Caroline Bonnet

Outcomes and safety of transcatheter pulmonary valve replacement in patients with large patched right ventricular outflow tracts

BACKGROUND Although globally accepted, the indication for implantation of the Melody(®) (Medtronic Inc., Minneapolis, MN, USA) transcatheter pulmonary valve is limited to the treatment of haemodynamically dysfunctional right ventricular outflow tract (RVOT) with right ventricle to pulmonary artery (PA) obstruction. The use of the Melody valve for haemodynamically significant isolated pulmonary regurgitation has not been evaluated. AIM We evaluated the outcomes of Melody valve insertion in patients with a large patched RVOT. METHODS We analysed procedural and short-term outcomes data from 13 patients who underwent Melody valve implantation for a large RVOT with significant pulmonary regurgitation as the primary lesion. RVOT preparation was done in all patients using the Russian dolls technique and/or the PA jailing technique. Melody valve insertion was performed concomitantly in 10 patients and after 1 to 3 months in three patients. RESULTS All procedures were successful. The mean follow-up period was 30 ± 4 months after the procedure. There was no incidence of stent fracture, migration or embolization. Only one patient who underwent the jailing technique developed a significant paraprosthetic leak and is scheduled for redilatation of the Melody valve. CONCLUSIONS Careful patient selection, balloon sizing and RVOT preparation with prestenting using the Russian dolls technique and/or the PA jailing technique are required to modify the RVOT for transcatheter valve implantation. Short-term follow-up showed competent valves with no stent fracture or migration and appears promising. Wider experience with long-term outcomes may be required to standardize the procedure in such a subset of patients.

Characteristics and long-term outcome of non-immune isolated atrioventricular block diagnosed in utero or early childhood: a multicentre study

AIMS The natural history of congenital or childhood non-immune, isolated atrioventricular (AV) block is poorly defined. METHODS AND RESULTS We retrospectively studied 141 children with isolated, non-immune AV block diagnosed in utero, or up to 15 years of age, at 13 French medical centres, between 1980 and 2009. Patients with structural heart disease or maternal antibodies were excluded. Atrioventricular block was asymptomatic in 119 (84.4%) and complete in 100 (70.9%) patients. There was progression to complete AV block in 29/41 (70.7%) patients with incomplete AV block over 2.8 ± 3.4 years (1-155 months), but all patients with incomplete AV block may not have been included in the study. Narrow QRS complex was present in 18 of 26 patients (69.2%) with congenital, and 106 of 115 (92.2%) with childhood AV block. Pacemakers were implanted in 112 children (79.4%), during the first year of life in 18 (16.1%) and before 10 years of age in 90 (80.4%). The mean interval between diagnosis of AV block and pacemaker implants was 2.6 ± 3.9 years (0-300 months). The pacing indication was prophylactic in 70 children (62.5%). During a mean follow-up of 11.6 ± 6.7 years (1-32 years), no patient died or developed dilated cardiomyopathy (DCM). The long-term follow-up was uncomplicated in 127 children (90.1%). CONCLUSION In this large multicentre study, the long-term outcome of congenital or childhood non-immune, isolated AV block was favourable, regardless of the patients age at the time of diagnosis. No patient died or developed DCM, and pacemaker-related complications were few.

Complications of paediatric interventional catheterisation: an analysis of risk factors.

OBJECTIVES To identify predictive factors of complications occurring during paediatric interventional catheterisation. BACKGROUND Interventional paediatric catheterisation is still burdened by a substantial risk. Risk factors, however, have rarely been investigated. METHODS We analysed prospectively 1,022 interventional procedures performed over a period of 8 years, excluding 260 procedures for atrial septostomy. We considered several patient-related variables, specifically age, weight, and gender, type of procedure, times required for fluoroscopy and the overall procedure, technical challenge, and the severity of the clinical condition. We also analysed variables linked to the environment, specifically the date of the examination, whether the operator remained in training, the novelty of the material, any breakdown in the installation, and errors made by the operator. We classified complications as those without clinical consequence, those which proved lethal, those requiring cardiopulmonary resuscitation, elective or emergency surgery, hospitalisation in the intensive care unit, and those leading to recatheterisation. RESULTS Our average incidence of complications was 4.1 per cent, which did not change significantly during the period of study. Of the patients, 4 died, 7 needed urgent surgery, 5 elective surgery, 3 hospitalisation in intensive care unit, and 8 recatheterisation. Independent risk factors for complications were technical challenge, critical clinical condition, operator in training, operator error, and breakdown of the installation. Young age was not associated with a higher risk of complications. Patients in whom no cause for complication could be found, either related to their own features or the environment, had a risk of complication of 1.4 per cent (95 per cent confidence intervals from 0.7 to 2.5 per cent). CONCLUSIONS Our data show that variables relating either to the patient or the environment of catheterisation are associated with an increased risk of procedural complications. Knowledge of the risk factors can improve the odds of paediatric interventional catheterisation.

