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[Display omitted] • The Trevisio DS flexible tip improves occluder position assessment before release. • Trevisio PAS studied the Trevisio DS during transcatheter closure of PFO and ASD. • 251 patients (144 PFO and 107 ASD) were enrolled at 22 European sites. • The Trevisio DS exhibited a high rate of technical success and excellent safety. The Amplatzer™ Trevisio™ Intravascular Delivery System (Trevisio DS; Abbott Laboratories, Chicago, IL, USA) facilitates the delivery of Amplatzer™ Occluders and features an ultraflexible tip, which improves assessment of occluder position before release. To assess the safety and efficacy of the Trevisio DS for transcatheter closure of patent foramen ovale and atrial septal defect. The Amplatzer™ Trevisio™ Intravascular Delivery System Post-Approval Study was a prospective, postmarket, single-arm, multicentre, observational study of the Trevisio DS. Enrolled patients were indicated for transcatheter closure of patent foramen ovale or atrial septal defect. In all procedures, the Trevisio DS was used to deliver Amplatzer™ Occluders. Technical success was defined as successful deployment and release of at least one occluder. Device- or procedure-related serious adverse events were tracked until discharge or day 7, whichever occurred earlier. The study enrolled 144 patients with patent foramen ovale and 107 patients with atrial septal defect at 22 European sites; 53 patients with atrial septal defect (49.6%) were aged < 18 years. The rate of technical success was 98.4% (97.2% for atrial septal defect, 99.3% for patent foramen ovale). There was one serious adverse event (0.4%), an acute periprocedural device embolization that occurred after occluder release in a patient with atrial septal defect; the device was retrieved percutaneously. This was determined by the implanter to be unrelated to the performance of the Trevisio DS. The Trevisio DS exhibited a high rate of technical success and an excellent safety profile during transcatheter closure of patent foramen ovale and atrial septal defect. [ABSTRACT FROM AUTHOR]

Superior sinus venosus defect is a communication between the right and left atrium located above the upper margin of the oval fossa, immediately inferior to the junction of the superior vena cava and the right atrium. It is systematically associated with partial anomalous pulmonary venous drainage, especially of the right upper pulmonary vein. Surgical repair has been the gold standard approach to close that defect. Introduced in 2014, percutaneous closure has gradually become a safe and effective alternative to surgery in carefully selected patients, although worldwide experience remains limited. This article provides an appraisal of the patients' selection process and a step-by-step description of the procedure as well as a comprehensive review of its outcomes. [Display omitted] • Surgical repair is the standard of care for superior sinus venosus defect with partial anomalous pulmonary venous drainage. However, transcatheter closure is emerging as a safe and effective alternative in carefully selected patients. • Knowledge of the anatomy of crossroads formed by the right upper pulmonary vein, superior vena cava, right atrium, and left atrium is key to appreciating the procedural concept. • Optimal patient selection requires the integration of multimodality imaging, 3D modeling, and balloon interrogation of the superior vena cava. • Modified techniques along with growing experience of interventionalists will further enhance safety and expand the role of transcatheter closure of SVD. [ABSTRACT FROM AUTHOR]

The aim of this study was to assess the diagnostic performances of 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET)/computed tomography (CT) in congenital heart disease (CHD) patients with pulmonary prosthetic valve or conduit endocarditis (PPVE) suspicion. PPVE is a major issue in the growing CHD population. Diagnosis is challenging, and usual imaging tools are not always efficient or validated in this specific population. Particularly, the diagnostic yield of 18F-FDG PET/CT remains poorly studied in PPVE. A retrospective multicenter study was conducted in 8 French tertiary centers. Children and adult CHD patients who underwent 18F-FDG PET/CT in the setting of PPVE suspicion between January 2010 and May 2020 were included. The cases were initially classified as definite, possible, or rejected PPVE regarding the modified Duke criteria and finally by the Endocarditis Team consensus. The result of 18F-FDG PET/CT had been compared with final diagnosis consensus used as gold-standard in our study. A total of 66 cases of PPVE suspicion involving 59 patients (median age 23 years, 73% men) were included. Sensitivity, specificity, positive predictive value, and negative predictive value of 18F-FDG PET/CT in PPVE suspicion were respectively: 79.1% (95% CI: 68.4%-91.4%), 72.7% (95% CI: 60.4%-85.0%), 91.9% (95% CI: 79.6%-100.0%), and 47.1% (95% CI: 34.8%-59.4%). 18F-FDG PET/CT findings would help to correctly reclassify 57% (4 of 7) of possible PPVE to definite PPVE. Using 18F-FDG PET/CT improves the diagnostic accuracy of the Duke criteria in CHD patients with suspected PPVE. Its high positive predictive value could be helpful in routine to shorten diagnosis and treatment delays and improve clinical outcomes. [Display omitted] [ABSTRACT FROM AUTHOR]

