The Registry of the International Society for Heart and Lung Transplantation: fourth official pediatric report—2000

doi:10.1016/S1053-2498(00)00243-6

The Journal of Heart and Lung Transplantation

Volume 20, Issue 1, January 2001, Pages 39-52

https://doi.org/10.1016/S1053-2498(00)00243-6 Get rights and content

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Data set and statistical methods

The fourth official pediatric report—2000 includes in its analysis 4,644 heart, 433 heart/lung, and 586 lung (single or double) transplantations performed through most of 1999. We are still collecting complete data on the patients transplanted in 1999. The centers that have contributed to this important data set include 226 heart, 67 heart/lung, 39 single lung, and 59 bilateral/double lung pediatric transplantation centers. We used the statistical methods described in the second official

Heart transplantation

The number of pediatric heart transplantations has plateaued since about 1996. As shown in Figure 1, the number of heart transplant procedures performed in 1999 should approximate the number performed in 1998 when the data set is complete. The shaded bars in Figure 1 show the absolute number of heart transplantation procedures, whereas the solid line indicates the number of centers. In 1994, the number of transplant centers that reported pediatric heart transplantations peaked, and a downward

Lung transplantation

In 1999, the number of centers performing lung transplantation plateaued. The actual number of lung transplant procedures appears to have decreased. Figure 15 shows the total number of pediatric lung transplants (vertical bars) by year. The solid line represents the total number of centers that reported transplant activity (n = 24). Figure 16 displays the indications for pediatric lung transplantation by age of the recipient. For infant recipients, congenital lung abnormality was the most

Heart/lung transplantation

The absolute number of patients who received heart/lung transplantation plateaued at around 20 patients per year, with 10 or fewer centers reporting pediatric heart/lung transplantations each year. Figure 24 provides a graphic display of the actual number of heart/lung transplants performed (shaded bars), and the number of centers (solid line) who performed them.

Figure 25 shows the indications for pediatric heart/lung transplantation in the 1- to-10-year-old age group as an overall percentage

Conclusion

The fourth official report of the pediatric registry, a component of the overall Registry of the International Society of Heart and Lung Transplantation, continues to document improving survival following heart transplantation. In contrast, the registry has noted no improvement in survival for lung and heart/lung recipients. The overall number of pediatric thoracic transplant recipients has remained stable for the past 3 years. As noted in previous reports, the number of centers that perform

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References (3)

J.D Hosenpud et al.
The Registry of the International Society for Heart and Lung Transplantationseventeenth official report—2000
J Heart Lung Transplant
(2000)

