Sunflower (Helianthus annuus) Seed Allergy.

BACKGROUND AND OBJECTIVE: Sunflower seed is one of the most common edible seeds and its consumption is growing. Case reports of sunflower seed allergy have been described since the 1970s. However, there are few data on the prevalence and clinical manifestations of sunflower seed allergy. To improve understanding of sunflower seed allergy. METHODS: We evaluated the clinical and immunological features of patients with sunflower seed allergy diagnosed in the Allergy Department of a tertiary hospital in Madrid over a 5-years period. RESULTS: Forty-seven patients reported adverse reactions after ingestion of sunflower seed and had specific sensitization to sunflower seed determined by skin prick test (median 8 mm) or specific IgE (median 1.10 kUA/L). Most had an adult-onset reaction to sunflower seed preceded by a history of atopy and other food allergies, predominantly to peach, peanut and nuts. Clinical presentation of sunflower seed allergy ranged from mild to severe, with a high proportion of patients suffering severe reactions, often undertreated. A variability in the severity of symptoms was seen on repeated exposures to sunflower seed on a same patient. Levels of sunflower seed IgE were strongly correlated with levels of IgE to non-specific lipid transfer proteins, while no significant differences were found in the severity of the reactions according to sensitization to those proteins. CONCLUSION: Our findings reveal a variability of clinical presentations of sunflower seed allergy on repeated exposures and an underuse of epinephrine in anaphylaxis. We highlight the importance of strict avoidance of sunflower seed and accurate prescription and administration of epinephrine in allergic patients.

Using CDOM spectral shape information to improve the estimation of DOC concentration in inland waters: A case study of Andean Patagonian Lakes.

For the last two decades different scientific disciplines have focused on lacustrine dissolved organic matter (DOM) given its importance in the biogeochemistry of carbon and in ecosystem functioning. New satellites supply the appropriate resolutions to evaluate chromophoric dissolved organic matter (CDOM) in inland waters, opening the possibility to estimate DOM at appropriate spatiotemporal scales. This requires, however, a robust relationship between CDOM and dissolved organic carbon (DOC). In this work, we evaluated the use of CDOM as a proxy of DOC in 7 Andean Patagonian lakes. Considering the entire data set, CDOM absorption coefficients (a355 and a440) were linearly related with DOC. Shallow lakes, however, drove this relationship showing a moderate relationship, whereas, deep lakes with lower colour presented a weaker relationship. Therefore, we assessed the use of CDOM spectral shape information to improve DOC estimates regardless of observed DOM differences due to climatic seasonality and lakes' morphometry. The use of well-known CDOM spectral shape metrics (i.e., S275-295 and a250:a365 ratio) significantly improved DOC estimation. Particularly, using a Gaussian decomposition approach we found that much of the variation in the spectral shape, associated with the variability of CDOM:DOC ratio, was explained by differences in two dynamic regions centred at 270 and 320 nm. A strong nonlinear relationship was found between the a270:a320 ratio and the DOC-specific absorption coefficients a*355 and a*440. This was translated into a further improvement in DOC estimation yielding the higher R2 and lower mean absolute differences (MAPD < 16%), either considering the entire data set or shallow and deep lakes separately. Our results highlight that incorporating the CDOM spectral shape information improves the characterization of the DOC pool of inland waters, which is particularly relevant for remote and/or inaccessible sites and has significant implications for the environmental management, biogeochemical studies and future remote sensing applications.

Hypersensitivity Reactions to Cancer Chemotherapy: Practical Recommendations of ARADyAL for Diagnosis and Desensitization.

Rapid drug desensitization has enabled first-line therapies in patients with drug hypersensitivity reactions to chemotherapeutic drugs including monoclonal antibodies. Desensitization is a safe and highly effective procedure, not only for IgE-mediated reactions, but also for those mediated by non-IgE mechanisms. The likelihood of breakthrough reactions during desensitization is low, and most are mild; in fact, moderate-to-severe reactions are infrequent. In this document, 16 allergy departments belonging to the Spanish research network ARADyAL present a review of the available scientific evidence and provide general guidelines for the diagnosis and management of drug hypersensitivity reactions to chemotherapeutic drugs and monoclonal antibodies. Emphasis is placed on the desensitization procedure.

Fluorescence components of natural dissolved organic matter (DOM) from aquatic systems of an Andean Patagonian catchment: Applying different data restriction criteria for PARAFAC modelling.

