Fate of infants with hypoplastic left heart syndrome listed for cardiac transplantation: a multicenter study.

BACKGROUND: Infants with hypoplastic left heart syndrome (HLHS) commonly undergo cardiac transplantation as primary management. METHODS: We examined outcomes of primary transplantation for unpalliated HLHS. We analyzed data from the 20 institutions of the Pediatric Heart Transplant Study Group, from January 1, 1993, through December 31, 1998, using actuarial and parametric survival analysis and competing outcomes analysis. RESULTS: During the 6 years studied, 1,234 patients were listed for cardiac transplantation; 262 patients (21.2%) had unpalliated HLHS. The number (and percentage) of patients with HLHS decreased from 58 (27% of patients listed) in 1993 to 30 (14%) in 1998. Overall, 25% of infants with HLHS died while waiting; primary cause of death was cardiac failure (50%). Of the remaining patients awaiting transplantation, 23 (9%) underwent Norwood/Fontan-type surgeries as interim palliation: 52% died. Ultimately, 175 patients underwent cardiac transplantation (67%); 50% received organs by 2 months after listing. Post-transplant actuarial survival was 72% at 5 years, with 76% of deaths (35/46) occurring within 3 months; early mortality was caused primarily by graft failure within the first 30 days after transplantation (in 54%). Among 1-month survivors, survival at 1 and at 5 years was 92% and 85%, respectively. Of the 262 patients listed with unpalliated HLHS, overall survival, taking into account mortality after listing and after transplantation, was 68% at 3 months and 54% at 5 years. CONCLUSIONS: Cardiac transplantation offers good intermediate survival for infants with unpalliated HLHS.     

Heart- and heart-lung transplantation in children

Since January 1987, 16 prepubertal children have undergone heart (13) or heart-lung (3) transplantation. Immunosuppression included cyclosporine and azathioprine and excluded steroids except in case of rejection. The indications for heart transplantation were hypoplastic left heart syndrome (4 infants, mean age = 2 months), congenital heart disease (4 patients, mean age = 5.7 years) and cardiomyopathy (5 patients, mean age = 2.8 years). There were 4 early deaths (acute graft failure in 2, pulmonary hypertension in 1, infection in 1) and 1 late death (heart failure at 3 months). The 8 survivors had a mean follow-up of 12 months (range 1-19 months). Late complications were minimal. There were 4 episodes of rejection in 2 patients. There was no infection, normal somatic growth and no systemic hypertension. Renal function remained within normal limits although mild-to-moderate tubulointerstitial lesions were found in 4 renal biopsies. Three children (9-11 years old) underwent heart-lung transplantation. The early postoperative course was difficult with 6 episodes of rejection and 5 infections. One patient died at 3 months from infectious complications. One child has a complete rehabilitation 8 months posttransplantation. The last patient is clinically well at 7 months but has a residual tracheal stenosis. The long-term fate of these children, and particularly the long-term effects of cyclosporine therapy are unknown. Heart and heart-lung transplantation remain under investigation but may be reasonable approaches for infants and children with end-stage cardiac and/or pulmonary disease.     

Intraaortic balloon pumping in children.

BACKGROUND: Intraaortic balloon pump (IABP) usage in young children remains a rarity because, first, there is a widespread misconception that the greater elasticity of the aorta in children may prevent effective augmentation, and second, children in low cardiac output states are more likely to have associated right ventricular failure and pulmonary problems. We report our experience with the use of IABP in children undergoing cardiac surgery in whom mechanical support was necessary for the treatment of refractory cardiac failure. METHODS: Over a 3-year period, 14 children were identified as requiring IABP support after cardiac surgery. Their median age was 3 years (range 7 days to 13 years) and their median weight was 13.3 kg (range 3.5-51 kg). Indications for IABP use were: failure to wean from cardiopulmonary bypass (5 patients), sudden deterioration in intensive therapy unit (ITU) (3 patients), progressive deterioration in ITU (4 patients), and prophylactic therapy for known poor preoperative ventricular function (2 patients). RESULTS: Ten of 14 patients (71%) were successfully weaned from the IABP, of whom 8 became long-term survivors (57%). Among the survivors, the mean duration of IABP usage was 127 h (range 12-260 h), while for those who died while on IABP, the mean duration was 15 h (range 8-24 h). The most major IABP-related complication encountered was mesenteric ischemia, which had a fatal outcome. CONCLUSIONS: IABP therapy, used as an adjunct to conventional medical treatment, can give properly selected young children in refractory low cardiac output states after heart surgery a greater than 50% chance of long-term survival.     