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From partnership to progress in the field of pediatric cardiomyopathy
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Future research directions in pediatric cardiomyopathy
2016, Progress in Pediatric Cardiology
Citation Excerpt :
Cardiomyopathy is a rare but serious disorder of the heart muscle, resulting in some of the worst pediatric cardiology outcomes, and it remains the leading cause of heart transplantation in children over one year of age [1–5]. Nearly 40% of children who present with symptomatic cardiomyopathy receive a heart transplant or die within the first 2 years, and the percentage of children receiving transplants has not declined over the past 10 years [5,6]. Studies in both the United States and Australia have shown that the incidence of pediatric cardiomyopathy is approximately 1 per 100,000 children and 8–12 times higher in the first year of life, making this disorder as prevalent as childhood cancers such as lymphoma, Wilms' tumor, and neuroblastoma [7,8].
Wearable cardioverter-defibrillator as a bridge to cardiac transplantation: A national database analysis
2015, Journal of Heart and Lung Transplantation
Life-threatening ventricular arrhythmias (VAs) and sudden cardiac death (SCD) are common in patients awaiting heart transplantation (HT), and the implantable cardioverter-defibrillator (ICD) is often used for primary prevention in this setting. Use of ICDs in these patients is not without risks and is sometimes contraindicated. The wearable cardioverter-defibrillator (WCD) may be a reasonable alternative to bridge the period of risk leading up to HT.
We obtained a convenience sample of patients prescribed an WCD as a bridge therapy to HT. The available data consisted of demographics, cardiac transplantation status, associated comorbidities, device use, device-stored electrocardiogram (ECG) and reason for discontinuing the WCD. Statistical analyses were performed using SPSS version 17 and GraphPad PRISM 5.
The registry included 121 patients consisting of 83 (69%) men and 38 (31%) women. The mean age was 44 ± 18 years. Mean ejection fraction was 25 ± 15%. Non-ischemic cardiomyopathy (CMP) was the underlying diagnosis in 67 (55%) patients, whereas 21 (17%) patients had ischemic CMP and 33 (27%) had a mixed or uncharacterized CMP. New York Heart Association Class III heart failure was present in 32% and 34% were in Class IV. Eighty-eight patients (73%) were being evaluated for HT or were on an HT waiting list, and 33 patients (27%) had had a prior HT, experienced rejection, and were awaiting re-transplantation. The patients wore the WCD for an average of 127 ± 392 days (median 39 days) with average daily use of 17 ± 7 hours (median 20 hours). Seven patients (6%) received appropriate WCD shocks. Fifty-one patients (42%) ended use after ICD implantation and 13 patients (11%) after HT. There were 11 deaths (9%).
A significant proportion of patients on the HT waiting list will have VA. WCD use in our study showed high compliance and efficacy and a low complication rate, suggesting that the WCD is a reasonable bridge therapy for preventing SCD in patients awaiting HT.
The promise of cardiovascular biomarkers in assessing children with cardiac disease and in predicting cardiovascular events in adults
2011, Progress in Pediatric Cardiology
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Time-to-transplant or death for children with cardiomyopathy has not improved during the past 35 years, and outcomes in the most developed nations are no better than those in developing nations [3–5]. The percentage of children with cardiomyopathy who received a heart transplant has not declined over the past 10 years, and cardiomyopathy remains the leading cause of heart transplantation in children over 1 year of age [6]. Heart failure is characterized by an inflammatory response that is associated with both disease severity and prognosis [7,8].
Cardiac and vascular biomarkers are used in the diagnosis, assessment, and prognosis of a variety of cardiovascular diseases in adults and children. For children, cardiac biomarkers are most often used to assess cardiomyopathy or congenital cardiovascular malformations or for monitoring during cardio-pulmonary bypass and pre- and post-heart transplantation. Because the risk for cardiovascular events in adulthood can be tracked from childhood, interest is high in identifying biomarkers of vascular inflammation in children that might also predict atherosclerotic events later in life. Here, we review the most common and promising biomarkers and comment on their current and potential uses in monitoring and predicting cardiovascular dysfunction.
Cardiomyopathy in childhood
2009, Paediatrics and Child Health
Citation Excerpt :
Although rare, the importance of cardiomyopathies lies in the fact that around one-third of cardiomyopathies will progress to death or require cardiac transplantation. Cardiomyopathies are the most common reason for cardiac transplant in childhood.1 Although major advances have been made in the management of childhood heart disease, there has been only minimal change in outcome over the past 30 years for those with cardiomyopathies.
Cardiomyopathies are a heterogeneous group of disorders of both genetic and non-genetic aetiology. Their importance lies in the fact that although the mortality for structural congenital heart disease has been reduced from around 40% to 5% in the past 40 years there has been little change in the prognosis for paediatric patients with cardiomyopathies. Around one-third of children with a cardiomyopathy will die or require transplantation. Advances in genetics over the past decade have resulted in the recognition that a large proportion of cardiomyopathies are genetic in origin. Management of these patients is best in centres offering cardiology, genetic and metabolic input. This article aims to briefly review the broad categories of cardiomyopathies encountered in childhood.
Pediatric cardiomyopathy as a chronic disease: A perspective on comprehensive care programs
2008, Progress in Pediatric Cardiology
Citation Excerpt :
The Pediatric Cardiomyopathy Registry (PCMR) reports survival rates after a diagnosis of dilated cardiomyopathy [2] with freedom from death or transplantation ranging from 69% at 1 year to 46% at 10 years and for idiopathic hypertrophic cardiomyopathy [3] 94% at 1 year to 85% at 10 years (Table 1). Cardiomyopathy is the leading cause of transplantation in children older than 1 year of age, and the percentage of children with cardiomyopathy who receive heart transplants has not declined over the past 10 years [4]. In 1993, the American Heart Association (AHA) reported that pediatric cardiovascular disease cost more than $8 billion per year in medical expenses and lost contributions to the US gross national product [5].
Substantial numbers of children with cardiomyopathy are now surviving into adulthood, making it essentially a chronic disease. As a chronic condition, it may be best treated through comprehensive, multidisciplinary treatment programs. Such programs have improved health outcomes and reduced costs in managing other pediatric chronic diseases and heart failure in adults, but the treatment and cost implications of programs for managing pediatric cardiomyopathy are unknown. We investigated the treatment and cost implications of establishing such programs by reviewing cost-effectiveness studies of similar programs, estimating the current inpatient costs of this diagnosis, and interviewing experts in the field about the need and desirability of these programs. According to our findings, comprehensive pediatric heart failure programs do exist, but they have not been evaluated or even described in the literature. Consensus among experts in the field is that comprehensive chronic care programs are highly desirable, and similar programs have reported tremendous cost savings through early and intensive management: the return on investment has been as high as 22 to 1. Another study reported that mean length of stay decreased from 83.9 to 10.6 days, mean annual admissions decreased from 2796 to 1622, and median hospital charges decreased from $26.1 million to $14.6 million. In conclusion, limited experience and strong circumstantial evidence suggest that, despite substantial costs, comprehensive multidisciplinary pediatric heart failure programs would be cost-effective and beneficial to patients, families, and institutions alike.

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Registry reportThe Registry of the International Society for Heart and Lung Transplantation: fourth official pediatric report—2000