Copyright of Archives of Cardiovascular Diseases is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart disease usually diagnosed during the first months of life. Without surgical treatment, ALCAPA carries a high mortality risk, and disease presentation in adulthood is rare. We describe the diagnosis and management of patients presenting with ALCAPA in adulthood. This multicenter French nationwide retrospective study included adult patients diagnosed from 1980 to 2014. Eleven adult patients (mean age: 38 ± 17 years) were analyzed. All patients were symptomatic, presenting with chest pain, palpitations, heart failure, or syncope. Electrocardiogram was abnormal in 8 (73%) patients. Echocardiogram showed a mildly depressed left ventricular ejection fraction of 50 ± 13%, kinetic abnormalities in 5 (45%) patients, and significant mitral regurgitation in 8 (73%) patients. Coronary angiography was performed in 10 (91%) patients and confirmed the diagnosis. Computerized tomography scan, magnetic resonance imaging, and myocardial scintigraphy were performed when deemed necessary. Ten patients underwent reconstructive surgery, but 1 patient was not operated because of age. Four patients experienced postoperative complications including cardiogenic shock, heart failure, renal failure, or additional surgery. After a median follow-up of 2.5 years, all 10 operated patients were alive and asymptomatic, and the nonoperated patient had died at the age of 70 from syncope related to ventricular tachycardia. ALCAPA may be diagnosed in adults. Although complications may occur postoperatively, long-term outcome is favorable in adult patients undergoing surgical correction. Surgery should be discussed as first-line therapy in adults with ALCAPA. [ABSTRACT FROM AUTHOR]

Background The long-term effectiveness of pulmonary arterial hypertension–specific drug therapy (PAH-SDT) in Eisenmenger syndrome is controversial. We investigated short-term and long-term hemodynamic changes under PAH-SDT and their associations with outcomes in a bicentric cohort. Methods Over 20 years, we included 69 patients with congenital heart disease, an indexed pulmonary vascular resistance (PVRi) >8 WU·m 2 , and 292 standardized catheterizations at baseline and after PAH-SDT initiation or intensification. Oxygen consumption was measured and the Fick principle applied to calculate indexed pulmonary output (Qpi) and PVRi. Results After PAH-SDT initiation or intensification, median (interquartile range) PVRi decrease was 5.1 WU·m 2 (−1.4, −12.6) ( p < 0.0001). Median Qpi and 6-minute walk test increases were +0.4 liter/min/m 2 (0.0, +0.9) ( p < 0.0001) and +49 m (+15, +93) ( p = 0.0003), respectively. Hemodynamic response combining increased Qpi with decreases in transpulmonary gradient and PVRi occurred in 68.0% of patients. After a median of 4.9 years, PVRi and Qpi changes were no longer significant. Over a median of 7.2 years, 23 (33.3%) patients met a composite criterion (death, n = 8; heart-lung transplantation or listing for transplantation, n = 15). The 15-year cumulative event rate was 49.2%. By multivariate analysis, independent predictors of events were superior vena cava oxygen saturation and hemodynamic response ( p = 0.048 and p < 0.0001). Conclusions In Eisenmenger syndrome, PAH-SDT induces early hemodynamic improvements, which decline over time. Hemodynamic changes under PAH-SDT vary across patients. Hemodynamic parameters at baseline and under PAH-SDT are associated with events. PAH-SDT may need to be individualized based on hemodynamic changes. [ABSTRACT FROM AUTHOR]

