The characterization of toll-like receptor repertoire in Pinna nobilis after mass mortality events suggests adaptive introgression.

The fan mussel Pinna nobilis is currently on the brink of extinction due to a multifactorial disease mainly caused to the highly pathogenic parasite Haplosporidium pinnae, meaning that the selection pressure outweighs the adaptive potential of the species. Hopefully, rare individuals have been observed somehow resistant to the parasite, stretching the need to identify the traits underlying this better fitness. Among the candidate to explore at first intention are fast-evolving immune genes, of which toll-like receptor (TLR). In this study, we examined the genetic diversity at 14 TLR loci across P. nobilis, Pinna rudis and P. nobilis × P. rudis hybrid genomes, collected at four physically distant regions, that were found to be either resistant or sensitive to the parasite H. pinnae. We report a high genetic diversity, mainly observed at cell surface TLRs compared with that of endosomal TLRs. However, the endosomal TLR-7 exhibited unexpected level of diversity and haplotype phylogeny. The lack of population structure, associated with a high genetic diversity and elevated dN/dS ratio, was interpreted as balancing selection, though both directional and purifying selection were detected. Interestingly, roughly 40% of the P. nobilis identified as resistant to H. pinnae were introgressed with P. rudis TLR. Specifically, they all carried a TLR-7 of P. rudis origin, whereas sensitive P. nobilis were not introgressed, at least at TLR loci. Small contributions of TLR-6 and TLR-4 single-nucleotide polymorphisms to the clustering of resistant and susceptible individuals could be detected, but their specific role in resistance remains highly speculative. This study provides new information on the diversity of TLR genes within the P. nobilis species after MME and additional insights into adaptation to H. pinnae that should contribute to the conservation of this Mediterranean endemic species.

Haplosporidium pinnae Parasite Detection in Seawater Samples.

In this study, we investigated the presence of the parasite Haplosporidium pinnae, which is a pathogen for the bivalve Pinna nobilis, in water samples from different environments. Fifteen mantle samples of P. nobilis infected by H. pinnae were used to characterize the ribosomal unit of this parasite. The obtained sequences were employed to develop a method for eDNA detection of H. pinnae. We collected 56 water samples (from aquaria, open sea and sanctuaries) for testing the methodology. In this work, we developed three different PCRs generating amplicons of different lengths to determine the level of degradation of the DNA, since the status of H. pinnae in water and, therefore, its infectious capacity are unknown. The results showed the ability of the method to detect H. pinnae in sea waters from different areas persistent in the environment but with different degrees of DNA fragmentation. This developed method offers a new tool for preventive analysis for monitoring areas and to better understand the life cycle and the spread of this parasite.

Marine heatwaves drive recurrent mass mortalities in the Mediterranean Sea.

Climate change is causing an increase in the frequency and intensity of marine heatwaves (MHWs) and mass mortality events (MMEs) of marine organisms are one of their main ecological impacts. Here, we show that during the 2015-2019 period, the Mediterranean Sea has experienced exceptional thermal conditions resulting in the onset of five consecutive years of widespread MMEs across the basin. These MMEs affected thousands of kilometers of coastline from the surface to 45 m, across a range of marine habitats and taxa (50 taxa across 8 phyla). Significant relationships were found between the incidence of MMEs and the heat exposure associated with MHWs observed both at the surface and across depths. Our findings reveal that the Mediterranean Sea is experiencing an acceleration of the ecological impacts of MHWs which poses an unprecedented threat to its ecosystems' health and functioning. Overall, we show that increasing the resolution of empirical observation is critical to enhancing our ability to more effectively understand and manage the consequences of climate change.

Natural hybridization between pen shell species: Pinna rudis and the critically endangered Pinna nobilis may explain parasite resistance in P. nobilis.