Laparoscopic nephroureterectomy to exclude upper urinary tract malignancy associated with analgesic nephropathy.

BACKGROUND AND PURPOSE: Analgesic abuse is a potential cause of end-stage renal disease. Such patients bear an elevated risk of developing malignancies, predominantly transitional-cell carcinoma. We report our experience with laparoscopic nephroureterectomy carried out in patients with analgesic nephropathy to exclude upper urinary tract malignancy. All patients were scheduled to be put on the waiting list for cadaveric renal transplantation. PATIENTS AND METHODS: Since 1996, nine women and two men with a long-term history of analgesic abuse have undergone laparoscopic nephroureterectomy at our hospital. The median age was 63 years (range 51-70 years). All patients had developed end-stage renal failure secondary to heavy analgesic abuse with a median duration of 14 years (range 7-40 years). The median interval from the beginning of hemodialysis to laparoscopic nephroureterectomy was 36 months (range 6-76 months). RESULTS: The median operative time was 99 minutes (range 55-170 minutes). There were no conversions to open surgery. Two complications occurred, and three patients required blood transfusions. The median hospital stay lasted 5 days (range 2-12 days), and the median convalescence was 20 days (range 6-44 days). In seven patients, histopathologic examination of the kidney revealed changes attributable to analgesic abuse. None of the patients had a transitional-cell carcinoma, but in two patients, a renal-cell carcinoma stage pT1cN0cM0 grade 2 was detected. CONCLUSION: Patients with analgesic nephropathy bear an elevated risk for the development of transitional-cell or renal-cell carcinoma. In these patients, laparoscopic nephroureterectomy combines minimally operative invasiveness with a maximum of diagnostic safety.     

Pediatric cardiac retransplantation: intermediate-term results

BACKGROUND: Cardiac retransplantation (re-CTx) in children is a controversial therapy, yet it remains the best treatment option to recipients with failing grafts. Our objective was to determine the incidence of re-CTx in a large pediatric population of recipients and evaluate the outcome of such therapy. METHODS: Between November 1985 and November 1999, 347 children underwent cardiac transplantation at the Loma Linda University Medical Center. Of these, 32 children were listed for re-CTx. Ten patients died while waiting, and 22 recipients underwent re-CTx. Median age at re-CTx was 7.1 years (range, 52 days to 20.1 years). RESULTS: Indications for re-CTx were allograft vasculopathy (n = 16), primary graft failure (n = 5), and acute rejection (n = 1). Two patients with primary graft failure underwent retransplantation within 24 hours of the first transplantation procedure while on extracorporeal membrane oxygenation support. Median time interval to re-CTx for the others was 7.2 years (range, 32 days to 9.4 years). Operative mortality for all cardiac re-CTx procedures was 13.6%. Causes of hospital mortality were pulmonary hypertension with graft failure (n = 2) and multiorgan failure (n = 1). Median hospital stay after re-CTx was 14.1 days (range, 6 to 45 days). There was one late death from severe rejection. Actuarial survival at 3 years for re-CTx was 81.9% +/- 8.9% compared with 77.3% +/- 2.6% for primary cardiac transplantation recipients (p = 0.70). CONCLUSIONS: Elective re-CTx can be performed with acceptable mortality. Although the number of patients undergoing retransplantation in this report is small and their long-term outcome is unknown, the intermediate-term survival after re-CTx is similar to that of children undergoing primary cardiac transplantation.     

