The waiting game: bridging to paediatric heart transplantation.

BACKGROUND: Although mechanical circulatory support might not increase the number of adults surviving to transplantation, because of the shortage of donor organs, the situation might be different for children. Our aim was to assess the effect of mechanical assist devices to bridge children with end-stage cardiomyopathy to heart transplantation. METHODS: A 5-year retrospective review was undertaken with data from the UK paediatric transplant programme and from bridging to transplant done at two paediatric transplant centres in the UK. FINDINGS: Between Jan 1, 1998 and Dec 31, 2002, 22 children with end-stage cardiomyopathy, median age 5.7 years (range 1.2-17), were supported by a mechanical assist device as a bridge to first heart transplantation, with a 77% survival rate to hospital discharge. Nine were supported by a paracorporeal ventricular assist device, six received transplantation, five survived to discharge (55%), with one late death. 13 were supported by extra-corporeal membrane oxygenation, and 12 were transplanted and survived to discharge (92%) with one late death. With urgent listing, the median waiting time for a heart was 7.5 days (range 1.5-22 days). The correlation between the proportion of patients bridged to transplantation and the proportion of patients dying while on the transplant waiting list was r=-0.93, p=0.02. INTERPRETATION: Our findings lend support to the hypothesis that a national mechanical assist programme to bridge children to transplantation can minimise the number dying while on the heart transplant waiting list. In the context of urgent listing and a short waiting time, extra-corporeal membrane oxygenation seems to provide the safest form of support.

Improved early outcome for end-stage dilated cardiomyopathy in children.

OBJECTIVE: To review the impact of management changes on the early outcomes of end-stage dilated cardiomyopathy in children. METHODS: We conducted a retrospective study of all consecutive children with end-stage dilated cardiomyopathy who received hospital treatment since 1992. Over the past 3 years the following management changes were made: (1) more aggressive use of mechanical cardiac assistance; (2) high priority listing for transplantation; and (3) ABO incompatible transplants for infants. Outcomes for 46 patients admitted between 1992 and 1999 (group I) were compared with 53 patients between 2000 and March 2003 (group II). RESULTS: In group I, 12 (26%) patients received mechanical support with recovery in 3 and transplantation in 5 (1 died). In group II, 19 (36%) patients received extracorporeal membrane oxygenation, with recovery in 5 and transplantation in 12 (all survived). The use of mechanical assistance was associated with high morbidity related to bleeding, end-organ failure, and long-term mechanical ventilation. Five patients in group II received ABO incompatible transplants and all survived. There have been no episodes of rejection or need for increased immunosuppressive therapy. Hospital mortality has been significantly reduced (group I, 37% vs group II, 11%; P <.05). CONCLUSIONS: Recent refinements in the management of end-stage dilated cardiomyopathy in children have significantly reduced early mortality. Identification of markers of early myocardial recovery and development of mechanical devices for longer term and more physiologic support are essential to achieve further improvements in outcome.

Depression in pediatric patients before and 1 year after heart or heart-lung transplantation.

BACKGROUND: Although depression is a significant psychiatric condition of childhood and adolescence and those with a chronic medical problem are at increased risk for developing depression, the prevalence of depression in children and adolescents undergoing heart and heart-lung transplantation has not been addressed. However, the role of psychosocial factors, such as mood state, in determining outcome is being increasingly acknowledged. METHODS: The Mood and Feelings Questionnaire, a rating scale of depressive symptoms, was administered to 58 children before transplantation and to 46 children after transplantation, with 24 completing both assessments. To compare children's and parents' ratings, both child and parent versions, with corresponding items, were utilized. RESULTS: Pre-transplant, the mean score on the child measure was significantly higher (p < 0.001) than that on the parent measure, with 24% and 21% on the child and parent measures, respectively, obtaining scores indicative of depression. Parents of children with acquired heart disease rated their children as having significantly more depressive symptoms than parents of children with congenital heart disease or cystic fibrosis. After transplantation, there was a reduction in mean scores on both the child and parent questionnaires and the pre-transplant differences between the different diagnostic groups were no longer apparent. Over time the numbers obtaining scores indicative of depression decreased by approximately 50%. CONCLUSIONS: Transplantation is associated with a reduction in the prevalence of depressive symptomatology. The role of original diagnosis in the manifestation of depression both before and after transplantation requires further investigation.