Copyright of Archives of Cardiovascular Diseases is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Even though several devices have been developed to facilitate occlusion of cardio vascular structures in adults, only a few types of plugs have been approved in selected pediatric indications. However, apart from patent ductus arteriosus (PDA) closure, little data has been published in children, particularly with regard to the heterogeneity of the procedures and outcomes. To describe the efficacy, safety, and characteristics of the utilization of the Amplatzer Vascular Plug (AVP) II and IV for cardiovascular percutaneous occlusions in children under 10 years. Retrospective multicenter (2006–2020, 6 academic centers) observational review of pediatric transcatheter occlusions using AVP II or IV. A total of 125 consecutive children (60 < 1 year, 145 lesions) underwent 136 successive procedures using 175 different devices (112 AVP IV, 63 AVP II) with a success rate of 98.5%. The mean device diameter was 7.7 ± 3.2 mm (4–20 mm). The median age and weight at implantation were 1.0 year (0.01–9.98 years) and 9.1 kg (1–69 kg), respectively. Procedures involved 55 PDA, 28 arterial bronchial or aorto-pulmonary collaterals, 18 sequestrations or Scimitar syndrome, 16 arteriovenous or veinovenous fistulae, 6 coronary fistulae, 6 vertical veins, 6 conduits, 5 ventricular septal defect, and 7 miscellaneous occlusions. The occlusion rate at Day1 was 96.3% and 100%, 6 months after, without hemolysis. Major adverse events (MAE) occurred in 5.1% mainly the need for surgery, without any related death. MAE was related to a lower weight (P = 0.04), a younger age (P = 0.02) and large PDA closure using AVP II (P < 0.01). AVP II and IV are safe and effective for the percutaneous treatment of numerous cardiovascular lesions in children under ten. In our multicenter experience, such devices represent a real "off-label" pediatric alternative in well-selected patients. [ABSTRACT FROM AUTHOR]

This study sought to investigate patient intermediate-term outcomes after transcatheter pulmonary valve replacement (TPVR) with Edwards SAPIEN valve. The Edwards SAPIEN valve, initially designed for percutaneous aortic valve replacement, has been approved for TPVR in patients with dysfunctional right ventricular outflow tracts (RVOT), but only short-term follow-up has been reported. From 2011 to 2016, 62 patients undergoing successful TPVR using the SAPIEN XT valve were consecutively included into the study. Primary efficacy and safety endpoints were defined as freedom from valve-reintervention and freedom from infective endocarditis at last follow-up, respectively. The primary efficacy outcome was met for 87,1% patients after a mean follow-up of 55 ± 21 months, corresponding to a freedom of reintervention at 5 years of 89% (95% CI 74.8–95.6%). Reinterventions were exclusively due to recurrent obstruction, no significant valvular insufficiency was observed. One case of infective endocarditis was reported, corresponding to a rate of 0.35% per patient-year (95% CI 0.01–2.00%). At 5 years freedom from infective endocarditis was 98.4% (95% CI 89.1–99.8%) (Fig. 1). Six patients died or were transplanted due to advanced cardiac failure, without relationship with TPVR. In univariate analysis, reintervention was associated with young age, a smaller tube-graft, a higher pulmonary valve gradient after the procedure and a ratio of largest implanted stent diameter to invasive balloon conduit diameter over 1.35. This study confirms the mid-term safety and efficacy of the Edwards SAPIEN XT valve in patients with dysfunctional RVOT, and identifies a patient profile associated with an uncertain benefit-risk balance. [ABSTRACT FROM AUTHOR]