Combined liver-kidney transplantation for primary hyperoxaluria type 1 in young children.

BACKGROUND: Primary hyperoxaluria type 1 (PH1) is a rare condition in which deficiency of the liver enzyme alanine:glyoxylate aminotransferase leads to renal failure and systemic oxalosis. Combined liver-kidney transplantation (LKT) is recommended for end-stage renal failure (ESRF) in adults, but management of infants and young children is controversial. We retrospectively reviewed six children who underwent LKT for PH1. METHODS: The median age at diagnosis was 1.8 years (range 3 weeks to 7 years). Two children presented with severe infantile oxalosis at 3 and 9 weeks, five patients had ESRF with nephrocalcinosis and systemic oxalosis, (median duration of dialysis 1.3 years), and one had progressive chronic renal failure. Four children underwent combined LKT, one child staged liver then kidney, and one infant had an isolated liver transplant. The median age at transplantation was 8.9 years (range 1.7-15 years). RESULTS: Overall patient survival was four out of six. The two infants with PH1 and severe systemic oxalosis died (2 and 3 weeks post-transplant) due to cardiovascular oxalosis and sepsis. The other four children are well at median follow-up of 10 months (range 6 months to 7.4 years). No child developed hepatic rejection and all have normal liver function. Renal rejection occurred in three patients. Despite maximal medical management, oxalate deposits recurred in all renal grafts, contributing to graft loss in one (one of the infants who died), and significant dysfunction requiring haemodialysis post-transplant for 6 months. CONCLUSIONS: LKT is effective therapy for primary oxalosis with ESRF but has a high morbidity and mortality rate in children who present in infancy with nephrocalcinosis and systemic oxalosis. We feel that earlier LKT, or pre-emptive liver transplantation, may be a better therapeutic strategy to improve the outlook for these patients.     

Post-cardiotomy mechanical circulatory support using a conventional bypass circuit in children.

OBJECTIVE: Mechanical circulatory support (MCS) is increasingly used after cardiotomy in children when conventional medical treatment fails. Poor overall survival and long-term outcome have been reported. We report our experience of post-cardiotomy MCS using a conventional bypass circuit. METHODS: Over a 4 year and 6 month period 10 patients, with a median age of 16 days (range 5 days to 16 years) required MCS. Eight patients required support for failure to wean from cardiopulmonary bypass during primary correction. Two patients required support for cardiac arrest or poor cardiac output in the postoperative period. RESULTS: The median duration of support was 43 h (range 26-146 h). Seven hospital survivors were alive and well at median follow-up of 18 months (range 4-36 months). One patient could not be weaned off support. Two more patients died after successful weaning from support. Complications in nine patients who could be weaned off support included renal failure (n=6), cerebrovascular events (n=3) and mediastinitis (n=2). CONCLUSIONS: Overall long-term survival (70%) and quality of recovery is usually good even though initial mortality and complication rates may be high. We think that post cardiotomy mechanical circulatory bypass using a conventional bypass circuit can offer a favourable outcome to selected patients.     

Analysis of risk factors for the development of posttransplant lymphoprolipherative disorder among 119 children who received primary intestinal transplants at a single center

Posttransplant lymphoproliferative disorder (PTLD) is a well-known complication after pediatric transplantation. We analyzed all potential risk factors to assess patient and graft outcomes of 119 children who received intestinal transplantations. MATERIALS AND METHODS: Between August 1994 and March 2005, 119 patients underwent cadaveric intestinal transplantation. Their median age at transplant was 1.4 years (range: 0.6-17), median weight was 9.5 kg (range: 4.7-67), and 57% were boys. The median follow-up among 49 ongoing survivors was 41 months (range: 4-121). All PTLD cases were biopsy proven. In the past 5 years, treatment included antiviral therapy, immunosuppression withdrawal, and use of rituximab. RESULTS: The incidence of PTLD was 11.8% (14/119). No patient experienced graft failure secondary to PTLD, while two patients died from PTLD (14.2%). The PTLD group was divided into an early onset group (<4 months, 6 of 14; 42.8%) and a late onset group (>2 years, 8 of 14; 57.2%). No patient experienced PTLD between 4 months and 2 years after transplantation. The use of OKT3 was the only significant risk factor for the development of PTLD. No factor was specifically associated with the early versus late development of PTLD. CONCLUSIONS: The only factor associated with a significantly higher risk of PTLD was the use of OKT3 to treat a rejection episode. Finally, since the the introduction of anti-CD20 antibodies as part of the treatment protocol for PTLD, the risk of death due to PTLD appears to have become manageably low.     

