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Published on: July 18, 2014
Heart transplantation in special patient populations
Kathryn R Armstrong1, Anne I Dipchand2
1Heart Centre, British Columbia Children's Hospital, 4480 Oak Street, Vancouver, Canada, V6H3N1.
Insights
Heart transplantation (HTx) in special populations requires careful consideration of medical, developmental, and psychosocial factors. Evaluating these aspects ensures optimal outcomes and quality of life for children with genetic conditions, myopathies, and intellectual delays.
Area of Science:
- Pediatric Cardiology
- Transplant Surgery
- Genetics
Background:
- Heart transplantation (HTx) is increasingly considered for pediatric patients with end-stage heart failure.
- Special patient populations, including those with genetic conditions, myopathies, mitochondrial disorders, connective tissue disorders (CTDs), and intellectual developmental delay, present unique challenges for HTx.
- Determining the best interests of the child, encompassing medical, physical, emotional, developmental, and psychosocial aspects, is paramount.
Purpose of the Study:
- To review the indications for HTx in special pediatric patient populations.
- To explore outcomes and survival rates for these patients.
- To highlight key considerations for evaluating suitability and optimizing post-transplant care.
Main Methods:
- Literature review focusing on heart transplantation in pediatric patients with specific underlying conditions.
- Analysis of existing data on outcomes, survival, and complications.
- Discussion of multidisciplinary evaluation principles aligned with ISHLT guidelines.
Main Results:
- HTx evaluation for these populations requires a comprehensive assessment of cardiopulmonary status, comorbidities, and psychosocial factors.
- Specific conditions like mitochondrial disorders and Noonan syndrome may pose unique risks, including accelerated disease progression or increased post-transplant complications.
- Further research is needed to fully understand long-term outcomes in these complex cases.
Conclusions:
- A holistic, patient-centered approach is essential for heart transplantation in children with complex underlying conditions.
- Careful pre-transplant evaluation and ongoing monitoring are crucial for maximizing survival and quality of life.
- Addressing the specific risks associated with genetic conditions, myopathies, CTDs, and intellectual delays is vital for successful HTx outcomes.
Abstract:
In this review of heart transplantation (HTx) in special patient populations, the indications for HTx for patients with underlying genetic conditions, myopathies, mitochondrial conditions, connective tissue disorders (CTDs), and intellectual developmental delay will be explored along with the outcomes and survival. A key principle for cardiologists looking after these patients who may develop end-stage heart failure and require HTx evaluation is to determine what is in the child's best interest from a medical, physical, emotional, developmental, and psychosocial perspective with a goal of providing the child not only survival but an acceptable quality of life within their underlying circumstances. This review highlights that much like clinicians do for any pediatric patient put forward for HTx and as the International Society of Heart Lung Transplantation (ISHLT) endorses an evaluation of the patient's cardiopulmonary status, comorbidities and psychosocial factors that may affect graft/patient survival should be considered for patients with underlying genetic conditions, myopathies, CTDs, and intellectual delays prior to transplantation. More research is required to better understand the risk and long-term outcomes of HTx in those with underlying mitochondrial conditions where HTx has accelerated the progression of disease in other organ systems and in conditions such as Noonan's syndrome, where patients have a higher risk of developing post-transplant complications such as post-transplant lymphoproliferative disorder.
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