Recently, Pinna nobilis pen shells population in Mediterranean Sea has plummeted due to a Mass Mortality Event caused by an haplosporidian parasite. In consequence, this bivalve species has been included in the IUCN Red List as "Critically Endangered". In the current scenario, several works are in progress to protect P. nobilis from extinction, being identification of hybrids (P. nobilis x P. rudis) among survivors extremely important for the conservation of the species.Morphological characteristics and molecular analyses were used to identify putative hybrids. A total of 10 individuals of each species (P. nobilis and P. rudis) and 3 doubtful individuals were considered in this study. The putative hybrids showed shell morphology and mantle coloration intermingled exhibiting both P. nobilis and P. rudis traits. Moreover, the analyses of 1150 bp of the 28S gene showed 9 diagnostic sites between P. rudis and P. nobilis, whereas hybrids showed both parental diagnostic alleles at the diagnostic loci. Regarding the multilocus genotypes from the 8 microsatellite markers, the segregation of two Pinna species was clearly detected on the PCoA plot and the 3 hybrids showed intermediate positions.This is the first study evidencing the existence of hybrids P. nobilis x P. rudis, providing molecular methodology for a proper identification of new hybrids. Further studies testing systematically all parasite-resisting isolated P. nobilis should be undertaken to determine if the resistance is resulting from introgression of P. rudis into P. nobilis genome and identifying aspects related to resistance.

Neuroplasticity during the transition period: How the adolescent brain can recover from aphasia. A pilot study.

BACKGROUND: Increasing clinical and scientific attention is given to the transition of neurological stages from child to adult. Data on brain plasticity during adolescence is interesting for providing adequate evidence-based medical attention to neurological conditions in this population. Acquired aphasia is well described in adults and children, but not in adolescence. OBJECTIVE: We describe a 5-year follow-up of language in three adolescent subjects with post-brain injury aphasia. METHODS: We analysed and scored formal aspects of language three times, language hemispheric dominance twice with dichotic listening test and functional magnetic resonance imaging (fMRI) brain activation patterns that supported expressive and comprehensive language during the recovery period. RESULTS: We found similarities to both paediatric and adult aphasia in these three adolescents. While the level of recovery resembled that of children with aphasia, a more efficient language rehabilitation occurred in those who remained with left hemispheric dominance in the chronic stage, as it is reported in adults. CONCLUSIONS: Our analysis and long-term follow-up provide data for a better understanding on how the injured brain matures during adolescence. More studies with larger samples will help to understand the function of the remaining networks and the recovery from injury in this particular age group.

Delineating the motor phenotype of SGCE-myoclonus dystonia syndrome.

OBJECTIVE: To perform phenotype and genotype characterization in myoclonus-dystonia patients and to validate clinical rating tools. METHOD: Two movement disorders experts rated patients with the Burke-Fahn-Marsden and Unified-Myoclonus rating scales using a video-recording protocol. Clinimetric analysis was performed. SGCE mutations were screened by Sanger sequencing and multiplex ligation-dependent probe amplification. RESULTS: 48 patients were included and 43/48 rated. Mean age at assessment was 12.9±10.5 years (range 3-51) and 88% were ≤18 years of age. Myoclonus was a universal sign with a rostro-caudal severity-gradient. Myoclonus increased in severity and spread to lower limbs during action tests. Stimulus-evoked myoclonus was observed in 86.8% cases. Dystonia was common but mild. It had a focal distribution and was action-induced, causing writer's cramp (69%) and gait dystonia (34%). The severity of both myoclonus and dystonia had a strong impact on hand writing and walking difficulties. The Unified Myoclonus Rating scale showed the best clinimetric properties for the questionnaire, action myoclonus and functional subscales, and exceeded the Burke-Fahn-Marsden scale in its utility in assessing functional impairment in MDS patients. Twenty-one different SGCE mutations were identified in 45/48 patients, eleven being novel (most prevalent p. Val187*, founder mutation in Canary Islands). CONCLUSION: This study quantifies the severity of the motor phenotype in SGCE-myoclonus dystonia syndrome, with a special focus on children, and identifies disabilities in gross and fine motor tasks that are essential for childhood development. Our results contribute to the knowledge of SGCE-related MDS in the early stage of evolution, where disease-modifying therapies could be initiated in order to prevent long-term social and physical burdens.