Fluorescence spectroscopy is a widely applied technique to characterize the composition of the fluorescent fraction of dissolved organic matter (DOM), allowing to infer sources and diagenetic state of soil, marine and freshwater DOM samples. The analysis of fluorescent DOM (FDOM) is often carried out by multi-way models such as parallel factor analysis (PARAFAC), which allows decomposing excitation-emission matrices (EEMs) obtained from DOM samples into their underlying chemical components. Some aspects of the performance/accuracy of the EEM-PARAFAC technique regarding the use of selective vs. non-selective EEM data are still in discussion. In this investigation, we evaluated the outcome of two different approaches (non-selective and selective) applied to study the composition of DOM from four headwater streams (Case 1) and from two neighboring shallow lakes (Case 2), all belonging to the same Andean watershed within Nahuel Huapi National Park in North Patagonia (Argentina). In both cases, the outcome of the PARAFAC performed to non-selective data (EEM datasets from all the streams and the two lakes) vs. selective datasets (EEMs from each stream and each lake treated separately) is compared on the basis of modelled fluorescent components. Regardless of the restriction criteria applied for the analyses, the results obtained indicated similar component loadings in the four streams and in the two lakes. The similarity of the outcomes likely relates to the low internal variation of the EEMs, since these are located in the same catchment, influenced by similar soils and vegetation which are the main DOM sources. Therefore, we conclude that the use of a small selective EEM dataset may not condition the validation of the FDOM components and their temporal dynamics.

IgE-Mediated Sensitization to Galactose-α-1,3- Galactose (α-Gal) in Urticaria and Anaphylaxis in Spain: Geographical Variations and Risk Factors.

BACKGROUND: The objectives of this study were to investigate the prevalence of sIgE to galactose-α-1,3-galactose (α-gal) in individuals with acute urticaria or anaphylaxis from different geographical areas of Spain and to evaluate the relevance of demographics and lifestyle as risk factors for this immune response. METHODS: Participants were recruited from allergy departments at 14 Spanish hospitals. Patients aged 18 years or older presenting with urticaria or anaphylaxis were enrolled into one of 2 arms: cases and controls. An interviewer-administered questionnaire collecting demographic data, lifestyle habits, and the presence of cofactors was obtained from each participant. sIgE to α-gal and total IgE were determined using ImmunoCAP. sIgE levels ≥0.35 kU/L were considered a positive result. RESULTS: The study population comprised 160 cases and 126 controls. The median age was 44 years. The overall prevalence of a positive result of sIgE to α-gal was 15.7%; this was higher in cases (26.3%) than in controls (2.4%). The sIgE anti-α-gal positivity rate ranged from 37.68% (rural) to 15.38% (semiurban), and 7.85% (urban). The rates of positivity were 46.32%, (Northern), 0.72% (Center), and 0% (Mediterranean). A positive result for sIgE to α-gal was associated with a history of tick bites, participation in outdoor activities, pet ownership, and ingestion of mammalian meats or innards before the onset of symptoms. Only alcohol consumption could be implicated as a cofactor. CONCLUSION: Sensitization to α-gal in patients with urticaria or anaphylaxis differs considerably between the 3 geographical areas studied and is related to tick bites.

Validation of the Spanish Version of the Food Allergy Quality of Life Questionnaire-Parent Form (S-FAQLQ-PF).

BACKGROUND: Food allergy is an emerging health problem. Several questionnaires can be used to establish health-related quality of life (HRQOL) in food allergy patients. Current questionnaires should be translated in such a way that they take account of the culture of the country in which they are to be used. Objective: To translate and perform a cross-sectional validation of the Food Allergy Quality of Life Questionnaire-Parent Form (FAQLQ-PF). METHODS: The parents of 54 children diagnosed with food allergy were recruited to assess the Spanish version of the FAQLQ-PF (S-FQLQ-PF). RESULTS: The S-FQLQ-PF was translated into Spanish according to WHO guidelines (including a forward-backward translation). The statistical analysis showed that feasibility, reliability, and internal consistency were very good for the global S-FAQLQ-PF score and for the different domains. Assessment of construct validity indicated that S-FAQLQ-PF has reduced capacity for measurement of HRQOL in younger children. Cross-sectional validation of the S-FAQLQ-PF demonstrated that HRQOL of a Spanish pediatric population was affected by patient age, severity of symptoms, and number of reactions. HRQOL was not affected by sex, food implicated, number of foods implicated, ingestion of the implicated food, or presence of anaphylaxis. CONCLUSIONS: Translation into Spanish and cultural validation of the FAQLQ-PF demonstrated the influence of factors, such as patient age, severity of symptoms, and number of reactions on the HRQOL of a pediatric Spanish population.

