Anomalous hepatic drainage and platypnea-orthodeoxia in cyanotic children.

The aetiology of cyanosis could be unclear in children, even for specialised paediatricians. Two cases were reported: first, a 6-year-old child with features of left isomerism and Fallot was fortuitously diagnosed with anomalous hepatic venous drainage before complete repair. Second, a newborn with an antenatal diagnosis of ductus venosus agenesis had an isolated intermittent right-to-left atrial shunt when upright, with favourable outcome, in contrast to the association with significant heart malformations including inferior caval vein interruption. Multimodality imaging and 3D printing helped to rule out extracardiac causes of persistent cyanosis and plan surgical repair.

Mitral annular disjunction and Pickelhaube sign in children with mitral valve prolapse: A prospective cohort study.

BACKGROUND: Mitral annular disjunction (MAD) and the Pickelhaube sign are identified as risk factors for malignant ventricular arrhythmias (VAs) and sudden cardiac death in adults with mitral valve prolapse (MVP); their prevalence and consequences in children have never been studied. OBJECTIVES: To determine the proportion of MAD in children with MVP, and its potential link with VAs. METHODS: A cohort of 49 consecutive children (mean age 12.8±3.0 years; 33 females) with MVP and comprehensive clinical arrhythmia (24-hour monitoring) and Doppler echocardiographic characterization, including pulsed-wave tissue Doppler (PWTD) of the lateral mitral annulus, was identified. The relationship between clinical and echocardiographic data and presence of VAs was studied. RESULTS: MAD was common (n=25; 51%). Only five patients had significant VAs (Lown grade>2) characterized by polymorphic premature ventricular contractions or couplets. MAD was not associated with VAs on 24-hour Holter monitoring, but an association was found between VAs and spiked high-velocity midsystolic signal>16cm/s on PWTD (Pickelhaube sign) (P=0.004), myxomatous mitral valve (P=0.004) and left ventricular dilatation (P=0.01). T-wave inversion in inferolateral leads on electrocardiogram was more frequent in patients with versus without the Pickelhaube sign (P=0.03). No difference was found between patients with or without MAD regarding sex, history of palpitation, severity of mitral regurgitation, aortic root diameter and incidence of connective tissue disorders. Myocardial fibrosis was detected in two of three patients who underwent a complementary cardiac magnetic resonance examination. CONCLUSIONS: MAD is common in children with MVP; its presence was not associated with significant VAs on 24-hour Holter monitoring, but the Pickelhaube sign and presence of myxomatous mitral valve may help to detect patients prone to significant VAs. Myocardial fibrosis can be detected by cardiac magnetic resonance in children with significant VAs.

Lympho-hematopoietic malignancies risk after exposure to low dose ionizing radiation during cardiac catheterization in childhood.

Pediatric patients with congenital heart disease (CHD) often undergo low dose ionizing radiation (LDIR) from cardiac catheterization (CC) for the diagnosis and/or treatment of their disease. Although radiation doses from a single CC are usually low, less is known about the long-term radiation associated cancer risks. We aimed to assess the risk of lympho-hematopoietic malignancies in pediatric CHD patients diagnosed or treated with CC. A French cohort of 17,104 children free of cancer who had undergone a first CC from 01/01/2000 to 31/12/2013, before the age of 16 was set up. The follow-up started at the date of the first recorded CC until the exit date, i.e., the date of death, the date of first cancer diagnosis, the date of the 18th birthday, or the 31/12/2015, whichever occurred first. Poisson regression was used to estimate the LDIR associated cancer risk. The median follow-up was 5.9 years, with 110,335 person-years. There were 22,227 CC procedures, yielding an individual active bone marrow (ABM) mean cumulative dose of 3.0 milligray (mGy). Thirty-eight incident lympho-hematopoietic malignancies were observed. When adjusting for attained age, gender and predisposing factors to cancer status, no increased risk was observed for lympho-hematopoietic malignancies RR/mGy = 1.00 (95% CI: 0.88; 1.10). In summary, the risk of lympho-hematopoietic malignancies and lymphoma was not associated to LDIR in pediatric patients with CHD who undergo CC. Further epidemiological studies with greater statistical power are needed to improve the assessment of the dose-risk relationship.

