First two decades of paediatric heart transplantation in Sweden - outcome of listing and post-transplant results.

AIMS: To evaluate outcome in the first generation of children with end-stage heart disease to whom heart transplantation was available. METHODS: Retrospective review of all 135 Swedish children <18 years old listed for heart transplantation 1989-2009, followed to December 31, 2009, including 74 (55%) with cardiomyopathy and 61 (45%) with congenital heart disease; 34 (25%) were infants (<1 year). Cumulative risk of requiring heart transplantation was 1:17,300 (11 patients who improved were omitted from outcome analysis). RESULTS: Waiting-list mortality was 31% (44% in infants). Median waiting time in 82 transplanted patients was 57 days (0-585 days). Post-transplant follow-up time was median 5.9 years (0.03-20.1 years), and actuarial survival was 92% at 1 year, 82% at 5 years, 76% at 10 years and 58% at 15 years. Survival after listing was 64% at 1 year, 58% at 5 years, 52% at 10 years and 40% at 15 years. Post-transplant complications included rejections (34%), malignancies (12%), renal failure (8%), coronary artery vasculopathy (6%) and re-transplantation (5%). Among 64 survivors, 84% were free of complications affecting prognosis. CONCLUSION: High waiting-list mortality and post-transplant attrition precluded 60% of this pioneer population from reaching adulthood. Functional status in survivors is generally good.

Heart transplantation across the antibodies against HLA and ABO.

We have intentionally performed heart transplantation in a 5-year-old child, despite the most unfavourable risk factors for patient survival; the presence of high level of antibodies against donor's human leucocyte antigen (HLA) class I/II and blood group antigens. Pretransplant treatment by mycophenolate mofetil, prednisolone, tacrolimus, intravenous immunoglobulin, rituximab, protein-A immunoadsorption (IA) and plasma exchange reduced antibody titres against the donor's lymphocytes from 128 to 16 and against the donor's blood group antigen from 256 to 0. The patient was urgently transplanted with a heart from an ABO incompatible donor (A(1) to O). A standard triple-drug immunosuppressive protocol was used. No hyperacute rejection was seen. Antibodies against the donor's HLA antigens remained at a low level despite three acute rejections. Rising anti-A(1) blood group antibodies preceded the second rejection and were reduced by two blood group-specific IAs and remained at a low level. The patient is doing well despite the persistence of donor-reactive antibodies.

Serial signal-averaged electrocardiography in children after cardiac transplantation.

In patients with myocytolysis detected in endomyocardial biopsy, there is a tendency towards a shift of ventricular electrical axes from normal to strain pattern on surface ECG. Their 12-lead signal-averaged electrocardiogram (SAECG) show a significant increase in filtered QRS duration (QRSD) compared with those with specimens without myocytolysis. Late potentials were generally found more frequently in individual SAECG leads than in the vector magnitude. An increase in filtered QRSD and the presence of late potentials compared with a baseline study emphasizes the need for endomyocardial biopsy. Consequently, no change in these parameters may preclude the indication for endomyocardial biopsy.

Increased QT dispersion and loss of sinus rhythm as risk factors for late sudden death after Mustard or Senning procedures for transposition of the great arteries.

Increased QT dispersion and loss of sinus rhythm were both associated with sudden cardiac death in patients with transposition of the great arteries after Mustard and Senning operations at the early and late postoperative periods. A combination of increased QT dispersion with loss of sinus rhythm increases the positive predictive value for sudden cardiac death in patients with transposition of the great arteries after Mustard and Senning procedures.