RATIONALE Brugada syndrome (BrS) is a cardiac ion channel disease that is caused by an autosomal dominant genetic abnormality. A ventricular septal defect is a common congenital heart disease, in which genetic defects play a significant role. PATIENT CONCERNS We report an extremely rare case of a 42-year-old male with congenital heart disease, who suffered recurrent syncope and gastrointestin...

Holt-Oram syndrome is a rare inherited disorder involving the hands, arms, and the heart. The defects involve carpal bones of the wrist and the thumb and the associated cardiac anomalies like atrial or ventricular septal defects. Congenital cardiac and upper-limb malformations frequently occur together and are classified as heart-hand syndromes. The most common amongst the heart-hand disorders ...

An analysis of 555 consecutive cases of congenital heart disease in Ceylon has shown that for the whole series interatrial septal defect is the commonest cardiac lesion; next is interventricular septal defect, followed by persistent ductus arteriosus. Interventricular and interatrial septal defects are equally common among all the patients below 16 years of age, but interventricular septal defe...

AIMS Pulmonary hypertension (PH) in asymptomatic patients is a rare cause of sudden death. This study aims to determine the incidence of this entity and raise awareness among pathologists. METHODS We retrospectively investigated 44 cases of sudden unexpected death in relation to PH in patients not on antihypertensive therapy. This is the largest pathological study reported. RESULTS We repor...

The haemodynamic effects and clinical manifestations of congenital heart lesions may be greatly influenced by developmental changes in the circulation. The sequential changes associated with abnormal communications between the systemic and pulmonary circulations are well typified by the effect on ventricular septal defect. The possible influences of ventricular septal defect on the foetal circu...

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 echocardiograp...

The affinity of some chromosomal anomalies for certain types of congenital heart disease is well known, e.g. atrioventricular defects in Down's syndrome and coarctation of the aorta in Turner's syndrome. Many reports on several types of congenital heart disease in close or more distant family relationships have aroused suspicion that genetic factors may be important also in other patients. Repo...

background: pulmonary arterial hypertension is a complication of most congenital heart diseases. we sought to assess the effect of sildenafil on patients suffering from pulmonary arterial hypertension in association with congenital heart disease on the basis of clinical and echocardiographic parameters and compare the catheterization and treatment results so as to evaluate the predictive value ...

Background: Cardiac conduction disorders are rare syndromes in neonates and children. According to the literature atrial septal defect, especially ostium secundum type, is associated with atrioventricular (AV) block. Wenckebach conduction heart block is very rare in a neonate; however, there is a dearth of research on the prevalence of this type of heart block regarding the neonates and pediatr...

Ventricular septal defects are the most common congenital heart defect. They vary greatly in location, clinical presentation, associated lesions, and natural history. The present article describes the clinical aspects of ventricular septal defects and current management strategies.