Análisis demográfico de una clínica de cardiopatías congénitas del Instituto Mexicano del Seguro Social, con interés en el adulto / Demographic analysis of a congenital heart disease clinic of the Mexican Institute of Social Security, with special interest in the adult

Resumen Introducción: Las cardiopatías congénitas (CC) en México se presentan con una cifra estimada entre 18,000-20,000 nuevos casos por año. La mayor parte de los centros que atienden a estos enfermos son exclusivamente pediátricos y el Instituto Mexicano del Seguro Social (IMSS) cuenta con una clínica que atiende a todas las edades. Objetivo: Analizar los aspectos demográficos de una clínica de CC del niño y del adulto del IMSS. Métodos: De 2011 a 2016 se realizó un estudio transversal de la clínica de CC de un hospital de tercer nivel del IMSS, incluyendo todos los pacientes con cardiopatía estructural confirmada de reciente diagnóstico. Se registró el sexo, la edad, la entidad federativa de referencia, el antecedente de embarazo y el tratamiento. Se dividió a la población en rangos de edades (< 2 años, 2.1-6 años, 6.1-10 años, 10.1-17 años y > 17.1 años). Se empleó estadística descriptiva y prueba de y2 en las variables cualitativas. Resultados: Tres mil cuatrocientos ochenta y tres enfermos con CC (relación hombre:mujer,0.8:1.2), las cardiopatías acianógenas de flujo pulmonar aumentado son el grupo más grande (47.2%); 25.6% fueron adultos y 35% de las mujeres con antecedente de al menos una gesta. En general la cardiopatía más frecuente fue la comunicación interauricular. La entidad federativa con mayor frecuencia de adultos de reciente diagnóstico fue Chiapas (33.82%); el 7% no fueron candidatos a ningún tratamiento por complicaciones inherentes a la cardiopatía. Conclusiones: Existe un diagnóstico tardío de la atención de las CC en la etapa adulta. Es necesario crear un registro nacional para promover nuevas políticas de salud y distribución de recursos destinados a estos pacientes.

Abstract Introduction: Congenital heart disease (CHD) has an incidence of 8-10 cases per 1000 live births. In Mexico, there are 18,000-20,000 new cases per year. Most tertiary care centers for CHD attend only pediatric population; the Mexican Institute of Social Security (IMSS) has a clinic that attends pediatric and adult population. Objective: To analyze the demographic aspects of the CHD clinic of IMSS. Methods: From 2011 to 2016 a cross-sectional study of the CC clinic of a third level hospital of the IMSS, including all patients with confirmed structural heart disease of recent diagnosis was carried out. The sex, age, reference entity, antecedent of pregnancy and treatment were registered. The population was divided into age ranges (< 2 years, 2.1-6 years, 6.1-10 years, 10.1-17 years and > 17.1 years). Descriptive statistics and y2 test were used in qualitative variables. Results: 3,483 patients with CHD (male:female ratio, 0.8:1.2) were included. Increased pulmonary flow acyanogenic cardiopathies were the most frequent CHD group (47.2%), with atrial septal defect being the most frequent diagnosis overall; 25.6% were adults and 35% of women with a history of pregnancy. Chiapas was Federal entity with greater proportion of patients diagnosed in the adult stage (33.82%); 7% were not candidates for any treatment for complications of the disease. Conclusions: CHD is a late diagnosis; it is necessary to create a national register to promote new health policies and a rational distribution of resources for these patients.

Prevalence of Congenital Heart Defects Associated with Down Syndrome in Korea

Congenital heart defect (CHD) is common in infants with Down syndrome (DS), which is the principle cause of mortality. However, there is no data available for the frequency and types of CHD in infants with DS in Korea. We investigated the frequency of CHD in infants with DS in Korea. After the survey on birth defects was conducted throughout the country, the prevalence of CHD in DS in 2005-2006 was calculated. This study was conducted based on the medical insurance claims database of the National Health Insurance Corporation. The number of total births in Korea was 888,263 in 2005-2006; of them, 25,975 cases of birth defects were identified. The prevalence of DS was 4.4 per 10,000 total births, accounting for 1.5% of all birth defects. Of the 394 infants with DS, 224 (56.9%) had a CHD. Atrial septal defect was the most common defect accounting for 30.5% of DS followed by ventricular septal defect (19.3%), patent duct arteriosus (17.5%), and atrioventricular septal defect (9.4%). Our study will be helpful to demonstrate the current status of DS and to identify the distribution of CHD in infants with DS in Korea.

