Coronary anomalies: what the radiologist should know

AbstractCoronary anomalies comprise a diverse group of malformations, some of them asymptomatic with a benign course, and the others related to symptoms as chest pain and sudden death. Such anomalies may be classified as follows: 1) anomalies of origination and course; 2) anomalies of intrinsic coronary arterial anatomy; 3) anomalies of coronary termination. The origin and the proximal course of anomalous coronary arteries are the main prognostic factors, and interarterial course or a coronary artery is considered to be malignant due its association with increased risk of sudden death. Coronary computed tomography angiography has become the reference method for such an assessment as it detects not only anomalies in origination of these arteries, but also its course in relation to other mediastinal structures, which plays a relevant role in the definition of the therapeutic management. Finally, it is essential for radiologists to recognize and characterize such anomalies.

Avaliação cefalométrica da posição do osso hióide em crianças respiradoras bucais

MATERIAL E MÉTODOS: em função das relações anatomofuncionais do osso hióide com o complexo craniofacial, realizou-se avaliação cefalométrica da posição do osso hióide em relação ao padrão respiratório. A amostra consistiu de 53 crianças, gênero feminino, com idades médias de 10 anos, sendo 28 respiradoras nasais e 25, bucais. As medidas cefalométricas horizontais, verticais e angulares foram utilizadas com a finalidade de determinar a posição do osso hióide. Estabeleceu-se uma comparação entre os grupos por meio do teste "t" de student, bem como correlação de Pearson entre as variáveis. RESULTADOS: Observou-se que não ocorreram diferenças estatísticas significativas para a posição mandibular e posição do osso hióide e o tipo do padrão respiratório. No Triângulo Hióideo, o coeficiente de correlação de 0,40 foi significativo entre AA-ENP (distância entre vértebra atlas e espinha nasal posterior) e C3-H (distância entre a terceira vértebra cervical e osso hióide) demonstrando uma relação positiva entre os limites ósseos do espaço aéreo superior e inferior. Para as medidas cranianas sugeriu-se uma relação entre a posição do osso hióide com a morfologia mandibular. CONCLUSÃO: Os resultados permitiram concluir que o osso hióide mantém uma posição estável, provavelmente, para garantir as proporções corretas das vias aéreas e não depende do padrão respiratório predominante.

Alterações da cavidade nasal e do padrão respiratório após expansão maxilar

A deficiência transversal da maxila é uma anomalia dentofacial relacionada à diminuição do diâmetro do arco maxilar. Este tipo de deformidade está muitas vezes associado a deficiência respiratória nasal, sendo um importante fator etiopatogênico. O tratamento desta atresia pode ser realizado através da Expansão Rápida da Maxila ou Expansão Rápida da Maxila Cirurgicamente Assistida, ficando na dependência da idade do paciente. Ambos os procedimentos levarão a mudanças na estrutura craniofacial, especialmente na cavidade nasal. OBJETIVO: Através da descrição de trabalhos levantados na literatura, procurou-se relatar a relação existente entre a expansão maxilar, cavidade nasal e resistência aérea nasal. MÉTODO: Foi realizada uma tradicional revisão assistemática da literatura, utilizando trabalhos do tipo experimentais, incluindo os que utilizaram Expansão Rápida da Maxila e Expansão Rápida da Maxila Cirurgicamente Assistida, e excluindo os que utilizaram Expansão Maxilar por Osteotomia Segmentada para o tratamento da atresia maxilar. RESULTADO: Tanto a Expansão Rápida da Maxila quanto a Expansão Rápida da Maxila Cirurgicamente Assistida levam a mudanças dentofaciais, especialmente sobre a cavidade nasal, causando um aumento da largura nasal, o que pode levar à diminuição da Resistência Aérea Nasal. CONCLUSÃO: Através dos estudos cefalométricos ântero-posteriores, fica evidente o aumento da largura da cavidade nasal após expansão maxilar.