Tracking a mass mortality outbreak of pen shell Pinna nobilis populations: A collaborative effort of scientists and citizens.

A mass mortality event is devastating the populations of the endemic bivalve Pinna nobilis in the Mediterranean Sea from early autumn 2016. A newly described Haplosporidian endoparasite (Haplosporidium pinnae) is the most probable cause of this ecological catastrophe placing one of the largest bivalves of the world on the brink of extinction. As a pivotal step towards Pinna nobilis conservation, this contribution combines scientists and citizens' data to address the fast- and vast-dispersion and prevalence outbreaks of the pathogen. Therefore, the potential role of currents on parasite expansion was addressed by means of drift simulations of virtual particles in a high-resolution regional currents model. A generalized additive model was implemented to test if environmental factors could modulate the infection of Pinna nobilis populations. The results strongly suggest that the parasite has probably dispersed regionally by surface currents, and that the disease expression seems to be closely related to temperatures above 13.5 °C and to a salinity range between 36.5-39.7 psu. The most likely spread of the disease along the Mediterranean basin associated with scattered survival spots and very few survivors (potentially resistant individuals), point to a challenging scenario for conservation of the emblematic Pinna nobilis, which will require fast and strategic management measures and should make use of the essential role citizen science projects can play.

Haplosporidium pinnae sp. nov., a haplosporidan parasite associated with mass mortalities of the fan mussel, Pinna nobilis, in the Western Mediterranean Sea.

This study provides morphological and molecular characterization of a new species, Haplosporidium pinnae), very likely responsible for mass mortality of fan mussels, Pinna nobilis, in the Western Mediterranean Sea. The parasite was found in dead or moribund P. nobilis but did not occur in healthy fan mussels from locations that were not affected by abnormal mortality. Histological examination of infected fan mussels showed uninucleate cells of a haplosporidan parasite throughout the connective tissue and hemolymph sinuses of the visceral mass and binucleate cells and, rarely, multinucleate plasmodia were also detected in the connective tissue. Additionally, stages of sporulation occurred in the epithelium of the host digestive gland tubules. Spores were slightly ellipsoidal with a hinged operculum in one pole. Typical haplosporosomes were not found with TEM but vesicles with two concentric membranes resembling haplosporosomes were abundant in the cytoplasm of the multinucleate plasmodia occurring in host digestive gland tubules. SEM analysis showed multiple structures on the spore surface; some spores had two or four long tape-like filaments attached to the spore wall. Phylogenetic analysis based on the SSU rDNA sequence placed this parasite within a large clade including species of the order Haplosporida, not in the Bonamia/Minchinia subclade or the subclade containing most Haplosporidium species, but within a subclade of Haplosporidium sp. from Penaeus vannamei. Our results suggested that H. pinnae and the parasite of P. vannamei may represent a distinct new genus within the order Haplosporida.

Rizotomía dorsal selectiva: revisión bibliográfica de esta técnica para el tratamiento de la espasticidad en la parálisis cerebral infantil / Selective dorsal rhizotomy: a review of the literature on this technique for the treatment of spasticity in infantile cerebral palsy

Introducción. La parálisis cerebral infantil conforma una entidad bien conocida cuya prevalencia ha variado poco a lo largo de los años. El subtipo más común es la diplejía espástica, y la espasticidad es el síntoma más incapacitante. Para su tratamiento es preciso una intervención multidisciplinar que aúna rehabilitación, uso de fármacos y cirugía ortopédica y del sistema nervioso, donde destaca la rizotomía dorsal selectiva. Objetivo. Mostrar una amplia revisión del uso, indicaciones y consecuencias a largo plazo de la rizotomía dorsal selectiva. Desarrollo. Se trata de una intervención mínimamente invasiva dirigida a disminuir la espasticidad en las extremidades inferiores con el fin de mejorar la deambulación, aminorar el dolor, facilitar los cuidados de la vida diaria y reducir la necesidad de cirugías ortopédicas. En la bibliografía hay una amplia variabilidad de criterios para su uso, y la principal indicación es la diplejía espástica con ausencia de distonía. Diversos países lo practican de forma rutinaria, mientras que en el nuestro no tenemos aún constancia de ello. Conclusiones. Tras la revisión de la bibliografía consideramos que existe suficiente experiencia como para afirmar que la rizotomía dorsal selectiva es una técnica segura y sencilla de la que muchos pacientes con espasticidad de las extremidades inferiores secundaria a parálisis cerebral infantil se pueden beneficiar a corto y largo plazo

