Scimitar syndrome is a rare congenital heart disease. It is divided into subgroups of infantile, adult, and multiple cardiac and extracardiac malformation. Most patients are diagnosed during infancy and occasionally in adolescence, but very few patients are older than 20 years of age, and only some cases have severe symptoms that require surgical correction. We report a case of a man 54 years o...

Background: Extracardiac malformations can be seen in 20-45% of infants with congenital heart disease (CHD). Chromosomal abnormalities exist in 5-10% of patients with CHD. The aim of this study was to assess the frequency of overt extra cardiac malformations in children with CHD. Methods: This descriptive epidemiologic study was conducted on 720 patients with CHD referred to the pediatric ca...

Ectopia cordis (EC) is a rare malformation due to failure of maturation of the midline mesodermal components of the chest and abdomen. It can be defined as a complete or partial displacement of the heart outside the thoracic cavity. It comprises 0.1% of congenital heart diseases. Common cardiac anomalies associated with EC are ventricular septal defect, atrial septal defect, and tetralogy of Fa...

Ectopia cordis is a rare congenital malformation in which the heart is located partially or totally outside the thoracic cavity. The estimated prevalence of ectopia cordis is 5.5-7.9 per million births and it comprises 0.1% of congenital heart diseases. Ectopia cordis is associated with other congenital heart diseases and various tissue and organ disorders. Common cardiac anomalies associated w...

The aim of this study was to evaluate the clincal value of multi-slice spiral computed tomography (MSCT) in the diagnosis of double aortic arch (DAA) and analyze the associated angiography and 3D reconstruction imaging to increase the accuracy of DAA diagnosis. Clinical and imaging data from 15 patients diagnosed with DAA by MSCT were summarized and compared with the corresponding surgical obse...

Malposition of the branch pulmonary arteries is a rare malformation with two forms. In the typical form, pulmonary arteries cross each other as they proceed to their respective lungs. The “lesser form” is characterised by the left pulmonary artery ostium lying directly superior to the ostium of the right pulmonary artery, without crossing of the branch pulmonary arteries. Malposition of the bra...

OBJECTIVE To analyse patient data to elucidate the apparent association between an abnormal karyotype and tricuspid regurgitation found during fetal echocardiography at early gestations. SETTING Tertiary referral centre for fetal medicine and cardiology. METHODS Fetuses between 11 and 14 weeks' gestation were selected for detailed echocardiography. Referral reasons were increased nuchal tra...

STUDY OBJECTIVE The aim was to make an epidemiological study of major congenital heart defects and to make comparisons between groups of heart defects regarding different epidemiological variables. DESIGN The cases with congenital heart defects were identified from two Swedish registries: the Registry of Congenital Malformations and the Child Cardiology Registry. A classification with nine gr...

OBJECTIVES To assess the proportion and pattern of extracardiac birth defects in children with congenital heart defects referred to a tertiary care institute. METHODS Cross-sectional observational study from January 2010 to June 2011. RESULTS Out of 560 children with congenital heart defects, 98 (17.5%) had extracardiac birth defects. Fifty-six had multiple congenital defects; 36 were syndr...

OBJECTIVE To review the association between congenital heart defects and extracardiac malformations. DATA SOURCES Scientific articles were searched in the Medline, Lilacs, and SciELO databases, using the descriptors "congenital heart disease," "congenital heart defects," "congenital cardiac malformations," "extracardiac defects," and "extracardiac malformations." All case series that specific...