Neonatal mortality: description and effect of hospital of birth after risk adjustment

OBJECTIVE: To assess the effect of hospital of birth on neonatal mortality. METHODS: A birth cohort study was carried out in Pelotas, Southern Brazil, in 2004. All hospital births were assessed by daily visits to all maternity hospitals and 4558 deliveries were included in the study. Mothers were interviewed regarding potential risk factors. Deaths were monitored through regular visits to hospitals, cemeteries and register offices. Two independent pediatricians established the underlying cause of death based on information obtained from medical records and home visits to parents. Logistic regression was used to estimate the effect of hospital of birth, controlling for confounders related to maternal and newborn characteristics, according to a conceptual model. RESULTS: Neonatal mortality rate was 12.7‰ and it was highly influenced by birthweight, gestational age, and socioeconomic variables. Immaturity was responsible for 65% of neonatal deaths, followed by congenital anomalies, infections and intrapartum asphyxia. Adjusting for maternal characteristics, a three-fold increase in neonatal mortality was seen between similar complexity hospitals. The effect of hospital remained, though lower, after controlling for newborn characteristics. CONCLUSIONS: Neonatal mortality was high, mainly related to immaturity, and varied significantly across maternity hospitals. Further investigations comparing delivery care practices across hospitals are needed to better understand NMR variation and to develop strategies for neonatal mortality reduction.

EVALUATION OF SUBLETHAL EFFECTS OF Ipomoea cairica LINN. EXTRACT ON LIFE HISTORY TRAITS OF DENGUE VECTORS

Plant derived insecticides have considerable potential for mosquito control because these products are safer than conventional insecticides. This study aimed to investigate sublethal activities of Ipomoea carica or railway creeper crude acethonilic extract against life history trait of dengue vectors, Aedes albopictus and Aedes aegypti. The late third instar larvae of Ae. albopictus and Ae. aegypti were exposed to a sublethal dose at LC50 and larvae that survived were further cultured. Overall, Ipomea cairica crude extracts affected the whole life history of both Aedes species. The study demonstrated significantly lower egg production (fecundity) and eggs hatchability (fertility) in Ae. albopictus. The sublethal dose of crude extracts reduced significantly the width of larval head capsule and the wing length of both sexes in both Aedes species. The significance of sublethal effects of I. cairica against Aedes mosquitoes was an additional hallmark to demonstrate further activity of this plant despite its direct toxicity to the larvae. The reduced reproductive capacity as well as morphological and physiological anomalies are some of the effects that make I. cairica a potential candidate to be used as a new plant-based insecticide to control dengue vectors.

Rectangular flaps technique for treatment of congenital hand syndactyly

The authors analysed a series of 22 patients undergoing surgical correction of congenital hand syndactyly by the rectangular flap technique. Using our evaluation method, we found that good functional and aesthetic results were obtained in 77.3% of the patients, with a complication rate of 13.6%. We concluded that the rectangular flap technique has a simple design, is easily reproducible by in-training staff, has good results, and can be applied on the majority of the syndactyly cases.

Relationship between plasma creatinine concentration and glomerular filtration in preterm newborn infants

Fluid management and dosage regimens of drugs in preterm infants should be based on the glomerular filtration rate. The current methods to determine glomerular flitration rate are invasive, time-consuming, and expensive. In contrast, creatinine clearance can be easy obtained and quickly determined. The purpose of this study was to compare plasma creatinine on the third and seventh day of life in preterm newborn infants, to evaluate the influence of maternal creatinine, and to demonstrate creatinine clearance can be used as a reliable indicator of glomerular filtration rate. We developed a prospective study (1994) including 40 preterm newborns (gestational age < 37 weeks), average = 34 weeks; birth weight (average) = 1840 g, in the first week of life. Inclusion criteria consisted of: absence of renal and urinary tract anomalies; O2 saturation 3 92%; adequate urine output (>1ml/kg/hr); normal blood pressure; absence of infections and no sympathomimetic amines in use. A blood sample was collected to determine plasma creatinine (enzymatic method) on the third and seventh day of life and creatinine clearance (CrCl) was obtained using the following equation: , k = 0.33 in preterm infant All plasma creatinine determinations showed normal values [third day: 0.78 mg/dl ± 0.24 (mean ± SD)and seventh day: 0.67 mg/dl ± 0.31 - (p>0.05)]. Also all creatinine clearance at third and seventh day of life were normal [third day: 19.5 ml/min ± 5.2 (mean ± SD) and seventh day: 23.8 ml/min ± 7.3 - (p>0,05)]. All preterm infants developed adequate renal function for their respective gestational age. In summary, our results indicate that, for clinical practice, the creatinine clearance, using newborn length, can be used to estimate glomerular filtration rate in preterm newborn infants.