Parental Electrocardiographic Screening Identifies a High Degree of Inheritance for Congenital and Childhood Nonimmune Isolated Atrioventricular Block

Background— The origin of congenital or childhood nonimmune isolated atrioventricular (AV) block remains unknown. We hypothesized that this conduction abnormality in the young may be a heritable disease. Methods and Results— A multicenter retrospective study (13 French referral centers, from 1980–2009) included 141 children with AV block diagnosed in utero, at birth, or before 15 years of age without structural heart abnormalities and without maternal antibodies. Parents and matched control subjects were investigated for family history and for ECG screening. In parents, a family history of sudden death or progressive cardiac conduction defect was found in 1.4% and 11.1%, respectively. Screening ECGs from 130 parents (mean age 42.0±6.8 years, 57 couples) were compared with those of 130 matched healthy control subjects. All parents were asymptomatic and in sinus rhythm, except for 1 with undetected complete AV block. Conduction abnormalities were more frequent in parents than in control subjects, found in 50.8% versus 4.6%, respectively (P<0.001). A long PR interval was found in 18.5% of the parents but never in control subjects (P<0.0001). Complete or incomplete right bundle-branch block was observed in 39.2% of the parents and 1.5% of the control subjects (P<0.0001). Complete or incomplete left bundle-branch block was found in 15.4% of the parents and 3.1% of the control subjects (P<0.0006). Estimated heritability for isolated conduction disturbances was 91% (95% confidence interval, 80%–100%). SCN5A mutation screening identified 2 mutations in 2 patients among 97 children. Conclusions— ECG screening in parents of children affected by idiopathic AV block revealed a high prevalence of conduction abnormalities. These results support the hypothesis of an inheritable trait in congenital and childhood nonimmune isolated AV block.

Mid-term effects of implanting stents for relief of aortic recoarctation on systemic hypertension, carotid mechanical properties, intimal medial thickness and reflection of the pulse wave

OBJECTIVE Primary implantation of stents is an accepted technique for treating aortic recoarctation, albeit that the effects of stenting on pressure profiles, carotid mechanical properties, intimal medial thickness, and reflection of the pulse wave have not been systematically investigated. METHODS Over the period from 1 January, 1999, to 31 December, 2002, we implanted stents to relieve aortic recoarctation in 15 patients, with a median age of 17 years, and a range from 7 to 29 years, with a median weight of 56 kilograms, ranging from 20 to 96 kilograms. Indications were a gradient of 20 millimetres of mercury or more measured in all, systemic hypertension at rest in 8, and systemic hypertension at exercise in all. Of the patients, 5 were receiving anti-hypertensive treatment. Before implantation of the stents, and after a mean follow-up of 22 months, all patients underwent an exercise test, vascular echography, and examination of the common carotid artery so as to determine its cross sectional compliance and distensibility, and the augmentation index. results: The stents were implanted successfully in all patients. The mean gradient was reduced from 27 to 4 millimetres of mercury (p < 0.001). Systolic blood pressure at rest diminished from 140 to 131 millimetres of mercury (p = 0.04), while hypertension at rest regressed in 4 patients. Systolic blood pressure at exercise diminished from 245 to 222 millimetres of mercury (p = 0.018), and hypertension at exercise regressed in 1 patient. Anti-hypertensive treatment is still required for 4 patients. A correlation was found between systolic blood pressure at rest and initial peak-to-peak gradient (r = 0.8), and between initial gradient and percentage reduction of systolic blood pressure at rest at follow-up (r = -0.73). Compliance and distensibility of the common carotid artery were not significantly modified, albeit that the intimal medial thickness diminished from 0.64 to 0.57 millimetres (p = 0.04), and the augmentation index decreased from 5 to -1 (p = 0.012). CONCLUSIONS Primary implantation of stents is effective in mid-term repair of aortic recoarctation. Although there is an improvement in systemic hypertension, the tensional profile and vascular sonography are not normalized. At long term follow-up, the suppression of an early reflection site of the pulse wave could decrease the wall stress of the great elastic vessels, reducing the thickness of the arterial walls.

CO 7 Outcomes after protein-losing enteropathy in univentricular hearts: A multicenter study

Suzanne Borrhomée*, Sébastien Hascoët, Alban-Elouen Baruteau, Jérôme Petit, Lucile Houyel, Marielle Gouton, Régine Roussin, Marianne Peyre, Mohammed Ly, Emre Belli, Emmanuel Lebret, Serge Demontoux, Virginie Lambert, Daniela Laux 1 Centre chirurgical Marie-Lannelongue, Pôle des cardiopathies congénitales, Centre de référence M3C des cardiopathies congénitales complexes, Le Plessis-Robinson, France.