Introduction. Infantile cerebral palsy is a well-known condition, the prevalence of which has varied only slightly over the years. The most common subtype is spastic diplegia, and spasticity is the most disabling symptom. Its treatment involves a multidisciplinary intervention that includes rehabilitation, the use of drugs, and orthopaedic and nervous system surgery, where selective dorsal rhizotomy is a prominent procedure. Aim. To present a thorough review of the use, indication and long-term consequences of selective dorsal rhizotomy. Development. It is a minimally invasive procedure aimed at reducing spasticity in the lower extremities in order to improve the ability to walk, lessen pain, facilitate care in everyday life and diminish the need for orthopaedic surgery. The literature contains a wide range of criteria for its use, and the main indication is spastic diplegia with the absence of dystonia. It is routinely performed in several countries, while we have no evidence of its application in ours. Conclusions. Following the literature review, we believe there is enough experience to state that selective dorsal rhizotomy is a safe and simple technique from which many patients with spasticity of the lower limbs secondary to infantile cerebral palsy can benefit in both the short and the long term

Stylohyoid Complex (Eagle) Syndrome Starting in a 9-Year-Old Boy.

Background There are only four previous pediatric reports of the glossopharyngeal neuralgic form of the stylohyoid complex syndrome. Stylohyoid complex has merely been described as cases of glossopharyngeal neuralgia in children. Case Report A 12-year-old boy came to our hospital because of recurrent episodes of severe cranial pain (9/10) lasting for 5 to 15 minutes. Pain affected the right tonsillar fossa, ear, and mastoid region. Since the start at the age of 9 years, the frequency of painful episodes has progressively increased: when admitted to our clinics 3 years later, the child was having up to five episodes daily in spite of analgesic, antiepileptic, and antidepressant drugs; he had abandoned school and leisure. Between episodes, neurological examination detected only discomfort to pressure on the right tonsillar fossa. Three-dimensional computed tomography images of the skull base showed an elongated right styloid process and bilateral calcification of the stylohyoid ligament. After surgical excision of the right styloid process and of part of the stylohyoid ligament, the glossopharyngeal painful episodes ceased. The patient remains asymptomatic seven years later. Conclusion In spite of its rarity in childhood, this debilitating but treatable syndrome should be kept in mind for the differential diagnosis of recurrent cranial pain in the pediatric population.

The Pen Shell, Pinna nobilis: A Review of Population Status and Recommended Research Priorities in the Mediterranean Sea.

The pen shell Pinna nobilis (also known as the fan mussel) is an endemic bivalve of the Mediterranean Sea. Threatened by human activities, it has been listed as an endangered and protected species under the European Council Directive 92/43/EEC since 1992. The ecological role of this species is of importance because it filters and retains large amounts of organic matter from suspended detritus contributing to water clarity. In addition, as a hard substrate in the soft-bottom seafloor, it provides a surface that can be colonized by other (floral and faunal) benthic species. Here, we provide an overview of all available published studies on the pen shell, compiling available data and summarizing current knowledge on the conservation status and viability of populations over the full range of the Mediterranean Basin. Additionally, we discuss the different practices in applied methodology and identify gaps and new research areas in order to render conservation programmes of the species more effective.

Environmental factors modulating the extent of impact in coastal invasions: The case of a widespread invasive caprellid (Crustacea: Amphipoda) in the Iberian Peninsula.