Renal and urinary findings in 20 patients with Williams-Beuren syndrome diagnosed by fluorescence in situ hybridization (FISH)

PURPOSE: Williams-Beuren syndrome is a rare multiple anomalies/mental retardation syndrome caused by deletion of contiguous genes at chromosome region 7q11.23. The aim of this work was to determine the frequency and the types of renal and urinary tract anomalies in 20 patients with Williams-Beuren syndrome. METHODS: The fluorescence in situ hybridization test using a LSI Williams syndrome region DNA probe was performed for all 20 patients to confirm the diagnosis of Williams-Beuren syndrome. A prospective study was performed in order to investigate renal and urinary aspects using laboratory assays to check renal function, ultrasonography of the kidneys and urinary tract, voiding cystourethrogram and urodynamics. RESULTS: Deletion of the elastin gene (positive fluorescence in situ hybridization test) was found in 17 out of 20 patients. Renal alterations were diagnosed in 5 of 17 (29%) the patients with the deletion and in 1 of 3 patients without the deletion. Fourteen patients with the deletion presented dysfunctional voiding. Arterial hypertension was diagnosed in 3 patients with deletions and 1 of these presented bilateral stenosis of the renal arteries. CONCLUSIONS: Due to the high incidence of renal and urinary abnormalities in Williams-Beuren syndrome, performing a systematic laboratory and sonographic evaluation of the patients is recommended.

On the influences of the El Niño, La niña and Atlantic Dipole Paterni on the Amazonian Rainfall during 1960-1998

The influence of the large-scale climatic variability dominant modes in the Pacific and in the Atlantic on Amazonian rainfall is investigated. The composite technique of the Amazon precipitation anomalies is used in this work. The basis years for these composites arc those in the period 1960-1998 with occurrences of extremes in the Southern Oscillation (El Niño or La Niña) and the north/south warm (or cold) sea surface temperature (SST) anomalies dipole pattern in the tropical Atlantic. Warm (cold) dipole means positive (negative) anomalies in the tropical North Atlantic and negative (positive) anomalies in the tropical South Atlantic. Austral summer and autumn composites for extremes in the Southern Oscillation (El Niño or La Niña) and independently for north/south dipole pattern (warm or cold) of the SST anomalies in the tropical Atlantic present values (magnitude and sign) consistent with those found in previous works on the relationship between Amazon rainfall variations and the SST anomalies in the tropical Pacific and Atlantic. However, austral summer and autumn composites for the years with simultaneous occurrences of El Niño and warm north/south dipole of the SST anomalies in the tropical Atlantic show negative precipitation anomalies extending eastward over the center-eastern Amazon. This result indicates the important role played by the tropical Atlantic in the Amazon anomalous rainfall distribution.

Total agenesis of the left pericardium

This is the report of a 46-year-old patient with the preoperative diagnosis of an atrial septal defect (ASD) of the ostium secudum type. After sternectomy, partial agenesis of the left pericardium was diagnosed. It is our opinion that, if the radiographic picture is suggestive of this entity, a clinical search for cardiopulmonary anomalies should be performed, because the majority of these associated anomalies can and should be surgically corrected.

Epidemiological characteristics of congenital heart diseases in Londrina, Paraná South Brazil

OBJECTIVE: To determine the prevalence and other epidemiological characteristics of congenital heart diseases. METHODS: A retrospective population based study of children who were born in Londrina, from January '89 to December '98 (80,262 live births). Diagnoses were confirmed through autopsy, surgery, catheterization, or echocardiography. RESULTS: A total of 441 patients was as certained what corresponds to a prevalence of 5.494:1,000 live births. Ventricular septal defect was the commonest lesion. A small number of transpositions of the great vessels and of left ventricular hypoplasia was observed. A high propation of ventricular septal defect (28.3%) and atrioventricular septal defects (8.1%) occurred. Fifty-one (11.35%) affected children had syndromic diseases and 52 (12.01%) children had nonsyndromic anomalies. CONCLUSION: The prevalence of congenital heart diseases in Londrina is in accordance with that of other regions of the globe. This prevalence also may reflect the reality in the southern region of Brazil, because population characteristics are very similar in the 3 southernmost Brazilian states.