Heart failure (HF) in children is a severe condition which diagnosis can be difficult [1,2]. We aimed at describing the incidence and outcome of new-onset HF with no know heart disease in the Loire-Atlantique area. We conducted a retrospective monocentric observational study between 2007 and 2016. We included all children under 16 years old with no known heart disease and new-onset HF confirmed by echocardiography. Data collected for the DIACARD study were used. One hundred and seventy-four children had a diagnosis of heart disease based on signs of HF during the study period. The incidence of new-onset HF in children was 1.92 per 100,000 including 0.91 per 100,000 due to cardiomyopathies and myocarditis (CM). 120 children had analyzable data including 60 with congenital heart diseases (CHD) and 57 with CM. Median age at diagnosis was 8 days (interquartile range 1–20.3 days) in CHD and 3 months (interquartile range 1–26 months) in CM. Among CHD, coarctation of the aorta was the most frequent diagnosis (21 children, 35%) followed by left-to-right shunts (12 children, 20%). Among CM, dilated cardiomyopathy (DCM) including myocarditis was the most frequent diagnosis (49 children, 86%). Overall one-year mortality rate was 17%, one-year neuro-motor sequel rate was 18%. More than half of the children with no known heart disease and new-onset HF are diagnosed with CHD. The incidence of CM in a French area is similar to the reported incidence in other industrialized countries [2,3]. Children diagnosed with HF signs have a high mortality rate. [ABSTRACT FROM AUTHOR]

Objective To assess the long-term results of the arterial switch operation (ASO) for Taussig-Bing Anomaly (TBA) and identify risk factors affecting outcomes. Methods Retrospective review and late follow-up was performed for all TBA patients from 1997 to 2010 (follow-up >3 years). Selection criteria included the absence of mitro-pulmonary continuity. Results Sixty-nine children underwent ASO at a median age of 24 days (interquartile range [IR] 11-125), with concomitant repair of aortic arch obstruction in 26 (37.7%). Complex coronary anatomy (n = 38; 55.0%) was common. Nine (13.0%) patients had staged repair. Hospital mortality was 5.8% (95% confidence interval [CI], 1.6%-14.2%; n = 4). Median follow-up was 11.2 years (IR 7.2-13.8). Subsequent mortality was confined to the first postoperative year (n = 5, 86% [95% CI, 78%-95%]), 1-, and 10-year survival). Overall mortality was related to coronary pattern (Yacoub types C and E vs A and D, multivariate, hazard ratio [HR] 12.2 [95% CI, 1.2-122.1], P = .03). At latest follow-up, 96% of the survivors are asymptomatic, with normal ventricular function. Cumulative incidence of reintervention at 10 years was 53% (95% CI, 28%-77%). Concomitant aortic arch obstruction was a predictor of reintervention (multivariate, HR 2.9 [95% CI, 1.1-7.4], P = .03). No mortality occurred upon reinterventions. Conclusions In the largest series to date of ASO for TBA, mortality is confined to the first postoperative year, and related to coronary artery pattern. Beyond the first year, needed reinterventions are frequent, but with sustained functional status and no mortality over >10 years follow-up. Aortic arch obstruction is the main predictor for reintervention. Despite a significant rate of early events, favorable long-term outcomes argue for use of the ASO in TBA patients. [ABSTRACT FROM AUTHOR]

Background Aortic valve replacement and management of complex left ventricular outflow tract obstruction in early life remains a surgical challenge. We assessed our experience with the Ross-Konno procedure. Methods Since 2000, 49 consecutive patients (24 neonates and infants) underwent the Ross-Konno procedure. Anatomic and clinical risk factors were analyzed. Results Median age was 12.2 months (0.0 to 194.4); 82% had previous valvotomy (surgical, n = 26; balloon, n = 7; balloon then surgical, n = 7) and 35% preceding arch repair (n = 17). Fifteen patients (31%) required concomitant procedures: mitral valve repair (n = 11), replacement (n = 1), endocardial fibroelastosis resection (n = 4), and aortic arch repair (n = 2). There were 5 hospital deaths (10.2%). Median follow-up was 63 ± 47 months. There were 4 late deaths (8.1%), all because of persistent pulmonary hypertension despite subsequent mitral procedures. Five-year actuarial survival and freedom from reoperation were 79.7% ± 6.1% and 68.6% ± 9.3%, respectively. Preoperative lower shortening fraction ( p = 0.005) was associated with early mortality, while concomitant mitral surgery and pulmonary hypertension ( p = 0.002) were associated with late mortality. Sixteen patients underwent 26 reoperations. Autograft function was normal in 30 of the 37 late survivors (81.1%), 4 (10.8%) had grade 2 regurgitation, and 3 (8.1%) required valve replacement. Conclusions Ross-Konno in children remains a high-risk procedure. Preoperative ventricular dysfunction is associated with significant early mortality and should favor conservative options. Mitral involvement substantially affects late outcome, survival for these patients relies on the efficacy in relieving mitral disease. Efforts to improve mitral repair in these infants are critically required as an alternative to univentricular pathways. [ABSTRACT FROM AUTHOR]