Identification of novel peptide biomarkers to predict safety and efficacy of cow's milk oral immunotherapy by peptide microarray.

BACKGROUND: Cow's milk oral immunotherapy (CM-OIT) is still an experimental treatment. The development of novel biomarkers to predict the safety and efficacy of CM-OIT is crucial to translate this treatment to common clinical practice. OBJECTIVE: To analyse long-term changes in IgE and IgG4 epitope binding profile induced by CM-OIT to identify safety and efficacy biomarkers. METHODS: We studied 25 CM-allergic children who underwent CM-OIT and seven non-treated CM-allergic children as controls. CM-OIT patients were classified as low, moderate, and high risk according to the number of allergic reactions (safety), time required to achieve desensitization (efficacy) and need of premedication. IgE and IgG4 peptide microarray immunoassay was performed using a library of overlapping peptides of CM proteins at baseline, after oral desensitization, and 6, 12, and 24 months of follow-up. RESULTS: Cow's milk oral immunotherapy induced a rapid increase of IgG4-binding epitopes and a slow decrease in IgE-binding epitopes. High-risk patients recognized a statistically significant higher number of IgE peptides in caseins at all the times studied. Similar but less pronounced changes were observed for IgG4-positive peptides. Clustering analysis grouped together the high-risk patients, and we identified 13 regions of caseins significantly differed between groups of patients. Bioinformatics analysis selected two sets of 16 IgE-binding peptides at baseline that predicted safety (R(2)  = 0.858) and efficacy (R(2)  = 0.732), respectively, of CM-OIT. CONCLUSION AND CLINICAL RELEVANCE: We found two sets of IgE-binding peptides that can be used as novel biomarkers to predict the safety and efficacy of CM-OIT before starting treatment.

SLIT: indications, follow-up, and management

La inmunoterapia específica sublingual (SLIT) ha demostrado ser un tratamiento seguro y eficaz para la alergia respiratoria. Sin embargo, aspectos como la selección del paciente, el empleo de dosis óptimas, sus efectos en el asma y a largo plazo, o el manejo de las reacciones adversas necesitan una mayor investigación. Además, la gran heterogeneidad de estudios realizados con SLIT y la aplicación de los mismos criterios que los que se aplican a la inmunoterapia subcutánea dificultan la obtención de conclusiones precisas y útiles al clínico que prescribe este tratamiento. Por ello, el grupo Quasar (QUality in the Administration of SLIT in Allergic Rhinitis), grupo de alergólogos con amplia experiencia clínica con SLIT, se propuso recoger el estado de la investigación actual y los datos reales disponibles con SLIT. Para ello, se establecieron grupos de trabajo en tres categorías distintas: selección del paciente candidato a recibir SLIT, eficacia del tratamiento y reacciones adversas. Se realizó una búsqueda de artículos representativos para cada tema, localizándose inicialmente en PubMed 850, de los que se seleccionaron y analizaron 314 para extraer la evidencia disponible con SLIT, incorporando finalmente 266 al documento. Se realizó la gradación de la evidencia para cada pregunta clínica según la clasificación de Oxford. El texto resultante fue evaluado hasta en tres ocasiones por todos los miembros del grupo hasta consensuar el documento final que revisa el cuerpo de la evidencia existente hasta el momento sobre SLIT, particularmente con pólenes, sobre los que existe un mayor número de artículos, y, para aquellos aspectos en los que se ha demostrado evidencia insuficiente, propone una alternativa consensuada entre los miembros del grupo Quasar. Finalmente, se proponen algoritmos de selección del paciente candidato para SLIT y de manejo de reacciones adversas (AU)

