Heart transplantation in children.

Cardiac transplantation has become a standard therapeutic option for a variety of cardiac conditions in childhood in which poor cardiac output without other surgical options exists in the face of maximized medical therapy. In this review, the common diagnoses resulting in heart transplantation, risk factors and outcomes of heart transplantation in children with otherwise inoperable heart disease are discussed.

A prospective evaluation of nesiritide in the treatment of pediatric heart failure.

This study sought to determine the potential of recombinant B-type natriuretic peptide (nesiritide) for the treatment of pediatric decompensated heart failure. Nesiritide is a widely used and effective treatment for decompensated heart failure (HF) in adults, but its safety and efficacy in pediatric patients is unclear. Outcomes of 55 separate nesiritide infusions of varying durations in 32 patients (13 males and 19 females; mean age, 8.01 years; range, 0.01-20.4) were evaluated prospectively. All patients received nesiritide in the intensive care unit. The starting dose (0.01 microg/kg/min) was titrated to a maximum of 0.03 microg/kg/min. All patients were monitored for clinical signs and symptoms, hemodynamics, urine output, electrolytes, oxygen requirements, and oral intake. Functional status was assessed by patients and/or their parents. All patients successfully underwent initiation and titration of nesiritide infusion. No hypotension or arrhythmias were noted during 478 cumulative days of therapy. Nesiritide was given safely with vasoactive medications. Mean urine output improved from 2.35 +/- 1.71 cc/kg/hr on the day before nesiritide initiation (baseline) to 3.10 +/- 1.94 cc/kg/hr on day 4 of treatment (p < 0.01). Serum creatinine decreased from 1.04 to 0.92 mg/dl (p = 0.096), mean central venous pressure from 13 to 7 mmHg (p = 0.018), and mean weight from 30.4 to 29.7 kg (p < 0.001) with therapy. Thirst, as subjectively assessed by patients old enough to respond, decreased with infusion in 31 of 42 cases (74%). Mean New York Heart Association functional class improved significantly (p < 0.001). Nesiritide infusion, alone or in combination, is a safe treatment for decompensated HF in pediatric patients. It is associated with decreased thirst and improved urine output and functional status, and it may be efficacious in the treatment of pediatric HF.

Left ventricular noncompaction cardiomyopathy in association with trisomy 13.

In recent years, left ventricular noncompaction (LVNC) has been recognized as a distinct form of cardiomyopathy with its own clinical presentation and natural history. More than 100 cases of LVNC have been described in children. Although LVNC has been described in association with metabolic disorders such as Fabry's disease or genetic disorders such as Roifman's syndrome, this case represents the first report of LVNC in a child with trisomy 13.

Echocardiographic predictors of adverse clinical events in children with dilated cardiomyopathy: a prospective clinical study.

OBJECTIVES: To compare tissue Doppler (TD) velocities between patients with dilated cardiomyopathy (DCM) and normal controls and to determine whether TD velocities, Tei index, right ventricular fractional area change, and left ventricular ejection fraction (LVEF) predict adverse clinical outcomes in children with DCM. METHODS: Prospective evaluation of children with DCM. RESULTS: 54 children with DCM and 54 age and sex matched control group participants were studied. Mitral inflow velocities were similar for both groups except for decreased mitral deceleration time in patients with DCM. Systolic and diastolic TD velocities at the mitral annulus (septal and lateral sides) and tricuspid annulus were significantly reduced in children with DCM compared with controls (p < 0.001 for each). By multivariate analysis, after adjustment for Tei index and right ventricular fractional area change, decreased LVEF and tricuspid velocity during early diastole (Ea) were predictors of the primary end point (PEP), a composite end point consisting of need for hospitalisation or the outcome transplantation or death. Tricuspid Ea velocity < 8.5 cm/s had 87% specificity and 60% sensitivity for reaching the PEP. LVEF < 30% had 68% specificity and 74% sensitivity for the PEP. Combined LVEF < 30% and tricuspid Ea < 11.5 cm/s had 100% specificity and 44% sensitivity for the PEP. CONCLUSIONS: Children with DCM have significantly lower TD velocities than normal controls. In such cases, lower LVEF (< 30%) is more sensitive but less specific than lower tricuspid Ea velocities (< 8.5 cm/s) in predicting which patients are at risk of hospitalisation, transplantation, or death.

Risk factors for neo-aortic root enlargement and aortic regurgitation following arterial switch operation.

