TGA like physiology

TGA like physiology – cyanotic heart disease with high pulmonary blood flow

Following congenital heart diseases have cyanosis with high pulmonary blood flood flow (TGA like physiology or transposition like physiology):

1. Dextro transposition of great arteries (D- TGA) with ventricular septal defect (VSD) and no pulmonary stenosis (PS)
2. Double outlet right ventricle (DORV) with VSD and no PS
3. Tricuspid atresia with VSD and no PS

TGA like physiologies can present with cyanosis and heart failure in very early life due to increased pulmonary blood flow as there is no pulmonary stenosis to restrict the pulmonary blood flow. D-TGA with VSD and no PS is a condition which can cause cyanosis on first day of life. This is in contrast to Tetralogy where the onset of cyanosis is delayed. Some of these patients may need pulmonary artery banding to reduce pulmonary blood flow if immediate surgical correction is not feasible. In case of D-TGA early surgical correction with arterial switch operation (Jatene operation) is ideal. TGA physiologies do not have hypercyanotic spells (cyanotic spells) and they do not give history of squatting which occurs in later life with TOF (tetralogy of Fallot) like physiologies.

Atrial switch operations were described for TGA prior to arterial switch. First was Senning operation using atrial flaps in 1957 [1]. Mustard operation using pericardium was described in 1963 [2]. First successful arterial switch had to wait till 1975.

References

1. Senning A. Surgical correction of transposition of the great vessels. Surgery. 1959 Jun;45(6):966-80.
2. Marathe SP, Talwar S. Surgery for transposition of great arteries: A historical perspective. Ann Pediatr Cardiol. 2015 May-Aug;8(2):122-8.