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.

Prevalence and Associated Factors of Long-term Growth Failure in Infants with Congenital Heart Disease Who Underwent Cardiac Surgery Before the Age of One.

Long-term growth failure can have negative impact on health (by increasing morbidity and mortality) and on neurodevelopmental outcomes. Its prevalence among children with congenital heart disease (CHD) is not well described. The aim of our study was to evaluate the prevalence of growth failure in a population of infants with CHD away from cardiac surgery and identify associated factors. We conducted a retrospective and multicentric study that included infants from the North of France who underwent cardiac surgery before the age of one, between January 2013 and December 2017. 331 infants were included among which 48% had a prenatal diagnosis, 15% had a genetic syndrome, and 15% were premature infants. Mean birth weight was 3 ± 0.6 kg. At surgery, 35% presented feeding difficulties (need for enriched formula and/or feeding tube) and 14% had growth failure (defined by Z-score weight for age < -2SD). 6-12 months after surgery, 16% still presented growth failure. Several associated factors were identified: prenatal diagnosis, genetic syndrome association, birth weight ≤ 3 kg, complex CHD (≥ 2 significative lesions, or double outlet right ventricle or single ventricle physiology), surgery after 30 days, and need for diuretic drug before surgery and/or still needed 1 month after surgery. Growth failure persists between 6 and 12 months after surgery in 16% of infants with CHD. More studies are needed to link growth failure and neurodevelopment, which is the new challenge for this aging population.

Exposure to low-dose ionising radiation from cardiac catheterisation and risk of cancer: the COCCINELLE study cohort profile.

PURPOSE: The COCCINELLE study is a nationwide retrospective French cohort set up to evaluate the risk of cancer in patients who undergone cardiac catheterisation (CC) procedures for diagnosis or treatment of congenital heart disease during childhood. PARTICIPANTS: Children who undergone CC procedures from 1 January 2000 to 31 December 2013, before the age of 16 in one of the 15 paediatric cardiology departments which perform paediatric CC in mainland France were included. The follow-up started at the date of the first recorded CC procedure until the exit date, that is, the date of death, the date of first cancer diagnosis, the date of the 18th birthday or the 31 December 2015, whichever occurred first. The cohort was linked to the National Childhood Cancer Registry to identify patients diagnosed with cancer and with the French National Directory for the Identification of Natural Persons to retrieve the patients' vital status. FINDINGS TO DATE: A total of 17 104 children were included in the cohort and followed for 110 335 person-years, with 22 227 CC procedures collected. Among the patients, 81.6% received only one procedure. Fifty-nine cancer cases were observed in the cohort. Standardised incidence ratios (SIRs) were increased for all-cancer (SIR=3.8, 95% CI: 2.9 to 4.9), leukaemia (SIR=3.3, 95% CI: 2.0 to 5.4), lymphoma (SIR=14.9, 95% CI: 9.9 to 22.5) and solid cancers excluding central nervous system (CNS) tumours (SIR=3.3, 95% CI: 2.0 to 5.5) compared with the general population. FUTURE PLANS: Dose reconstruction is currently underway to estimate individual cumulative doses absorbed to relevant organs, including red bone marrow and brain for respectively haematologic disorders and CNS tumours risk estimation. A dose-response analysis will be conducted with consideration to confounding factors such as age at exposure, gender, predisposing factors to cancer and other sources of medical diagnostic low-dose ionising radiation.

French national survey on infective endocarditis and the Melody™ valve in percutaneous pulmonary valve implantation.

BACKGROUND: Percutaneous pulmonary valve implantation (PPVI) is a routine treatment for dysfunctional right ventricular outflow tract. Infective endocarditis (IE) is a major concern. AIM: To report French experience with the Melody™ valve (Medtronic Inc., Minneapolis, MN, USA). METHODS: All patients who underwent PPVI were recorded in a multicentre French national survey. Demographic and procedural data were collected from patients with IE. Bacterial identification, diagnostic tools and outcome were recorded. RESULTS: Forty-five cases of IE were diagnosed in 43 patients. The cumulative IE incidence was 11.8% (95% confidence interval [CI] 8.5-15.9). The annualized IE incidence was 3.6% (95% CI 0-4.8). Freedom from IE was 96.3% and 85.8% at 12 months and 60 months, respectively. IE incidence did not change during the study period. The mean interval between PPVI and IE was 2.6±2.1 years (range, 5 days to 7.3 years). Fifteen patients with IE required intravenous antibiotics only. Seven patients had early interventional cardiac catheterization to relieve severe right ventricular outflow tract obstruction. Twenty-four patients had surgical valve replacement (six urgently; nine semi-urgently; nine electively). Staphylococcus aureus IE required surgery in all but one patient. Three patients died before any treatment. Three additional patients died, giving a mortality rate of 14%. Global survival in the total cohort of patients who received a Melody valve was excellent (96.5% at 5 years). When comparing survival curves between the IE and non-IE groups, death and cardiovascular events were statistically significantly higher in the IE group (log-rank P<0.0001). CONCLUSION: Melody valve IE is a severe complication following PPVI. The annualized IE incidence in this cohort was similar to rates reported in other studies. With rapid diagnosis and adequate treatment, outcome has improved, and unfavourable outcome is mainly associated with S. aureus.