Understanding the respective roles of environment and interspecific interactions in shaping species' distributions is a critical aspect in determining the potential impacts of newcomer species on occupied habitats. The invasive caprellid amphipod Caprella scaura has successfully spread along southern Europe in a short time period, coinciding with a decline in the population of an ecologically similar congener, Caprella equilibra. To understand the mechanisms underlying the establishment success of this aggressive species and its potential role in shaping C. equilibra's distribution, we analyze the biotic and abiotic factors involved in the patterns of distribution and co-occurrence of both species along the Iberian Peninsula and northern Africa. Our analyses support that C. scaura is competitively displacing C. equilibra from the study area, but also point out the critical role of salinity and temperature in modulating this interaction and limiting the invasive success of C. scaura on the Iberian North-Atlantic coast.

Human Stressors Are Driving Coastal Benthic Long-Lived Sessile Fan Mussel Pinna nobilis Population Structure More than Environmental Stressors.

Coastal degradation and habitat disruption are severely compromising sessile marine species. The fan shell Pinna nobilis is an endemic, vulnerable species and the largest bivalve in the Mediterranean basin. In spite of species legal protection, fan shell populations are declining. Models analyzed the contributions of environmental (mean depth, wave height, maximum wave height, period of waves with high energy and mean direction of wave source) versus human-derived stressors (anchoring, protection status, sewage effluents, fishing activity and diving) as explanatory variables depicting Pinna nobilis populations at a mesoscale level. Human stressors were explaining most of the variability in density spatial distribution of fan shell, significantly disturbing benthic communities. Habitat protection affected P. nobilis structure and physical aggression by anchoring reveals a high impact on densities. Environmental variables instead played a secondary role, indicating that global change processes are not so relevant in coastal benthic communities as human-derived impacts.

The Panama Canal and the transoceanic dispersal of marine invertebrates: evaluation of the introduced amphipod Paracaprella pusilla Mayer, 1890 in the Pacific Ocean.

Although the Panama Canal is one of the major corridors for shipping and potential dispersal of marine invaders in the tropics, little is known about the effect that the Canal has had on the distribution of marine biota. In this study, we (a) document the existence of established populations of the Western Atlantic caprellid amphipod Paracaprella pusilla, Mayer, 1890 for the first time at the Pacific entrance to the Canal, (b) review its distribution in the Pacific Ocean, and (c) evaluate possible mechanisms of introduction. The confirmed distribution of P. pusilla in the Pacific Ocean is limited to Australia, Hawaii, and Panama, despite earlier published reports from Chile and China. Laboratory experiments demonstrated intolerance of P. pusilla to freshwater, causing 100% mortality, and suggest invasion of the Pacific coast of Panama occurred through the Canal via ships' ballast water or by secondary spread via ships (ballast water or hull fouling) from another Pacific region.

Partial anomalous pulmonary venous connection to the superior vena cava.

We describe the surgical technique of reimplantation of the right superior pulmonary vein into the left atrium in 2 patients with partial anomalous pulmonary venous connection to the superior vena cava without atrial septal defect. A right axillary minithoracotomy is done through the fourth intercostal space. The pulmonary vein is detached from its origin in the superior vena cava. This is sutured with 6-0 reabsorbable polydioxanone suture (Ethicon, Somerville, NJ). A lateral clamp is applied to the left atrium, and the pulmonary vein is reimplanted. The patient is extubated in the operating room. Neither cardiopulmonary bypass nor blood transfusion was required. It is simple, safe, and reproducible.

[Pulmonary atresia with intact ventricular septum. Perforation and pulmonary valvuloplasty using a modified mechanical technique. Medium-term follow-up]. / Atresia pulmonar con septo íntegro. Perforación y valvuloplastia pulmonar mediante técnica mecánica modificada. Seguimiento a medio plazo.