Heart transplantation in children: risk factors of early and late mortality

In order to identify predictive risk factors of poor outcome following heart transplantation in children, we performed a retrospective analysis of our pediatric recipient population: 31 children, aged 15 days to 15 years (mean = 5.2 +/- 4.9 years). The preoperative diagnosis was cardiomyopathy in 17 (55%), congenital heart disease in 13 (42%) and end-stage valvular disease in 1 (3%). There were 5 operative deaths: hyperacute rejection (2), low cardiac output syndrome (3); 4 in-hospital deaths: infection (2), multiorgan failure (2) and 4 late deaths: acute rejection (1), chronic rejection (1), lymphoma (1), unknown (1). The actuarial probability of survival (+/- SE) was 62% +/- 10% at 1 year and 53% +/- 12% at 2 years. Univariate analysis was used to evaluate the following risk factors: age, diagnosis, hemodynamic decompensation, previous cardiac surgery, ischemic time of the graft, technique of graft preservation, preoperative pulmonary artery pressure, occurrence of postoperative low cardiac output syndrome (LCOS) with pulmonary hypertension (PHT). The occurrence of early LCOS with PHT significantly increased both early and late mortality (78% early mortality, 100% overall mortality). This syndrome occurred in 9 patients (29%) and was attributed to primary graft failure in 2, increased pulmonary vascular resistances in 6 and multiple factors in 1. Although not significant, two factors may increase early survival: young age (less than or equal to 1 year) at operation and improved technique of graft preservation.     

Antenatal oligohydramnios of renal origin: long-term outcome.

BACKGROUND: Prognosis of fetuses with renal oligohydramnios (ROH) is often still regarded as poor. Neonatal complications and the long-term follow-up of fetuses with ROH in two pediatric centres are described. Method. 23 fetuses (16 males, 7 females) were included as patients. Primary diseases included congenital anomalies of the kidney and urinary tract (n = 16), autosomal recessive polycystic kidney disease (n = 4) and renal tubular dysgenesis (n = 3). The analysis includes retrospective chart review. RESULTS: Seven children died (30%), the majority (n = 4, 17%) within the neonatal period due to pulmonary hypoplasia and renal insufficiency. Fourteen patients (61%) required postnatal mechanical ventilation for a median of 4 (range 1-60) days; 11 infants had an associated pneumothorax. All 16 surviving children have chronic kidney disease (CKD) at a current median age of 5.7 years (range 0.5-14.5), managed conservatively in eight patients [median glomerular filtration rate 51 (range 20-78) ml/min/1.73 m(2)]. Eight patients reached end-stage renal disease at a median age of 0.3 years (range 2 days to 8.3 years), including one patient with pre-emptive kidney transplantation. Five of the patients requiring dialysis underwent successful renal transplantation at a median age of 3.5 years (range 2.5-4). Growth was impaired in seven children requiring growth hormone treatment. Cognitive and motor development was normal in 12 (75%) of the 16 patients and showed a delay in four children, including two with associated syndromal features. CONCLUSION: ROH is not always associated with a poor prognosis and long-term outcome in survivors is encouraging. The high incidence of neonatal complications and long-term morbidity due to CKD requires a multidisciplinary management of these children.     