325 Clinical presentation and long-term clinical outcomes of non immune, isolated atrioventricular block diagnosed in utero or early childhood

Results: Pregnancies were bichorionic-biamniotic (BCBA) in 103 cases and monochorionic in 112 (biamniotic 92-MCBA, monoamniotic 20-MCMA) and the remaining 11 were unknown. Overall the 2 foetuses were affected in 35 cases (15.4%) with the two CHDs belonging to the same group in 23 (65.7%). The two foetuses had a CHD in 35% of MCMA, 14.1% of MCBA and in 10.7% of BCBA. The most frequent defects were conotruncal defects (n=71) with concordance in 13/71 (8/20 MCBA, 3/5 MCMA). For other groups concordance in all pregnancies, in MCBA and in MCMA were respectively: right outflow tract obstructions 5/48, 0/33, 0/0; obstructive left heart diseases 7/42, 2/13, 1/1; laterality defects 2/36, 0/6, 2/12; ventricular septal defects 5/19, 1/9, 0/0; atrioventricular septal defects 3/9, 1/2, 0/0; functionally univentricular heart 1/15, 0/5, 0/1. MCBA pregnancies were more frequent in right outflow tract obstructions (33/48; 69% – p<0.01) and discordance between twins was constant (pulmonary stenosis in the recipient twin) and was associated with proven TTTS in 22/33. MCMA were more frequent in laterality defects (12/36; 33%-p<0.01) and discordance between twin of was observed in 10/12.

345 Safe treatment of infantile hemangiomas with propranolol despite baseline bradycardia

Background: The prevalence of obesity in children is increasing worldwide. We used 2D speckle strain imaging to investigate whether severely overweight children without hypertension, dyslipidemia, diabetes or sleep apnea, show early cardiac abnormalities. We also investigated the relation between these myocardial features and severity of obesity, fat mass percentage, inflammation and insuline resistance index.

242 Clinical presentation and long-term outcome of non immune and isolated atrioventricular block when congenital or diagnosed during childhood: a French multicentric study on 141 patients

Purpose When isolated and non immune, prevalence of congenital and childhood atrioventricular blocks (AVB) is extremely low and little is known about their natural history. Methods a multicentric study retrospective from 1980 to 2009 allowed inclusion of 141 children from 13 French referral centers. Included children presented an AVB diagnosed in utero, at birth or during childhood before the age of 15 years, without structural heart abnormalities and without maternal antibodies. Results 26 congenital and 114 childhood AVB were included. Symptoms lead to diagnosis in 15,6% whereas AVB was asymptomatic in 84,4%. 73% AVB were complete and 26,2% AVB were incomplete at first presentation. 21 of these incomplete blocks (56,7%) progressed to permanent complete AVB. Narrow QRS complexes were found in 69,2% congenital and 91,2% childhood AVB. In the 112 (79,4%) implanted children, mean duration between AVB diagnosis and pacemaker implantation was 35 months. Pacemaker primo-implantation occurred during the first year of life for 18 children and 90 children (63.8%) were paced before 10 years old. Pacing was required for symptomatic bradycardia in 37,5% whereas prophylactic cardiac pacing accounted for 61,6%. The median follow-up was 96 months (from 6 to 384 months). 85,1% experienced no complication and neither dilated cardiomyopathy nor death had occurred at last follow-up. Pacemaker-related complications appeared in 11,6%. Conclusion We describe the largest reported experience with isolated and non immune congenital and childhood AVB. Such a block is a nodal damage from unknown origin that may postnatally progress in incomplete forms. Outcome is not influenced by age at diagnosis. Prognosis is very good with no late-onset dilated cardiomyopathy, a few pacemaker-related complications in the modern technological era and no death at last follow-up.

310 Aorto-pulmonary anastomosis in tuberous sclerosis and cardiac tumor with severe right ventricular outflow tract obstruction (a case report)

Primary cardiac tumors are rare with an estimated incidence of 0.27% in pediatric autopsies. The most common type of cardiac tumor identified in infancy and childhood is rhabdomyoma. We report a case of prenatal diagnosis of multiples cardiac rhabdomyoma at 29 weeks of gestation. Full-term delivery was straightforward without hydrops and dysrhythmia. In the immediate post-natal period, cyanosis appeared, and echocardiography showed multiple cardiac rhabdomyoma and severe right ventricular outflow tract obstruction (Image 1). Aprostadil perfusion was necessary. Aorto-pulmonary anastomisis was performed with success. The diagnosis of cardiac rhabdomyoma was confirmed histologically and tuberous sclerosis by molecular genetic analysis. Renal echocardiography was normal and cerebral MRI was not perfomed. Fourteen months later, neurodevelopment was normal. Echocardiography confirmed regression of the cardiac tumors with disappearance of the severe right ventricular outflow tract obstruction. ECG monitoring was normal. Conclusion The natural history of most cardiac rhabdomyoma is favorable with tumors regressing (completely or partially). Surgical resection of the tumors is required for severe ventricular outflow tract obstruction. Aorto-pulmonary anastomosis, as in our case with severe right ventricular outflow tract obstruction, is an alternative treatment because regression and even complete resolution of more than 80% of the tumors occurs during infancy and early childhood. Download : Download full-size image