The objectives of this study were to evaluate changes in dimension of the neo-aortic annulus, aortic root, and aortic anastomosis following arterial switch operation (ASO) and to identify risk factors for developing abnormal neo-aortic root enlargement and aortic regurgitation (AR). Prior studies report development of neo-aortic root dilatation and AR in a small subset of patients after ASO. Predisposing factors for neo-aortic root dilatation and development of moderate/severe AR are poorly understood. We performed a retrospective review of all patients with d-transposition of the great arteries (d-TGA) or double-outlet right ventricle with subpulmonary ventricular septal defect (VSD) who underwent ASO from May 1986 to January 2001. Serial echocardiograms were reviewed to measure neo-aortic annulus, root, and anastomosis diameter (z scores) and to determine progression of AR. Potential risk factors were assessed for developing neo-aortic root enlargement and AR. There were 119 patients (44 female and 75 male): 73 patients had simple d-TGA, 36 had d-TGA with ventricular septal defect, and 10 had a Taussig-Bing heart. The median duration of follow-up was 65 months (range, 12-180). The median neo-aortic root (z = 0.55+/-2.2; p < 0.01) and aortic annulus dimensions (z = 1.57+/-1.75; p < 0.01) were significantly increased over the study period. Aortic anastomosis diameter correlated with growth of the ascending aorta (z = 0.55+/-1.24). Development of severe neo-aortic root enlargement was associated with prior pulmonary artery (PA) banding (p < 0.01), the presence of a VSD (p = 0.03), and Taussig-Bing anatomy (p < 0.01) but was independent of coronary arterial anatomy, coronary arterial transfer technique, or associated lesions (p > 0.05). At latest follow-up, there was no or trivial AR in 88 patients, mild AR in 29 patients, and moderate to severe AR in 3 patients. Risk factors for developing mild or worse AR included severe or rapid neo-aortic root dilatation (p < 0.01). Only 3 patients required surgical intervention for AR. Despite the significant prevalence of neo-aortic root enlargement at intermediate follow-up after ASO, there is a low incidence of significant AR. Prior PA banding, the presence of VSD, and Taussig-Bing anatomy are risk factors for severe root enlargement. Surgical intervention for AR was rare (2%), however, serial surveillance of such patients is vital to monitor for neo-aortic root enlargement and potential aortic valve dysfunction.

Long-term follow-up of arrhythmias in pediatric orthotopic heart transplant recipients: incidence and correlation with rejection.

BACKGROUND: Arrhythmias in adult orthotopic heart transplant (OHT) recipients are common and have been used as predictors of rejection. Because of the paucity of information in pediatric OHT recipients, the purpose of this study was to determine the incidence and correlation of arrhythmias with rejection or with coronary artery disease (CAD) in children. METHODS: We retrospectively reviewed the records, electrocardiograms (ECGs), and 24-hour ambulatory ECGs of patients who underwent OHT from January 1984 to December 1999. We excluded arrhythmias occurring in the first 2 weeks after OHT. RESULTS: Sixty-nine patients underwent OHT, received triple-immunosuppression therapy, were discharged home, and have been followed for a mean of 4.7 years (0.3-13 years). Each patient had an average of 10 ECGs and three 24-hour ECGs. Twenty-six patients had 33 arrhythmias: sinus bradycardia (n = 9), atrial tachycardia (n = 9), ventricular tachycardia (n = 3), and Wenckebach periodicity (n = 6). Sinus bradycardia was treated with theophylline in 8 patients, and 2 required pacemakers. Atrial tachycardias (atrial flutter in 4 patients and atrial ectopic tachycardia in 5) were treated with digoxin, propranolol, or procainamide. Ventricular tachycardia was treated with mexiletine, lidocaine, and amiodarone. There were 65 episodes of rejection, 20 of which were moderate/severe (> or =3B). Only Wenckebach was associated with the presence of either rejection or CAD (p < 0.05). CONCLUSIONS: We noted clinically significant arrhythmias in 38% of the pediatric OHT recipients. Sinus bradycardia, atrial tachyarrhythmias, and ventricular tachycardia occurred with the same frequency. Only new-onset Wenckebach periodicity was noted in the presence of either CAD or rejection. No arrhythmia was of negative predictive value for rejection or CAD. From this data, we suggest that new-onset Wenckebach prompt evaluation for rejection or CAD.

Neoaortic root dilation associated with left coronary artery stenosis following arterial switch procedure.