Congenital heart disease in children with Down syndrome in Duhok city

To find out the most common types of congenital heart defects among Down syndrome children in Duhok/Kurdistan and the effects of parental consanguinity on the pattern of congenital heart defects. Down syndrome is the most common chromosomal abnormality due to a trisomy of chromosome number 21, commonly associated with congenital heart defects. A study was done in Heevi Pediatric Teaching Hospital and Early Detection Childhood Disability Center in Duhok city, from the period of January 2010 to January 2011. Congenital heart defects were detected in 44/80 [55%] children. Ventricular Septal Defect was the most common 18/44, followed by atrioventricular septal defect 12/44, atrial septal sefect 7/44, Tetralogy of Fallot 4/44, patent ductus arteriosus, hypetrophic obstructive cardiomyopathy 2/44. Consanguinity was found in 22 [27%]of all parents. Slightly higher frequency of congenital heart defects was documented in Down syndrome children from the population of Duhok city with a high consanguineous marriage rate. The frequencies of specific lesions were differing to those reported internationally; ventricular septal defect was the most frequently detected in this study

Prevalence of Birth Defects in Korean Livebirths, 2005-2006

We investigated the livebirths prevalence and occurrence pattern of birth defects in Korea. After the survey on birth defects was done in 2,348 medical institutions around the nation, the birth defect prevalence of livebirths in 2005-2006 was calculated. This study was based on the medical insurance claims database of the National Health Insurance Corporation. The number of livebirths in Korea was 883,184 from 2005-2006, and 25,335 cases of birth defects were notified to our study, equivalent to a prevalence of 286.9 per 10,000 livebirths. Anomalies of the circulatory system were the most common defects, accounting for 43.4% of birth defects with a prevalence of 124.5 per 10,000 livebirths. It was followed by the musculoskeletal system anomalies, the digestive system anomalies, and the urinary system anomalies. The five major birth defects based on the ranking of prevalence were atrial septal defect, ventricular septal defect, hydronephrosis, patent ductus arteriosus, and cleft lip/palate. Birth defects in livebirths were associated with a high proportion of low birthweight, prematurity, multiple births and advanced maternal age. The prevalence of birth defects in Korea is similar to or lower than those reported in developed countries. Our study suggests baseline data to explain the current status of birth defects and to establish a registry system of birth defects in Korea.

Análisis de microdeleciones en 22q11 en pacientes colombianos con cardiopatía congénita no sindrómica / Analysis of microdeletions in 22q11 in Colombian patients with congenital heart disease

Los defectos cardiacos conforman las malformaciones congénitas más frecuentes, con una incidencia que se ha estimado entre 4 y 12 por 1000 en recién nacidos vivos. Estos tienen una etiología multifactorial en la que convergen la predisposición genética y los factores ambientales. A partir de 1990 se ha relacionado este tipo de patologías con microdelección 22q11. Se determinó la frecuencia de la microdeleción 22q11 en pacientes con cardiopatía congénita no sindrómica. Se analizaron 61 pacientes con cardiopatía congénita, a partir de ADN de sangre periférica y posterior amplificación, mediante PCR multiplex del gen TUPLE1 y del STR D10S2198, visualización electroforesis en geles de agarosa y análisis densitométrico para determinar dosis génica. Se encontraron 3 pacientes con microdeleción 22q11, para una frecuencia de 4,9%. Esta microdeleción se asoció en dos de los casos a Tetralogía de Fallot y en el otro a Defecto Septal Atrial (DSA). En conclusión, la frecuencia de microdeleción 22q11 en la población analizada es de 4,9%. Dentro de los casos de Tetralogía de Fallot, la microdeleción estaba presente en el 7,4% y en los DSA corresponde al 11,1%.