Cardiovascular events in perimembranous ventricular septal defect with left ventricular volume overload: a French prospective cohort study (FRANCISCO).

The long-term prospective multi-centre nationwide (French) observational study FRANCISCO will provide new information on perimembranous ventricular septal defect with left ventricular overload but no pulmonary hypertension in children older than 1 year. Outcomes will be compared according to treatment strategy (watchful waiting, surgical closure, or percutaneous closure) and anatomic features of the defect. The results are expected to provide additional guidance about the optimal treatment of this specific population, which is unclear at present. BACKGROUND: The management of paediatric isolated perimembranous ventricular septal defect (pmVSD) with left ventricle (LV) volume overload but no pulmonary arterial hypertension (PAH) remains controversial. Three therapeutic approaches are considered: watchful waiting, surgical closure, and percutaneous closure. We aim to investigate the long-term outcomes of these patients according to anatomic pmVSD characteristics and treatment strategy. METHODS: The Filiale de Cardiologie Pediatrique et Congénitale (FCPC) designed the FRANCISCO registry, a long-term prospective nationwide multi-centre observational cohort study sponsored by the French Society of Cardiology, which enrolled, over 2 years (2018­2020), patients older than 1 year who had isolated pmVSD with LV volume overload. Prevalent complications related to pmVSD at baseline were exclusion criteria. Clinical, echocardiographic, and functional data will be collected at inclusion then after 1, 5, and 10 years. A core lab will analyse all baseline echocardiographic data to depict anatomical pmVSD features. The primary outcome is the 5-year incidence of cardiovascular events (infective endocarditis, sub-aortic stenosis, aortic regurgitation, right ventricular outflow tract stenosis, tricuspid regurgitation, PAH, arrhythmia, stroke, haemolysis, heart failure, or death from a cardiovascular event). We plan to enrol 200 patients, given the 10% estimated 5-year incidence of cardiovascular events with a 95% confidence interval of ±5%. Associations linking anatomical pmVSD features and treatment strategy to the incidence of complications will be assessed. CONCLUSIONS: The FRANSCICO study will provide the long-term incidence of complications in patients older than 1 year with pmVSD and LV volume overload. The results are expected to improve guidance for treatment decisions.

The slow-flat-slow sequence in malignant vasovagal syncope.

We present the electroencephalographic and electrocardiographic tracing obtained in an 8-year old boy who experienced malignant vasovagal syncope during the recording. This tracing illustrates the highly specific "slow-flat-slow" sequence described in cases of syncope induced by severe cerebral hypoperfusion.

Screening for neurodevelopmental disorders in children with congenital heart disease.

The aim of this study was to evaluate the frequency of neurodevelopmental disorders (NDD) in children with significant congenital heart disease (CHD) and to determine associated factors to NDD and frequency of follow-up in developmental therapies. Two hundred and ten children with significant CHD aged from 6 to 66 months were enrolled over a period of six months. The Ages & Stages Questionnaire Third Edition in French (ASQ-3) was used to assess neurodevelopmental domains. NDD were defined if cut-off scores were ≤ - 1SD. - 1SD corresponded to "Monitor" range: children with minor or emerging disorders; - 2SD corresponded to "Refer" range: children exhibiting neurodevelopmental delays. Forty children were in "Monitor" range and 86 in "Refer" range. NDD rate was 60.0% (n = 126, 95% CI, 53.4 to 66.6%). There was no difference regarding CHD severity (p = 0.99). Only the presence of non-cardiac disease (OR = 2.14; 95% CI, 1.11 to 4.20) was associated with NDD. Forty-six children with NDD had no developmental follow-up (among them 21 were in "Refer" range (10%)) despite this being available.Conclusion: Children with significant CHD are at risk for NDD regardless of CHD severity. Systematic and early monitoring in a specific care program is required. Barriers that prevent access of care must be identified.Trial registration: Neurodevelopmental Disorders in Children With Congenital Heart Disease. NeuroDis-CHD. NCT03360370. https://clinicaltrials.gov/ct2/show/NCT03360370 What is Known: • Children with CHD are at risk for neurodevelopmental disorders and behavioural problems impacting their social adaptation, academic achievements and quality of personal and family life even in adulthood. What is New: • Children with CHD are at risk for neurodevelopmental disorders regardless of the complexity of the CHD. • Even with the availability of appropriate developmental services, children with CHD are not correctly followed, highlighting the need of a specific program of care for a better outcome. Local barriers that prevent access of care of those children must be identified.

