22759              CONGENITAL TRANSPOSITION OF THE GREAT VESSELS

The patient was a boy aged 2 who suffered from congenital transposition of the great vessels.  At age 3 weeks a palliative atrial septal defect was made.  Thereafter his development was only a little retarded and he was otherwise well.  His last admission was for elective reconstruction.  Catheter and angiography studies 3 months previously had shown complete transposition, mild pulmonary stenosis, a small ventricular septal defect and an atrial septal defect.  At operation Mustard’s procedure was performed in which a pericardial patch is sewn into the atria to form a baffle directing blood into the appropriate vessels, thus producing haemodynamic correction although leaving the transposition unchanged.  Post-operatively there was massive pulmonary oedema and he died 36 hours later from ventricular fibrillation.

The specimen shows the aorta (with attached arch and great vessels) arising from a greatly hypertrophied right ventricle.  The pulmonary artery arises from the left ventricle.  There is a large defect in the upper part of the interventricular septum.  The results of surgery may be seen sewn into the common chamber thus formed, running between the orifices of the vena cava and bridging over the mitral valve(ie has been cut open at autopsy).  This serves to divert systemic venous blood enters the atria through the pulmonary veins (the orifices of which can be seen) flows over the patch, through the tricuspid valve to the right ventricle and thence to the systemic circulation.  There is also a pericarditis as a result of surgery.