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Single Ventricle Babies: The Fontans Have Come of Age


Monica Horn, RN, CCRN, CCTC
mhorn@chla.usc.edu

Carolyn Buckley, MSW
cbuckley@chla.usc.edu

Helene Morgan, MSW
Children's Hospital Los Angeles
Los Angeles, CA, USA
hmorgan@chla.usc.edu



Transplant Coordinator

When earlier literature is compared with the more recent data describing staged surgical palliation for single ventricle infants, skilled surgical technique and precise medical management seem required for better survival. As these children have matured to adolescence, medical experience has revealed improved longevity and quality of life. Over time, these longer term observations have additionally revealed complications possibly associated with the Fontan physiology [1].

With increased access to fetal echocardiography, healthcare providers may now be able to counsel parents of single ventricle infants as early as the defect becomes evident. Treatment choices at time of diagnosis may include three main choices for parents of these children: comfort care, staged surgical palliation, or orthotopic heart transplantation. Depending therapy chosen, patients may be referred to selected experienced, larger volume programs that offer their chosen option and are successful in such interventions [2]. Centers skilled in surgical palliation may show preference for that pathway due to concerns related to transplantation such as limited donor availability and desire to avoid immunosuppression [3]. However, as Fontan patients age after all surgical palliations have been exhausted, referral for heart transplantation may once again be considered when heart failure or other associated complications demand a different course.

Decreased survival has been reported in heart transplant patients after the Fontan procedure, compared to those with other heart disorders [4]. Multiple previous chest surgeries and allo-sensitization may influence transplant candidacy and challenge management [5]. Other morbidities associated with passive, rather than pulsatile, blood flow of the Fontan circulation over extended time are: ventricular failure, liver disease, plastic bronchitis, protein-losing enteropathy (PLE), thrombosis/stroke, decreased oxygen saturations, and arrhythmias. Ventricular assist device support may not be successful or even offered due to single ventricle anatomical challenges and increased mortality risk.

Considering anatomic and physiologic challenges alone, the medical transplant evaluation for such a referral may prove to be quite complex. Add the nursing assessment and psychosocial preparation piece, and the undertaking becomes colossal.

For the failing Fontan, medical therapy may consist of referral to Transplant, Palliative Care or Both [6, 7]. Effective medical, nursing and psychosocial support for the failing palliated single ventricle patient and family must involve collaboration of the multidisciplinary team. Up to this point in their lives, Fontan patients have survived some very extreme life experiences including multiple chest surgeries, lengthy hospitalizations, frequent clinical monitoring and chronic illness states. They may bring a lot more "wounds" to the transplant evaluation than their old surgical incisions.

Transplant Social Worker

During the psychosocial assessment, a multitude of factors must be evaluated within a limited amount of time. These include patient's/family's cognitive understanding of transplant process, emotional readiness and motivation for transplant, adherence to medication regimen and treatment plan, adequate income and access to health care, and reliability of support system. Risk factors such as history of substance abuse, psychiatric illness, maladaptive coping and poor communication with medical team must also be taken into account. In the pediatric setting, it is imperative to consider the developmental stages of the child and provide age appropriate discussions and interventions.

During the evaluation phase, patients and parents are asked to make many important decisions within a short time frame. Parents have little time to process medical information while facing the unknown survival outcome of their child. The Transplant Social Worker and Palliative Care Social Worker can guide families through the decision making process by assisting family in clarifying their goals of care based on the family's values and beliefs. These interventions can be particularly helpful as the disease progresses or complications/risk factors arise.

Many pediatric patients and families will face a prolonged hospitalization following transplant listing. The unfamiliar hospital environment, multiple care providers, and the different routines in the CTICU can increase the patient's and family's anxiety. Supportive interventions for hospitalized children include creating a daily schedule, visits from hospital school teacher, and play therapy through Child Life Services, Music and Art Therapists to assist the child in coping. Social Workers can provide individual counseling to parents, referrals to community support services or parent to parent support networks, and suggest financial assistance programs to meet the short term needs during a lengthy hospitalization. Through frequent visits with the patient and family, social workers gain a clear understanding of the impact of the illness on the patient and family and can relay clinical impressions to the child's medical and nursing providers.

