Short- and long-term survival of children treated with ventricular assist devices in Spain, based on 15 years' experience.

OBJECTIVES: To describe the use of ventricular assist devices (VAD) in children in Spain and to identify variables related to survival. METHODS: This is an observational cohort study of all children younger than 18 years of age who underwent an initial implantation of a VAD at any of the 6 paediatric heart transplant centres from May 2006 to December 2020. Subjects were identified retrospectively from each hospital's database. RESULTS: Paracorporeal VADs were implanted in 118 children [pulsatile (63%), continuous (30.5%) or both types (5.9%)]. Small children (<0.7 m2 of body surface area) comprised the majority of this cohort (63.3%). Overall, 67% survived to VAD explantation, and 64.9% survived to hospital discharge. Non-central nervous system haemorrhage (39%) and stroke (38.1%) were the most common complications. Body weight <5 kg, congenital heart disease, pre-implantation bilirubin >34 µmol/l and bridge to decision strategy were associated with a higher mortality at hospital discharge and in the long-term. Interagency registry for mechanically assisted circulatory support (INTERMACS) status 1 and cardiac arrest prior to VAD implantation were related to long-term mortality, whereas pre-implantation renal replacement therapy and extracorporeal membrane oxygenation were not related to mortality. CONCLUSIONS: In Spain, 67% of the VAD-supported children have been bridged to heart transplantation or to recovery. Body weight lower than 5 kg, congenital heart disease diagnosis, cholestatic liver dysfunction, bridge to decision as VAD strategy, INTERMACS-1 status and cardiac arrest were pre-implantation variables related to mortality, whereas pre-implantation renal replacement therapy and extracorporeal membrane oxygenation were not.

Pulmonary Arteriovenous Malformation and Embolic Myocardial Infarction in a Patient With Hereditary Hemorrhagic Telangiectasia.

This report describes a case of embolic myocardial infarction secondary to a pulmonary arteriovenous malformation. Pulmonary arteriovenous malformations are rare and mostly congenital and are inherited as an autosomal dominant disorder known as hereditary hemorrhagic telangiectasia. Myocardial infarction is an uncommon complication in patients with untreated pulmonary arteriovenous malformations. (Level of Difficulty: Advanced.).

Éxito de la cirugía combinada de trasplante bipulmonar y reparación de cardiopatía congénita en paciente pediátrico / Successful combined bilateral lung transplant and congenital heart disease repair in a pediatric patient

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Valve-Sparing Tetralogy of Fallot Repair With Intraoperative Dilation of the Pulmonary Valve. Mid-Term Results.

Deleterious long-term effects of chronic pulmonary regurgitation after repair of tetralogy of Fallot have become evident during the last decades. Subsequently, some groups have developed strategies to spare the pulmonary valve function at the time of repair with good early results. However, mid-term outcomes are scarce in the literature and in some cases controversial. The aim of our study is to report our results mid-term with valve-sparing repair of tetralogy of Fallot. We retrospectively reviewed patients undergoing tetralogy of Fallot repair and having preservation of the pulmonary valve with intraoperative dilation at our institution. From June 2009 through June 2017, 42 patients underwent valve-sparing tetralogy of Fallot repair. Median age and weight at surgery were 5.2 months and 7.2 kg. Median preoperative pulmonary valve diameters and Z scores by echocardiography were 6.4 mm (range 4.5-11 mm) and -2.3 (range -1.3 to -4.5). No patient died in our series. For a median follow-up of 45 months, the pulmonary valve has grown by Z score (P < 0.0001) as well as the pulmonary trunk (P= 0.00216). Significant pulmonary regurgitation has developed in 9 patients (21.4%). No patient has required reintervention/reoperation for recurrent right ventricular outflow tract obstruction. Patients with tetralogy of Fallot who had valve-sparing repair with intraoperative dilation of the pulmonary valve show good early and mid-term results with respect to right ventricular outflow tract obstruction. The pulmonary valve annulus and the pulmonary trunk grow through follow-up. Progressive development of significant pulmonary regurgitation is seen in more than 20% of patients. Long-term data with this approach and comparison with a population of patients undergoing a transannular patch repair are required to establish the real utility of this approach.

