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.

A Bicentric Propensity Matched Analysis of 158 Patients Comparing Porcine Versus Bovine Stented Bioprosthetic Valves in Pulmonary Position.

BACKGROUND AND OBJECTIVES: Pulmonary valve replacement (PVR) is the most common operation in adults with congenital heart disease (CHD). There is controversy regarding the best bioprosthesis. We compare the performance of stented bioprosthetic valves (the Mosaic [Medtronic™] porcine pericardial against Carpentier Perimount Magna Ease [Edwards™] bovine) in pulmonary position in patients with CHD. METHODS: Between January 1999 and December 2019, all the PVRs were identified from hospital databases in 2 congenital heart centres in Spain. Valve performance was evaluated using clinical and echocardiographic criteria. Propensity score matching was used to balance the 2 treatment groups. RESULTS: Three hundred nineteen patients were retrospectively identified. After statistical adjustment, 79 propensity-matched pairs were available for comparison Freedom from reintervention for the porcine cohort was 98.3%, 96.1%, and 91.9% at 3, 5, and 10 years and 100%, 98%, and 90.8% for the bovine cohort (p=0.88). Freedom from structural valve degeneration (SVD) for the porcine cohort was 96.9%, 92.8% and 88.7% at 3, 5, and 10 years and 100%, 98%, and 79.1% for the bovine cohort (p=0.38). Bovine prosthesis was associated with a reintervention hazard ratio (HR), 1.12; 95% confidence intervals (CIs), 0.24-5.26; p=0.89 and SVD HR, 1.69 (0.52-5.58); p=0.38. In the first 5 years, there was no difference in outcomes. After 5 years, the recipients of the bovine bioprosthesis were at higher risk for SVD (reintervention HR, 2.08 [0.27-16.0]; p=0.49; SVD HR, 6.99 [1.23-39.8]; p=0.03). CONCLUSIONS: Both bioprosthesis have similar outcomes up to 5 years, afterwards, porcine bioprosthesis seem to have less SVD.

The European Registry for Patients with Mechanical Circulatory Support (EUROMACS): third Paediatric (Paedi-EUROMACS) report.

OBJECTIVES: A third paediatric report has been generated from the European Registry for Patients with Mechanical Circulatory Support (EUROMACS). The purpose of EUROMACS, which is operated by the European Association for Cardio-Thoracic Surgery, is to gather data related to durable mechanical circulatory support for scientific purposes and to publish reports with respect to the course of mechanical circulatory support therapy. Since the first report issued, efforts to increase compliance and participation have been extended. Additionally, the data provided the opportunity to analyse patients of younger age and lower weight. METHODS: Participating hospitals contributed pre-, peri- and long-term postoperative data on mechanical circulatory support implants to the registry. Data for all implants in paediatric patients (<19 years of age) performed from 1 January 2000 to 31 December 2020 were analysed. This report includes updates of patient characteristics, implant frequency, outcome (including mortality rates, transplants and recovery rates) as well as adverse events including neurological dysfunction, device malfunction, major infection and bleeding. RESULTS: Twenty-five hospitals contributed 537 registered implants in 480 patients. The most frequent aetiology of heart failure was any form of cardiomyopathy (59%), followed by congenital heart disease and myocarditis (15% and 14%, respectively). Competing outcomes analysis revealed that a total of 86% survived to transplant or recovery or are ongoing; at the 2-year follow-up examination, 21.9% died while on support. At 12 months, 45.1% received transplants, 7.5% were weaned from their device and 20.8% died. The 3-month adverse events rate was 1.59 per patient-year for device malfunction including pump exchange, 0.7 for major bleeding, 0.78 for major infection and 0.71 for neurological events. CONCLUSIONS: The overall survival rate was 79.2% at 12 months following ventricular assist device implant. The comparison of survival rates of the early and later eras shows no significant difference. A focus on specific subgroups showed that survival was less in patients of younger age (<1 year of age; P = 0.01) and lower weight (<20 kg; P = 0.015). Transplant rates at 6 months continue to be low (33.2%).

External Validation of 3 Risk Scores in Adults with Congenital Heart Disease.

