Rationale and design of a randomized controlled clinical trial of a resilience-building intervention in adults with congenital heart disease.

BACKGROUND: Adults with congenital heart disease (ACHD) are at risk for lower quality of life (QOL) and psychological health. Behavioral interventions to meet their psychosocial needs are lacking. The aim of this study is to evaluate the feasibility of implementing the Promoting Resilience in Stress Management (PRISM) intervention in ACHD and its efficacy in increasing resilience in this population. METHODS: We designed a phase II randomized controlled clinical trial of patients with moderate or complex ACHD, physiological stages C or D. Enrolled participants will be randomized to receive PRISM or usual care. PRISM is a manualized, skills-based behavioral intervention comprised of four one-on-one sessions targeting resilience resources (stress-management, goal-setting, cognitive reframing, meaning making), an optional session on advance care planning, and a facilitated family meeting. Participants in both groups will complete study questionnaires at enrollment and 3-months later. The primary aim is to describe feasibility, namely the proportions of patients who a) enroll in the study among those eligible, and b) complete the PRISM intervention among those randomized to that arm. We will also evaluate PRISM's efficacy by using linear regression models to compare changes in mean resilience scores between assigned groups. In exploratory analyses, we will evaluate effects on QOL, psychological distress, perceived competence for health care management, and comfort with advance care planning. DISCUSSION: This study will provide rigorous evidence to determine the feasibility and efficacy of a brief intervention to promote resilience and psychosocial health in ACHD. Findings may guide the development of a future multi-site effectiveness study. CLINICAL TRIAL REGISTRATION: NCT04738474.

Key Psychosocial Health Outcomes and Association With Resilience Among Patients With Adult Congenital Heart Disease.

BACKGROUND: Adult congenital heart disease (ACHD) can negatively impact quality of life (QOL). Strengthening resilience may improve this and other psychosocial outcomes important for living a meaningful life. OBJECTIVES: The purpose of this study was to describe resilience and key psychosocial health outcomes in ACHD and evaluate the associations between resilience and these outcomes. METHODS: We conducted a prospective cohort study of outpatients with moderate or complex ACHD between May 2021 and June 2022. Participants completed surveys at baseline and 3 months, evaluating resilience (Connor-Davidson Resilience Scale-10), health-related QOL (EQ5D-3L, linear analog scale), health status (Euroqol visual analog scale), self-competence (Perceived Competence Scale), and psychological symptom burden (Hospital Anxiety and Depression Scale) and distress (Kessler-6). RESULTS: The mean participant age (N = 138) was 41 ± 14 years, 51% were female, and 83% self-identified as non-Hispanic White. ACHD was moderate for 75%; 57% were physiologic class B. Mean baseline resilience score (Connor-Davidson Resilience Scale-10) was 29.20 ± 7.54. Participants had relatively good health-related QOL, health status, and self-competence, and low psychological symptom burden and distress. Higher baseline resilience was associated with better values of all outcomes at 3 months (eg, 1 point higher resilience was associated with 0.92 higher linear analog scale; 95% CI: 0.52-1.32) with or without adjustment for demographics. After further adjusting for the baseline psychosocial measure, only the association between resilience and QOL measures at 3 months remained statistically significant. CONCLUSIONS: Resilience is positively associated with health-related QOL for outpatients with moderate or complex ACHD, though relationships are small in magnitude. Study findings can guide the application of resilience-building interventions to the ACHD population.

Design, Creation, and 13-Month Performance of a Novel, Web-Based Activity for Education in Primary Cardiology Palliative Care.

Cardiovascular disease (CVD) clinicians who care for seriously ill patients frequently report that they do not feel confident nor adequately prepared to manage patients' palliative care (PC) needs. With the goal, therefore, of increasing PC knowledge and skills amongst interprofessional clinicians providing CVD care, the ACC's PC Workgroup designed, developed, and implemented a comprehensive PC online educational activity. This paper describes the process and 13-month performance of this free, online activity for clinicians across disciplines and levels of training, "Palliative Care for the Cardiovascular Clinician" (PCCVC). A key component of PCCVC is that it is tailored to the lifelong learner; users can choose and receive credit for the activities that meet their individual learning needs. This webinar series was well-subscribed, and upon completion of the modules, learners reported better self-perceived abilities related to palliative care competencies. We propose PCCVC as a model for primary PC education for clinicians caring for individuals with other serious or life-shortening illnesses.

Palliative Care Education in Cardiovascular Disease Fellowships: A National Survey of Program Directors.