Antenatal diagnosis of double-outlet left atrium.

In this study, we describe a fetus with double-outlet atrium associated with complex arrangement of the ventricles and the great vessels. Various presentations of this malformation not described antenatally are discussed.

Transcatheter closure of coronary artery fistulas in infants and children: A French multicenter study.

OBJECTIVES: The short-term efficacy and safety of transcatheter closure (TCC) for the management of coronary artery fistulas (CAFs) was investigated in pediatric patients. BACKGROUND: CAFS are rare with potentially severe complications and their management is still a matter of debate. Percutaneous closure appears to be the treatment of choice in anatomically suitable vessels but limited data are available in the pediatric population. METHODS: This retrospective, observational, multicenter, national study included patients under 16 years of age who underwent TCC of a congenital CAF. Patients with additional congenital heart defect were excluded. RESULTS: 61 patients (36 girls, 25 boys) with a median age of 0.6 year [0-15.4] at diagnosis and 3.9 years [0-16] at procedure were included. The CAF was large in 48 patients (79%); it was distal in 23 (38%) and proximal in 22 (36%). Most patients (77%) were asymptomatic at diagnosis. Clinical signs of congestive heart failure were present in seven patients (11%). Perioperative complications included three cases of ST elevation myocardial infarction (exclusively during attempted closure of a distal CAF), three devices migrations, and one case of leg ischemia. One patient died after surgical closure of a large distal CAF that could not be closed by TCC. Follow-up data were collected for 43 patients (70%) for a median of 91 days [0-4,824]. The Kaplan-Meyer estimate for complete occlusion at 2 years was 73 ± 7.6%. CONCLUSION: TCC in the pediatric population appears to be effective and associated with few complications.

Stenting in paediatric and adult congenital heart diseases: A French multicentre study in the current era.

BACKGROUND: Many stents are used "off-label" during the management of congenital heart diseases (CHD). AIMS: To describe indications for, results of, and adverse events associated with stenting in CHD in current practice. METHODS: Participation in this study was proposed to all catheterization laboratories that specialize in CHD in France (M3C network). All paediatric and adult CHD cases with stent implantation in 2013 were included retrospectively. RESULTS: Overall, 207 stents were implanted in 151 patients across 11 centres. Median age was 13.7 years (range, 5 days to 70.1 years). Main procedure indications were branch pulmonary artery angioplasty (n=46, 29.1%), aortic (re)coarctation stenting (n=43, 27.2%), percutaneous pulmonary valve implantation (n=32, 20.2%) and ductus arteriosus stenting (n=14, 8.9%). The main stents implanted were the CP Stent™ (n=61, 29.5%), the Max™ LD stent (n=43, 20.8%), the Valeo(®) stent (n=28, 13.5%) and valved stents (n=30, 14.5%). Procedures were considered successful in 96.8% of cases (95% confidence interval [CI] 92.8-99.0%). Adverse events were observed in 23 procedures (14.7%, 95% CI 9.5-21.0%). Ductus arteriosus stenting (odds ratio 12.4, 95% CI 2.0-77.5; P<0.01) and pulmonary revalvulation (odds ratio 5.9, 95% CI 1.1-32.3; P=0.04) were risk markers for stent-related adverse events. CONCLUSIONS: Stents are used in various CHD catheterization procedures, from infancy to adult age. The adverse events rate is significant and is related to the type of procedure.

