Psychosocial Outcomes: New Horizons in Medical and Surgical Fontan Management...

Psychosocial functioning is the interplay between psychological factors (internal processes including neurocognitive functioning, cognitive and intellectual functioning, executive functioning, mood, anxiety, temperament, and personality factors) and social factors (levels of social support including family supports, school supports for children, employment, social interactions with peers, community involvement, and areas where they may feel productive in their social roles).

The presence of a congenital heart defect, especially a Fontan, often results in enormous emotional and financial strain on families, with continual effects from ongoing medical care and routine disruptions.

Early life experiences of families with Fontan patients have profound impact and these experiences resonate across a lifetime.

Children with complex CHD are at risk of neurological and cognitive impairment, and Fontan patients may be especially vulnerable.

A multidisciplinary clinic at Children's includes psychology, developmental pediatricians, cardiologists, social work, education consultant, OT, nutrition, and speech and language professionals, focusing on evaluation, consultation, and coordination of care for especially vulnerable children followed into adulthood.

As adults, Fontan patients remain at risk for significant sequelae and face significant and very unique life stressors that place them at increased risk for ongoing distress.

Typical neurodevelopmental issues in Fontan patients include lower IQ, difficulty with math and language-based academic achievement, impairments in expressive and receptive language skills and written expression skills (including organization and planning of expression), executive functioning deficits (behavioral and metacognitive), flexible attention shifting problems (distractibility and hyperfocus), poor planning and organization problems, difficulty with time management, diminished fine and gross motor skills, working memory issues, and slowed processing speed.

A very high number of Fontan patients are left-handed (anecdotal observation).

Approximately 1 in 3 adults with CHD in North America experience difficulty with depression and/or anxiety.

Adolescents with single ventricle congenital heart disease have a 5-fold increase in rates of anxiety disorders as well as ADHD compared with their healthy control peers.

Adults with CHD may have significantly higher risk of PTSD than in the general population.

The chronic impact over time of disruption contributes to PTSD risk, and parents early on also show signs of PTSD.

Mitigating psychosocial factors for adults with Fontan include differences in body image or perceptions of scarring, perceived health status or severity of disease, poor social support or social anxiety, performance anxiety, tendency to compare themselves and feel different than others with difficulties in social situations, communication skill deficits extending into adulthood, lack of awareness of how these factors impact self-adjustment and ability to interact with others and coping skills, perceived or real lack of independence, impulsivity or poor problem solving, ongoing difficulty maintaining employment for physical or psychological reasons, and contributing financial strain.

The case patient is a 15-year-old female with complex medical history resulting in a Fontan, who had feeding difficulties early on requiring a G-tube in early childhood.

Extra medical requirements such as G-tubes can contribute to changes in parent-child interactions and family dynamics.

The case patient has an extensive medication regimen, could verbalize her medication list, was very aware of all medications, and was reported to take them consistently; on adaptive scales, self-care and health management was really high.

Children and young adults who tend to be anxious do a really nice job of taking care of their healthcare regimen and are very hypervigilant about that area.

The case patient lives in a rural area with her mother and 16-year-old brother; parents are divorced (contentious early on), and she does not have consistent contact with her father.

The case patient had friends in school but recently moved, lost established friends, and her closest friend had moved away with no contact; the family as a whole is pretty isolated and mother is under quite a bit of stress.

The case patient just transitioned into high school with academic concerns surrounding reading comprehension, math reasoning, retention of complex academic information (especially abstract reasoning), and applied skills including written expression.

The case patient missed quite a bit of school for medical reasons (appointments and procedures), and over time there were increased days staying home because she didn't feel good, probably could have gone to school at least part of the day, which played into anxiety and comfort of being home.

Loss of school days results in losing instruction time, piled-up assignments, and missing social interaction and positive social experiences that contribute to sense of satisfaction and competency.

At presentation, the case patient was complaining of fluttering heart and a lot of somatic body pain; she denied anxiety when asked directly, but her mother reported she reported feeling panicky and was panting (significant signs of anxiety).

The case patient is described as very meek, very self-conscious, and often very keyed up or on edge; at school she would not ask for help and wanted to be under the radar if at all possible.

The case patient worried about missing assignments, which could lead to more procrastination and avoidance of school; she did not view herself as being as capable as peers, and any talk about differences led her to report feeling stupid or not smart.

During evaluation, the case patient presented as extremely polite, arrived a bit late, very cooperative, very soft-spoken, difficult to get her to engage, does not initiate; on verbal components there was a lot of query and exploring needed to get information; if not sure of an answer she was hesitant to take a guess; on nonverbal tasks her approach was disorganized; she seemed anxious and when things became more challenging she tended to shut down and become tearful, appeared embarrassed.

On the WISC-5 (standardized cognitive measure), the case patient's full-scale IQ is 82, which falls in the low average range when compared to other children her age.

The case patient's verbal index was in the average range, nonverbal in the low average range, and her weakest areas of performance were working memory and processing speed.

Academically, the case patient's stronger areas are basic phonetic decoding, reading accuracy, sentence completion, spelling, and numerical sequencing (basic rote skills).

The case patient has difficulty with higher-level reading comprehension, reading fluency (reads slowly, has to reread for accuracy of understanding), math calculation and reasoning, and listening recall.

In the classroom, the case patient often feels overwhelmed and very anxious, feels like she's behind the eight ball at all times, has difficult time processing what's being instructed (feels it's going too fast), doesn't ask questions, often does not bring home materials needed or actual assignment requirements because she has hard time copying from the board and keeping up with her planner.

Low-level organizational and processing issues contribute to further withdrawal and feeling of inadequacy in the case patient.

The case patient reports a high level of test anxiety, with her mind going blank and difficulty recalling any information.

Diagnostic rating scales (BRIEF - Behavior Rating Inventory of Executive Function) distributed to school personnel, mother, and patient showed significant executive dysfunction in areas of organization, planning, initiating, task persistence, and attention dysregulation.

The case patient met criteria for ADHD, predominantly inattentive presentation (not the hyperactive or impulsive components).

Additional ratings indicated significant levels of anxiety and somatic or health-related complaints, a lot of dysphoria, and sense of inadequacy, best represented with a diagnosis of generalized anxiety disorder.

The initial care plan included encouragement to pursue ongoing counseling (with the psychologist or options close to home); the family was very closed in and other than medically presenting were not really likely to come in, and despite voicing interest they did not come back for psychotherapy.

The school was willing to accommodate with a shortened school week (3 partial days per week) to allow time for medical planning and appointments while condensing core instruction time, with assistance in core instruction areas so the patient could demonstrate mastery without excessive assignments compiling.

All listed psychosocial factors (limited social support, low self-confidence and performance anxiety, isolation) are contributing factors to patient outcomes, with the relative importance dependent on where the family is at; isolation is a significant part because it plays into low sense of competency and further social withdrawal, not integrating back into the community.

Clinicians should assume that Fontan patients are going to have adjustment issues, which normalizes the challenges and gives credence to them, starting the conversation very early that emotional and mental support is a basic part of the care plan (equal opportunity, not selective for those not doing well).

Having conversations about mental health very early is important because there is still a lot of misconception about what that represents; conversations should start early with parents as well to normalize that they are juggling a lot and this disrupts work and the entire family.

A solution-focused approach asking 'what is working' taps into patient and family strengths.