Pushing Boundaries in Paediatric Heart Transplantation

Cardiology in the Young, 2024

In the 1980s, heart transplantation was the first successful treatment for infants born with hypoplastic left heart syndrome. Infants who have required heart transplantation benefit from immunologic "advantages," including long-term survival free from cardiac allograft vasculopathy. Currently ~90% of children undergoing a heart transplant are reaching their first-year anniversary and the clinical practices of paediatric heart transplantation have dramatically improved. These successes are largely attributed to research sponsored by the Pediatric Heart Transplant Study Group, the International Society of Heart and Lung Transplantation and, more recently, the Non-profits Enduring Hearts and Additional Ventures. Despite these successes, the field is challenged to increase progress to achieve longterm survival into adulthood. The wait-list mortality, especially among infants, is unacceptably high often leading to palliative measures that can increase post-transplant mortality. Cardiac allograft vasculopathy remains a major cause for progressive graft loss of function and sudden death. The relative tolerance seen in immature recipients has not been translated to modifying older recipients' post-transplant outcomes. The modifiable cause(s) for the increased risks of transplantation in children of different ethnicities and races require definition. Addressing these challenges faces the reality that for-profit research favours funding adult recipients, with ~10-fold greater numbers, and their more modest longevity goals. Advocacy for funding "incentives" such as the Orphan Drug rules in the United States and upholding principles of equity and inclusion are critical to addressing the challenges of paediatric heart transplant recipients worldwide.

Circulation, 2007

Revista chilena de pediatria, 2017

Pediatric heart transplantation is an effective therapy to treat advanced heart failure in children. To analyze the immediate and mid-term results of pediatric patients listed for heart transplantation. Registration of patients admitted to our transplant protocol between October 2001 and July 2016 were reviewed, analyzing demographic data, diagnosis, status at the time of listing, waiting time until transplantation, donor data, use of ventricular assist device, hemodynamic data, complications and global mortality. Thirthy patients where included with a mean age of 9.4 years (1 month to 15 years). The most frequent diagnosis was dilated cardiomyopathy in 24 patients (80%). The status was I (urgency) in 19 cases and II in 11 cases. Ten patients died on the waiting list (33.3%) at an average of 52 days (13-139 days). Fourteen were transplanted (46.7%), with a waiting time of 199.6 days (4-586 days). Nine patients required mechanical support (30%). All patients received triple associati...

Paediatrics and Child Health, 2011

Paediatric heart transplantation is the last remaining treatment option for children in end-stage heart failure. Approximately 30 heart transplants are performed in the UK each year, between two specialist centres. Approximately two-thirds of recipients have suffered from a cardiomyopathy (mainly dilated), with the remainder mainly being born with congenital heart disease that is, or has become unamenable to conventional surgery. Over the course of the last 20 years, improvements in surgical expertise, intensive care techniques and immunosuppressive strategies have vastly improved the outlook for these children, and it is predicted that the majority of grafts implanted today will last at least for 15 years. This inevitably means that patients transplanted as young children will require retransplant during adolescence or early adulthood, and with organ donation in the UK currently at severely low levels, the current chances of a second transplant are poor. The goals of transplant programmes must therefore be to postpone transplantation as long as possible and careful management of the post-transplant phase, in order to improve the longevity of donated organs. This article reviews the current practice of paediatric heart transplantation in the UK.

Pediatric Cardiology and Cardiac Surgery, 2018

Heart transplant remains an important treatment option for end-stage heart failure in children who have failed maximum medical management. Although the outcome of heart transplant has significantly improved due to advances in perioperative management and immunosuppression, commonly, it is not a permanent solution. We still encounter multiple problems in managing these patients before and after transplant, not only with hemodynamic derangement, but also with functional deterioration of multiple organ systems. Shortage of donor hearts in association with wait-list mortality remains a major ongoing problem, especially for infants. Importantly, transplant for patients with congenital heart disease has unique challenges, including complexity of surgical reconstruction, coagulation abnormalities, allosensitization, and specific problems related to single ventricular palliation. Even after successful transplant, chronic complications emerge as inevitable challenges, including rejection, in...

Circulation, 2014

The Thoracic and Cardiovascular Surgeon, 2007

Objective: Early and late mortality have significantly improved during recent decades in pediatric patients after heart transplantation (HTx). Nevertheless early and late morbidity and mortality are influenced by acute rejection, cardiac allograft vasculopathy (CAV), malignancy, renal failure, and graft failure. Methods: We evaluated our results after HTx in children under the age of 18 years with 23 years of follow-up. Perioperative characteristics, probability of survival, and time-related morbidity were retrospectively analyzed. Results: We included 169 pediatric HTx recipients, transplanted between 05/1986 and 05/2010. One hundred and one were males with a median age of 8.7 (0.02-23.2) years at the time of HTx. Main preoperative diagnoses were cardiomyopathy (CMP) (n = 139) with a median survival of 7.0 (0-23.2) years and congenital heart disease (CHD) (n = 30), median survival 11.3 (0-19.9) years. Overall survival at 1, 5, 10, and 15 years was 87%, 76%, 68%, and 50%, respectively. Patient survival was significantly reduced in patients with 0-1 year at the time of HTx versus 1-10 and 11-18 years: 2.3 (0-13.2) years versus 1-10 years = 8.6 (0-23.2) years; 11-18 years = 5.9 (0.003-18.5) years. Fifty-one patients were on mechanical circulatory support as a bridge-to-HTx with increased early but not late mortality. Ten patients underwent retransplant due to acute or chronic graft failure after a median posttransplant time of 12.25 (0.3-17.45) years. Late mortality was influenced by rejection, infection, posttransplant lymphoproliferative disease (PTLD) (11.8%), or CAV with an incidence of 25% at 5 years, 50% at 10 years, and approximately 75% at 15 years. Conclusions: Pediatric HTx is a safe and effective treatment for terminal heart failure. In our experience, there is no adverse effect of previous cardiac assist device implantation in long-term follow-up. Virtually all anatomic malformations are amenable to orthotopic HTx. Significant progress has been achieved in controlling rejection through improved immunosuppression and noninvasive rejection monitoring. #

Journal of Cardiac Surgery, 2008

Heart failure has been recognized as a major public health problem. Its incidence and prevalence is increasing and imposes substantial burden on the health care system. Despite much progress in development of many new drugs and innovations in palliative surgical strategy, nontransplant cardiac surgical procedures and the use of mechanical assist devices, pediatric heart transplantation remains the best treatment option for patients with end-stage heart failure. So far, more than 6000 pediatric heart transplantations have been performed worldwide. This article reviews some clinical aspects of pediatric heart transplantation, including the history, indications and contraindications, donor evaluation and recipient management, surgical techniques, risk factors of mortality, and survival of pediatric heart transplantation. The short-and long-term outcomes of pediatric heart transplantation are encouraging. However, the lack of donor hearts still hampers its clinical application.

Seminars in Thoracic and Cardiovascular Surgery, 2004

Pediatric cardiac transplant patients present many challenges to the medical community. These include such things as complex evaluations, preoperative heart failure support, complex operative interventions, and postoperative challenges in management. In spite of these challenges, survival outcomes for children undergoing a heart transplant have improved dramatically over the last two decades. Semin Thorac Cardiovasc Surg 16:404-409