Long-term results of cardiac transplantation from live donors: the domino heart transplant.

BACKGROUND: Hearts explanted from the recipients of heart-lung transplants provide a unique source of transplants from live donors. This article presents long-term results with this procedure at our center. METHODS: We performed a retrospective chart review of domino transplantations performed in our institution between 1989 and 1998. RESULTS: We analyzed 131 domino transplants (123 orthotopic, 8 heterotopic). Domino hearts were from patients with cystic fibrosis (69%), primary pulmonary hypertension (15%), and other diagnoses (16%). The mean recipient pulmonary vascular resistance (PVR) was 3.1 Wood units, 25% of patients having values >4 Wood units. Thirty-day mortality was 13%. The 1-, 5-, and 10-year graft survival was 75% (70% confidence interval [CI], 65-74), 70% (70% CI, 65-74), and 58% (70% CI, 52-64), respectively. Patients with PVR >4 Wood units had 1-year survival (76%; 70% CI, 69-84) similar to that of patients with PVR of < or =4 units (74%; 70% CI, 69-80). Recipients of hearts from patients with cystic fibrosis survived longer (5-year survival, 76%; 70% CI, 71-82) vs 65% for non-cystic fibrosis hearts (70% CI, 57-74) p = 0.09). One-year survival was decreased after transplantation of hearts from female donors (66%; 70% CI, 60-72)) compared with hearts from male donors (85%; 70% CI, 79-90); p = 0.06). Late deaths caused by coronary artery disease and malignancy were uncommon. CONCLUSION: Although the rate of early mortality after domino transplantation was slightly higher than after cadaveric transplantation, we noted a remarkably low long-term attrition rate in recipients of domino grafts, up to 10 years. In addition, successful transplantation of patients with high PVR supports the hypothesis that heart-lung recipients may provide superior donor hearts for this patient group, many of whom traditional listing criteria would exclude.

Outcome in adult patients after arterial switch operation for transposition of the great arteries.

BACKGROUND: The arterial switch operation (ASO) is currently the treatment of choice in neonates with transposition of the great arteries (TGA). The outcome in childhood is encouraging but only limited data for long-term outcome into adulthood exist. METHODS AND RESULTS: We studied 145 adult patients (age>16, median 25 years) with ASO followed at our institution. Three patients died in adulthood (mortality 2.4/1000-patient-years). Most patients were asymptomatic and had normal left ventricular function. Coronary lesions requiring interventions were rare (3 patients) and in most patients related to previous surgery. There were no acute coronary syndromes. Aortic root dilatation was frequent (56% patients) but rarely significant (>45 mm in 3 patients, maximal-diameter 49 mm) and appeared not to be progressive. There were no acute aortic events and no patient required elective aortic root surgery. Progressive neo-aortic-valve dysfunction was not observed in our cohort and only 1 patient required neo-aortic-valve replacement. Many patients (42.1%), however, had significant residual lesions or required reintervention in adulthood. Right ventricular outflow tract lesions or dysfunction of the neo-pulmonary-valve were frequent and 8 patients (6%) required neo-pulmonary-valve replacement. Cardiac interventions during childhood (OR 3.0, 95% CI 1.7-5.4, P<0.0001) were strong predictors of outcome (cardiac intervention/significant residual lesion/death) in adulthood. CONCLUSIONS: Adult patients with previous ASO remain free of acute coronary or aortic complications and have low mortality. However, a large proportion of patients require re-interventions or present with significant right sided lesions. Life-long cardiac follow-up is, therefore, warranted. Periodic noninvasive surveillance for coronary complications appears to be safe in adult ASO patients.

Two generations of identical twins with ELN deletion.

We report a family with three generations of an ELN deletion. The grandfather was normal except for two inguinal herniotomies. The first generation identical twins had supravalvular aortic and multiple peripheral pulmonary artery stenoses. The second generation twins died during the neonatal period of myocardial infarcts.

Prospective psychological evaluation of pediatric heart and heart-lung recipients.