Elevated right ventricular pressures are not a contraindication to liver transplantation in Alagille syndrome

BACKGROUND: Elevated right ventricle pressure resulting from pulmonary artery stenoses may affect outcome and survival after liver transplantation in patients with Alagille syndrome. METHODS AND RESULTS: Between 1984 and 1997, among 444 pediatric liver transplant recipients, 17 had liver transplantation for Alagille syndrome (mean age 3.5 years, range 1.2-13 years), mainly because of poor quality of life with intractable pruritus, and failure to thrive. All patients had pulmonary artery stenosis. In 10 patients considered to have elevated RV pressure on ECG and/or Doppler-echocardiography, a cardiac catheterization was performed before liver transplantation. Mean RV systolic pressure was 55 mmHg (median 49.5 mmHg, range 35-98 mm Hg), mean RV to left ventricular systolic pressure ratio 0.53 (median 0.53, range 0.29-0.78) with a ratio above 0.5 in 6 patients (median 0.66, range 0.5-0.8). All patients underwent successful liver transplantation. Five patients died 1 to 9 months after transplantation from noncardiac causes. In two of them, cardiac catheterization before transplantation showed a RV to left ventricular pressure ratio of 0.51 in one and 0.37 in the second. In the three others, echocardiography before transplantation estimated RV pressures below 0.5 systemic pressures. At follow-up (median 6 years, range 1.5-15 years), liver tests were normal in all, none complained of pruritus and body weight was normalized in 70%. None of the patients presented cardiac symptoms, arrhythmias, or worsening of their cardiac status. CONCLUSIONS: Liver transplantation can be performed safely in children with Alagille syndrome, even in the presence of elevated right ventricular pressure.     

Immunosuppression withdrawal after auxiliary liver transplantation for acute liver failure

BACKGROUND: The potential for immunosuppression withdrawal is the rationale for auxiliary liver transplantation (AUX) in patients with acute liver failure (ALF). PATIENTS AND METHODS: Forty-four AUX were performed in 28 adults and 16 children with ALF secondary to seronegative hepatitis (n = 20; 45%), paracetamol hepatotoxicity (n = 14; 32%), acute viral hepatitis (hepatitis B virus [HBV] n = 3, Epstein-Barr virus n = 1; 9%), drug-induced hepatitis (n = 3; 7%), autoimmune hepatitis (n = 2; 5%), and mushroom poisoning (n = 1; 2%). All patients fulfilled the King's College Hospital transplant criteria for ALF. After partial hepatectomy, 38 patients received a segmental auxiliary graft and six, a whole auxiliary graft. Immunosuppression was based on calcineurin inhibitors and steroids. RESULTS: Thirty-four patients (77%) are alive after a median follow-up of 30 months (range 4 to 124). Eight adults and two children died of sepsis (n = 6; 14%) at a median interval of 30 days (range 2 to 66), intraoperative cardiac failure (n = 1), brain edema on postoperative day 8 (n = 1), sudden death on day 35 (n = 1), and multiple organ failure associated with HBV recurrence 4 years after transplantation (n = 1). Three patients underwent retransplantation for small-for-size graft syndrome with sepsis on postoperative day 15 (n = 1) and for ductopenic rejection 4 and 15 months after AUX (n = 2). In 10/31 (32%) survivors (6/18 adults and 4/13 children) immunosuppression was completely withdrawn after a median of 19 months. CONCLUSION: Complete immunosuppression withdrawal can be achieved in a significant proportion of patients after AUX for ALF.     

Mechanical circulatory support for the treatment of children with acute fulminant myocarditis

