Characteristics and outcomes of the Spanish registry for pediatric patients with bicuspid aortic valve (REVAB).

INTRODUCTION AND OBJECTIVES: Bicuspid aortic valve (BAV) disorder is the most common congenital heart disease. The aim of this study was to describe the characteristics of 0- to 18-year olds with BAV in a population-based registry. METHODS: Data from all pediatric patients were obtained from the Spanish registry for pediatric patients with bicuspid aortic valve (REVAB) (< 18 years). For data analysis, patients with BAV were divided into 2 groups by their features: isolated BAV and BAV with associated congenital heart disease. RESULTS: We included 1681 patients from 33 hospitals. Males accounted for 69.6% (n = 1158). Valve morphology was horizontal in 63.4% (n = 1012) and pure (Sievers type 0) in 28.4% (n=469). Isolated BAV was present in 63.7% (n=1060), and concomitant left-sided obstructive lesions in 23.4% (n=390). Interventions were required in 8.6% (n=145). CONCLUSION: These data represent the first large, population-based description of the clinical presentations and outcomes of patients enrolled in the Spanish registry for pediatric patients with bicuspid aortic valve.

Changes in Ventilation Practices for Bronchiolitis in the Hospital Ward and Need for ICU Transfer over the Last Decade.

There is limited evidence of the potential benefits of the use of high-flow nasal cannula (HFNC) for the management of bronchiolitis in the ward. Our aim is to describe the ventilation trends for bronchiolitis in our hospital along with the introduction of an HFNC ward protocol and to determine the need for respiratory support escalation and transfer to an intensive care unit (ICU). A retrospective analytical observational study of children < 12 months old requiring admission for a first RSV bronchiolitis episode in a single centre from January 2009 to December 2018. The sample was divided into four groups according to the type of respiratory support that would ensure the clinical stability of the infants on admission. A total of 502 infants were recruited. The total number and percentage of patients admitted in the ward grew progressively over time. Simultaneously, there was an increase in HFNC and, paradoxically, an increase in ICU transfers. The risk of failure was higher for those who required HFNC or CPAP for clinical stabilisation in the first 12 h after admission. Moreover, the risk of failure was also higher in children with standard oxygen therapy promptly escalated to HFNC, especially if they had atelectasis/viral pneumonia, coinfections or a history of prematurity. Despite the limitations of a retrospective analysis, our study reflects usual clinical practice and no correlation was found between the usage of HFNC and a shorter length of hospital stay or less time spent on oxygen therapy.

Spanish registry of percutaneous VSD closure with NitOcclud Lê VSD Coil device: lessons learned after more than a hundred implants.

INTRODUCTION AND OBJECTIVES: The NitOcclud Lê VSD Coil was specifically designed for transcatheter occlusion of ventricular septal defects (VSD) and became available for this purpose in August 2010. Our objective was to describe the Spanish experience of this technique and analyze its reliability and short- to mid-term efficacy. METHODS: National multicenter observational study, which retrospectively recruited all patients (of any age) with VSD (of any location or type) who underwent percutaneous NitOcclud occlusion, using an intention-to-treat analysis, until January 2019. RESULTS: A total of 117 attempts were made to implant at least 1 NitOcclud in 116 patients in 13 institutions. The median [range] age and weight was 8.6 [0.4-69] years and 27 [5.8-97] kg, respectively. In 99 patients (85%), the VSD was an isolated congenital defect. The location was perimembranous in 95 (81%), and 74 (63%) of them were aneurysmatic. The mean fluoroscopy time was 34 [11.4-124] minutes. Of the 117 attempts, 104 were successful (89%) with a follow-up of 31.4 [0.6-59] months. At the last review, final complete occlusion of the defect without residual shunt or with only a minimal shunt was achieved in 92.3% (no shunt, n=73; trivial shunt, n=23). Four patients required a second procedure for residual shunt occlusion. Two devices had to be surgically explanted due to severe hemolysis. There were no deaths or other major complications. CONCLUSIONS: The NitOcclud device can be used successfully for a wide anatomical spectrum of VSD. The main issue is residual shunt, but its incidence decreases over time. The incidence of hemolysis was very low and no permanent changes were detected in atrioventricular conduction.

Pediatrics Spanish Registry of Percutaneous Melody Pulmonary Valve Implantation in Patients Younger Than 18 Years.

INTRODUCTION AND OBJECTIVES: A decade has passed since the first Spanish percutaneous pulmonary Melody valve implant (PPVI) in March 2007. Our objective was to analyze its results in terms of valvular function and possible mid-term follow-up complications. METHODS: Spanish retrospective descriptive multicenter analysis of Melody PPVI in patients < 18 years from the first implant in March 2007 until January 1, 2016. RESULTS: Nine centers were recruited with a total of 81 PPVI in 77 pediatric patients, whose median age and weight were 13.3 years (interquartile range [IQR], 9.9-15.4) and 46kg (IQR, 27-63). The predominant cardiac malformation was tetralogy of Fallot (n = 27). Most of the valves were implanted on conduits, especially bovine xenografts (n = 31). The incidence of intraprocedure and acute complications was 6% and 8%, respectively (there were no periprocedural deaths). The median follow-up time was 2.4 years (IQR, 1.1-4.9). Infective endocarditis (IE) was diagnosed in 4 patients (5.6%), of which 3 required surgical valve explant. During follow-up, the EI-related mortality rate was 1.3%. At 5 years of follow-up, 80% ± 6.9% and 83% ± 6.1% of the patients were free from reintervention and pulmonary valve replacement. CONCLUSIONS: Melody PPVI was safe and effective in pediatric patients with good short- and mid-term follow-up hemodynamic results. The incidence of IE during follow-up was relatively low but was still the main complication.

Tetralogy of Fallot with absent pulmonary valve syndrome, right aortic arch, and disconnected left pulmonary artery.

We present a rare case of a two-week-old infant with tetralogy of Fallot , absent pulmonary valve syndrome , right aortic arch, and disconnected left pulmonary artery (LPA) whose origin was from ductal ligament adjacent to the left subclavian artery. One-stage surgical correction, including closure of ventricular septal defect (VSD), LPA reconstruction and reconnection to the pulmonary trunk, reduction in size of the right pulmonary artery (RPA), and right ventricular outflow tract reconstruction with valved conduit, was successfully performed with good clinical mid-term outcome.