OBJECTIVE One-and-a-half ventricle repair is a surgical option for complex cardiac anomalies characterized by right-ventricle hypoplasia or dysfunction. The long-term result analyses or large clinical reviews are rare. The aim of this study is to evaluate the long-term functional results of this surgical procedure. METHODS The 29 patients, who underwent one-and-a-half ventricle repair from Ju...

The surgical correction of the transposition of the great arteries (TGA) is divided into three procedures by the methods of switching i.e., (1) intra-atrial, (2) intra-ventricular, and (3) via the great arteries. Arterial correction is considered as an ideal technique physiologically as well as anatomically. With the above in mind, twenty formalin-preserved hearts of TGA were used to carry out ...

The lung was biopsied in 20 children with double inlet ventricle and pulmonary hypertension aged 2 months to 14 years. Eleven patients had two patent atrioventricular valves, three atresia of the right valve, and six hypoplasia of the left valve. Severe pulmonary arterial medial hypertrophy occurred in the nine children less than 1 year of age. The findings did not suggest a period of normality...

DESCRIPTION A 13-month-oldmale child born of a second degree consanguineous marriage presented with delayed milestones; notably, he was not able to pull to sit or stand and he was swaying from side to side when pulled to sit. He had bilateral post-axial polydactyly (figure 1). He never experienced any respiratory difficulty and there was no history of apnoeic episodes. The central nervous syste...

Correlative angiographic-anatomical studies in 19 cases of pulmonary atresia with intact ventricular septum showed the following relations between the angiographic appearance of the pulmonary valve and the morphology of the right ventricle. (1) Doming of the pulmonary valve was associated with a nearly normal-sized right ventricle and a wide infundibulum patent to the level of the pulmonary val...

  Cartilage hair hypoplasia (CHH), is a rare cause of metaphyseal chondrodysplasia and short stature. Other features included hair abnormality, immunodeficiency, anemia, gastrointestinal disorders (Hirschsprung disease, celiac, …) and increased risk of cancer. The disease is an autosomal recessive disorder and previously has not been reported in Iran. We report a 9-year-old boy diagnosed as car...

Correlative angiographic-anatomical studies in 19 cases of pulmonary atresia with infarct ventricular septum showed the following relations between the angiographic appearance of the pulmonary valve and the morphology of the right ventricle. (1) Doming of the pulmonary valve was associated with a nearly normal-sized right ventricle and a wide infundibulum patent to the level of the pulmonary va...

OBJECTIVE To assess the efficacy of arterial duct angioplasty in maintaining adequate ductal patency in neonates with critical pulmonary valve stenosis. PATIENTS Two neonates presenting with cyanosis due to critical pulmonary valve stenosis with severe right ventricular hypoplasia underwent percutaneous balloon dilatation of the pulmonary valve. Despite successful dilatation, both remained cy...