We describe a patient who was diagnosed with d-transposition of the great arteries, with intact ventricular septum, who underwent arterial switch procedure on day 5 of life. Over the subsequent years, he developed progressive neoaortic root dilation with a Z score of up to 7.2. At 5 years of age, he presented with myocardial infarction. Cardiac catheterization demonstrated a markedly dilated aortic root with kinking and stenosis of the left main coronary artery into the left anterior descending coronary artery. He underwent emergency left internal mammary artery bypass grafting to the left anterior descending coronary artery. Although he required left ventricular assist device (LVAD) support in the early post-operative period, he recovered with a left ventricular ejection fraction of 52% on the most recent follow-up.

Usefulness of routine surveillance biopsies in children more than one year after orthotopic heart transplantation.

This retrospective study examines the usefulness of routine biopsies following the first year after transplant. This study found that routine biopsies detect few episodes of rejection in the first year after transplant and were less useful than nonroutine biopsies.

Sudden death and cardiovascular collapse in children with restrictive cardiomyopathy.

BACKGROUND: Restrictive cardiomyopathy (RCM) is rare in children, and the prognosis is poor. In the present study, we evaluated all pediatric patients with RCM who were at our institution during a 31-year period to determine the clinical outcome and cause of death. Those who sustained sudden, unanticipated cardiac arrests were evaluated for risk factors that are predictive of sudden death. METHODS AND RESULTS: Eighteen consecutive patients were reviewed. Presentation, clinical course, laboratory data, and histopathological evidence of ischemia were compared between patients with and without sudden death events. The results demonstrated that patients who were at risk for sudden death were girls with chest pain, syncope, or both at presentation and without congestive heart failure. Although not statistically significant for sudden death, Holter monitor evidence of ischemia predicted death within months. Histopathological evidence of acute or chronic ischemia was found in the majority of patients, with acute ischemia more common among those who sustained sudden death events. CONCLUSIONS: All children with RCM are at risk for ischemia-related complications and death, and some are at risk of sudden death. In the present study, patients at risk of sudden death appeared well and had no evidence of ongoing heart failure but often had signs or symptoms of ischemia characterized by chest pain, syncope, or both. ECGs and Holter monitors may be useful screening tools. The use of beta-blockade, the placement of an implantable cardioverter-defibrillator, and preferential status 1A or B listing for cardiac transplantation are proposed for pediatric patients with RCM and evidence of ongoing ischemia.

Long-term results of triple-drug-based immunosuppression in nonneonatal pediatric heart transplant recipients.

BACKGROUND: Few reports document long-term results of pediatric cardiac transplantation in which triple therapy (cyclosporine, azathioprine, and corticosteroids) was the mainstay of immunosuppression. This report details a single center's pediatric transplant experience and analyzes the relative contributions of selected pre/posttransplant risk factors on long-term morbidity and mortality. METHODS: Retrospective data were collected for all non-neonatal pediatric transplant recipients including: presenting diagnosis, cardiac hemodynamics (particularly pulmonary vascular resistance index), donor ischemic time, occurrence of postoperative infections, episodes of allograft rejection, incidence of posttransplant lymphoproliferative disease or coronary artery disease (CAD), and overall survival. Analysis of single variables and a Cox-proportional hazards model were utilized to determine the impact of pre/posttransplant risk factors on long-term survival. RESULTS: From 1984 to 1995, 64 patients (mean age, 8.3 years), 46 of whom had cardiomyopathy and 18 who had inoperable complex congenital heart disease, underwent cardiac transplantation and received triple-drug immunosuppression. Orthotopic transplantation was performed unless the pulmonary vascular resistance index remained >6 um2 (despite use of pulmonary vasodilator). One patient required heterotopic transplantation. Average donor ischemic time was 217 min. An average of 1.2 rejection episodes/patient occurred (average follow-up period: 50 months). No patient developed posttransplant lymphoproliferative disease, but 22 patients (34%) developed CAD. Overall survival was 80%, 60%, and 57% at 1, 5, and 10 years, respectively. Of outcome variables analyzed, rejection frequency was significantly increased in patients who subsequently developed CAD, but the presence of CAD was not significantly correlated with mortality. CONCLUSION: Triple-drug-based immunosuppressive maintenance therapy in pediatric heart transplant recipients results in good long-term graft survival.

Cardiac transplantation for pediatric patients. With inoperable congenital heart disease.

