Introduction: Along with advances occurring in cardiopulmonary bypass (CPB) including improved anesthesia intubation and early surgical interventions, it has become possible to correct life-threatening congenital heart anatomic abnormalities in patients. Our study aimed to collect information from one of the most important centers of pediatric cardiac surgery in Iran since there is limited info...

Two sisters with Ehlers-Danlos syndrome, inherited as an autosomal recessive trait, and congenital heart disease are herein reported. One was a 20-year-old woman with Ehlers-Danlos syndrome and multiple aphthous stomatitis, bronchial asthma, an emphysematous lung, a ventricular septal defect and a bilateral inguinal hernia due to hyperextensibility and joint hypermobility. The other was a 17-ye...

introduction pulmonary vein stenosis includes narrowing of one or more pulmonary veins that may have congenital or acquired causes. congenital pulmonary vein stenosis is a rare condition and also a rare cause for pulmonary hypertension. since identifying the etiology of pulmonary hypertension plays a crucial role in medical and surgical planning, we decided to introduce a rare case of pulmonary...

OBJECTIVE To analyse the spectrum of congenital heart malformations, the frequency of extracardiac malformations, and the proportion of chromosome aberrations among fetuses sent for necropsy. MATERIAL Necropsies were performed on 815 fetuses-448 induced abortions (55%), 220 spontaneous abortions (27%), and 147 stillbirths (18%)-during a seven year period (1991-97) in the department of patholo...

the most frequent congenital heart defects in the neonatal period are ventricular septal defects. ventricular septal aneurysms can rarely develop from an interventricular septal (ivs) defect in adults. we describe a 47-year-old man with an aneurysm in the ivs growing towards the right ventricle, which was confirmed by cardiac computed tomographic angiography and was missed by echocardiography.

Aim. To identify dysmorphic features and cardiac, skeletal, and urogenital anomalies in patients with congenital hypothyroidism. Patients and Methods. Seventeen children with congenital primary hypothyroidism were recruited. Cause for congenital hypothyroidism was established using ultrasound of thyroid and (99m)Tc radionuclide thyroid scintigraphy. Malformations were identified by clinical exa...

holt-oram syndrome is an autosomal dominant disorder, characterised by skeletal abnormalities of the upper limb associated with congenital heart defect, mainly atrial and ventricular septal defects. skeletal defects exclusively affect the upper limbs in the preaxial radial ray distribution and are bilateral and asymmetrical. they range from clinodactyly, absent or digitalised thumb, hypoplastic...

OBJECTIVE Ambient hypoxia impairs the airway epithelial Na transport, which is crucial in lung edema reabsorption. Whether chronic systemic hypoxemia affects airway Na transport has remained largely unknown. We have therefore investigated whether chronic systemic hypoxemia in children with congenital heart defect affects airway epithelial Na transport, Na transporter-gene expression, and short-...