BACKGROUND: Viral myocarditis may follow a rapidly progressive and fatal course in children. Mechanical circulatory support may be a life-saving measure by allowing an interval for return of native ventricular function in the majority of these patients or by providing a bridge to transplantation in the remainder. METHODS: A retrospective chart review of 15 children with viral myocarditis supported with extracorporeal membrane oxygenation (12 patients) or ventricular assist devices (3 patients) was performed. RESULTS: All patients had histories and clinical findings consistent with acute myocarditis. The median age was 4.6 years (range 1 day-13.6 years) with a median duration of mechanical circulatory support of 140 hours (range 48-400 hours). Myocardial biopsy tissue demonstrated inflammatory infiltrates or necrosis, or both, in 8 (67%) of the 12 patients who had biopsies. Overall survival was 12 (80%) of 15 patients, with 10 (83%) survivors of extracorporeal membrane oxygenation and 2 (67%) survivors of ventricular assist device support. Nine (60%) of the 15 patients were weaned from support, with 7 (78%) survivors; the remaining 6 patients were successfully bridged to transplantation, with 5 (83%) survivors. All survivors not undergoing transplantation are currently alive with normal ventricular function after a median follow-up of 1.1 years (range 0.9-5.3 years). CONCLUSION: Eighty-percent of the children who required mechanical circulatory support for acute myocarditis survived in this series. Recovery of native ventricular function to allow weaning from support can be anticipated in many of these patients with excellent prospects for eventual recovery of full myocardial function.     

Lung transplantation in very young infants.

INTRODUCTION: Established successes with adult lung transplantation have laid the foundation for extension of this therapeutic modality to infants and children dying of end-stage pulmonary disease. The purpose of this report is to convey our experience with 19 infants undergoing lung transplantation before the age of 6 months. METHODS: Six patients with predominantly pulmonary vascular disease and 13 patients with primarily pulmonary parenchymal disease have undergone bilateral sequential lung transplantation at our institution since 1990. Mean age at transplant was 104 +/- 44 days, and mean weight was 4.9 +/- 1.6 kg. RESULTS: Although early mortality (32%, 6/19) was higher than that previously reported for older pediatric age groups, long-term survival was similar (44% at a maximum follow-up of 6 years). Although anastomotic complications and infections occurred at a rate approximating that seen in older pediatric age groups, episodes of acute rejection appear to occur with decreased frequency. Similarly, at a mean follow-up of 3 years, only 2 (15%) of 13 long-term survivors have evidence of bronchiolitis obliterans. The functional residual capacity, as measured on infant pulmonary function tests, has gradually increased as the children have grown, suggesting that lung growth is occurring. CONCLUSIONS: Bilateral lung transplantation is a viable alternative in infants dying of end-stage pulmonary disease. Efforts directed toward avoiding the complications that lead to early posttransplant mortality combined with the seemingly lower incidence of early and late rejection may provide long-term results better than those in other age groups.     

Cardiac transplantation for hypertrophic cardiomyopathy: a valid therapeutic option.

BACKGROUND: Hypertrophic cardiomyopathy is a rare indication for cardiac transplantation, with only anecdotal reports in the literature. Transplantation has been proposed to patients with hypertrophic cardiomyopathy who remained symptomatic despite optimal medical treatment or who progressed to congestive heart failure, and when conventional surgical correction was not or was no longer possible. In this report, we analyze the clinical results of cardiac transplantation in these patients. METHODS: We retrospectively reviewed complete clinical data and prospectively gathered complete follow-up data for 14 patients who underwent orthotopic cardiac transplantation for hypertrophic cardiomyopathy at the Montreal Heart Institute and Ste-Justine Hospital between 1984 and 2001. RESULTS: Ten male (71.4%, 5 adults and 5 children) and 4 female (28.6%, 2 adults and 2 children) patients underwent heart transplantation for hypertrophic cardiomyopathy. The median age of the recipients in the pediatric group was 13 years (range, 6-16) and was 40 years (range, 22-46) in the adult group. Median duration of follow-up was 9.5 years (mean, 8.8 +/- 4.8 years; range, 1-18) and was 100% complete. We found no operative mortality and found 2 late deaths related to coronary graft atherosclerosis. Long-term survival at 5, 10, and 15 years was 100%, 85%, and 64%, respectively. Freedom from acute rejection at 1, 5, and 10 years was 73%, 32%, and 9%, respectively. The remaining 11 survivors exhibit few symptoms and are currently in New York Heart Association Class I or II. CONCLUSIONS: Cardiac transplantation is a valid therapeutic option for patients with symptomatic hypertrophic cardiomyopathy who do not respond to optimal medical management and who are not candidates for conventional surgical treatment. The long-term outcome is excellent in these patients.     

