 patients (21.9%), there was no impact on late care strategy. Of the 872 terminations of pregnancy and intrauterine fetal deaths, 189 fetopsies were available: 16 (8.5%) different diagnoses, 27 (14.3%) major differences, and 60 (31.7%) minor differences. CONCLUSIONS Correcting fetal cardiac diagnosis after birth can lead to significant changes in neonatal (10.6%) and late (7.4%) care strategies. Tools should be developed to try to improve the accuracy of pre-natal diagnosis of CHD. Clinicians should be cautious when predicting required treatment and outcomes during pre-natal counseling.

Neonatal management and outcomes of prenatally diagnosed CHDs

OBJECTIVES The aim of this study was to determine the probability of intervention at birth after prenatal diagnosis of CHD. METHODS A 10-year retrospective study including all foetuses with a prenatally diagnosed CHD and those delivered in a tertiary-care cardiac centre between January, 2002 and December, 2011 was carried out. Patients were classified into eight groups according to the anticipated risk of neonatal intervention. RESULTS The need for urgent intervention and/or PGE1 infusion within the first 48 hours of life was 47% (n=507/1080): 72% (n=248) for CHD at risk for a Rashkind procedure, 77% (n=72) for CHD with ductal-dependent pulmonary flow, 13% (n=22) for CHD with potentially ductal-dependent pulmonary flow, 94% (n=62) for CHD with ductal-dependent systemic flow, 29% (n=88) for CHD with potentially ductal-dependant systemic flow, 50% (n=4) for total anomalous pulmonary venous connection, and 17% (n=1) for CHD with atrio-ventricular block. In all, 34% of the patients received PGE1 infusion and 21.4% underwent urgent catheter-based or surgical interventions; 10% of patients without anticipated risk (n=10) underwent an early intervention; 6.7% (n=73) of the patients died; and 55% (n=589) had an intervention before discharge from hospital. CONCLUSION Half of the neonates with foetal CHD benefited from an urgent intervention or PGE1 infusion at birth. We recommend scheduled delivery and in utero transfer for transposition of the great arteries, double-outlet right ventricle with sub-pulmonary ventricular septal defect, total anomalous pulmonary venous connection, CHD with atrio-ventricular block with heart rate <50, all ductal-dependant lesions, and CHD with potentially ductal-dependant systemic flow.

Percutaneous pulmonary Melody® valve implantation in small conduits

BACKGROUND The Melody® pulmonary valve has received approval for the treatment of dysfunctional right ventricular (RV) outflow tract conduits ≥16mm. AIMS To investigate technical and clinical outcomes in patients who underwent percutaneous pulmonary valve implantation (PPVI) in conduits ≤16mm. METHODS Eleven patients were enrolled retrospectively between 2000 and 2015 in a cardiac referral centre. RESULTS The PPVI indications were obstruction (n=5); regurgitation (n=1); and mixed lesions (n=5). Pre-stenting was performed during the procedure in seven cases. Three of the remaining four patients had previous stent implantation. The mean maximal dilatation balloon diameter/surgical initial diameter ratio was 1.3. Procedural haemodynamics showed a decrease in peak RV to pulmonary artery gradient (mmHg) from 50 (14-86) to 11 (5-23) (P<0.001), in RV systolic pressure (mmHg) from 69 (35-110) to 40 (25-50) (P=0.006) and in RV/aortic systolic pressure ratio from 0.8 (0.4-1.0) to 0.4 (0.3-0.6) (P=0.004). Acute device-related complications occurred in four patients (36.4%): two confined conduit tears managed with placement of a covered stent; and two vascular access complications. Mean follow-up after PPVI was 3.9 years (41 days to 6.7 years). At last follow-up, one patient had undergone surgical conduit replacement for recurrent conduit stenosis, one had percutaneous pulmonary valve dilatation and two had developed bacterial endocarditis and undergone surgical conduit replacement. Freedom from valve dysfunction at 1, 2 and 3 years was 91%, 91% and 82%, respectively. CONCLUSIONS PPVI is feasible in small conduits ≤ 16mm with good procedural and early haemodynamic results, postponing the need for surgery. These results are not sustainable over time. Larger studies are needed to determine if bare-metal stent implantation as a bridge to surgical replacement provides the same results in this specific population.

0535: A novel approach to exploration of complex congenital heart diseases through 3D CT scans

Introduction One of the key success factors of the surgical treatment of complex congenital heart defects is a detailed preoperative exploration. The main issue is the lack of detailed visibility of the intracardiac anatomical structures. 2D echocardiography provides a partial view and doesn’t provide fine anatomical details. Novel imagery technology with 3D cardiac tomography (CT) may represent a valuable tool to explore complex congenital heart defects. Objective To determine the quality and limitations of this technique to study the intracardiac anatomy in complex outflow tract defects. The secondary objective is to generate the CT scan views in preoperative assessment fitting precisely to the surgical views. Methods and results We performed CT scans with high resolution in 15 heart specimens with double outlet right ventricle. All 3D images were produced with a 3D reconstruction platform from Paris Descartes University. The 3D view from the right ventricle showed the anatomic details for all hearts. We described the VSD, its localization, borders and surface. We described also the relationship with the aorta and the pulmonary trunk and the conal septum (length and orientation). The 3D view from the right atrium was more difficult then expected and needs to be improved. Conclusion This study summarizes the role of 3D scan reconstruction in congenital heart diseases as an imaging modality to increase the precision of preoperative assessment.