Recent studies have reported the expanding use of transplantation as the definitive option for pediatric patients with inoperable congenital heart disease. This study compares perioperative risk factors and outcomes in pediatric patients who received heart transplants for congenital heart disease with those in pediatric patients who received heart transplants for cardiomyopathy. Retrospective data collected on 40 consecutive pediatric patients undergoing cardiac transplantation from 1 January 1990 through 31 January 1995 provided the following results: 26 patients with cardiomyopathy (mean age, 7.6 years) and 14 patients with congenital heart disease (mean age, 7.2 years) underwent heart transplantation. Between groups, no significant difference was detected in waiting time for a donor heart (cardiomyopathy = 85 days, range = 2 to 409; congenital heart disease = 126 days, range = 9 to 396; P = NS); in donor/recipient weight ratio (1.27 +/- 0.34 vs 1.27 +/- 0.28, P = NS); or in ischemic times (209 +/- 92 minutes vs 248 +/- 70 minutes, P = NS). Cardiopulmonary bypass times accounted for the only significant difference (73 +/- 21 minutes vs 102 +/- 29 minutes, P = 0.003). No significant difference was found in the number of infection episodes, total days hospitalized, rejection episodes, or incidence of transplant coronary artery disease. Forty-month actuarial survival was 88% +/- 6% and 92% +/- 7% for cardiomyopathy and congenital heart disease transplant recipients, respectively (P = NS). We conclude that post-transplantation morbidity and mortality in patients with previous congenital heart disease are not significantly different from morbidity and mortality in patients with cardiomyopathy. Transplantation should be considered an acceptable therapeutic option for patients with congenital heart disease when surgical repair of the native heart is not possible.

New mutations in the KVLQT1 potassium channel that cause long-QT syndrome.

BACKGROUND: Long-QT syndrome (LQTS) is an inherited cardiac arrhythmia that causes sudden death in young, otherwise healthy people. Four genes for LQTS have been mapped to chromosome 11p15.5 (LQT1), 7q35-36 (LQT2), 3p21-24 (LQT3), and 4q25-27 (LQT4). Genes responsible for LQT1, LQT2, and LQT3 have been identified as cardiac potassium channel genes (KVLQT1, HERG) and the cardiac sodium channel gene (SCN5A). METHODS AND RESULTS: After studying 115 families with LQTS, we used single-strand conformation polymorphism (SSCP) and DNA sequence analysis to identify mutations in the cardiac potassium channel gene, KVLQT1. Affected members of seven LQTS families were found to have new, previously unidentified mutations, including two identical missense mutations, four identical splicing mutations, and one 3-bp deletion. An identical splicing mutation was identified in affected members of four unrelated families (one Italian, one Irish, and two American), leading to an alternatively spliced form of KVLQT1. The 3-bp deletion arose de novo and occurs at an exon-intron boundary. This results in a single base deletion in the KVLQT1 cDNA sequence and alters splicing, leading to the truncation of KVLQT1 protein. CONCLUSIONS: We have identified LQTS-causing mutations of KVLQT1 in seven families. Five KVLQT1 mutations cause the truncation of KVLQT1 protein. These data further confirm that KVLQT1 mutations cause LQTS. The location and character of these mutations expand the types of mutation, confirm a mutational hot spot, and suggest that they act through a loss-of-function mechanism or a dominant-negative mechanism.

Association of parvovirus B19 genome in children with myocarditis and cardiac allograft rejection: diagnosis using the polymerase chain reaction.

BACKGROUND: Inflammatory diseases of the heart, including myocarditis and cardiac transplant rejection, are important causes of morbidity and mortality in children. Although viral infection may be suspected in either of these clinical conditions, the definitive etiology is often difficult to ascertain. Furthermore, the histology is identical for both disorders. Coxsackievirus has long been considered the most common cause of viral myocarditis; however, we previously demonstrated by polymerase chain reaction (PCR) analysis that many different, and sometimes unexpected, viruses may be responsible for myocarditis and cardiac rejection. In this study, we describe the association of parvovirus genome identified through PCR analysis of cardiac tissue in the clinical setting of myocarditis and cardiac allograft rejection. METHODS AND RESULTS: Myocardial tissue from endomyocardial biopsy, explant, or autopsy was analyzed for parvovirus B19 using primers designed to amplify a 699-base pair PCR product from the VP1 gene region. Samples tested included those obtained from patients with suspected myocarditis (n=360) or transplant rejection (n=200) or control subjects (n=250). Parvoviral genome was identified through PCR in 9 patients (3 myocarditis; 6 transplant) and no control patients. Of the 3 patients with myocarditis, 1 presented with cardiac arrest leading to death, 1 developed dilated cardiomyopathy, and the other gradually improved. Four of the 6 transplant patients had evidence of significant rejection on the basis of endomyocardial biopsy histology. All transplant patients survived the infection. CONCLUSIONS: Parvovirus is associated with myocarditis in a small percentage of children and may be a potential contributor to cardiac transplant rejection. PCR may provide a rapid and sensitive method of diagnosis.