BACKGROUND AND OBJECTIVES: Adults with congenital heart disease (CHD) are an increasing group of patients thanks to the survival of over 85% of children with CHD. 20% of these patients shall warrant a surgical procedure during their life span. However, currently there is no one risk score that assess correctly the mortality of these procedures. Thus, we analyse the risk scores used at our institution. METHODS: From May 1991 till June 2017, 608 procedures in adults with CHD were performed. The 3 risk scores (risk adjustment for congenital heart surgery [RACHS-1], Aristotle, and Euroscore I) of each procedure were analysed. We used area under the receiver operating characteristic curve (c-index) to measure model discrimination, and Hosmer-Lemeshow (H-L) statistic along with calibration plots to measure calibration. RESULTS: There was no statistical difference between the area under the curve for the 3 scores (χ²=0.58 with 2 df, p=0.750). There was no evidence of lack of fit for RACHS-1 (H-L, χ²=2.61; p=0.271) and Aristotle score (H-L, χ²=5.69; p=0.459). However, there was evidence in lack of calibration in the Euroscore I scoring system (H-L, χ²=33.69; p<0.001). The calibration slope for RACHS-1 was 0.912, for Aristotle (stratified in risk groups) was -0.14 and for Euroscore 1 (stratified in risk groups) was 0.46. CONCLUSIONS: RACHS-1 seems to be best risk scoring system for calculating mortality applied to surgery in adults with CHD.

Factores de riesgo asociados a la cirugía del adulto con cardiopatía congénita: 22 años de experiencia. ¿Quién debe intervenirlos? / Risk factors for surgery of congenital heart disease in adults: Twenty-two years of experience. Who should operate them?

Objetivo: Analizar la asociación entre mortalidad en cirugía de cardiopatías congénitas del adulto y los factores relacionados con el paciente y la intervención. Método: Estudio descriptivo de intervenciones por cirujanos con actividad habitual en cardiopatías congénitas (238), cardiopatías adquiridas (117) y residentes (108). Se evaluó la asociación de la mortalidad con el riesgo y complejidad quirúrgica, actividad habitual del cirujano, y tiempo de circulación extracorpórea y de pinzamiento aórtico, mediante modelos de regresión logística. Resultados: Se incluyeron 463 cirugías (442 con circulación extracorpórea) entre 1991 y 2012. Edad mediana de intervención: 34 años (52.8% mujeres); primera cirugía 295, reintervención 168. La puntuación mediana del Aristóteles fue 6.8, con complejidad significativamente mayor tras reestructurarse la Unidad en 2001. La mortalidad hospitalaria total fue del 3.9%. La mortalidad se asoció significativamente al número de intervenciones previas (OR: 5.02; IC 95%: 1.44-17.52), intervenciones por cirujanos de cardiopatía adquirida (OR: 3.53; IC 95%: 1.14-10.98), Aristóteles alto (OR: 1,64; IC 95%: 1.18-2.29), y tiempos prolongados de extracorpórea (OR: 1.13; IC 95%: 1.07-1.19). Conclusiones: La mortalidad en cirugía de cardiopatía congénita en adultos es baja. Las intervenciones de alta complejidad, tiempos elevados de extracorpórea y múltiples reintervenciones se asocian con mayor mortalidad. La participación de cirujanos especialistas en cardiopatías congénitas se asocia con mejores resultados.

Objective: To assess the association between mortality in surgery of congenital heart disease in adults, and factors related to patients and operations. Method: Descriptive study of operations performed by specialized surgeons in congenital heart surgery (238), adult acquired surgery (117), and specialty residents (108). The association of mortality with surgical risk and complexity, specialization of surgeon, cardiopulmonary by-pass and aortic cross clamping was assessed fitting logistic regression models. Results: A total of 463 operations were included (442 with cardiopulmonary by-pass) in the study performed between 1991 and 2012. Median age at surgery: 34; 52.8% were women. First surgery: 295, reoperation: 168. Median score of Aristotle was 6.8, with significantly higher complexity since 2001, after restructuring the Unit. Overall hospital mortality was 3.9%. Mortality was significantly associated to number of previous surgeries (OR: 5.02; 95%CI: 1.44-17.52), operations by acquired heart disease surgeons (OR: 3.53; 95%CI: 1.14-10.98), higher Aristotle (OR: 1,64; 95%CI: 1.18-2.29), and high cardiopulmonary by-pass time (OR: 1.13; 95%CI: 1.07-1.19). Conclusions: Surgery of congenital heart disease in adults has been performed with low mortality. High complexity interventions, prolonged cardiopulmonary by-pass times and multiple reoperations were associated to higher mortality. Participation of cardiac surgeons specialized in congenital heart disease is associated with better outcomes.

[Risk factors for surgery of congenital heart disease in adults: twenty-two years of experience. Who should operate them?]. / Factores de riesgo asociados a la cirugía del adulto con cardiopatía congénita: 22 años de experiencia. ¿Quién debe intervenirlos?