Copyright of Archives of Cardiovascular Diseases is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Archives of Cardiovascular Diseases is the property of Elsevier B.V. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background. The long-term results and indications of the arterial switch operation (ASO) for transposition of the great arteries (TGA) and anatomic left ventricular outflow tract obstruction (LVOTO) remain undetermined. The aims of this study were to determine long-term outcomes and prognostic factors in this specific population. Methods. Between 1986 and 2011, 55 patients with TGA and anatomic LVOTO underwent ASO. Anatomic LVOTO was defined as an echocardiographic peak LVOT gradient exceeding 20 mm Hg associated with an anatomic narrowing. Forty-three patients had a ventricular septal defect. Median follow-up was 7.9 ± 6.5 years (maximum, 25 years). Univariate and multivariate risk analyses for late LVOTO, aortic regurgitation, LVOT reintervention, and death were performed. Results. The early mortality rate was 11% (n = 6); 2 deaths were LVOTO-related. At the latest follow-up, 3 patients (5%) had a LVOTO, 7 (13%) had moderate aortic regurgitation, and 4 (7%) had LVOT reoperation. Actuarial freedom from LVOT reoperation was 90% ± 5% at 10 and 15 years. The mean LVOT peak gradient was 3 ± 9 mm Hg at the latest follow-up. A preoperative pulmonary valve z-score of less than -1.7 (odds ratio, 19; p = 0.02) and an atrioventricular valve-related LVOTO (odds ratio, 15; p = 0.02) are independent predictors of recurrent LVOTO. A preoperative pulmonary valve z-score of less than -1.8 is an independent predictor of LVOT reoperation (odds ratio, 17; p = 0.03). The LVOT gradient per se and the presence of ventricular septal defect or a bicuspid valve do not influence outcomes. Conclusions. Long-term outcomes of ASO for patients with TGA and anatomic LVOTO are satisfactory in selected patients. A lower preoperative pulmonary valve z-score and complex multilevel atrioventricular valve-related LVOTO are independent predictors of recurrent LVOTO and LVOT reoperation. TGA/LVOTO patients with pulmonary valve z-score exceeding -1.8 and resectable valvular or subvalvular LVOTO, or both, should be candidates for ASO, regardless of the severity of the LVOT peak gradient. [ABSTRACT FROM AUTHOR]

Background: Idiopathic pulmonary arterial hypertension (IPAH) remains a progressive fatal disease. Palliative Potts shunt has been proposed in children displaying suprasystemic IPAH. Methods: A retrospective multicenter study was performed to evaluate Potts shunt in pediatric IPAH. Results: Between 2003 and 2010, 8 children with suprasystemic IPAH and in World Health Organization functional class IV despite medical pulmonary arterial hypertension therapy underwent Potts shunt. Age at IPAH diagnosis ranged from 4 to 180 months (median age, 64 months). Surgical procedure was performed in a mean delay of 41.9 ± 54.3 months (range, 4 to 167 months; median delay, 20 months) after IPAH diagnosis. Mean size of the Potts shunt was 9.25 ± 3.30 mm. Two patients, whose medical pulmonary arterial hypertension therapy had been interrupted just after surgery, died at postoperative days 11 and 13 of acute pulmonary hypertensive crisis. After a mean follow-up of 63.7 ± 16.1 months, the 6 children who were discharged from the hospital were alive. Functional status improved markedly in the 6 survivors, with a World Health Organization functional class I (n = 4) or II (n = 2) at last follow-up, consistent with significant improvement of 6-minute-walk distance (302 ± 95 m [51% ± 20% of theoretical values] versus 456 ± 91 m [68% ± 10% of theoretical values]; p = 0.038) and decrease of brain natriuretic peptide levels (608 ± 109 pg/mL versus 76 ± 45 pg/mL; p = 0.035). No Potts shunt was found to be restrictive at last echocardiography. Conclusions: Palliative Potts shunt constitutes a new alternative to lung transplantation in severely ill children with suprasystemic IPAH, carrying a prolonged survival and persistent improvement in functional capacities. [Copyright &y& Elsevier]