A method providing bidirectional control of coil delivery in occlusions of patent ductus arteriosus with shallow ampulla and Pott's shunts.

A novel method using a snare and bioptome to provide bidirectional control of a Gianturco coil for occlusion of a patent ductus arteriosus with a shallow ampulla and Pott's shunts is presented. This method greatly reduces the risk of coil embolization and optimizes coil position in difficult cases.

Outcomes among pediatric heart transplant recipients.

Postoperative cytomegalovirus prophylaxis with cytomegalovirus immunoglobulin or ganciclovir has decreased the incidence of cytomegalovirus disease in cytomegalovirus-negative recipients of cytomegalovirus-positive donor organs. In adults, these drugs have also been used to treat recipients who developed symptomatic cytomegalovirus disease. This report describes outcomes of predominantly cytomegalovirus-negative pediatric cardiac transplant recipients of cytomegalovirus-positive donor organs who received cytomegalovirus immunoglobulin plus ganciclovir as cytomegalovirus prophylaxis, as well as results of this combination therapy when used to treat cytomegalovirus disease. We reviewed the records of children who received donor hearts at our institution between 1989 and 1994. Cytomegalovirus-negative patients who received cytomegalovirus-positive donor organs were given prophylaxis consisting of ganciclovir (5 mg/kg every 12 hours for 14 days, followed by maintenance dosage of 5 to 6 mg/kg every day for 14 days) plus 7 scheduled cytomegalovirus immunoglobulin infusions. Cytomegalovirus infection was documented by culture, polymerase chain reaction, and cytomegalovirus immunoglobulin M seroconversion of a 4-fold or greater rise in cytomegalovirus immunoglobulin G titers. After infection, patients were diagnosed with cytomegalovirus disease when they developed clinical symptoms. These episodes were treated with cytomegalovirus immunoglobulin infusions plus ganciclovir (5 mg/kg every 12 hours) until symptoms resolved. Of 40 cardiac transplant recipients, 10 cytomegalovirus-negative and 9 cytomegalovirus-positive patients received cytomegalovirus-positive donor organs. Five patients (3 of whom were seronegative and had received dual-agent prophylaxis) developed cytomegalovirus disease, which resolved with dual-agent therapy. During an average 15-month follow-up period, no significant morbidity or mortality was attributable to cytomegalovirus disease. Post-transplant dual-therapy cytomegalovirus prophylaxis appears to be as safe and effective in children as in adults, when our results are compared with the published results of studies in adults. Dual-agent treatment eradicated symptoms among patients who developed cytomegalovirus disease. This regimen may allow safer use of the cytomegalovirus-positive donor pool for pediatric recipients.

Update on pediatric heart transplantation. Long-term complications.

Despite evolution to "higher-order" immunosuppressive agents that better control cell-mediated allograft rejection and, therefore, short- and intermediate-term survival, allograft vasculopathy and PTLD remain factors that limit extended graft survival. While improved basic science "bench" techniques have enabled investigation of their pathogenesis at the subcellular and molecular levels, each scientific advance leads the way to the next horizon of complex questions. Only through a more complete understanding of the etiology and pathophysiology of these processes will we be able to design strategies to combat these complications.

Restrictive cardiomyopathies in childhood. Etiologies and natural history.