Cantrell Syndrome. Case report of an adult

Cantrell syndrome is characterized by defects that involve the diaphragm, abdominal wall, pericardium, heart, and lower region of the sternum. It is a rare entity, usually diagnosed at birth and accompanied by high mortality due to the complexity and gravity of the anomalies. In this report, we present a 32-year-old male patient, who was diagnosed in infancy but who reached adult age asymptomatic.

Tetralogy of Fallot and hypertrophic cardiomyopathy: a rare association

Tetralogy of Fallot is known as the most common cyanotic congenital heart disease and has a prevalence of 10% of all congenital heart diseases. Although many other heart anomalies may coexist, the association of tetralogy of Fallot and hypertrophic cardiomyopathy is extremely rare. We report this association in a 15-month-old female, cyanotic since birth, in her first hospital admission for diagnosis and treatment of recurring cyanotic crises. In addition, a review of the literature and of the problems related to the treatment is provided.

Epidemiological study of congenital heart defects in children and adolescents: analysis of 4,538 cases

OBJECTIVE: To analyze the frequency and prevalence of congenital heart defects in a tertiary care center for children with heart diseases. METHODS: We carried out an epidemiological assessment of the first medical visit of 4,538 children in a pediatric hospital from January 1995 to December 1997. All patients with congenital heart defects had their diagnoses confirmed at least on echocardiography. The frequency and prevalence of the anomalies were computed according to the classification of sequential analysis. Age, weight, and sex were compared between the groups of healthy individuals and those with congenital heart defects after distribution according to the age group. RESULTS: Of all the children assessed, 2,017 (44.4%) were diagnosed with congenital heart disease, 201 (4.4%) with acquired heart disease, 52 (1.2%) with arrhythmias, and 2,268 (50%) were healthy children. Congenital heart diseases predominated in neonates and infants, corresponding to 71.5% of the cases. Weight and age were significantly lower in children with congenital heart defects. Ventricular septal defect was the most frequent acyanotic anomaly, and tetralogy of Fallot was the most frequent cyanotic anomaly. CONCLUSION: Children with congenital heart defects are mainly referred during the neonatal period and infancy with impairment in gaining weight. Ventricular septal defect is the most frequent heart defect.

Congenital Heart Disease as a Warning Sign for the Diagnosis of the 22q11.2 Deletion

Background: To alert for the diagnosis of the 22q11.2 deletion syndrome (22q11.2DS) in patients with congenital heart disease (CHD). Objective: To describe the main CHDs, as well as phenotypic, metabolic and immunological findings in a series of 60 patients diagnosed with 22q11.2DS. Methods: The study included 60 patients with 22q11.2DS evaluated between 2007 and 2013 (M:F=1.3, age range 14 days to 20 years and 3 months) at a pediatric reference center for primary immunodeficiencies. The diagnosis was established by detection of the 22q11.2 microdeletion using FISH (n = 18) and/or MLPA (n = 42), in association with clinical and laboratory information. Associated CHDs, progression of phenotypic facial features, hypocalcemia and immunological changes were analyzed. Results: CHDs were detected in 77% of the patients and the most frequent type was tetralogy of Fallot (38.3%). Surgical correction of CHD was performed in 34 patients. Craniofacial dysmorphisms were detected in 41 patients: elongated face (60%) and/or elongated nose (53.3%), narrow palpebral fissure (50%), dysplastic, overfolded ears (48.3%), thin lips (41.6%), elongated fingers (38.3%) and short stature (36.6%). Hypocalcemia was detected in 64.2% and decreased parathyroid hormone (PTH) level in 25.9%. Decrease in total lymphocytes, CD4 and CD8 counts were present in 40%, 53.3% and 33.3%, respectively. Hypogammaglobulinemia was detected in one patient and decreased concentrations of immunoglobulin M (IgM) in two other patients. Conclusion: Suspicion for 22q11.2DS should be raised in all patients with CHD associated with hypocalcemia and/or facial dysmorphisms, considering that many of these changes may evolve with age. The 22q11.2 microdeletion should be confirmed by molecular testing in all patients.