Pulmonary prosthetic valve and conduit infective endocarditis (PPVE) is a major issue in the growing congenital heart disease (CHD) population. Diagnosis is challenging and usual imaging tools are not always efficient nor validated in this specific population. Particularly, the diagnostic yield of 18F-fluorodeoxyglucose (18F-FDG) Positron Emission Tomography (PET)/Computed Tomography (CT) remains poorly studied in PPVE. The Aim of this study was to assess the diagnostic performances of 18F-FDG PET/CT in CHD patients with PPVE suspicion. A retrospective multicenter study was conducted in 8 French tertiary centers. Children and adult CHD patients who underwent a 18F-FDG PET/CT in the setting of PPVE suspicion between January 2010 and May 2020 were included. The cases are initially classified as definite, possible or rejected PPVE regarding the modified Duke criteria (DC) and finally by the Endocarditis Team consensus. The result of 18F-FDG PET/CT had been compared to final diagnosis consensus used as gold-standard in our study. Sixty-six cases of PPVE suspicion involving 59 patients (median age 23, 73% male) were included. Sensitivity, specificity, positive predictive value and negative predictive value of 18F-FDG PET/CT in PPVE suspicion were respectively: 79.1% (68.4–91.4), 72.7% (60.4–85.0), 91.9% (79.6–100) and 47.1% (34.8–59.4). 18F-FDG PET/CT findings would help to rightly reclassify 43% of possible PPVE to definite PPVE. Using 18F-FDG PET/CT improves the diagnostic accuracy of the Duke Criteria in CHD patients with suspected PPVE. Its high positive predictive value could be helpful in routine to shorten diagnosis and treatment delays and improve clinical outcomes. [ABSTRACT FROM AUTHOR]

To clarify the clinical characteristics and outcomes of children with SCN5A-mediated disease and to improve their risk stratification. A multicentre, international, retrospective cohort study was conducted in 25 tertiary hospitals in 13 countries between 1990 and 2015. All patients ≤ 16 years of age diagnosed with a genetically confirmed SCN5A mutation were included in the analysis. There was no restriction made based on their clinical diagnosis. A total of 442 children {55.7% boys, 40.3% probands, median age: 8.0 [interquartile range (IQR) 9.5] years} from 350 families were included; 67.9% were asymptomatic at diagnosis. Four main phenotypes were identified: isolated progressive cardiac conduction disorders (25.6%), overlap phenotype (15.6%), isolated long QT syndrome type 3 (10.6%), and isolated Brugada syndrome type 1 (1.8%); 44.3% had a negative electrocardiogram phenotype. During a median follow-up of 5.9 (IQR 5.9) years, 272 cardiac events (CEs) occurred in 139 (31.5%) patients. Patients whose mutation localized in the C-terminus had a lower risk. Compound genotype, both gain- and loss-of-function SCN5A mutation, age ≤ 1 year at diagnosis in probands and age ≤ 1 year at diagnosis in non-probands were independent predictors of CE. In this large paediatric cohort of SCN5A mutation-positive subjects, cardiac conduction disorders were the most prevalent phenotype; CEs occurred in about one-third of genotype-positive children, and several independent risk factors were identified, including age ≤ 1 year at diagnosis, compound mutation, and mutation with both gain- and loss-of-function. [ABSTRACT FROM AUTHOR]