BACKGROUND: Palliative care (PC) is an essential component of high-quality care for people with cardiovascular disease (CVD). However, little is known about the current state of PC education in CVD training, including attitudes toward integration of PC into training and implementation of PC by the program's leadership. METHODS: We developed a nationwide, cross-sectional survey that queried education approaches, perspectives and barriers to PC education in general CVD fellowship training. The survey was distributed to 392 members of the American College of Cardiology Program Director (PD) listserv, representing 290 general CVD fellowships between 1/2023 and 4/2023. We performed descriptive and ꭕ2 analyses of survey data. RESULTS: Of the program's representatives, 56 completed the survey (response rate = 19.3%). Respondents identified themselves as current PDs (89%), associate PDs (8.9%) or former PDs (1.8%), representing a diverse range of program sizes and types and regions of the country. Respondents reported the use of informal bedside teaching (88%), formal didactics (59%), online or self-paced modules (13%), in-person simulation (11%), and clinical rotations (16%) to teach PC content. Most programs covered PC topics at least annually, although there was variability by topic. We found no associations between program demographics and type or frequency of PC education. Most respondents reported dissatisfaction with the quantity (62%) or quality (59%) of the PC education provided. Barriers to PC education included an overabundance of other content to cover (36%) and perceived lack of fellow (20%) or faculty (18%) interest. Comments demonstrated the importance of PC education in fellowship, the lack of a requirement to provide PC education, difficulty in covering all topics, and suggestions of how PC skills should be taught. CONCLUSIONS: In a national survey of CVD educational leadership concerning approaches to PC education in CVD training, respondents highlighted both challenges to implementation of formal PC curricula in cardiology training and opportunities for comprehensive PC education.

Peak oxygen consumption by smartwatches compared with cardiopulmonary exercise test in complex congenital heart disease.

OBJECTIVE: To evaluate for correlation between exercise capacity as assessed by peak oxygen consumption (pVO2) measurement during a cardiopulmonary exercise test (CPET) and smartwatches reporting this parameter in patients with adult congenital heart disease (ACHD) complex lesions. METHODS: A prospective study that included patients with ACHD either a Fontan circulation or a right ventricle supporting the systemic circulation who underwent two separate CPETs at least 1 year apart. Generalised estimating equations linear regression was performed to identify factors associated with correlation between smartwatch and CPET-derived pVO2. RESULTS: 48 patients (71% with a Fontan circulation, 42% females, mean age 33±9 years) underwent two CPETs between May 2018 and May 2022 with echocardiograms performed within 6 months of each CPET. Apple Watch was the predominant smartwatch used (79%). Smartwatch and CPET measured peak heart rate (Pearson correlation=0.932, 95% CI (0.899, 0.954)) and pVO2 (0.8627, 95% CI (0.8007, 0.9064) and 0.8634, 95% CI (0.7676, 0.9215) in the first and second CPET, respectively) correlated well, with smartwatch-measured pVO2 values measuring higher by a mean of 3.146 mL/kg/min (95% CI (2.559, 3.732)). Changes in pVO2 between the first and the second CPET also correlated well (Pearson correlation=0.9165, 95% CI (0.8549, 0.9525)), indicating that for every 1 mL/(min kg) change in CPET-measured pVO2, there was a corresponding 0.896 mL/(min kg) change in the smartwatch-measured pVO2. CONCLUSION: Both absolute values and changes over time in pVO2 as measured by smartwatches and CPETs correlate well in patients with complex ACHD.

Definitions of resilience and resilience resource use as described by adults with congenital heart disease.

Background: Adult congenital heart disease (ACHD) is a lifelong illness that presents ongoing challenges to quality of life. Fostering personal resilience resources to sustain well-being can enhance patients' psychosocial health. Objective: We aimed to describe patients' resilience experiences: how they understand, develop, and utilize resilience resources in managing ACHD. Methods: We conducted a qualitative study of patients with ACHD. Participants were recruited using maximum variation sampling. Individual, semi-structured interviews were conducted June 2020 to August 2021. We queried approaches to managing ACHD-related stress and experiences with resilience and analyzed responses with thematic analysis. Results: Participant (N = 25) median age was 32 years (range 22-44); 52% identified as female and 72% non-Hispanic white. Participants' anatomic ACHD was moderate (56%) or complex (44%); physiologically, 76% were functional class C or D. Participants described various resilience resources, which map to an established resilience framework: 1) internal resources: maintaining positivity, self-directed activity, and setting goals; 2) external resources: social support; 3) existential resources: purpose, gratitude, and cultivating health. Even among participants who reported feeling unfamiliar (8/25) with the term "resilience," all participants shared experiences reflecting resilience developed while living with ACHD. Conclusion: ACHD-relevant resilience resources may help patients and clinicians navigate ACHD-related stress and promote psychosocial well-being.

