Since the first cardiac transplant operation in Cape Town in 1967 the annual total number of cardiac transplants worldwide has increased, except over the last few years when a plateau has been noted. Despite thJs, the numbers of cases involving young patients have continued to rise, mainly owing to an increase in the numbers in the younger age groups and tbe fact tbat cardiac transplantation is now also perionned In patients with complex congenital cardiac abnormalities where the resuJts of conventional surgical procedures are unsatisfactory. That this does not seem to be the situation at Groote Schuur Hospital, Cape Town, prompted a review of our results In patients aged 20 years and younger. There was no operative mortality. The actuarial survival of patients at 1 year was 9Z,8% and at 5 years 72,4%. The actuarial graft survival for this group of patients was 94% at 1 year and 68,8% at 5 years. Analysing patients accepted for transplantation in the period between 1988 and 1990, it was found that all patients aged 30 years or younger underwent operation. A simuJtaneous review of potential donors referred to Groote Schuur Hospital indicated that there is a sufficient donor-organ supply for young patients in this country.
The surgical repair of an 8 kg infant with inllinonary annular hypoplasia and a diffuse stenosis of the main left and right pulmonary arttries (Land RPAs) from their origin to their bifurcations is reported. The condition occurred as aD isolated congenital heart defect. The preoperative RV peak systolic pressure was 218 mmHg and tbe RV/LV ratio exceeded 2,3. Seven months after surgery this RV/LV ratio bad improved to 0,50 by ecbocardiography. A two-patch technique was utilised in tbe reconstruction, and the aorta was tnmsected to improve exposure.
Progressive familial heart block (PFHB) is an autosomal dominant inherited disorder that is usually asymptomatic. Affected members of such families are diagoosed on any of the foUowing electrocardiographic features: sinus bradycardia (SB), right bundle-branch block (RBBB), left anterior or left posterior hemlblocks, second-degree atrioventricular (A V) blocks aod complete beart block with broad or narrow QRS complexes. The aim of this study was to evaluate the effort test as a diagnostic aid to identity PFHB family members at risk. Seventy-nine pFHB members were subjected to an effort test (38 witb abnormal electrocardiograms (ReGs) and 41 with normal ECGs at rest). The commonest finding was the Inability of a significant number of members with abnormal ECGs to reach their target heart rate (X"" P < 0,05). Other ECG changes were also commoner in this group (Xl, p <0,0(5).