INTRODUCTION AND OBJECTIVES: In patients with pulmonary atresia with intact ventricular septum (PAIVS), radiofrequency-assisted perforation of the valve is the most widely used initial therapy when the anatomy is favorable. We report our experience with a modified mechanical technique that gave good results. METHODS: Between November 2001 and October 2006, valve opening was carried out successfully in 11 consecutive neonates with a favorable anatomy (i.e., Alwi groups A and B, and tricuspid valve Z-score -1.1 [1.3]). The technique involved snare-assisted anterograde or retrograde perforation with the soft tip of a special guidewire used for chronic total coronary artery occlusions, use of an arteriovenous loop, and progressive balloon dilatation from a diameter of 2 mm to a maximum diameter of 9.6 [1.2] mm. RESULTS: Valve opening was achieved in all patients, and right ventricular (RV) systolic pressure fell from 97 [17] mmHg to 48 [13] mmHg (P< .001). No pericardial effusion or cardiac tamponade was observed, though one neonate died 24 hours after the procedure due to pulmonary embolism. Six patients (54%) were discharged without any further intervention, while 4 (36%) required an additional increase in pulmonary blood flow. During the follow-up period of 25 [21] months, two patients died. Eight (72%) survived and were in New York Heart Association functional class 1. Two required additional surgery on the outflow tract, one of whom also needed a one-and-a-half ventricular repair. Data indicate that the valves remain open as RV structures grow, though without any change in the tricuspid valve Z-score. CONCLUSIONS: Pulmonary valvuloplasty using a mechanical technique proved effective in patients with PAIVS. Modification of the standard mechanical technique by using the soft tip of a special guidewire used for chronic total coronary artery occlusions was less aggressive and improved results. In patients with a favorable anatomy, results were comparable to those obtained using the radiofrequency technique.

Atresia pulmonar con septo íntegro. Perforación y valvuloplastia pulmonar mediante técnica mecánica modificada. Seguimiento a medio plazo / Pulmonary atresia with intact ventricular septum. Perforation and pulmonary valvuloplasty using a modified mechanical technique. Medium-term follow-up

Introducción y objetivos. En la atresia pulmonar con septo íntegro, si la anatomía es favorable, el tratamiento inicial más extendido es la apertura valvular mediante radiofrecuencia. Presentamos nuestra experiencia mediante una técnica mecánica modificada con buenos resultados. Métodos. Entre noviembre de 2001 y octubre de 2006 realizamos apertura valvular en 11 casos consecutivos con anatomía favorable (grupo A y B de Alwi e índice z tricúspide de ­1,1 ± 1,3), mediante perforación anterógrada guiada por lazo o retrógrada mediante guías especiales para la obstrucción crónica total coronaria por su parte blanda y circuito arteriovenoso y dilatación progresiva con balón monorraíl coronario desde 2 mm de diámetro hasta un máximo de 9,6 ± 1,2 mm. Resultados. Se realizó la apertura valvular en todos los casos con reducción de la presión sistólica del ventrículo derecho de 97 ± 17 a 48 ± 13 mmHg (p < 0,001). No se observaron derrame ni taponamiento en ningún caso, y un neonato falleció a las 24 h por un tromboembolismo pulmonar. Seis casos (54%) fueron dados de alta sin otro procedimiento, y 4 (36%) precisaron un flujo pulmonar adicional. El seguimiento fue de 25 ± 21 meses. Dos pacientes fallecieron, mientras que 8 (72%) sobrevivieron y se encontraban en situación funcional I. Dos precisaron cirugía adicional del tracto de salida y en uno de ellos, además, del ventrículo y medio. Persisten datos de desobstrucción valvular con crecimiento de estructuras en el ventrículo derecho, aunque sin cambios en el índice z valvular. Conclusiones. La valvuloplastia pulmonar con técnica mecánica sigue siendo válida en la atresia pulmonar con septo íntegro. La modificación de la técnica mecánica clásica mediante guías especiales para la obstrucción crónica total coronaria por su parte blanda es menos agresiva y mejora sus resultados. En esta serie con anatomía favorable los resultados son superponibles a los obtenidos mediante radiofrecuencia (AU)