Main operating room deliveries for patients with high-risk cardiovascular disease.

BACKGROUND: High-risk cardiovascular disease (CVD) prevalence in pregnant patients is increasing. Management of this complex population is not well studied, and little guidance is available regarding labour and delivery planning for optimal outcomes. OBJECTIVE: We aimed to describe the process for and outcomes of our centre's experience with the main operating room (OR) caesarean deliveries for patients with high-risk CVD, including procedural and postpartum considerations. STUDY DESIGN: We performed a retrospective evaluation of pregnant patients with high-risk CVD who delivered in the main OR at a large academic centre between January 2010 and March 2021. Patients were classified by CVD type: adult congenital heart disease, cardiac arrest, connective tissue disease with aortopathy, ischaemic cardiomyopathy, non-ischaemic cardiomyopathy or valve disease. We examined demographic, anaesthetic and procedure-related variables and in-hospital maternal and fetal outcomes. Multidisciplinary delivery planning was evaluated before and after formalising a cardio-obstetrics programme. RESULTS: Of 25 deliveries, connective tissue disease (n=9, 36%) was the most common CVD type, followed by non-ischaemic cardiomyopathy (n=5, 20%). Scheduled deliveries that went as initially planned occurred for six patients (24%). Fourteen (56%) were unscheduled and urgent or emergent. Patients in modified WHO Class IV frequently underwent unscheduled, urgent deliveries (64%). Most deliveries were safely achieved with neuraxial regional anaesthesia (80%) and haemodynamic monitoring via arterial lines (88%). Postdelivery intensive care unit stays were common (n=18, 72%), but none required mechanical circulatory support. There were no in-hospital maternal or perinatal deaths; 60-day readmission rate was 16%. Some delivery planning was achieved for most patients (n=21, 84%); more planning was evident after establishing a cardio-obstetrics programme. Outcomes did not differ significantly by CVD group or delivery era. CONCLUSIONS: Our experience suggests that short-term outcomes of pregnant patients with high-risk CVD undergoing main OR delivery are favourable. Multidisciplinary planning may support the success of these complex cases.

Palliative Care Across the Life Span for Children With Heart Disease: A Scientific Statement From the American Heart Association.

AIM: This summary from the American Heart Association provides guidance for the provision of primary and subspecialty palliative care in pediatric congenital and acquired heart disease. METHODS: A comprehensive literature search was conducted from January 2010 to December 2021. Seminal articles published before January 2010 were also included in the review. Human subject studies and systematic reviews published in English in PubMed, ClinicalTrials.gov, and the Cochrane Collaboration were included. Structure: Although survival for pediatric congenital and acquired heart disease has tremendously improved in recent decades, morbidity and mortality risks remain for a subset of young people with heart disease, necessitating a role for palliative care. This scientific statement provides an evidence-based approach to the provision of primary and specialty palliative care for children with heart disease. Primary and specialty palliative care specific to pediatric heart disease is defined, and triggers for palliative care are outlined. Palliative care training in pediatric cardiology; diversity, equity, and inclusion considerations; and future research directions are discussed.

Sudden cardiac death as a consequence of Cor triatriatum sinistrum in an adult.

Cor triatriatum sinistrum (CTS) is a rare congenital cardiac malformation in which the left atrium is divided by a fenestrated membrane, which can restrict blood flow and cause symptoms of congestive heart failure. Rarely, the condition can present in adulthood. This case report illustrates a case of sudden cardiac death (SCD) due to the sequelae of untreated CTS. To date, there are no reported cases of SCD attributable to CTS. Learning objectives: Cor triatriatum sinistrum is among the rarest of congenital heart diseases. In this case report, we describe the prevalence, etiology, diagnosis, and management of this disease.

Adults with CHD balancing motivations and concerns in pregnancy decision-making.

