A pair of monozygotic twin girls is reported with concordance for 3 congenital cardiac abnormalities: (1) secundum atrial septal defect, (2) aneurysm of the membraneous ventricular septum, and (3) electrocardiographic frontal plane left axis deviation. A review of the published materials shows a 9.5 per cent incidence of concordance for congenital heart disease among monozygotic twins. In those...

Congenital abnormalities of coronary arteries may predispose to life-threatening sudden cardiac events. We present a case of aborted sudden cardiac death in a patient who was diagnosed as having single coronary artery originating from right coronary sinus. The vessell divided into critically stenosed left main trunk and significantly narrowed right coronary artery. The patient was successfully ...

Left ventricular non-compaction (LVNC) is a rare congenital cardiomyopathy characterized by the presence of an extremely thickened endocardial layer with prominent trabeculations and deep recesses in communication with ventricular chamber and determining a typical spongeous aspect [1]. Noncompaction of the ventricular myocardium is thought to be a congenital arrest of endomyocardial embryogenes...

some syndromes that are characterized by abnormalities of the skull, facial bones, and mandibule, most of these patients are from the pediatric population. for the anaesthetic management of patients with various craniofacial dysostosis are as follows: 1) the necessary for careful evaluation of the airway by simply observing the patient. 2) evaluation of the patient for abnormalities of the hear...

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...

Background and Objective: Congenital diaphragmatic hernia (CDH) is an anomaly of the cardiopulmonary system maturation process that results from both a global embryopathy concomitant mechanical compression abdominal contents during fetal maturation. This in pulmonary hypertension, hypoplasia, cardiac dysfunction, requiring intense critical care management. The patients with highest risk CDH are...

BACKGROUND In Brazil, since 2001, the congenital abnormalities have represented the second cause of death in children younger than one year, with cardiovascular defects being responsible for 39.4% of these deaths. OBJECTIVES To establish the prevalence and the characteristics of the congenital cardiac malformations in pediatric necropsies performed in Hospital Regional da Asa Sul, Brasília, D...

Pulmonary agenesis is a very rare condition and many of them are associated with a variety of cardiac and non-cardiac malformations. We report an eight-month old girl with chronic lung infection due to right sided pulmonary agenesis without any associated major cardiac or non-cardiac abnormalities. The case brings in forth the importance of investigating any infant presenting with features of c...

Mutations in cardiac Na(+) and K(+) channels can disrupt the precise balance of ionic currents that underlies normal cardiac excitation and relaxation. Disruption of this equilibrium can result in arrhythmogenic phenotypes leading to syncope, seizures, and sudden cardiac death. Congenital defects result in an unpredictable expression of phenotypes with variable penetrance, even within single fa...

Background Cardiac MRI (CMRI) is considered the “gold” standard for non-invasive characterization of cardiac function and viability as it allows the assessment of regional and global cardiac function, mass, volumes, myocardial perfusion, and tissue characterization. With its excellent soft-tissue differentiation, CMRI is the most important imaging modality for differentiating tumour from thromb...