Lower mini-sternotomy in congenital heart disease: just a cosmetic improvement?

OBJECTIVES: Lower mini-sternotomy represents a minimally invasive surgical technique that has been utilized for the repair of a wide variety of congenital heart defects with excellent surgical and cosmetic outcomes. However, clinical improvements provided for this technique beyond cosmetic results are controversial. The aim of our study is to report our results with lower mini-sternotomy for the repair of congenital heart malformations and compare them with a matched group with a full median sternotomy approach. METHODS: From 2010 through 2013, 105 consecutive congenital patients (81 paediatric) underwent lower mini-sternotomy at our centre (Group 1). We analysed in-hospital and follow-up outcomes, and compare them with an age-sex-diagnosis-type of surgery-matched group (Group 2). Both groups were managed following the same clinical protocols. RESULTS: In Group 1, age at the time of surgery was 12 ± 17 years (range from 0.2 to 64.6 years). In this group, 81 patients were paediatric and 62 were female. Operative techniques were atrial septal defect (n = 72), ventricular septal defect (n = 24) and atrioventricular canal repairs (n = 9). There were no deaths or major in-hospital complications. Two adult patients required conversion to full median sternotomy. For a medium follow-up of 1.5 years (range from 1 month to 5 years), there were no deaths, reinterventions or reoperations and no significant residual defects were found. Compared with Group 2, patients in Group 1 had longer cardiopulmonary bypass times (58.71 ± 19.08 vs 45.39 ± 20.45, P < 0.001) and cross-clamp times (32.75 ± 13.11 vs 23.22 ± 13.93, P < 0.001), higher rate of early extubation (96 vs 85%, P = 0.018) and lower rate of postoperative complications (11.6 vs 22.3%, P = 0.034). CONCLUSIONS: At our centre, lower mini-sternotomy represents a safe alternative for the repair of congenital heart defects in paediatric and adult populations. Cardiopulmonary bypass and cross-clamp times were longer in the mini-sternotomy group. However, these patients showed earlier extubation and less postoperative complications when compared with patients with a full sternotomy approach. Combined with improved cosmetic outcomes, lower mini-sternotomy could represent the technique of choice for these populations.

Valve-sparing tetralogy of Fallot repair with intraoperative dilation of the pulmonary valve.

Deleterious long-term effects of pulmonary regurgitation after tetralogy of Fallot (TOF) repair have become evident during the last two decades. Subsequently, different groups have developed strategies aimed at preserving the pulmonary valve function. However, the results of these approaches are not well known. From July 2009 through March 2012, 38 patients underwent primary repair of TOF at the authors' institution. Of these, 12 children (7 boys) underwent attempted pulmonary valve-sparing surgery with intraoperative dilation of the pulmonary valve. The technical details as well as the echocardiographic preoperative and follow-up data for this repair were recorded, with a special focus on the feasibility of the technique and the effects on pulmonary valve function. No patient in the series died. At repair, the median age was 6 months (range 3.4-126 months), and the median weight was 7.6 kg (range 4.7-47 kg). Intraoperative dilation of the pulmonary valve was technically feasible for all the patients. Two patients had unsuccessful dilation and underwent a transannular patch procedure. During a median follow-up period of 22 months (range 6-30 months), the pulmonary valve diameter and z-score improved significantly. Moreover, the annular size normalized, whereas the mean right ventricular outflow tract (RVOT) gradient remained at the mild level (median, 24 mmHg; range 12-36 mmHg). At the most recent follow-up evaluation, three patients showed moderate pulmonary regurgitation. Intraoperative dilation of the pulmonary valve in patients undergoing TOF repair is feasible and provides good relief of obstruction. Moreover, the pulmonary valve annulus grows through the follow-up period. Longer follow-up studies are needed to evaluate the exact role of this strategy in this population.

Configuración «en cobra» del dispositivo Amplatzer de cierre de la comunicación interauricular: ¿se puede evitar? / Cobra-like deformation of amplatzer devices used for closing atrial septal defects: can it be avoided?

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