Cardiac defects are the most frequent congenital malformations, with an incidence estimated between 4 and 12 per 1000 newborns. Their etiology is multifactorial and might be attributed to genetic predispositions and environmental factors. Since 1990 these types of pathologies have been associated with 22q11 microdeletion. In this study, the frequency of microdeletion 22q11 was determined in 61 patients with non-syndromic congenital heart disease. DNA was extracted from peripheral blood and TUPLE1 and STR D10S2198 genes were amplified by multiplex PCR and visualized in agarose gels. Gene content was quantified by densitometry. Three patients were found with microdeletion 22q11, representing a 4.9% frequency. This microdeletion was associated with two cases of Tetralogy of Fallot and a third case with atrial septal defect (ASD). In conclusion, the frequency for microdeletion 22q11 in the population analyzed was 4.9%. The cases that presented Teratology of Fallot had a frequency for this microdeletion of 7.4% and for ASD of 11.1%.

Prevalence of congenital heart disease, Kanpur, India.

Our aim was to study the prevalence, age-wise distribution, and clinical spectrum of congenital heart disease (CHD) at a multi-speciality corporate hospital in North India. A retrospective analysis of records of 10,641 patients over a five-and-a-half year period was done. Clinical examination, echocardiography and color doppler were used as diagnostic tools. A prevalence of 26.4 per 1000 patients was observed. VSD (ventricular septal defect) was the commonest lesion (21.3%), followed by ASD (atrial septal defect) in 18.9% and PDA (patent ductus arteriosus) in 14. 6%. Tetralogy of Fallot was the commonest cyanotic heart disease (4.6%). Maximum number of children with heart disease (82.9%) were diagnosed between 0-3 years of age.

Congenital heart disease in Nepalese children.

The present status of congenital heart disease (CHD) from the persepective of the children's health in Nepal is not yeat available. Cross-sectional analysis of all children with heart disease attending Kanti Children Hospital was performed. VSD, ASD, obstructive valvular lesions and cyanotic heart diseases were the most common CHDs. Present data indicate the need for increased levels of cardiac care in children, correctional surgeries, monitoring of the patients and awareness among caregivers of CHD patients.

Congenital heart disease in Saudi Arabia: current epidemiology and future projections

To provide an overview of the epidemiology of congenital heart disease, the results of epidemiological studies done in 4 regions of Saudi Arabia [August 1988-February 2000] and 2604 individuals with congenital heart disease were evaluated. Ventricular septal defect was the commonest lesion [33.9%] followed by atrial septal defect [18.1%]. Overall, sex distribution was similar; for 3 conditions, more males than females were affected. Of 2269 [59%] presenting in the first year of life, 566 [24.9%] had neonatal congenital heart disease. Down syndrome was the commonest cause. Distribution of specific lesions and sex distribution was similar to findings from other parts of the world; however, the overall detection rate at 1 year of age was lower

Patent foramen ovale in a cohort of young patients with cryptogenic ischemic stroke