Specific sublingual immunotherapy (SLIT) has been proved to be a safe and effective approach in respiratory allergy. However, further research is required on aspects such as patient selection, use of optimal dosing, effects on asthma, long-term effects, and management of adverse reactions. In addition, the widely heterogeneous nature of studies on SLIT performed to date and the application of the criteria for subcutaneous immunotherapy make it difficult for the prescribing clinician to draw accurate and useful conclusions. Therefore, the QUASAR Group (QUality in the Administration of SLIT in Allergic Rhinitis), which comprises allergologists with broad clinical experience in SLIT, investigated the latest research findings and available data on this approach. Working parties were formed in 3 different categories: selection of candidates for SLIT, treatment efficacy, and adverse reactions. We performed a PubMed search for articles that were representative of each category and found 850. From these, we finally selected 266 articles, which were reviewed to retrieve data on SLIT. Evidence for each clinical question was graded according to the Oxford classification. The resulting text was evaluated on 3 occasions by all the members of the group until the final version was agreed upon. In this version, we review available evidence on SLIT, particularly with pollens, which is the subject of most articles. In areas where evidence is insufficient, an alternative agreed upon by the members of the QUASAR group is presented. Finally, we propose algorithms for selecting candidates for SLIT and for management of adverse events (AU)

SLIT: indications, follow-up, and management.

Specific sublingual immunotherapy (SLIT) has been proved to be a safe and effective approach in respiratory allergy. However, further research is required on aspects such as patient selection, use of optimal dosing, effects on asthma, long-term effects, and management of adverse reactions. In addition, the widely heterogeneous nature of studies on SLIT performed to date and the application of the criteria for subcutaneous immunotherapy make it difficult for the prescribing clinician to draw accurate and useful conclusions. Therefore, the QUASAR Group (QUality in the Administration of SLIT in Allergic Rhinitis), which comprises allergologists with broad clinical experience in SLIT, investigated the latest research findings and available data on this approach. Working parties were formed in 3 different categories: selection of candidates for SLIT, treatment efficacy, and adverse reactions. We performed a PubMed search for articles that were representative of each category and found 850. From these, we finally selected 266 articles, which were reviewed to retrieve data on SLIT. Evidence for each clinical question was graded according to the Oxford classification. The resulting text was evaluated on 3 occasions by all the members of the group until the final version was agreed upon. In this version, we review available evidence on SLIT, particularly with pollens, which is the subject of most articles. In areas where evidence is insufficient, an alternative agreed upon by the members of the QUASAR group is presented. Finally, we propose algorithms for selecting candidates for SLIT and for management of adverse events.

Validez de un método fotométrico de medición de partículas en aire para el control de quirófanos y otras salas de ambiente controlado en los hospitales / Validity of a photometric method of counting particles in air for control of operating rooms and clean rooms in hospitals

Objetivo: Validar un sistema de medici¨®n fotom¨¦trica de part¨ªculas en aire para realizar los controles de bioseguridad ambiental de quir¨®fanos y otras salas de ambiente controlado en los hospitales. Material y m¨¦todos: Se realizaron un total de 144 mediciones pareadas de aire, 88 en ocho quir¨®fanos con sistemas de ventilaci¨®n convencional y tres niveles de filtraci¨®n y 56 en nueve habitaciones sin instalaciones de ventilaci¨®n. Se midieron part¨ªculas de entre 0,1 a 10 ¦Ìm en mg/m3 por m¨¦todo fotom¨¦trico. Simult¨¢neamente, en cada sala se realiz¨® impacto de aire en placas de Petri con medio de cultivo mediante aparato aspirador de cabezal perforado, obteniendo tras cinco d¨ªas de incubaci¨®n recuento de unidades formadoras de colonias (UFC)/1000L. Finalmente se realiz¨® el c¨¢lculo de los puntos de corte ¨®ptimos para la medici¨®n de part¨ªculas mediante Curvas ROC para discriminar tres niveles de contaminaci¨®n. Resultados: El coeficiente de correlaci¨®n entre las dos mediciones fue 0,779 (p<0,001). Los puntos de corte fueron para 0 UFC. 0,015, para >=10 UFC, 0,037, y para >=100 UFC, 0,053. La sensibilidad para estos puntos fue de 95,83%,100% y 100%, y los ¨ªndices Kappa registraron 0,51, 0,88 y 0,75 respectivamente. Conclusi¨®n: En nuestro estudio la correlaci¨®n entre las medidas es notable; la medici¨®n fotom¨¦trica de part¨ªculas puede ser una alternativa para llevar a cabo alguno de los controles rutinarios de bioseguridad (AU)