Short and long-term outcomes of alcohol septal ablation with the trans-radial versus the trans-femoral approach: A single center-experience.

BACKGROUND: Although the trans-radial approach (TR) has been applied to various subsets of patients in percutaneous coronary intervention, the feasibility, efficacy, acute procedural and long-term outcomes of TR versus trans-femoral approach (TF) for alcohol septal ablation (ASA) have not yet been determined. OBJECTIVES: The aim of this study was to compare the short and long-term outcomes of ASA with the TR approach compared to the TF approach. METHODS: We retrospectively analyzed 240 patients who underwent an ASA procedure at our institution from November 1999 to November 2015. The TR approach was performed in 172 cases and the TF approach in the remaining 68 cases. RESULTS: The use of TR approach progressively increased from 62% in 1999-2005 to 91% in 2011-2015 (p=0.0001). The TF and TR group had similar age, baseline NYHA class (NYHA 3 or 4) and mean left ventricular outflow tract peak gradient before ASA. Total contrast used (TR: 73.2±47.2ml; TF: 88.7±49.3ml, p=0.11), total radiation Air kerma area product (TR: 43.7±48.0Gycm(-2); TF: 55.9±48.2Gycm(-2); p=0.39) and peak left ventricular outflow tract gradient immediately after ASA (TR: 19.1±19.6mmHg; TF: 20.4±18.0mmHg, p=0.63) were similar in both groups. Procedural success was 91.9% and 91.2% in the TR and TF groups, respectively (p=0.53). At 30days, there was 2 intra-hospital death (1 in TF and 1 in TR), 1 major stroke in the TF group and 1 coronary artery dissection in the TR group. Vascular complications were less frequent in the TR group (0.58% vs. 7.3%; p=0.002). The mean length of follow-up was 4.56±4.34years (IQR 0.69-8.2; median 2.92years; maximum: 15.5years). By Kaplan-Meier estimate, the observed survival in the overall cohort was comparable to the expected survival for a sex and age-matched comparable general French population at 10years (86.9 vs. 83.6%, p=0.88). Survival was similar between the TR and TF group (92.1% vs. 89.7% at 6years, respectively; p=0.71). CONCLUSIONS: Alcohol septal ablation from the radial approach can be performed with similar acute and long-term success, but with lower vascular complications compared to the femoral approach.

Significant persistent ductus arteriosus in infants less or equal to 6 kg: percutaneous closure or surgery?

BACKGROUND: Percutaneous closure of large persistent ductus arteriosus using the Amplatzer duct occluder is an alternative to surgery. However, this device is not recommended in infants weighing less than 6 kg. AIM: To evaluate the safety and effectiveness of this procedure in low-body-weight infants. METHODS: We reviewed retrospectively data for infants weighing less or equal to 6 kg who underwent percutaneous closure of significant persistent ductus arteriosus using the Amplatzer duct occluder in France between 1998 and 2007. RESULTS: Data for 58 patients (mean weight: 5 kg, range: 3.4-6; mean age: 5.5 months, range: 2.1-15.3) were reviewed. Mean angiographic persistent ductus arteriosus minimal diameter was 3.7 mm (range: 1-7.5). Implantation of the Amplatzer duct occluder was successful in 89.7% of cases. In six (10.3%) patients, the device was not implanted because it would have led to significant aortic obstruction. One procedure-related death occurred in a 4 kg infant (1.7%). Major and minor complications occurred in 6.9 and 31.0% of patients, respectively. Persistent ductus arteriosus diameter greater than 3.7 mm, type C (tubular shape) and diameter/patient weight ratio greater than 0.91 were significantly associated with an unsuccessful procedure and/or major complications. During a median 10-month follow-up, no late device embolization occurred. CONCLUSIONS: Although percutaneous closure of significant persistent ductus arteriosus with the Amplatzer duct occluder is effective in low-body-weight infants, the level and severity of complications indicate surgery as first-line treatment, at least until further studies are done to assess the safety and effectiveness of the new Amplatzer duct occluder II in low-body-weight infants.

Efficiency of transcatheter closure of atrial septal defects in small and symptomatic children.

