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To establish the medium-term results of our transplant population, we retrospectively reviewed the charts of 51 consecutive patients who underwent orthotopic heart transplantation between July 1988 and April 1995. These patients comprised two groups: group A consists of 26 patients (age 6 days to 16.4 years, median 1.4 years) with no previous heart surgery, and group B consists of 25 patients (ages 0.1 to 14.3 years, median 8.3 years), all of whom had heart surgery before undergoing transplantation. There was no difference between these groups in early or late survival rates, and neither age at transplantation nor sex was an indicator of survival. There have been 14 deaths, six early (before hospital discharge) and eight late. Early deaths have predominantly been attributed to long-term ventilation and hemodynamic instability before transplantation, and late deaths to graft coronary artery disease (n = 4), acute coronary vasculitis (n = 3), and acute cellular rejection (n = 1). Although infection has resulted in significant morbidity (57 hospital admissions), there have been no late deaths resulting from infection. Sepsis accounts for four early deaths in chronically ill patients. Orthotopic heart transplantation in the pediatric patient with and without previous heart surgery is a viable option for those with end-stage heart disease and those in whom other surgical options carry a prohibitively high mortality rate.

This study defines the total sodium-23 magnetic resonance spectroscopy (23Na MRS) signal from in vivo heterotopic rat heart transplants in the early post-transplant period and examines the utility of this noninvasive method for monitoring allograft rejection. Measurements were performed at 4.7 T. Syngeneic (n = 6) and allogeneic (n = 6) donor hearts were transplanted into the neck of recipient rats. There were 27 MRS observations between days 0 and 29 post-transplant. Heart grafts were excised at various intervals post-transplant for histologic examination. Allogeneic heart grafts rejected between days 4 and 5 post-transplant while syngeneic grafts continued to beat. All hearts showed ischemic damage. Allogeneic hearts showed cellular rejection by Day 1. 23Na MRS showed a steady elevation in signal in the 3 days prior to rejection and a sharp rise after rejection. 23Na MRS accurately identified full rejection and was also sensitive to the rejection process.

An 18-year-old man whose sister has classic Kartagener's syndrome was found to have sinusitis, bronchiectasis, and corrected transposition with normal visceral situs. Congenital complete heart block was secondary to absence of conducting-system pathways between a small posterior atrioventricular node and the transposed His bundle and bundle branches. No anterior atrioventricular node was present. Prosthetic valvular replacement of the left-sided (morphologic right) atrioventricular valve was complicated by coronary arterial occlusion by suture, with subsequent myocardial infarction. The case appears to represent an unusual variant of Kartagener's syndrome with the abnormality of laterality being expressed as corrected transposition.

A pathogenetic mechanism for the formation of tetralogy of Fallot is postulated. We propose that the right ventricular ejection stream is divided into a transseptal aortic stream and an infundibular pulmonary stream during embryonic life before the ventricular septum is closed. The surgically unaltered hearts from 77 cases of tetralogy of Fallot were examined, and measurements made of certain morphologic features. A malformed, stenotic pulmonary valve was found in most all cases and accounts for a divided ejection stream. Infundibular stenosis is shown to be a postnatally acquired, progressive lesion. The detailed anatomic features of the heart and great vessels in tetralogy of Fallot are explained as a response of the developing heart and great vessels to the abnormal blood flow pattern of a divided right ventricular ejection stream. This pathogenetic mechanism, compared to previously proposed hypotheses, appears better able to account for the malformation complex of tetralogy of Fallot.