OBJECTIVE: To assess the association between mortality in surgery of congenital heart disease in adults, and factors related to patients and operations. METHOD: Descriptive study of operations performed by specialized surgeons in congenital heart surgery (238), adult acquired surgery (117), and specialty residents (108). The association of mortality with surgical risk and complexity, specialization of surgeon, cardiopulmonary by-pass and aortic cross clamping was assessed fitting logistic regression models. RESULTS: A total of 463 operations were included (442 with cardiopulmonary by-pass) in the study performed between 1991 and 2012. Median age at surgery: 34; 52.8% were women. First surgery: 295, reoperation: 168. Median score of Aristotle was 6.8, with significantly higher complexity since 2001, after restructuring the Unit. Overall hospital mortality was 3.9%. Mortality was significantly associated to number of previous surgeries (OR: 5.02; 95%CI: 1.44-17.52), operations by acquired heart disease surgeons (OR: 3.53; 95%CI: 1.14-10.98), higher Aristotle (OR: 1,64; 95%CI: 1.18-2.29), and high cardiopulmonary by-pass time (OR: 1.13; 95%CI: 1.07-1.19). CONCLUSIONS: Surgery of congenital heart disease in adults has been performed with low mortality. High complexity interventions, prolonged cardiopulmonary by-pass times and multiple reoperations were associated to higher mortality. Participation of cardiac surgeons specialized in congenital heart disease is associated with better outcomes.

Effects of ultraviolet radiation (UVA+UVB) on young gametophytes of Gelidium floridanum: growth rate, photosynthetic pigments, carotenoids, photosynthetic performance, and ultrastructure.

This study investigated the effects of radiation (PAR+UVA+UVB) on the development and growth rates (GRs) of young gametophytes of Gelidium floridanum. In addition, photosynthetic pigments were quantified, carotenoids identified, and photosynthetic performance assessed. Over a period of 3 days, young gametophytes were cultivated under laboratory conditions and exposed to photosynthetically active radiation (PAR) at 80 µmol photons m(-2) s(-1) and PAR+UVA (0.70 W m(-2))+UVB (0.35 W m(-2)) for 3 h per day. The samples were processed for light and electron microscopy to analyze the ultrastructure features, as well as carry out metabolic studies of GRs, quantify the content of photosynthetic pigments, identify carotenoids and assess photosynthetic performance. PAR+UVA+UVB promoted increase in cell wall thickness, accumulation of floridean starch grains in the cytoplasm and disruption of chloroplast internal organization. Algae exposed to PAR+UVA+UVB also showed a reduction in GR of 97%. Photosynthetic pigments, in particular, phycoerythrin and allophycocyanin contents, decreased significantly from UV radiation exposure. This result agrees with the decrease in photosynthetic performance observed after exposure to ultraviolet radiation, as measured by a decrease in the electron transport rate (ETR), where values of ETRmax declined approximately 44.71%. It can be concluded that radiation is a factor that affects the young gametophytes of G. floridanum at this stage of development.

Bioabsorption of cadmium, copper and lead by the red macroalga Gelidium floridanum: physiological responses and ultrastructure features.

Heavy metals, such as lead, copper, cadmium, zinc, and nickel, are among the most common pollutants found in both industrial and urban effluents. High concentrations of these metals cause severe toxic effects, especially to organisms living in the aquatic ecosystem. Cadmium (Cd), lead (Pb) and copper (Cu) are the heavy metals most frequently implicated as environmental contaminants, and they have been shown to affect development, growth, photosynthesis and respiration, and morphological cell organization in seaweeds. This paper aimed to evaluate the effects of 50µM and 100µM of Cd, Pb and Cu on growth rates, photosynthetic pigments, biochemical parameters and ultrastructure in Gelidium floridanum. To accomplish this, apical segments of G. floridanum were individually exposed to the respective heavy metals over a period of 7 days. Plants exposed to Cd, Cu and Pb showed discoloration of thallus pigmentation, chloroplast alteration, especially degeneration of thylakoids, and decrease in photosynthetic pigments, such as chlorophyll a and phycobiliproteins, in samples treated with Cd and Cu. Moreover, cell wall thickness and the volume of plastoglobuli increased. X-ray microanalysis detected Cd, Cu and Pb absorption in the cell wall. The results indicate that Cd, Pb and Cu negatively affect metabolic performance and cell ultrastructure in G. floridanum and that Cu was more toxic than either Pb or Cd.

Photoacclimation responses of the brown macroalga Sargassum Cymosum to the combined influence of UV radiation and salinity: cytochemical and ultrastructural organization and photosynthetic performance.

