Restrictive Dermopathy Associated With Transposition of the Great Arteries and Microcolon

Restrictive dermopathy associated with transposition of the great arteries and microcolon: a rare neonatal entity with new symptoms.

BACKGROUND Restrictive dermopathy is a very rare autosomal recessive skin disorder. The typical pathologic findings are striking: microstomia, micrognathia, thin but very tight translucent skin that tears spontaneously, and arthrogryposis multiplex. The mechanisms behind this disease are unknown. OBSERVATIONS We describe for the first time a newborn girl with restrictive dermopathy, transposi...

A 33-day-old Infant with the Transposition of the Great Arteries; A Rare Case Report

Dextro-transposition of the great arteries (d-TGA) is the one most common cyanotic congenital heart disease in neonates. The discordant ventriculoarterial arrangement results in parallel circulation, it so is vital to understand the management. We report a rare interesting but critical case of 33-day-old boy who developed cyanosis and had transposition of great arteries combined with interrupte...

Transposition of the great arteries

Transposition of the great arteries (TGA), also referred to as complete transposition, is a congenital cardiac malformation characterised by atrioventricular concordance and ventriculoarterial (VA) discordance. The incidence is estimated at 1 in 3,500-5,000 live births, with a male-to-female ratio 1.5 to 3.2:1. In 50% of cases, the VA discordance is an isolated finding. In 10% of cases, TGA is ...

Transposition of the great arteries.

Many patients with ventriculoarterial discordance have survived to adulthood. Those with complete transposition of the great arteries have often had an atrial switch procedure (Mustard or Senning operation) performed, which leaves the morphological right ventricle (RV) supporting the systemic circulation. RV failure and tricuspid regurgitation are common. Some patients may ultimately require ca...

Transposition of the great arteries.