Describe tranposition of great arteries with intact ventricular septum?
Transposition of great arteries with intact ventricular septum (or small VSD): There is usually inadequate mixing, which occurs at the level of a patent foramen ovale or a patent ductus arteriosus. (Less commonly, there may be an arties septal defect of adequate size).
Inadequate intercirculatory mixing results in early presentation in the immediate neonatal period with cyanosis, which may initially be mild but rapidly progresses in prominence. As the ductus arteriosus constricts, the intercirculatory mixing further decreases and there is onset of features of severe systemic hypoxemia and acidosis, within the first week of life. Most of these babies are good-weight babies, otherwise well-preserved and apart from the cyanosis physical examination findings may be unrewarding. S1 is normal and S2 may be single (due to antero-posterior relationship of the great arteries which pushed the pulmonary artery behind the aorta).There may an ejection systolic murmur usually soft, less than grade216 which may represent a functional murmur produced by increased flow across the left ventricular outflow, or a closing PDA.
Persistence of a large PDA may result in milder cyanosis and more proininent tachypnea, but classical signs of PDA like continuous murmur and bounding peripheral pulses may be absent in >SO per cent of these patients.
Prompt diagnosis and emergent management of transposition of great arteries intact ventricular septum is of critical importance as TGAIIVS is a lethal combination of defects.