OBJECTIVE: To study psychological adjustment in children and adolescents before undergoing heart or heart-lung transplantation and to reevaluate them at 6, 12, and 24 months after surgery. METHODS: Previously validated measures of behavior and depression were administered to children, their parents, and teachers. Parents also completed a measure of distress. RESULTS: Before transplantation, participants and parents obtained significantly higher scores on measures of behavior and psychological distress than children with no health problems and their parents, but 1 year after transplant, there were no significant differences between the two groups. Over time, there were significant improvements in depression and behavior scores in the group who had undergone transplantation. CONCLUSION: Despite improvements in psychological functioning over time, a significant minority of children and adolescents undergoing transplantation are at risk for psychological adjustment difficulties. Psychological interventions to reduce morbidity need to be implemented and evaluated.

Bridge to transplantation with a left ventricular assist device for systemic ventricular failure after Mustard procedure.

After Mustard or Senning procedures, transplantation remains the only option for some patients who present at late stage with severe systemic (right) ventricular failure. In some circumstances these patients may require urgent mechanical circulatory support to bridge them to transplantation. The use of mechanical support poses considerable potential and actual specific problems both in terms of insertion and management of the device. We report the case of a 17-year-old patient who had a left ventricular assist device implanted from the right ventricle to the aorta for "end-stage" heart failure 15 years after the Mustard procedure. The specific problems are discussed and a management scheme is proposed.

Longitudinal assessment of psychological functioning in children after heart or heart-lung transplantation.

BACKGROUND: Despite the increasing numbers of pediatric heart and lung transplants being performed worldwide, longitudinal psychological evaluation of children and adolescents undergoing transplantation remains uncommon. The objective of this study was to assess psychological functioning in a group of patients at 12 months and 3 years after transplantation. METHODS: Thirty-four children and adolescents (mean age at 12-month assessment: 7.9 years [range 1.3 to 15.3 years]) were evaluated after heart (n = 24) or heart-lung (n = 10) transplantation for congenital heart disease (CHD; n = 10), cystic fibrosis (n = 1), cardiomyopathy (CM; n = 21) or primary pulmonary hypertension (n = 2). Standardized measures were used to assess development, cognitive function, mood state and behavior at each test occasion. RESULTS: Measures of developmental, cognitive and academic function were within the normal range with each test and showed stability over time. About 33% of patients had behavior problems at each test occasion, which is higher than the 10% reported for the normal population, but the prevalence of depression fell from 23% at 12 months to 13% at 3 years. Although there were no significant differences between heart and heart-lung recipients, children with a pre-transplant diagnosis of CHD had poorer scores on cognitive and behavioral parameters than those with CM. In particular, while the prevalence of behavior problems showed a slight decrease over time in the CM group, it increased from 33% at 12 months to 75% at 3 years in the CHD group. CONCLUSIONS: A number of pediatric patients continue to have psychological difficulties 3 years after transplant. Initial diagnosis is an important factor in post-transplant psychological functioning, with a diagnosis of CHD appearing to be a risk factor for greater psychological morbidity, at least in the short and medium term. Further follow-up must address whether such differences persist in the longer term. Patients at risk for poorer psychological outcome need to be identified so that interventions can be implemented to reduce psychological morbidity.

Adherence in adolescents and young adults following heart or heart-lung transplantation.

To assess the prevalence and some potential correlates of non-adherence to medications in adolescent and young adult transplant patients. Fifty patients who had undergone heart or heart-lung transplantation 1.4-14.9 yr (mean 8.8 yr) previously completed the Beliefs about Medication Questionnaire (BMQ), Perceived Illness Experience (PIE) scale and a demographics questionnaire. Medical notes were reviewed for information regarding previous psychiatric referral, rejection episodes and complications and noted concerns about adherence. Forty (80%) completed questionnaires were received. Non-adherence determined from the questionnaires was associated with forgetting to take medication and was classified as unintentional non-adherence. Such non-adherence was reported by 11 (28%) patients. Seven patients (18%) showed evidence from their records of deliberate non-adherence, which was classified as intentional. Whilst intentional non-adherence was associated with depression and transplant-related lymphoma, unintentional non-adherence and perceived difficulties with medications were associated with high scores on the PIE preoccupation with illness and BMQ concerns subscale and with drinking alcohol. Future research is required to determine whether unintentional non-adherence results in significant medical complications in the longer term and how a reduction in the prevalence of non-adherence can be facilitated.

