An improved echocardiographic rejection-surveillance strategy following pediatric heart transplantation.

BACKGROUND: The unique demands of cardiac transplantation in infancy have led to non-invasive rejection-surveillance strategies. ECHO-A is a multiparametric, two-dimensionally guided, M-mode analysis algorithm that assigns an empirically derived score for deviations of recipient parameters to age-adjusted, population-based normal values. A cumulative ECHO-A score > or =4 is highly predictive of endomyocardial biopsy Grade > or =3 and of cellular rejection. METHODS: This study determined whether modifying ECHO-A to score for deviations of recipient parameters from the recipient's baseline would improve the predictive power of ECHO-A. We reanalyzed 701 consecutive echocardiograms of 18 pediatric cardiac transplant recipients (median age at transplantation, 142 days) and based scoring on significant (Z score > or =1) deviation from the patients' baseline means (ECHO-B). RESULTS: Eight episodes of treated rejection occurred during the first year after transplantation (median, 1.4 years). Approximately 10% (72) of the analyses had ECHO-A scores > or =4 that were not associated with treated rejection and were considered false positives. We identified parameters that contributed to the false-positive evaluations and calculated patient-specific baseline mean +/- standard deviation. The ECHO-B, in comparison with ECHO-A, decreased the number of false positives from 72 to 10, increased specificity from 90% to 99%, and increased the positive predictive value about 4-fold (10% to 44%). With treated rejection episodes, ECHO-B increased ECHO-A scores in 7 of 8 recipients and increased the mean score from 6 to 8. CONCLUSIONS: analysis algorithm based on change from baseline improved the positive predictive power without reducing the negative predictive value of multiparametric quantitative analyses of echocardiograms following pediatric heart transplantation.

Experience of FK506 immune suppression in pediatric heart transplantation: a study of long-term adverse effects.

BACKGROUND: Immunosuppression with FK506 for pediatric heart transplantation has been used in this institution since 1989. This study reports the unique toxicity of this macrolide agent in these heart transplant recipients. METHODS: Between October 1989 and August 1994, 49 patients were managed with FK506, which was the initial primary agent in 38 patients. The remaining 11 were switched from cyclosporine A because of persistent rejection or side effects from the cyclosporine A or prednisone. Data were obtained retrospectively from medical records. RESULTS: Mean duration of follow-up was 29 months (median 37 months, range 3 to 96 months). Twenty-nine patients (59%) were receiving FK506 alone; 20 patients (41%) were receiving additional treatment with azathioprine, prednisone, or methotrexate. There were seven deaths. Twenty patients (41%) had elevated creatinine levels between 1 to 2 mg/dl. Five patients (11%) had levels greater than 2 mg/dl. Two patients with preexisting renal dysfunction while receiving cyclosporine A had chronic renal failure 32 and 36 months after switching to FK506 and required kidney transplantation. Hyperkalemia was a persistent finding in 26 patients. Of eight patients with hypertension, four had preexisting disease while receiving cyclosporine A; two had impaired renal function, and two were receiving prednisone. Severe anemia developed in eight patients (16%), two of whom had parvovirus. Moderate anemia developed in 21 patients (43%). Eosinophilia occurred in 19 patients; 11 of 19 patients (58%) had allergic symptoms. There was one case of diabetes mellitus. There were 12 significant infections with four infection-related deaths. Lymphoproliferative disease was noted in three patients, two of whom survived. Gastrointestinal symptoms, including chronic diarrhea, recurrent abdominal pain, and reflux esophagitis were present in 10 patients. CONCLUSIONS: In our experience, anemia, renal toxicity, hyperkalemia, chronic diarrhea, and allergies were the most common adverse effects of FK506. Unlike cyclosporine A, hypertension, gingival hyperplasia, coarsening of facial features, and hirsutism were not seen.

Thrombolysis with tissue-type plasminogen activator following cardiac surgery in children.

Three children with major intracardiac or vascular thrombosis following cardiac operations were treated with intravenous recombinant tissue-type plasminogen activator. The first patient, aged 10 yr, developed a left atrial thrombus following replacement of the mitral valve with a Björk-Shiley prosthesis. The second patient, aged 16 months, had a right atrial thrombus following a modified Fontan procedure for tricuspid atresia. Both were successfully treated with a short course of intravenous tissue plasminogen activator. The third patient, aged 19 months, developed the Budd-Chiari syndrome with occlusion of the inferior caval vein following a modified Fontan operation for double inlet left ventricle. Even though near-complete thrombolysis and recanalization of the inferior caval vein was achieved with three courses of tissue plasminogen activator on successive days, she died with failure of multiple organs. In all cases, the diagnosis was established by cross-sectional and Doppler echocardiography, and the response to therapy was monitored using the same technique. Thrombolytic therapy with systemic tissue-type plasminogen activator was safe and effective in the postoperative period, with no major haemorrhagic complications.

Distal ductal origin of the right pulmonary artery: prospective diagnosis and primary repair in infancy.

Absence of the proximal segment of the right pulmonary artery was diagnosed by echocardiography in two neonates. In both a right-sided duct communicated with the right pulmonary artery at the hilum of the lung. Successful surgical repair was undertaken at seven months and 10 weeks, respectively