Objective: Validate a photometric on-air particle measurement system in order to perform ambient bio-security checks in operating rooms and other clean rooms within hospitals. Material and methods: A total of 144 coupled air samples were performed, 88 in 8 different operating rooms with conventional ventilation systems and 3-level filtering and 56 in 9 different rooms without specific ventilation systems. Particles were measured in size between 0,1 to 10 ¦Ìm with the photometric system in mg/m3. Simultaneously in the rooms are performed the sampling air method with impact on culture media. Incubation period is 5 days and count is performed in colony-forming units (CFU/1000L). Finally, optimal cut-points in particles measure were calculated using ROC curves in order to discriminate the three different contamination levels. Results: Spearma´s correlation coefficient was 0,779 p<0,001. Sensitivity for cut-points in particle counting for the different contamination levels was 95,83% ,100% and 100% respectively, and the corresponding Kappa indexes were 0,51; 0,88 and 0,75. Conclusion: Correlation among measurements is notable. Particle measurement can be a worthy alternative for some of the routine bio-security checks (AU)

Latex allergy: Position Paper.

Correct management of latex allergy is essential to ensure adequate care of patients who are allergic to latex, which is ubiquitous in the health care setting. In this Position Paper, the Latex Committee of the Spanish Society of Allergology and Clinical Immunology provides guidelines for the management of latex allergy.

Adaptation and validation of the Spanish version of the Quality Of Life in Latex Allergy questionnaire (QOLLA).

OBJECTIVE: The aim of the present study was to translate into Spanish and transculturally adapt the Quality of Life in Latex Allergy questionnaire (QOLLA) in order to provide a validated instrument for use in research and daily practice. METHODS: Patients diagnosed with latex allergy were invited to participate in an observational prospective multicenter study to validate the Spanish version of the QOLLA following the recommendations of the World Health Organization. The study included 3 phases--feasibility, reliability, and cross-sectional validation-and was approved by the Ethics Committee of Hospital Ramón y Cajal. RESULTS: Mean time to complete the questionnaire was 4.7 minutes. The maximum score was 28 (mean, 7.7; median, 4).The SF-12 score ranged from 25.8 to 51.6 in the physical domain and from 20.8 to 61.5 in the mental domain. Internal consistency was excellent (Cronbach alpha, 0.9348). The kappa index fluctuated between 0.40 and 0.93. A kappa of 0.84 was obtained for the global score in 5 categories. Sixty patients were included to evaluate construct validity. Mean age was 39 years and 49 patients were women (80%). The global score ranged between 0 and 30 (mean, 11.69; median, 11). Spearman correlation coefficients between the QOLLA and a visual analog scale and the SF-12 physical, mental, and severity scales according to the researcher were--0.47, 0.37, 0.29, and 0.54, respectively. CONCLUSION: The QOLLA is a feasible, valid, and reliable instrument for the measurement of disease-specific quality of life in adult patients diagnosed with latex allergy. It could play an important role in determining suitable treatment for latex-allergy.

Dust inputs and bacteria influence dissolved organic matter in clear alpine lakes.

Remote lakes are usually unaffected by direct human influence, yet they receive inputs of atmospheric pollutants, dust, and other aerosols, both inorganic and organic. In remote, alpine lakes, these atmospheric inputs may influence the pool of dissolved organic matter, a critical constituent for the biogeochemical functioning of aquatic ecosystems. Here, to assess this influence, we evaluate factors related to aerosol deposition, climate, catchment properties, and microbial constituents in a global dataset of 86 alpine and polar lakes. We show significant latitudinal trends in dissolved organic matter quantity and quality, and uncover new evidence that this geographic pattern is influenced by dust deposition, flux of incident ultraviolet radiation, and bacterial processing. Our results suggest that changes in land use and climate that result in increasing dust flux, ultraviolet radiation, and air temperature may act to shift the optical quality of dissolved organic matter in clear, alpine lakes.

Skin prick test predictive value on the outcome of a first known egg exposure in milk-allergic children.