Patent ductus arteriosus (PDA) is very common in extremely low birth weight babies and may contribute to prolonged mechanical ventilation, bronchopulmonary dysplasia, renal failure, periventricular leukomalacia and necrotizing enterocolitis. When PDA closure is indicated with failure of medical therapy (cyclooxygenase inhibitors or paracetamol), surgical ligation is usually performed with excellent results. Transcatheter closure has been recently developed with promising results but comparative studies with surgical ligation are lacking. To compare results and outcomes after transcatheter closure using the Amplatzer Piccolo device versus surgical closure in 2 matched groups of pre-term infants weighing ≤ 3000 g. One hundred and forty seven babies (3 tertiary centres) were retrospectively analysed. Sixty-four patients who underwent transcatheter closure were compared with 83 matched surgical patients, using Wilcoxon signed-rank tests. PDA closure was successful in all surgical and transcatheter cases. During NICU course, mortality was 6.3% (n = 4) after transcatheter closure and 12% (n = 10) after surgery (P = 0.24). Median duration of mechanical ventilation was shorter after transcatheter closure than after surgical ligation (3 vs. 5 days, P = 0.035). In babies undergoing duct closure before 4 weeks of age the difference between transcatheter and surgical closure for mechanical ventilation during the NICU course was even more pronounced (3 vs. 9 days, P = 0.022). When transcatheter closure was performed before 4 weeks, babies were discharged home earlier as compared to those who underwent closure later in life (39 + 1 vs. 41 + 5 weeks, P = 0.021). Such difference was not found in the surgical group. Transcatheter PDA closure in low birth weight babies is safe, effective and associated with improved mechanical ventilation as compared to surgery. It may offer shorter hospital stay when performed earlier in life. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background Idiopathic pulmonary arterial hypertension (IPAH) remains a progressive fatal disease. Palliative Potts'shunt has been proposed in children displaying supra-systemic IPAH. Methods A retrospective multicenter study to evaluate Potts'shunt in pediatric IPAH. Results Between 2003 and 2010, 8 children with supra-systemic IPAH and in WHO functional class IV despite medical PAH therapy underwent Potts'shunt. Age at IPAH diagnosis ranged from 4 to 180 months (median age: 64 months). Surgical procedure was performed in a mean delay of 41.9±54.3 months (from 4 to 167 months, median delay: 20 months) after IPAH diagnosis. Mean size of the Potts'shunt was 9.25±3.30 mm. Two patients, whose medical PAH therapy had been interrupted just after surgery, died at post-operative day 11 and 13 from acute pulmonary hypertensive crisis. After a mean follow-up of 63.7±16.1 months, the 6 children who were discharged from hospital were alive. Functional status improved markedly in the 6 survivors with a WHO functional class I (n=4) or II (n=2) at last follow-up, consistent with significant improvement of 6 minute-walk distance [302±95 (51±20% of theoretical values) vs 456±91 meters (68±10% of theoretical values), p=0.038] and decrease of brain natriuretic peptid (BNP) levels (608±109 vs 76±45 pg/ml, p=0.035). No Potts'shunt was found restrictive at last echocardiography. Conclusion Palliative Potts'shunt constitutes a new alternative to lung transplantation in severely ill children with supra-systemic IPAH, carrying a prolonged survival and persistent improvement in functional capacities. [ABSTRACT FROM AUTHOR]