Percutaneous treatment of neonatal aortic coarctation presenting with severe left ventricular dysfunction as a bridge to surgery.

BACKGROUND: Neonatal aortic coarctation presenting with multiorgan failure is a life threatening condition. OBJECTIVE: Our aim was to investigate whether emergency balloon dilation improved the prognosis of neonates with aortic coarctation who present with multiorgan failure. METHODS: We studied all neonates referred over a period of 6 years with aortic coarctation and left ventricular dysfunction, with or without multiorgan failure. During the first half of the period, from January, 2001 to December, 2003, patients were treated surgically after regression of the multiorgan failure. During the second half, from January, 2004, to January, 2007, patients not responding to medical management were referred for balloon dilation of the coarcted segment. RESULTS: Among 113 and 181 neonates, respectively, treated during the 2 periods, 40 and 36 had left ventricular dysfunction (p less than 0.01), and 11 and 23 had multiorgan failure (p less than 0.01). Prior to 2004, 6 deaths occurred before surgery. After 2004, 3 out of 14 patients (21%) treated by balloon angioplasty died in consequence of the procedure.During the period of the study, the global mortality of patients with left ventricular dysfunction diminished from 17.5 to 13.8%, this difference not being statistically significant. Mortality due to multiorgan failure, however, diminished from 54 to 13% (p less than 0.01). Multiorgan failure was reversed under medical treatment in 45% of patients, but in 87% under the regime of medical combined with interventional treatment (p less than 0.01). CONCLUSIONS: To the best of our knowledge, this is the first series of neonates with aortic coarctation and multiorgan failure receiving a palliative treatment by balloon dilation. Although the prognosis of this condition remains severe, emergency balloon dilation can diminish mortality, providing a bridge to surgery in severely ill patients.

Experimental off-pump transventricular pulmonary valve replacement using a self-expandable valved stent: A new approach for pulmonary incompetence after repaired tetralogy of Fallot?

OBJECTIVE: Off-pump valve replacement using self-expandable stents is an emerging technique for pulmonary valve disease. However, significant limitations are the lack of easily available valve substitute to be inserted within the stent and, in the setting of repaired tetralogy of Fallot, the existence of huge pulmonary trunk. We report the first experimental results of a transventricular approach using a decellularized porcine xenograft mounted in a self-expandable stent. METHODS: Pulmonary valve replacement was realized in 15 lambs by direct access of the infundibulum through a left thoracotomy, combined with pulmonary artery banding. Animals were followed by transthoracic echocardiography and, after control hemodynamic study, were electively killed either at day 7, month 1, or month 4 after implantation. RESULTS: Implantation succeeded in all lambs. Two animals died after implantation (1 pneumothorax and 1 endocarditis). Doppler echocardiographic follow-up did not show any significant transvalvular gradient and showed only mild pulmonary regurgitation. The hemodynamic control before termination revealed a systolic pulmonary valve gradient of 18.5 +/- 12.4 mm Hg at 1 week (n = 4), 13.5 +/- 10.6 mm Hg at 1 month (n = 4), and 4.3 +/- 4.9 mm Hg at 4 months (n = 5). Gross examination demonstrated the presence of connective tissue between the valved stent and pulmonary wall, which increased with time. CONCLUSION: Fifteen lambs underwent successful deployment of a self-expandable valved stent in the pulmonary position using a transventricular approach. This technique combined with pulmonary artery banding could be a therapeutic option for pulmonary insufficiency after repair of tetralogy of Fallot with a transannular patch.