Outcome of renal transplantation in children with pericardiopleural effusion

INTRODUCTION: Children with end-stage renal disease may present with pericardiopleural effusion secondary to volume overload and overhydration. The present study was designed to investigate the efficacy and safety of renal transplantation in these pediatric patients. METHODS: From 1981 to 2001, six of 20 patients (30%) under 18 years old who received renal transplants showed pericardiopleural effusion after serial pretransplant imaging studies. These patients also displayed associated diseases, such as congestive heart failure (n = 3), ascites (n = 2), and splenomegaly (n = 2). The recipients included five boys and one girl of mean age of 12.7 years (range, 8 to 17 years), all of whom had undergone hemodialysis before transplantation. The waiting time for grafts ranged from 1.3 to 6 years (mean = 2.6 years). Episodes of acute pulmonary edema had been observed in three patients pretransplant. RESULTS: One recipient died with a functioning graft due to heart failure with acute pulmonary edema at 4 months after transplantation. Acute rejection episodes were observed in three, and chronic rejection in two children. The median follow-up was 11 years (range = 6 to 16 years) in the other five recipients, all of whom presently survive with functioning grafts. The posttransplant mean serum creatinine levels at 1 year, 3 years, and 5 years were 1.54 +/- 0.44, 1.74 +/- 0.56, and 1.92 +/- 0.56 mg/dL, respectively. CONCLUSION: Renal transplantation in children displaying pericardiopleural effusion was associated with a high success rate. However, these patients must be followed closely with regular cardiopulmonary evaluation since their condition may deteriorate.     

Short- and long-term outcomes of combined cardiac and renal transplantation with allografts from a single donor

Coexisting end-stage heart and kidney failure can be treated by combined cardiac and renal transplantation. This study reviews the short- and long-term outcomes after such a procedure over a 16-year period at a single institution.All patients who underwent single-donor simultaneous heart and kidney transplantation during the period of March 1986 to April 2002 (including heart retransplantation) were included (n = 13). They were listed for combined heart and kidney transplantation as they fulfilled our criteria for irreversible end-stage organ failure. Retrospective review of patient data from the transplant database, patient case notes and post-mortem reports were carried out.The mean (SD) recipient age was 45 (12) years and there were 2 females. The mean pre-operative creatinine level was 724 (415) micromol/liter with 9 patients (69.2%) on continuous ambulatory peritoneal dialysis and 2 patients (15.4%) on hemodialysis prior to transplantation. The 30-day mortality rate was 15.4% (2 of 13). For surviving patients the mean creatinine level at hospital discharge was 158 (93) micromol/liter. The mean number of acute cardiac rejection episodes per 100 patient-days was significantly lower (p = 0.01) than that for the heart-only transplant group (n = 760) during the same period. The median (interquartile range) post-operative survival was 1,969 (620 to 3,468) days. The actuarial survival rates (95% confidence interval) at 1 and 10 years were 77% (54% to 100%) and 67% (40% to 94%), respectively, and were not significantly different from the isolated heart transplant population (p = 0.68). Only 1 episode of acute renal rejection was diagnosed on clinical grounds, which was treated accordingly. There was no renal allograft loss in the long-term survivors.Combined cardiac and renal transplantation with allografts from the same donor has acceptable short- and long-term outcomes for patients with coexisting end-stage cardiac and renal failure. This group of patients may also experience fewer acute rejection episodes post-operatively.     