Beyond the first year after pediatric heart or heart-lung transplantation: Changes in cognitive function and behaviour.

With the increasing use and improved survival rates of heart and lung transplantation as treatments for children with end-stage heart or lung disease, attention is focusing on the longer term psychological implications of these procedures. This paper focuses on the changes in cognitive development and behaviour in a group of 47 children who were seen 12 months and 2 yr after transplantation. There were 24 boys and 23 girls, mean age at transplantation was 8.3 yr (s.d. 5.3 yr), with a range of 0.3-15.1 yr. Assessments were made of developmental level, cognitive ability and problem behaviours, using previously validated measures, and comparisons were made with physically healthy children. For children under three and a half years of age there was a decrease over time in scores on all developmental parameters, with the change reaching significance on the scale assessing eye-hand coordination and on the overall IQ. Whilst all scores were within the normal range, they were at a significantly lower level than those of the healthy children. In contrast, there were no changes over time on any measures of cognitive or academic ability for older children, with correlations between 12 month and 2 yr scores being highly significant. The rate of behaviour problems at home at 12 months was 22%, compared with 34% at 2 yr post-transplant, which was higher than that found in the healthy children. Conversely, there was a drop in the prevalence of behaviour problems at school from 23% at 12 months to 9% at 2 yr. It is concluded that a significant minority of children and adolescents experience psychological difficulties 2 yr after transplant, with particular areas of concern focusing on development in the younger children and the occurrence of behaviour problems at home across the age-range.

Developmental and behavioral status of infants and young children awaiting heart or heart-lung transplantation.

OBJECTIVE: Research into cognitive development and behavior in children undergoing heart or heart-lung transplantation has focused mainly on older children, with investigation of children undergoing transplantation as infants or toddlers being largely retrospective. This study was conducted, therefore, to obtain pretransplant baseline measures of development and behavior for preschool-aged children. METHODS: Children <3.5 years old were assessed before transplantation (n = 35) and compared with a group of children awaiting conventional cardiac surgery, a group undergoing bone marrow transplantation, and a group of healthy children. Development was measured by using the Ruth Griffiths Mental Development Scales, and behavior was measured with the Achenbach Child Behavior Checklist. RESULTS: Within the transplant group, 17 had congenital heart disease (CHD), and 18 had cardiomyopathy (CM). Although the overall mean developmental scores were within the normal range for the transplant, conventional cardiac surgery, and bone marrow transplantation groups, scores were significantly lower than those of the healthy group. Within the transplant group, those with CHD had a significantly lower mean developmental quotient than those with CM. Furthermore, the CHD patients obtained significantly lower scores than those with CM on areas of development covering locomotor abilities, speech and hearing, eye-hand coordination, and performance. CONCLUSIONS: In common with other groups of ill children, patients awaiting heart or heart-lung transplantation are at risk for developmental delay. Diagnosis is a salient factor in determining outcome in most areas of development. Psychosocial interventions need to be targeted to maximize developmental potential before transplantation.

Pediatric cardiothoracic domino transplantation: the psychological costs and benefits.

The first domino transplants were carried out in the UK in 1987, since which time 52 such procedures have been carried out involving patients within the paediatric cardiothoracic transplant programmes of Harefield and Great Ormond Street Hospitals. Although there are medical advantages in using domino organs--such as the ability for preoperative cross-matching, the heart not being subjected to the biochemical changes of brain death and less post-transplant coronary artery disease in the recipients of domino hearts compared with the recipients of hearts from cadaveric donors--the psychological sequelae for both donor and recipient have not been previously studied. The objective of this study was to identify the main psychological themes for patients involved in the domino programmes at the two hospitals, focusing on those situations where both patients were cared for in the same tertiary centre. Patients and their families were interviewed during routine outpatient clinic visits. Negative themes identified by patients included anxiety, guilt, resentment and anger if either patient had a poor outcome or suffered significant complications, disappointment and low self-esteem for potential donors whose heart was not used and recipient awareness of donor characteristics. Positive themes included gratefulness, comfort for the recipient that someone had not had to die for them directly and the benefit to the donor of giving their heart to another patient. In conclusion, domino transplantation has many medical advantages but there are significant negative psychological concomitants which need to be addressed within the multi-disciplinary management of these patients.