OBJETIVO: A associação das anormalidades do septo interatrial - forame oval patente (FOP) e aneurisma de septo interatrial (ASA) - com acidente vascular cerebral isquêmico (AVCI) ainda é questão de incerteza para muitos autores. No entanto, vários estudo mostram que em pacientes jovens tais anormalidades podem estar relacionadas à gênese de eventos isquêmicos. Nosso objetivo é descrever a prevalência do FOP e ASA em uma coorte de pacientes jovens (< 55 anos) com AVCI e acompanhar estes pacientes após fechamento cirúrgico ou por prótese endovascular. MÉTODO: Em 21 meses de estudo, identificamos todos os pacientes com menos de 55 anos de idade admitidos em nosso hospital por AVCI. Consideramos AVCI como criptogenético quando não havia uma causa provável para AVCI. Todos os pacientes foram submetidos ao ecocardiograma transesofágico. O fechamento por prótese endovascular foi o procedimento oferecido àqueles com alguma anormalidade do septo interatrial. Os pacientes foram acompanhados mensalmente e submetidos a antiagregação plaquetária com AAS ou clopidogrel. RESULTADOS: Foram admitidos 32 pacientes jovens com AVCI. Após ampla investigação, 29 receberam o diagnóstico de AVCI criptogênico. Destes, 12 (12/29 - 41,3%) apresentaram alguma anormalidade do septo interatrial; sendo que em 7 havia a associação de FOP e ASA. Dez pacientes foram submetidos a fechamento endovascular percutâneo e 2 foram submetidos a fechamento cirúrgico. Até o momento, nenhum paciente relatou recorrência do evento isquêmico e 2 pacientes relataram melhora das crises de enxaqueca (14 meses de seguimento). CONCLUSÃO: Nossa pequena série de casos está de acordo com outros estudos e sugere uma possível relação entre anormalidades do septo interatrial e AVCI em pacientes jovens. Maiores estudos são necessários para comprovar esta associação e para definir a melhor conduta terapêutica.

Pattern of congenital heart disease in Northern Jordan

To study the pattern of congenital heart diseases in Northern Jordan. Design: A prospective study of 170 children with congenital heart disease. Setting: Princess Rahmah Teaching Children Hospital. All cases of congenital heart diseases in Northern Jordan over two years period starting January 1994. Result: A single heart defect was seen in 90% of cases with Ventricular Septal Defect [VSD] being the commonest seen in 44% followed by Atrial Septal Defect [ASD] in 13.5%. Of the 10% with combined or complex heart defects, a combined VSD and ASD were the commonest seen in six paients [35%]. The overall male to female sex ratio are 1.1:1.0. There is identical sex distribution for VSD. Male predominance is seen in pulmonary stenosis, aortic stenosis, atrioventricular canal defects and fallot tetralogy. Female predominance is seen in ASD, Patent Ductus Arteriosus and Transposition of Great Arteries. Half of the patients with congenital heart diseases are diagnosed by three months and three quarter by the age of six months. This is the first study on congenital heart diseases in Northern Jordan that shows a VSD being the commonest, corrobaratim previous reports but have an unexplained higher incidence of ASD

Análise dos fatores de risco na correçäo cirúrgica do defeito septal atrioventricular de forma total / Risk factors analysis in the surgical repair of complete atrioventricular septal defect

Pacientes com defeito septal atrioventricular de forma total (DSAVT) frequentemente apresentam insuficiência cardíaca intratável e hipertensäo arterial pulmonar nos primeiros meses de vida, e apenas uma minoria sobrevive sem tratamento cirúrgico precoce. Por essa razäo, indica-se a correçäo definitiva para alterar favoravelmente a história natural da doença. Entretanto, vários fatores säo responsáveis pela alta mortalidade cirúrgica. O presente trabalho estuda a experiência na correçäo cirúrgica do DSAVT com o objetivo de identificar alguns fatores de risco estatisticamente significativos para a ocorrência de morte operatória. Analisaram-se, retrospectivamente, 52 pacientes submetidos, entre janeiro de 1974 e dezembro de 1990, a cirurgia definitiva para correçäo de DSAVT no Royal Brompton and National Heart and Lung Institute, sendo estudadas as seguintes variáveis: idade, peso, sexo, ano de operaçäo, presença de síndrome de Down, grau de regurgitaçäo da valva AV, bandagem prévia do tronco pulmonar, presença de anomalias associadas, pressäo sistólica pulmonar, duplo orifício mitral, classificaçäo do defeito segundo Rastelli, emprego de parada circulatória e técnica de correçäo (1 x 2 retalhos). Todos os fatores foram avaliados isoladamente, mediante a análise univariada. Para determinar quais os fatores que, independentemente da açäo de outros, contribuíram significativamente para maior mortalidade cirúrgica, foi utilizada a análise multivariada com regressäo logística. A análise multivariada demonstrou que o baixo peso na época da operaçäo e a técnica de correçäo com um retalho aumentam significativamente a mortalidade cirúrgica.