Impairment of renal function following liver transplantation

BACKGROUND: Although renal insufficiency following liver transplantation is not infrequent, only limited reports describe the incidence and progression of the kidney disease. METHODS: This single-centre retrospective analysis after successful liver transplantation between January 1985 and March 2002 defined the baseline serum creatinine at 50 days after liver transplantation to represent the renal function. The primary end-point was an increase of serum creatinine by more than 50% above the baseline. RESULTS: Long-term data were available for 162 patients (84 women, 78 men) who received 167 liver transplants. The median serum creatinine level at 50 days after liver transplantation was 1.0 mg/dL (range 0.5-3.5 mg/dL). The median serum creatinine increased to 1.2 mg/dL (0.4-9.8 mg/dL) at the end of follow-up. Six patients (4%) experienced end-stage renal failure. Forty-one patients (25%) showed a 50% increase in the serum creatinine. Kaplan-Meier analysis revealed that 43% and 48% of patients had a deterioration of renal function at 10 and 15 years after liver transplantation, respectively. Patients at risk showed an increase of serum creatinine by 0.25 mg/dL/y. Only the recipient age was an independent risk factor for deterioration of renal function. CONCLUSIONS: Although there is a high risk for the impairment of renal function after liver transplantation, progression of renal disease is slow and rarely results in end-stage renal failure within 10-15 years. However, patients at risk should be identified early to prevent further decline in renal function.     

Heart transplantation in children after mechanical circulatory support with pulsatile pneumatic assist device.

BACKGROUND: Mechanical support with a pulsatile pneumatic ventricular assist device (VAD) is a complex rescue procedure performed in children with untreatable cardiogenic shock. Its impact on early and long-term survival after subsequent heart transplantation (HTx) remains to be determined. METHODS: We reviewed retrospectively the course of 95 children (median age, 8 years; range, 8 days-17 years; body weight, 24 kg; range, 3-110 kg) who underwent HTx. Group A, the elective-HTx group, consists of 33 children who were treated as outpatients before transplantation. Group B, the emergency-HTx group, has 44 children who were critically ill and hospitalized before transplantation but without ventricular assist devices, whereas Group C, the VAD-HTx group, consists of 18 children resuscitated and supported with pulsatile pneumatic VADs for a median time of 20 days. RESULTS: Overall actuarial survival after cardiac transplantation was 86% at 1 month, 82% at 1 year, and 78% at 5 years, without significant differences among the 3 sub-groups. Group A had the best long-term survival rate, 88% at 1 month, 88% at 1 year, and 80% at 5 years. Group B had a survival rate of 88% at 1 month, 82% at 1 year, and 79% at 5 years. Group C had a survival rate of 72% at 1 month, 72% at 1 year, and 72% at 5 years. We found no differences in neurologic outcome, acute cardiac rejection, or transplant failure. The survival rate was significantly better in the children with cardiomyopathy compared with those with congenital heart defects (p = 0.014). CONCLUSIONS: Bridging to HTx with a pulsatile pneumatic VAD is a safe procedure in pediatric patients. After HTx, overall survival of these children is similar to that of patients who were bridged with inotropes or who were awaiting heart transplantation electively.     

Human hepatocyte isolation and relationship of cell viability to early graft function

Hepatocyte transplantation is emerging as an additional modality of treatment for patients with acute liver failure or liver-based metabolic disorders. The procedure requires isolation of high-quality hepatocytes from unused donor livers. Hepatocytes were isolated from 20 donor livers (11 right lobes, 3 left lateral segments, 6 whole livers) using a collagenase perfusion technique. Cell viability (median 56%, range 13-95%) and yield (median 1.4 x 10(9) cells, range 2.0 x 10(6)-1.8 x 10(10) cells) varied according to the tissue available. Fatty livers rejected for transplantation gave lower cell viability (median 45%, range 25-59%). There was a significant correlation between age of donor (median 21 years, range 7-66 years) and viability of isolated hepatocytes in vitro (r = -0.683, p = 0.001). The 13 segments of livers were from reduced/split grafts used for clinical transplantation in 9 children and 4 adults. There was no significant correlation between in vitro cell viability and clinical parameters including intensive care stay, serum aspartate aminotransferase,and international normalized ratio (in the first 7 days), and allograft rejection or other early posttransplant complications, in patients transplanted with the corresponding tissue.