Time from first symptoms to diagnosis, called time to diagnosis, is related to prognosis in several diseases. The aim of this study was to assess time to diagnosis in children with new-onset heart failure (HF) and assess its consequences and determinants. A retrospective population-based observational study was conducted between 2007 and 2016 in a French tertiary care center. We included all children under 16 years old with no known heart disease, and HF confirmed by echocardiography. With logistic regression used for outcomes and a Cox proportional-hazards model for determinants, analyses were stratified by HF etiology: congenital heart diseases (CHD) and cardiomyopathies/myocarditis (CM). A total of 117 children were included (median age [interquartile range (IQR)] 25 days (6–146), 50.4% were male, 60 had CHD and 57 had CM). Overall median (IQR) time to diagnosis was 3.3 days (1.0–21.2). The frequency of 1-year mortality was 17% and 1-year neuromotor sequel 18%. Death at 1 year was associated with low birth weight for all patients (adjusted odds ratio 0.24, 95% confidence interval [CI] 0.08–0.68) and time to diagnosis below the median with CM (0.09, 0.01–0.87) but not time to diagnosis above the median for all patients (0.59, 0.13-2.66). Short time to diagnosis was associated with clinical severity on the first day of symptoms for all patients (adjusted hazard ratio 3.39, 95% CI 2.01–5.72), and young age with CM (0.09, 0.02–0.41). In children with new-onset HF presenting in our region, median time to diagnosis was short. Long time to diagnosis was not associated with poor outcome. • In pediatric new-onset heart failure, median time to diagnosis was short. • Excess mortality was not associated with long time to diagnosis but with low birth weight. • Early severity signs were the main determinant of short time to diagnosis. [ABSTRACT FROM AUTHOR]

Patent ductus arteriosus (PDA) is common in preterm infants and can contribute to morbidity and mortality. We aimed to compare results and outcome of transcatheter closure using the Amplatzer Piccolo Occluder versus surgical closure in 2 matched groups of preterm infants weighing <3000 g. A total of 147 babies from three tertiary centres were retrospectively analysed. Sixty-four babies undergoing catheter closure were compared with 83 matched surgical cases. Patent ductus arteriosus closure was successful in all cases. During neonatal unit course, mortality was 6.3% (n = 4) after catheterization and 12% (n = 10) after surgery (p = 0.24). Median duration of mechanical ventilation was shorter after catheterisation than after surgery (3 vs 5 days, p = 0.035). Before 4 weeks of age the difference between transcatheter and surgical closure for mechanical ventilation was even more pronounced (3 vs 9 days, p = 0.022). Additionally, when catheterisation was performed before 4 weeks, babies were discharged home earlier as compared to those who underwent closure later in life (39+1 vs. 42+1 weeks, p = 0.021). Such difference was not found in the surgical group. Transcatheter closure of patent ductus arteriosus is safe, effective and is associated with shorter mechanical ventilation than after surgery. Hospital stay might be shorter when performed earlier in life. • Premature babies benefit from cardiac catheter technique to close PDA • Shorter ventilation course compared with surgical ligation • Benefit of early PDA closure; both in ventilation and hospital stay [ABSTRACT FROM AUTHOR]

Background: Inherited cardiac conduction disease is a rare bradyarrhythmia associated with mutations in various genes that affect action potential propagation. It is often characterized by isolated conduction disturbance of the His-Purkinje system, but it is rarely described as a syndromic form.Objectives: The authors sought to identify the genetic defect in families with a novel bradyarrhythmia syndrome associated with bone malformation.Methods: The authors genetically screened 15 European cases with genotype-negative de novo atrioventricular (AV) block and their parents by trio whole-exome sequencing, plus 31 Japanese cases with genotype-negative familial AV block or sick sinus syndrome by targeted exon sequencing of 457 susceptibility genes. Functional consequences of the mutation were evaluated using an in vitro cell expression system and in vivo knockout mice.Results: The authors identified a connexin-45 (Cx45) mutation (p.R75H) in 2 unrelated families (a de novo French case and a 3-generation Japanese family) who presented with progressive AV block, which resulted in atrial standstill without ventricular conduction abnormalities. Affected individuals shared a common extracardiac phenotype: a brachyfacial pattern, finger deformity, and dental dysplasia. Mutant Cx45 expressed in Neuro-2a cells showed normal hemichannel assembly and plaque formation. However, Lucifer yellow dye transfer and gap junction conductance between cell pairs were severely impaired, which suggested that mutant Cx45 impedes gap junction communication in a dominant-negative manner. Tamoxifen-induced, cardiac-specific Cx45 knockout mice showed sinus node dysfunction and atrial arrhythmia, recapitulating the intra-atrial disturbance.Conclusions: Altogether, the authors showed that Cx45 mutant p.R75H is responsible for a novel disease entity of progressive atrial conduction system defects associated with craniofacial and dentodigital malformation. [ABSTRACT FROM AUTHOR]