Medical and end-of-life decision making in adolescents' pre-heart transplant: A descriptive pilot study.

BACKGROUND: Adolescents and young adults undergoing heart transplantation experience risks of morbidity and mortality both pre- and post-transplant. To improve end-of-life care for this population, it is necessary to understand their medical and end-of-life decision-making preferences. AIM: (1) To examine adolescent/young adult decision-making involvement specific to heart transplant listing, and (2) to characterize their preferences specific to medical and end-of-life decision making. DESIGN: This cross-sectional research study utilized survey methods. Data were collected from October 2016 to March 2018. SETTING/PARTICIPANTS: Twelve adolescent and young adult patients listed for heart transplant (ages = 12-19 years) and one parent for each were enrolled at a single-center, US children's hospital. RESULTS: Consistent with their preferences, the majority of adolescent/young adult participants (82%) perceived a high level of involvement in the decision to be listed for transplant. Patient involvement in this decision was primarily by way of seeking advice or information from their parents and being asked to express their opinion from parents. Despite a preference among patients to discuss their prognosis and be involved in end-of-life decision making if seriously ill, only 42% of patients had discussed their end-of-life wishes with anyone. Few parents recounted having such discussions. Preferences regarding the timing and nature of end-of-life decision-making discussions varied. CONCLUSIONS: Although young people are involved in the decision to pursue heart transplantation, little attention is paid to involving them in discussions regarding end-of-life decision making in a manner that is consistent with individual preferences.

Generalized and specific anxiety in adolescents following heart transplant.

Mental health concerns are associated with worse outcomes after adult heart transplant. Illness-specific anxiety is associated with worsened psychological well-being after other solid organ transplants but has never been characterized after pediatric heart transplant. This single-center cross-sectional study aimed to evaluate illness-specific and generalized anxiety after heart transplantation in adolescents. A novel 12-item PHTF, GAD-7, and the PedsQL were administered. Univariate associations of demographics, clinical features, and medication adherence as measured by immunosuppression standard deviation with the PHTF and GAD-7 scores were evaluated. Internal consistency and validity of the PHTF were examined. In total, 30 patients participated. The most common illness-specific fears were retransplantation, rejection, and more generally post-transplant complications. The PHTF had good internal consistency (Cronbach α = .88). Construct validity was demonstrated between PHTF and GAD-7 (r = .62) and PedsQL (r = -.54 to -.62). 23% endorsed moderate to severe generalized anxiety symptoms. More severe symptoms were associated with older age at survey (P = .03), older age at listing (P = .01) and having post-transplant complications (P = .004). Patients with moderate or severe symptoms were more likely to report late immunosuppression doses (P = .004). Illness-specific and generalized anxiety may be prevalent after pediatric heart transplant. Screening for anxiety in adolescents post-transplant may identify those at risk for adverse outcomes including non-adherence. The PHTF is a brief, valid, and reliable instrument identifying illness-specific anxiety in this population.

Acute Hemodynamic Effects of Negative Extrathoracic Pressure in Fontan Physiology.

We sought to assess acute hemodynamic changes after implementation of negative extrathoracic pressure (NEP) in spontaneously breathing ambulatory Fontan patients with symptomatic heart failure. We hypothesized that application of NEP would result in an acute decrease in pulmonary artery pressure. Ten patients with clinical evidence of Fontan failure underwent baseline hemodynamic catheterization while breathing spontaneously. Hemodynamic measurements were then repeated after 30 min of continuous NEP. After 30 min of continuous NEP, 4/10 patients had a decrease in their Fontan pressure by 2 mmHg and one patient had a decrease by 1 mmHg. There were three patients that had an increase in Fontan pressure by 2 mmHg. In 7/10 patients, indexed pulmonary vascular resistance decreased by an average of 31%. In symptomatic Fontan patients with a favorable hemodynamic response to NEP during catheterization, potential benefit of longer-term NEP to improve clinical status should be explored.