The photoacclimation responses of the brown macroalga Sargassum cymosum were studied to determine its cytochemical and ultrastructural organization, as well as photosynthetic pigments and performance. S. cymosum was cultivated in three salinities (30, 35 and 40 psu) under four irradiation treatments: PAR-only, PAR + UVA, PAR + UVB and PAR + UVA + UVB. Plants were exposed to PAR at 70 µmol photons m(-2) s(-1), PAR + UVB at 0.35 W m(-2) and PAR +UVA at 0.70 W m(-2) for 3 h per day during 7 days in vitro. Growth rate was not significantly affected by any type of radiation or salinity. The amount of pigments in S. cymosum was significantly influenced by the interaction of salinity and radiation treatments. Compared with PAR-only, UVR treatments modified the kinetics patterns of the photosynthesis/irradiance curve. After exposure to UVR, S. cymosum increased cell wall thickness and the presence of phenolic compounds. The number of mitochondria increased, whereas the number of chloroplasts showed few changes. Although S. cymosum showed insensitivity to changes in salinity, it can be concluded that samples treated under four irradiation regimes showed structural changes, which were more evident, but not severe, under PAR + UVB treatment.

Effects of brefeldin A on the endomembrane system and germ tube formation of the tetraspore of Gelidium floridanum (Rhodophyta, Florideophyceae).

Gelidium floridanum W.R. Taylor tetraspores are units of dispersal and are responsible for substrate attachment. This study aimed to examine evidence of direct interaction between germ tube formation and Golgi activity during tetraspore germination of G. floridanum. After release, the tetraspores were incubated with brefeldin A (BFA) in concentrations of 4 and 8 µM over a 6 h period. The controls and treatments were analyzed with light, fluorescence (FM4-64 dye) and transmission electron microscopy. In the control samples, the Golgi bodies were responsible for germ tube formation. In contrast, BFA-treated samples were observed to inhibit spore adhesion and germ tube formation. These tetraspores also showed an increase in volume (≥30 µm width). BFA treatment also resulted in the disassembly of Golgi cisternae and the formation of vesiculated areas of the cytoplasm, blocking the secretion of protein and amorphous matrix polysaccharides. When stained with FM4-64, the control samples showed fluorescence in the apical region of the germ tube, but the treated samples showed an intense fluorescence throughout the cytoplasm. From these results, we can conclude that the germ tube is formed by the incorporation of vesicles derived from Golgi. Thus, vesicle secretion and Golgi organization are basic processes and essential in adhesion and tube formation. By blocking the secretion of protein and amorphous matrix polysaccharides, BFA treatment precluded tetraspore germination.

Chimney technique for mitral valve replacement in children.

Severe mitral stenosis is unusual in children, but it represents an important challenge for surgeons because of the scarcity of solutions. Several mitral percutaneous and surgical valvuloplasties are performed repetitively to delay mitral valve replacement. Most of the time these procedures show discouraging results. When mitral valve replacement is performed, the annulus may not be large enough to fit a substitute. We present, to our best knowledge, a new technique to implant a large prosthesis in a small annulus without negatively affecting the opening of the leaflets.

Comparison of outcomes in adults with congenitally corrected transposition with situs inversus versus situs solitus.

The long-term outcome of patients with congenitally corrected transposition of the great arteries is mainly determined by progressive morphologically tricuspid valve regurgitation, heart block, atrial arrhythmias, and/or systemic ventricular dysfunction. Situs abnormalities have been reported in ≤34% of cases, but whether clinical differences exist between a situs inversus and situs solitus arrangement has not yet been studied. The clinical records of 38 adults with congenitally corrected transposition of the great arteries (mean age 40 ± 15 years) followed for a mean period of 7.4 years were reviewed. Of these 38 patients, 8 presented with situs inversus and 30 with situs solitus. No significant differences were found between the 2 groups in age, gender, ventricular septal defect, pulmonary tract stenosis, previous surgical repair, or duration of follow-up. However, none of the patients with situs inversus presented with an Ebstein-like anomaly of the morphologically tricuspid valve and none developed nonoperative-related complete atrioventricular block compared to 15 (50%; p = 0.013) and 11 (42%; p = 0.032) of the patients with situs solitus, respectively. At follow-up, 2 patients with situs inversus (25%) presented with sustained atrial arrhythmia, severe tricuspid regurgitation, or severe systemic right ventricular systolic dysfunction compared to 22 (73%) of 30 those with situs solitus (p = 0.034). No patient with situs inversus presented with cardiac death or severe heart failure compared to 12 (40%) of 30 with situs solitus (p = 0.038). In conclusion, Ebstein-like anomaly or spontaneous complete atrioventricular block are rare in patients with congenitally corrected transposition of the great arteries with situs inversus, and late complications are uncommon. The long-term outcome of patients with situs inversus was significantly better than that for patients with situs solitus.