P7 Impact of precision prenatal diagnostic of congenital heart diseases on perinatal and long-term management

Objective To evaluate the impact of precising prenatal diagnosis of congenital heart diseases (CHD) on perinatal and long-term management. Methods Over a 10-year period, 1258 neonates with a prenatally diagnosed CHD and 189 fetal autopsies after termination of pregnancy were included. Changes in CHD diagnosis were classified as totally different, similar but leading to changes in neonatal management, and similar without changes on initial management. The impact on long-term outcome was considered negative if the final diagnosis was a more complex CHD precluding the planned biventricular repair, or if additional surgical interventions were needed, or if the complexity level of the Aristotle score was increased. The impact on outcome was considered positive if biventricular repair was possible while not planned prenatally, or if the number of surgical interventions was reduced, or if the complexity level of the Aristotle score was lower. Results The post-natal diagnosis was imprecise in 30.2% of the cases: completely different in 2.9%, led to changes in initial management in 8%, and did not affect initial management in 19.3%. Imprecision in the prenatal diagnosis had a negative impact on long-term outcome in 4.9% of the cases, and a positive impact in 4.1%. In the fetal autopsy group (mean term 26 weeks), the diagnosis was imprecise in 54.5% of the cases: completely different in 8.5%, could have led to changes in postnatal management in 14.3%, and with minor differences that would not have led to changes in management in 31.7%. In both groups, the most frequent differences were anomalies of the outflow tract anatomy (43%), and the systemic or pulmonary veins (25%). Conclusion Imprecision of prenatal diagnosis of CHD changes early management in 11% of the cases, and impacts long-term outcome in 9% of the cases. Improvement of CHD diagnosis for anatomy of the outflow tract and main veins should help to reduce impact on postnatal management and outcome.

0460: Radiation dose reduction in pediatric coronary CT: assessment of effective dose and image quality

Aim To assess the impact of different scanning protocols on radiation dose and image quality for pediatric coronary computed tomography. Materials and Methods 100 children underwent coronary computed tomography after arterial switch operation from November 2012 to March 2014. Scans were done using two different scanner models without difference in scanning and image reconstruction parameters: Lightspeed VCT and Discovery HD750, 64-slice from GE Healthcare. Two consecutive changes in scanning protocols were performed: 1) the use of adaptive statistical iterative reconstruction (ASIR) instead of filtered back projection (FBP) for image reconstruction; 2) the optimization of scan acquisition parameters (current and tube voltage reduction). Premedication (beta-blocker) was used for all children to obtain heart rate Results Patients were divided in three age groups: 0-4, 5-7 and 8-18 years. After adjustment for scan settings, median ED decreased by 28% (3.9 mSv, IQR 2.8-4.2), 40% (0.9 mSv, IQR 0.6-2.6) and 65% (0.7 mSv, IQR 0.5-0.9) for 0-4, 5-7 and 8-18 years age groups (p Conclusions Coronary CT can be obtained at very low radiation doses in pediatric patients using ASIR and prospective ECG-triggered acquisition with optimized scan parameters.

SFP PC-61 – Dix ans de transfert in utero des cardiopathies congénitales de diagnostic anténatal

Objectif Etudier l’interet du transfert in utero des cardiopathies congenitales dans un centre tertiaire de reference. Methode et resultats Entre janvier 2002 et decembre 2011, 2130 fœtus ont ete inclus, dont 1258 nouveau-nes vivants, 799 interruptions de grossesse et 73 morts fœtales in utero. Le transfert in utero des nouveau-nes vivants a ete considere comme indiscutable a priori pour 899 patients (71,5%) pour les raisons suivantes: necessite de manœuvre de Rashkind dans 344 cas, risque de coarctation dans 272 cas, ductodependance pulmonaire certaine dans 107 cas, systemique certaine dans 93 cas, necessite d’intervention neonatale dans 83 cas. La justification du transfert a ete analysee a posteriori pour 359 patients: ductodependance pulmonaire possible pour 156 cas, systemique possible pour 35 cas, complement diagnostique pour 94 cas, tolerance neonatale pour 51 cas. A posteriori, le transfert a ete considere comme non justifie pour 231 nouveau-nes (18,5%) et justifies pour 128 nouveau-nes (10%) dont 65 ont eu une intervention neonatale et 63 une modification majeure de leur diagnostic cardiologique. Conclusion Notre etude a permis de montrer que 4 transferts in utero sur 5 dans notre centre tertiaire etaient justifies tant sur le plan diagnostic que therapeutique.

SFP CO-41 - Impact des anomalies extracardiaques sur le pronostic des cardiopathies congénitales de diagnostic anténatal

Objectif Etudier l’influence des anomalies extracardiaques dans le choix d’interrompre la grossesse et dans la survie des nouveau-nes atteints de cardiopathies congenitales de diagnostic antenatal. Methode et resultats Entre janvier 2002 et decembre 2011, 2057 foetus ont ete inclus, dont 1258 nouveau-nes vivants et 799 interruptions de grossesse. La prevalence des anomalies extracardiaques majeures etaient de 18,6%. Parmi les nouveau-nes, 121 avaient une anomalies extracardiaque majeure mais seulement 55 etaient diagnostiquees avant la naissance. Les anomalies extracardiaques majeures etaient significativement associees au groupe des interruptions de grossesse par rapport aux nouveau-nes vivants (31 vs 4%, p Conclusion Les anomalies extracardiaques sont associees a un plus fort taux d’interruption de grossesse et de deces chez les nouveau-nes vivants.