Palliative Care Social Worker

Palliative Care brings a unique perspective to how families cope and make medical decisions. Often mislabeled as the "death team," palliative care as a source of support for families and staff has become integrated into the transplant landscape. How and when they are introduced to the families varies from program to program, depending on many factors. Whether a baby is diagnosed prenatally or postnatally, and how the trajectory of their medical course is presented to the parents at the time of diagnosis, however, is a factor that is out of the control of the transplant team. In an ideal world, the palliative care team would be introduced at the time of diagnosis or soon after, to facilitate communication with the many teams involved and to assist with medical decision-making and symptom management.

When the palliative care team is introduced when transplantation becomes the treatment option, the palliative care team can be instrumental in providing additional support in ensuring that the family understands the information that has been presented, assessing the patient's and family's goals and hopes in the context of what quality of life means to them, and considering the needs of all family members in light of the changes that will impact each person post-transplantation. When there is seamless collaboration and communication between the transplant and palliative care teams, the potential for holistic care is maximized as patients, family members, and staff receive the tools that they need to cope adaptively through this transitional time.

Summary

It's truly amazing that surgical and medical advances have allowed many single ventricle patients' lifespans to be extended and quality of life to be improved. When referred to the transplant team, medical evaluation includes assessment for prediction of relative success or failure of this potential intervention so that the family and patient are able to make educated decisions. However, we must not be focused only on previous procedural histories and current anatomic and physiologic measurements.

Adolescent and adult congenital single ventricle patients have been coping with illness and advanced medical interventions their entire lives. If a candidate is referred late into the heart failure course, the multidisciplinary transplant team must mobilize and be prepared to intervene very quickly to complete the evaluation before more decompensation occurs. Earlier referral or consultation with Palliative Care prior to end-stage heart failure may allow the family and candidate more time for adjustment and thought, well before a formal transplant evaluation which demands full attention to education and training about transplant lifestyle expectations that ensure acceptable survival.

These patients have probably experienced many different healthcare team members in institutions and have adapted in various individual styles in order to tolerate much of it. Sometimes a professional self-reminder that the focus of healthcare providers' roles must be to ensure that families and patients receive adequate education, so that they may make their own choices, may prove helpful to avoid the pitfalls of complicating their decisions with our own personal views.

In addition to education and valuable supportive counseling for the patient and family, perhaps the busy medical, nursing and ancillary care teams who care for these patients would benefit from regular supportive solution-driven conferences aimed at enhancing their own skills for effectively working with complex patients who may experience emotional crises during the course of continued treatment. ■

Disclosure Statement: The author has no conflicts of interest to disclose.


References:
1. Simpson KE, Everitt MD. Fontan Associated Liver Disease: Reversible with Cardiac Transplantation, or "Past the Point of No Return?": ISHLT Links Sep 2013.
2. Karamlou T, Diggs B, Ungerleider R, et al. Evolution of treatment options and outcomes for hypoplastic left heart syndrome over an 18-year period. J Thorac Cardiovasc Surg 2010;139:119-27.
3. Bhama JK, Shulman J, Bermudez CA, et al. Heart transplantation for adults with congenital heart disease: Results in the modern era. J Heart Lung Transplant 2013; 32:499-504.
4. Simpson KE, Kirklin JK, Naftel DC, et al. Survival of Fontan Patients After Heart Transplant; Has Survival Improved in the Current Era? Circulation. 2014; 130: A16311.
5. Thrush PT, Hoffman TM. Pediatric heart transplantation-indications and outcomes in the current era. J Thorac Dis 2014; 6(8): 1080-1096.
6. Ellinger MK, Rempel GR. Parental Decision Making Regarding Treatment of Hypoplastic Left Heart Syndrome. NANN-Advances in Neonatal Care. 2010; 10 (6): 316-322.
7. AAP Committee on Bioethics and Committee on Hospital Care. Palliative Care for Children. Pediatrics. August 2000; 106 (2).




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