Platypnea-orthodeoxia syndrome after failed percutaneous closure of secundum atrial septal defect.

We report a 51-year-old patient with platypnea-orthodeoxia syndrome after percutaneous closure of a secundum atrial septal defect, an unusual complication of this modality of treatment. Echocardiography, the main diagnostic technique in the present case, showed that one of the percutaneous device's rims was fixed to the anterior wall of the inferior cava vein. Furthermore it showed that the blood flowed from the inferior cava vein, through the defect in the atrial septum, into the left atria.

Incidence and predictors of sudden cardiac arrest in adults with congenital heart defects repaired before adult life.

Many adult survivors of repaired congenital heart disease (CHD) are at premature risk of death. Sudden cardiac arrest (SCA) is 1 of the leading causes of death but little is known about determinants for SCA in adults with repaired lesions. We sought to determine incidence and risk factors for SCA in a study population of 936 adults with previously repaired CHD who had completed follow-up at a single tertiary center during a mean period of 9 ± 7 years. Mean age at first examination in our institution was 21 ± 7 years. Diagnostic categories included tetralogy of Fallot (216), coarctation of the aorta (157), transposition complexes (99), single ventricle (55), and other CHD (409). During a total follow-up of 8,387 person-years, 22 patients (2.6 per 1,000 person-years) presented with SCA. Incidence of SCA varied widely between specific lesions; the highest incidence was observed in transposition complexes (10 per 1,000 person-years). Independent predictors of SCA were retrospectively identified using multivariate Cox proportional hazard modeling. Age at initial examination and severely impaired subaortic ventricular systolic function were independent risk factors for SCA (severe subaortic ventricular systolic dysfunction, adjusted hazard ratio 29, 95% confidence interval 11 to 72, p <0.001). SCA occurred in 23% of patients with severe subaortic ventricular systolic dysfunction versus 0.7% of patients with nonsevere decreased subaortic ventricular function (p <0.001). In conclusion, severe subaortic ventricular systolic dysfunction is a dominant multivariate predictor of SCA in an unselected population of adult survivors after surgery for CHD. Our data support the consideration of primary prevention strategies in these patients.

Management of congenital tracheal stenosis in infancy.

OBJECTIVE: Congenital tracheal stenosis (CTS) is a very infrequent malformation. Till recently, the outlook for these patients was dismal because medical management was the only way of treatment. Surgical and endoscopical techniques developed in the last years have improved the prognosis. We review the short- and long-term outcomes of a single institution experience in the management of children with CTS, comparing different treatment modalities. METHODS: Between 1991 and 2004, 19 cases of CTS have been managed in our Unit. Respiratory symptoms varied from mild stridor on exertion to severe distress. Bronchoscopy was performed for diagnostic purposes in all cases; other imaging techniques (computed tomography (CT), magnetic resonance imaging (MRI), bronchography, angiography, doppler-ultrasound) were performed on an individual basis. According to clinical and endoscopical features, patients were classified into three groups. The following data have been studied in each case: sex, age at diagnosis and treatment, anatomical type, associated anomalies, treatment modality, complications, outcome and time of follow-up. RESULTS: Ten boys and nine girls have been included in this study. Age at diagnosis ranged from 3 days to 7 years (median, 4 months) and 84% of cases showed associated anomalies. Five patients presented mild or no symptoms and have been managed expectantly. The other 14 cases were operated on because of persistent or severe clinical symptoms. The following procedures were performed: slide tracheoplasty (n = 7), costal cartilage tracheoplasty (n = 5), tracheal resection and reconstruction (n = 3), endoscopical dilatation (n = 3), stent placement (n = 1), and laser resection (n = 1). Three patients required two or more procedures and surgical survival rate is 78%. Overall mortality in the series is 21% and all survivors (15 patients) are asymptomatic or show mild symptoms with respiratory infections only. Follow-up is complete, ranging from 8 months to 12.3 years (mean, 5 years). CONCLUSIONS: Bronchoscopy is our preferred diagnostic tool. Selection of the type of treatment depends on the patient's clinical status and the anatomical pattern of the stenosis. In symptomatic cases with short-segment stenosis (<30% of total tracheal length), we prefer tracheal resection with end-to-end anastomosis; for long-segment stenosis (>30%), slide tracheoplasty is our procedure of choice.