OBJECTIVES: We report the multicentric French experience with transcatheter closure in children weighing 15 kilograms or less, with the aim of assessing the efficacy of the procedure in this age group. PATIENTS: We included all children weighing 15 kilograms or less, and seen between January, 1997, and June, 2004, who had successful transcatheter closure of an interatrial communication within the oval fossa. RESULTS: Transcatheter closure was performed in 35 patients weighing 15 kilograms or less, of whom 14 were male and 21 female. The procedures were undertaken in 8 different centres, the patients having a median age of 3 years, with a range from zero to 6.2 years, and a mean weight of 13 kilograms, with a range from 3.6 to 15 kilograms. All the patients were symptomatic, with associated cardiac malformations present in 4 cases, and extracardiac anomalies in 4 patients, including Down's syndrome in 3, and Adams Oliver syndrome in the other case. In 1 patient, emergency cardiac surgery was needed 24 h after the procedure to correct a previously undiagnosed divided right atrium. No other complication occurred. After a median follow-up of 2 years, with a range from 0.5 to 5.2 years, all the patients are asymptomatic, except for one long-standing patient with bronchodysplasia. In 1 other patient, a small residual bidirectional shunt was detected by echocardiography. No patient presented significant arrhythmia. In the patients followed-up for more than 12 months, we found a significant gain in weight gain. CONCLUSION: Transcatheter closure of an interatrial communication within the oval fossa is efficient in children weighing 15 kilograms or less, and can be proposed as a first line of treatment in symptomatic patients. Children with retarded growth tend to have complete recovery within one year of closure.

Transcatheter closure of congenital ventricular septal defects: results of the European Registry.

AIM: To report the experience of 23 tertiary referral European Centres on transcatheter closure of congenital ventricular septal defects (VSD). METHODS AND RESULTS: Implantation of transcatheter devices was attempted in 430 patients (pts) with congenital VSDs until July 2005. The following anatomic types were present: 119 muscular, 250 perimembranous, 16 multiple, 45 residual post-surgery. Median VSD size was 7 mm (range 3-22), fluoroscopy time 33 min (range 3-146). Devices implanted were Amplatzer muscular or membranous devices in 364, PDA devices in 12, ASD devices in seven, Starflex in seven, and coils in nine patients. Procedure was successful in 410 cases (95%). COMPLICATIONS: device embolization in five cases (surgery in two, catheter retrieval in three), aortic regurgitation in 14 cases (two of which requiring surgery), tricuspid regurgitation in 27 cases (no surgery was necessary), minor rhythm disturbances in 10 pts, death in one patient, complete heart block (cAVB) in 16 pts [perimembranous 12 of 250 (5%), muscular one of 119 (0.8%), residual post-surgery VSD three of 45 (6.7%)]. CAVB was transient in six patients, requiring permanent pace-makers in 10 cases (3.8%) (six early, four late). In the multivariate analysis, the only variable associated with a risk of the occurrence of complication was age (P=0.012) and weight (P=0.0035). In the univariate analysis, risk factors for the development of cAVB were, device type (P=0.03) and VSD location (P=0.05). After the multivariable Cox proportional hazards analysis, no risk factor was found. CONCLUSION: Transcatheter closure of congenital VSDs offers encouraging results. COMPLICATIONS are limited; the most relevant one seems to be the device related to cAVB in perimembranous VSD. More experience and long-term follow-up are mandatory to assess safety and effectiveness of this procedure as an alternative to conventional surgery.

Perforation of the atretic pulmonary valve. Long-term follow-up.

OBJECTIVES: We evaluated the long-term results of perforation of the pulmonary valve in patients with pulmonary atresia with an intact ventricular septum (PA-IVS). BACKGROUND: Interventional perforation of the pulmonary valve is considered the elective first stage treatment for PA-IVS, particularly in patients with a tripartite right ventricle (RV) and normal coronary circulation. However, the long-term results of this procedure are lacking. METHODS: Between January 1991 and December 2001, 39 newborns with a favorable form of PA-IVS underwent attempted perforation of the pulmonary valve. We evaluated the early and long-term outcomes. RESULTS: Median tricuspid and pulmonary z values were -1.2 and -2.4, respectively. Perforation was successful in 33 patients. Among them, 17 needed neonatal surgery, 13 did not need any surgery, and 3 had elective surgery after the first month of life. There were two procedure-related deaths, seven nonfatal procedural complications, and four postsurgical deaths. Compared with patients needing neonatal surgery, those having no or elective surgery had a higher incidence of a tripartite RV and a higher median tricuspid z value (92% vs. 53%, p = 0.04 and -1.7 vs. -0.5, p = 0.03). At a median follow-up of 5.5 years (range 0.5 to 11.5), survival was 85% and freedom from surgery was 35%. Five patients, four of whom had neonatal surgery, underwent a partial cavo-pulmonary connection. CONCLUSIONS: Our results show that this technique, although burdened by non-negligible mortality and morbidity, is effective in selected patients with a normal-sized RV. Preselection of patients allows interventional or surgical biventricular correction in the majority of cases.

Transcatheter closure of traumatic ventricular septal defect: an alternative to surgical repair?

A 19-year-old man with multiple-system injuries including a serious head injury and two poorly tolerated traumatic ventricular septal defects, was admitted to our hospital. Transcatheter closure of the cardiac defects was attempted instead of surgical repair because the required anticoagulation for cardiopulmonary bypass could precipitate intracranial bleeding. The two ventricular septal defects were successfully closed with Amplatzer devices, but the patient remained in hemodynamically unstable condition and subsequently died. Transcatheter closure of traumatic ventricular septal defect is an alternative to surgical repair, although it remains a hazardous procedure and requires experienced anesthesia management.