OBJECTIVE It has not been clear that Voluven (6% hydroxyethyl starch 130/0.4) may be administered in pediatric patients safely. The purpose of this study was to determine if Voluven could be used for blood volume expansion and hypovolemia prevention in pediatric patients with congenital heart disease. PATIENTS AND METHODS 50 pediatric patients with congenital heart disease were recruited in t...

V entricular septal defect (VSD) is defined as a structural abnormality of the heart; defect in any portion of the ventricular septum with blood flow at the level of ventricles through this defect. It accounts 25% of congenital heart disease; 1 and remains one of the major causes of morbidity. 2 Ventricular septal defect is the most common lesion of congenital heart disease worldwide, 3 with a ...

BACKGROUND Service experiences for adults with congenital heart disease have not been reported in our country. OBJECTIVE To describe the basic clinical profile of adults with congenital heart disease in an outpatient tertiary care center. METHODS We compiled data on age, gender, place of residence, primary diagnosis, and secondary diagnoses of 413 patients treated for seven years. RESULTS...

OBJECTIVE Noonan syndrome is a clinically homogeneous but genetically heterogeneous condition. Type 1 Noonan syndrome is defined by the presence of a mutation in the PTPN11 gene, which is found in approximately 40% of the cases. Phenotype descriptions and cardiac defects from cohorts with Noonan syndrome were delineated in the "pregenomic era." We report the heart defects and links to gene dysf...

conclusions opium has teratogenic effect on cardiovascular system. results from 225 children under study 23.5% had addicted parents based on the variables of the study but the rate was only 2.3 for the control group. the difference between these two groups was significant and the most common form of heart disease was congenital ventricular septal defect. background opium abuse is one of the wid...

Asymmetric septal hypertrophy, or ASH, is a genetically determined myocardial disorder that is transmitted as an autosomal dominant trait. ASH is characterized by a disproportionately thickened ventricular septum that contains numerous hypertrophied, bizarrely-shaped and disorganized cardiac muscle cells. Disproportionate hypertrophy of the ventricular septum has also been observed in associati...

BACKGROUND About half of people with Down syndrome (DS) exhibit some form of congenital heart disease (CHD); however, trisomy for human chromosome 21 (Hsa21) alone is insufficient to cause CHD, as half of all people with DS have a normal heart, suggesting that genetic modifiers interact with dosage-sensitive gene(s) on Hsa21 to result in CHD. We hypothesize that a threshold exists in both DS an...

This report describes a family with frequent recurrence of congenital heart disease in multiple generations. Eight members had atrial septal defect (ASD) of the fossa ovalis type and seven members had other forms of congenital heart disease. One branch of the pedigree showed a predominance of ASD with prolonged atrioventricular (A-V) conduction and initially suggested an autosomal dominant gene...

Thank you for your Letter to the Editor [1] regarding our report ‘Tricuspid valve repair in a case with congenital absence of left thoracic pericardium’. We appreciate your comments and agree that congenital elements in partial and complete absence of pericardum must be considered. Associated anomalies including mitral stenosis, atrial septal defect, patent ductus arteriosus and tetralogy of Fa...

Congenital heart disease (CHD) is one of the risk factors for developing infective endocarditis (IE). Right-sided IE occurs in 5%–10% cases, and pulmonary valve (PV) involved less than 2% such patients. Literature data are few, optimal treatment methods, indications surgery, types operative techniques still under debate. We present an adult patient with a rare combination ventricular septal def...