Introduction and objectives. In patients with pulmonary atresia with intact ventricular septum (PAIVS), radiofrequency-assisted perforation of the valve is the most widely used initial therapy when the anatomy is favorable. We report our experience with a modified mechanical technique that gave good results. Methods. Between November 2001 and October 2006, valve opening was carried out successfully in 11 consecutive neonates with a favorable anatomy (i.e., Alwi groups A and B, and tricuspid valve Z-score ­1.1 [1.3]). The technique involved snare-assisted anterograde or retrograde perforation with the soft tip of a special guidewire used for chronic total coronary artery occlusions, use of an arteriovenous loop, and progressive balloon dilatation from a diameter of 2 mm to a maximum diameter of 9.6 [1.2] mm. Results. Valve opening was achieved in all patients, and right ventricular (RV) systolic pressure fell from 97 [17] mmHg to 48 [13] mmHg (P<.001). No pericardial effusion or cardiac tamponade was observed, though one neonate died 24 hours after the procedure due to pulmonary embolism. Six patients (54%) were discharged without any further intervention, while 4 (36%) required an additional increase in pulmonary blood flow. During the follow-up period of 25 [21] months, two patients died. Eight (72%) survived and were in New York Heart Association functional class 1. Two required additional surgery on the outflow tract, one of whom also needed a one-and-a-half ventricular repair. Data indicate that the valves remain open as RV structures grow, though without any change in the tricuspid valve Z-score. Conclusions. Pulmonary valvuloplasty using a mechanical technique proved effective in patients with PAIVS. Modification of the standard mechanical technique by using the soft tip of a special guidewire used for chronic total coronary artery occlusions was less aggressive and improved results. In patients with a favorable anatomy, results were comparable to those obtained using the radiofrequency technique (AU)

Isolated pseudohypoplasia of the right ventricle.

We present a case of a 12-year-old girl with severe cyanosis due to abnormal moderator band producing a hidden trabecular component of the right ventricle, mimicking isolated hypoplasia of the right ventricle. A marked hypoplasia was confirmed by echocardiography and catheterization. At operation an anomalously thickened moderator band obstructing the apical infundibulum was found. Repair consisted of a section of the moderator band and closure of the atrial septal defect. Postoperatively the right ventricle showed normal dimension and function.

Respiratory infection in congenital cardiac disease. Hospitalizations in young children in Spain during 2004 and 2005: the CIVIC Epidemiologic Study.

OBJECTIVES: To evaluate the rate of hospitalization for acute respiratory tract infection in children less than 24 months with haemodynamically significant congenital cardiac disease, and to describe associated risk factors, preventive measures, aetiology, and clinical course. MATERIALS AND METHODS: We followed 760 subjects from October 2004 through April 2005 in an epidemiological, multicentric, observational, follow-up, prospective study involving 53 Spanish hospitals. RESULTS: Of our cohort, 79 patients (10.4%, 95% CI: 8.2%-12.6%) required a total of 105 admissions to hospital related to respiratory infections. The incidence rate was 21.4 new admissions per 1000 patients-months. Significant associated risk factors for hospitalization included, with odds ratios and 95% confidence intervals shown in parentheses: 22q11 deletion (8.2, 2.5-26.3), weight below the 10th centile (5.2, 1.6-17.4), previous respiratory disease (4.5, 2.3-8.6), incomplete immunoprophylaxis against respiratory syncytial virus (2.2, 1.2-3.9), trisomy 21 (2.1, 1.1-4.2), cardiopulmonary bypass (2.0, 1.1-3.4), and siblings aged less than 11 years old (1.7, 1.1-2.9). Bronchiolitis (51.4%), upper respiratory tract infections (25.7%), and pneumonia (20%) were the main diagnoses. An infectious agent was found in 37 cases (35.2%): respiratory syncytial virus in 25, Streptococcus pneumoniae in 5, and Haemophilus influenzae in 4. The odds ratio for hospitalization due to infection by the respiratory syncytial virus increases by 3.05 (95% CI: 2.14 to 4.35) in patients with incomplete prophylaxis. The median length of hospitalization was 7 days. In 18 patients (17.1%), the clinical course of respiratory infection was complicated and 2 died. CONCLUSIONS: Hospital admissions for respiratory infection in young children with haemodynamically significant congenital cardiac disease are mainly associated with non-cardiac conditions, which may be genetic, malnutrition, or respiratory, and to cardiopulmonary bypass. Respiratory syncytial virus was the most commonly identified infectious agent. Incomplete immunoprophylaxis against the virus increased the risk of hospitalization.