Restrictive cardiomyopathy is rare in childhood and little is known about the causes and outcome. This lack of information results in extrapolation of adult data to the care and management of children, who might require different treatment from that of adults. This study was undertaken retrospectively to evaluate the causes and natural history of restrictive cardiomyopathy in childhood. Twelve cases of restrictive cardiomyopathy were identified by database review of patient records from 1967 to 1994. The cases were selected on the basis of echocardiographic and cardiac catheterization criteria. Charts were reviewed for the following variables: age, sex, cause, right-and left-sided hemodynamics, pulmonary vascular resistance index, shortening fraction, therapy, and outcome. There were 6 males and 6 females with a mean age of 4.6 years at presentation (median, 3.4 yr; range, 0.9 to 12.3 yr). Etiologies included hypertrophic cardiomyopathy in 3 patients, cardiac hypertrophy with restrictive physiology in 3, idiopathic in 2, familial in 2 (twins), "chronic eosinophilia" in 1, and "post inflammatory" with no definitive causes in 1. At presentation the mean shortening fraction was 33% +/- 2% (mean +/- SEM), average right ventricular pressures were 44/13 +/- 3/1, average left ventricular pressures were 88/25 +/- 4/3, and the mean pulmonary vascular resistance index was 3.4 +/- 1.3 U.m2 (n = 9), but increased to 9.9 +/- 3.1 U.m2 (n = 5, p = 0.04) by 1 to 4 years after diagnosis. Four of the 12 patients had embolic events (1, recurrent pulmonary emboli; 1, saddle femoral embolus; 2, cerebrovascular accidents) and 9 of 12 died within 6.3 years despite medical therapies, which included diuretics, verapamil, propranolol, digoxin, and captopril. In conclusion, restrictive cardiomyopathy in childhood is commonly idiopathic or associated with cardiac hypertrophy, and the prognosis is poor. Embolic events occurred in 33% of our patients, and 9 of 12 patients died within 6.3 years. Within 1 to 4 years of diagnosis, patients may develop a markedly elevated pulmonary vascular resistance index; therefore, transplantation should be considered early.

Diagnosis, surveillance, and epidemiologic evaluation of viral infections in pediatric cardiac transplant recipients with the use of the polymerase chain reaction.

BACKGROUND: Viral infections, particularly those caused by cytomegalovirus, are a major cause of postoperative morbidity and mortality in heart transplant recipients. These infections have classically been diagnosed by history, physical examination, peripheral viral cultures, and serologic studies. These methods are often time-consuming and lack sensitivity. Positive viral cultures from the heart are rarely obtained, and viral myocarditis and acute cellular rejection are unable to be differentiated histologically. We have therefore used the polymerse chain reaction to diagnose possible viral infection in pediatric heart transplant recipients with findings consistent with acute unexplained rejection. METHODS: Polymerase chain reaction was used as an aid to diagnose cytomegalovirus infection of cardiac tissue obtained by right ventricular endomyocardial biopsy and follow its long-term course. In addition, polymerase chain reaction was used to diagnose infection of the heart by other viruses in patients with clinical and histologic evidence of rejection, especially those with unexplained late rejection or chronic rejection. Polymerase chain reaction primers were designed to amplify nucleic acid sequences from cytomegalovirus, parvovirus, adenovirus, herpes simplex virus, Epstein-Barr virus, and the RNA viruses of the Enterovirus family. RESULTS: Forty patients underwent serial right ventricular endomyocardial biopsy (129 samples) for rejection surveillance with positive results obtained in 41 samples (32%) from 21 patients. Viral genome amplified included cytomegalovirus in 16 samples, adenovirus in 14, enterovirus in 6, parvovirus in 3, and herpes simplex virus in 2. In 13 of the 21 patients positive for viral genome (62%), endomyocardial biopsy histologic scores were consistent with multifocal moderate to severe rejection (Internal Society for Heart and Lung Transplantation scores of 3A or greater). CONCLUSIONS: Polymerase chain reactions may be used as a rapid and sensitive method to evaluate postoperative viral infections in heart transplant recipients, especially in those with late-onset rejection or chronic rejection. Polymerase chain reaction may also be useful in the serial analysis of cytomegalovirus status in transplant recipients. The use of multiple viral primers improves the diagnostic evaluation of these patients and may lead to a better understanding of the epidemiologic characteristics of posttransplantation viral infections and the cause of late or chronic rejection.

Heart transplantation in an 8-month-old girl. 10th anniversary report.

In 1984, Dr. Denton A. Cooley led a surgical team that implanted a cardiac allograft in an 8-month-old girl who had end-stage cardiac disease secondary to endocardial fibroelastosis. At that time, experience with cardiac transplantation in infants was limited, and the long-term effects of the procedure were cause for concern. Ten years later, our patient is a healthy 4th-grade student who enjoys a remarkably normal life. She has grown and developed quite satisfactorily, and her heart has enlarged in proportion to her overall somatic growth. Long-term immunosuppression has produced no adverse effects, and the child's medical problems have differed little from those of her peers. This landmark case has yielded preliminary answers to a number of important questions about cardiac transplantation in infants and has confirmed our original opinion that the procedure is well warranted in selected patients.