Children with milk allergy have higher incidence of other food allergies, especially egg allergy. The objective of this study was to ascertain the accuracy of the prick test in children with IgE-mediated milk allergy for diagnosing egg allergy. Children under the age of 1 yr who came consecutively to Allergy Department 2003-05, and were diagnosed with IgE-mediated milk allergy were selected for this study. Egg introduction was completely avoided until the age of 14 months when clinical history, skin prick tests (SPT), specific-IgE antibodies determination and egg challenge test were performed. One hundred and four milk-allergic children were included. At least one positive prick test to any egg allergen was found in 65 out of the 104 (62.5%). Thirty-eight (36.5%) were allergic to egg proteins as well. Prick tests with egg white and ovomucoid (OVM) had the best diagnostic performances showing the largest areas under the receiver operating characteristic curve. The optimal diagnosis cut-off point was 6 mm for egg white and 5 mm for OVM. The positive likelihood ratios for these cut-off points were: 2.95 (95% CI: 1.74-4.99) for egg white prick test, and 20 (95% CI: 2.9-143.7) for OVM prick test. Children with specific IgE-mediated cow's milk allergy must be closely followed as a risk group for egg allergy. Early diagnosis is necessary and the SPT has shown itself to be a very useful tool for diagnosing immediate IgE reactions to egg on first known exposure.

Prevalence of allergy and anaphylactic symptoms in 210 adult and pediatric patients with mastocytosis in Spain: a study of the Spanish network on mastocytosis (REMA).

BACKGROUND: Mast cells (MCs) play a key role in allergic diseases through the release of inflammatory mediators, which are responsible of allergic symptoms. Mastocytosis is characterized by an abnormal proliferation and accumulation of mast cells, in which mediators are released intermittingly or continuously. Despite these clinical similarities, few studies have addressed the presence of allergic symptoms in mastocytosis patients, including anaphylaxis. OBJECTIVE: A prospective evaluation was carried out to study the prevalence of allergic diseases in patients with mastocytosis and their impact on the natural history of mastocytosis. METHODS: A questionnaire was given to 210 patients with mastocytosis to evaluate the history of asthma, rhinitis, conjunctivitis, atopic dermatitis, urticaria and anaphylaxis. Patients underwent total IgE, Phadiatop infant (aeroallergens and food allergens), specific IgE to latex and to Anisakis simplex determinations. Skin tests were done to 72 patients. RESULTS: The prevalence of allergy, as defined by clinical symptoms associated to specific IgE, was 23.9%. Total IgE level was significantly higher in patients with allergy as compared with patients without allergy (median 58 vs. 16.5 kU/L, P<0.0001). Anaphylactic symptoms were present in 36 patients (22%), in nine the allergen was identified. Males had more allergy and anaphylactic symptoms than females (61.5% vs. 38.5% and 72% vs. 28%, respectively). CONCLUSIONS: Allergic diseases coexist in patients with mastocytosis with similar frequency as compared with the general population. Anaphylactic symptoms are more prevalent in males with mastocytosis and in patients with elevated IgE. CAPSULE SUMMARY: The prevalence of allergy in mastocytosis is similar to the general population. Anaphylactic symptoms are more prevalent in males and in patients with elevated IgE. The coexistence of atopy does not influence mastocytosis-associated symptoms.

Structure and dynamics of food webs in Andean Lakes

Se ha realizado un estudio de los sistemas de alimentos de los lagos oligotróficos en la región de Bariloche. Se nota diferencias comparados con los lagos del hemisferio norte. Se podrían crear cambios con la incorporación de salmonidas exóticas y distintas especies de árboles

[Fallot's tetralogy with severe symptomatology during the first year of life (author's transl)]. / Tetralogía de Fallot grave en el primer año de vida. Aspectos clínicos e implicaciones quirúrgicas

Seventeen cases of Fallot's Tetralogy with severe symptomatology during the first year of life are reviewed. In all of them, a cardiac catheterization and angiocardiography were performed. In 2 cases the pulmonary outflow tract was atretic. In general, the clinical, radiologic, electrocardiographic and hemodynamic findings were similar to those previously reported, although in 3 patients of the present series atypical electrocardiographic findings were found. Pronounced narrowing and horizontal orientation of the infundibular chamber, and accentuated hypoplasia of the pulmonary artery branches and of the left heart cavities and were all unfavourable findings for total correction frequently found in the present material. Minimal or non-existent aortic dextroposition was observed in association with cases of Fallot's Tetralogy with extreme severity. Surgery was indicated as an emergency measure in the majority of the cases. Palliatives procedures were performed in 10 patients, and corrective surgery in one. Surgical results agree with the extreme severity that this lesion has, when it gives rise to important symptomatology so early in the life.