Pediatric Patients With Advanced Heart Disease Want to Be Heard

Many adolescent and young adults with advanced heart disease want to be actively involved in decisions about their care, a new survey shows. But often the parents of these patients prefer to face these difficult choices themselves without burdening their child.

Lead author Melissa K. Cousino, PhD (Michigan Medicine, Ann Arbor), a pediatric psychologist specializing in the needs of patients with congenital heart disease, noted that innovations in technologies and interventions have greatly improved outcomes in recent years. Amid this good news, less talked about are the patients who, despite these advancements, continue to be at high risk of morbidity and mortality.

“Clinically what I see is that these are not parents that are trying to withhold information from their children—it is a parental desire to protect their children from hard stuff,” said Cousino. “And I think that parents often [take] it upon themselves, when they have a child or a teen or even a young adult with these critical, chronic medical conditions, to shoulder as much burden as they can.”

In supporting these families, she said it’s important to help parents “understand that their children oftentimes can handle these conversations and do indeed want to be involved.”

A Disconnect in Preferences

Cousino and colleagues conducted their survey, which was published recently in JAMA Network Open, through the heart failure/transplant service at a Midwestern US children’s hospital between July 2018 and April 2021.

A total of 56 adolescent/young adult patients aged 12 to 14 years with advanced heart disease, and 53 of their parents, agreed to take part. Patients were a median of 17.8 years old, two-thirds were male, and three-quarters identified as white. Most of the parent respondents (84.3%) were female.

The most common diagnoses were posttransplant with complications (26.4%), single-ventricle congenital heart disease (22.6%), anthracycline cardiomyopathy (20.8%), and dilated cardiomyopathy (17.0%). Median age at the time of diagnosis was 7.7 years.

Of the adolescent/young adult surveyed, 43.5% preferred to have active, patient-led decision-making about heart disease management. Only 18.9% wanted their doctors to make the decision based on their knowledge of the disease and treatment, while 26.5% preferred shared decision making with their parents or physicians. None selected the response of: “My parent(s) should make the decision using all that he/she knows about the treatment.”

Yet 35.3% of parents preferred that they and their children’s physician, working together, be the ones making decisions on the patient’s behalf. Only 11.8% said decisions were entirely up to the adolescent/young adult.

Fully 86.8% of patients wanted to talk about adverse effects or risks of treatment, 84.9% wanted to discuss procedural and/or surgical details, and 79.2% wanted to speak about their prognosis. More than half (56.6%) said that if they were very ill they’d like to be involved in making end-of-life decisions; still, 83.0% said they wanted their parents to be involved and 49.1% wanted their doctors to be involved. Most (86.8%) thought it was the parents’ role to start the conversation about end-of-life plans, while 50.9% said cardiologists should initiate such discussions.

Among the parents, 72.5% said their child would want to take part in end-of-life decision-making.

The adolescent/young adult patients were more apt to desire an active role in decision-making if they had worse functional status and if they’d had a longer time since their cardiac diagnosis. Demographic factors like sex, current age, and racial/ethnic background had no impact on their preferences.

Conversations About Preferences Varies

How these hard topics are handled currently varies from center to center, and among clinicians, said Cousino. “There are some centers where assessment of communication preferences is a really regular part of practice or standard of care.” At others, these conversations are less common.

“There seemed to be a bit of a disconnect between the hopeful discourse and the realistic kind of discourse in terms of prognosis and outcomes,” she told TCTMD. “And while I am not one who feels strongly that we should be talking about those things if that's not in line with what patients and families desire, what I realized was there was no research or science on exactly that: what do our pediatric and young adult patients, as well as their families, desire when it comes to understanding risk, outcomes, and some of these more difficult topics that are just not always at the forefront in pediatric medicine?”

The aim of the survey was not to dictate a particular approach, but rather to develop “a standardized way of making sure we are asking patients what they want to be shared” in communication with their healthcare team, she said, “specifically as it relates to ‘high-stakes’ cardiac management.”

Palliative care has gotten growing recognition over the years but the multidisciplinary, dedicated teams that provide such care, much like mental health professionals, can sometimes be stretched thin.

“What I think we really want to move towards is more primary palliative care skills within specialties like cardiology, where you do see high disease burden and morbidity and mortality,” said Cousino. Cardiologists can learn ways to assess how patients and their families want to communicate—and begin conversations about decision-making, advanced-care planning, or symptom burden management.

For clinicians new to these topics, a good place to start is the American Heart Association scientific statement on palliative care in children that came out earlier this year, she suggested. Cardiologists also can refer patients to specialty palliative care when it’s helpful.

Cousino said their research in this area continues. One project involves interviews—rather than a survey with set answers that respondents can choose among—to learn more about the complex concerns surrounding the choices made in young people with advanced heart disease. Another is the creation of a physician-directed communication tool to help facilitate these conversations in clinical practice.