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...

Hypoplastic left heart syndrome is a relatively common cardiac malformation, accounting for 4-9% of children born with congenital heart disease. Since 1981 the mainstay of treatment has been the Norwood series of operations which have been variously modified, most recently using an RV-PA conduit (Sano shunt). With these surgical modifications and with increased experience in perioperative care ...

When most of the centers in India do not entertain the patients with hypoplastic left heart syndrome, the original authors of the article in the present issue should be congratulated for the attempt of treating this patient with hybrid procedure. It’s a spectacular beginning. The development of staged reconstructive surgery for hypoplastic left heart syndrome (HLHS) by Norwood and colleagues in...

A total of 92 participants, 50 younger (mean age 26.3 years) and 42 older (mean age 63.8 years), were tested for visual-field asymmetries. On a word-matching task, a right-visual-field (RVF) advantage increased with age, consistent with the theory that right-hemispheric function shows relatively greater decline with age than left-hemispheric function. On a figural-comparison task, a left-visual...

Left ventricular hypoplasia complicates other forms of congenital heart disease in addition to the classic ‘‘hypoplastic left heart syndrome.’’ Within this heterogeneous group, subtle anatomic differences determine surgical management and ultimate prognosis. Thus, an individualized approach is necessary to optimize outcomes in this complex population.