BACKGROUND: While the incidence of pregnancy has increased among individuals with adult CHD, little has been described about considerations and experiences of patients with adult CHD regarding pregnancy. OBJECTIVE: We aimed to explore patients' motivations, concerns, and decision-making processes regarding pregnancy. METHODS: In April 2019-January 2020, we conducted in-depth telephone interviews with patients (n = 25) with simple, moderate, or complex adult CHD, who received prenatal care at the University of Washington during 2010-2019 and experienced a live birth. Transcripts were analysed using thematic analysis. RESULTS: Participants described motivations for pregnancy as both internal desires (motherhood, marriage fulfillment, biological connection, fetal personhood, self-efficacy) and external drivers (family or community), as well as concerns for the health and survival of themselves and the fetus. Factors that enabled their decision to maintain a pregnancy included having a desire that outweighed their perceived risk, using available data to guide their decision, planning for contingencies and knowing their beliefs about termination, plus having a trusted healthcare team, social support, and resources. Factors that led to insurmountable risk in subsequent pregnancies included desire having been fulfilled by the first pregnancy, compounding risk with age and additional pregnancies, new responsibility to an existing child, and reduced healthcare team and social support. CONCLUSIONS: Understanding individuals' motivations and concerns, and how they weigh their decisions to become or remain pregnant, can help clinicians better support patients with adult CHD considering pregnancy. Clinician education on patient experiences is warranted.

A World of Maximalist Medicine: Physician Perspectives on Palliative Care and End-of-life for Patients With Pulmonary Arterial Hypertension.

CONTEXT: Physicians who specialize in pulmonary arterial hypertension (PAH) care for patients facing a serious, life-limiting illness. Palliative care is underutilized in patients with PAH, and little is known about how best to provide palliative care to this patient population. OBJECTIVES: Using a qualitative approach, assess physicians' perspectives on barriers and facilitators to the use of palliative care in PAH. METHODS: Participants were board-certified pulmonologists and cardiologists recruited from the Pulmonary Hypertension Association's list of physician specialists and academic center websites. We performed one-on-one semi-structured interviews that were recorded, transcribed, and analyzed using thematic analysis. RESULTS: Twelve physicians participated in the study, with a median age of 48.5 years and 20.5 years of clinical experience caring for patients with PAH. We identified the following themes and associated barriers and facilitators to effective implementation of palliative care for patients with PAH: a tailored approach to the individual patient; a PAH-specialist-led culture of care; effective collaboration with palliative care clinicians; and limitations imposed by health systems. CONCLUSION: PAH physicians are open to palliative care for their patients and are willing to partner with palliative care clinicians to implement this effectively and in the right setting. Areas for targeted improvement in enhancing palliative care for patients with PAH exist, especially enhancing collaboration between PAH physicians and palliative care specialists and navigating barriers in health systems.

Peri-procedural code status for transcatheter aortic valve replacement: Absence of program policies and standard practices.

BACKGROUND: Little is known about policies and practices for patients undergoing Transcatheter Aortic Valve Replacement (TAVR) who have a documented preference for Do Not Resuscitate (DNR) status at time of referral. We investigated how practices across TAVR programs align with goals of care for patients presenting with DNR status. METHODS: Between June and September 2019, we conducted semi-structured interviews with TAVR coordinators from 52/73 invited programs (71%) in Washington and California (TAVR volume > 100/year:34%; 50-99:36%; 1-50:30%); 2 programs reported no TAVR in 2018. TAVR coordinators described peri-procedural code status policies and practices and how they accommodate patients' goals of care. We used data from the Society of Thoracic Surgeons/American College of Cardiology Transcatheter Valve Therapy Registry, stratified by programs' DNR practice, to examine differences in program size, patient characteristics and risk status, and outcomes. RESULTS: Nearly all TAVR programs (48/50: 96%) addressed peri-procedural code status, yet only 26% had established policies. Temporarily rescinding DNR status until after TAVR was the norm (78%), yet time frames for reinstatement varied (38% <48 h post-TAVR; 44% 48 h-to-discharge; 18% >30 days post-discharge). For patients with fluctuating code status, no routine practices for discharge documentation were well-described. No clinically substantial differences by code status practice were noted in Society of Thoracic Surgeons Predicted Risk of Mortality risk score, peri-procedural or in-hospital cardiac arrest, or hospice disposition. Six programs maintaining DNR status recognized TAVR as a palliative procedure. Among programs categorically reversing patients' DNR status, the rationale for differing lengths of time to reinstatement reflect divergent views on accountability and reporting requirements. CONCLUSIONS: Marked heterogeneity exists in management of peri-procedural code status across TAVR programs, including timeframe for reestablishing DNR status post-procedure. These findings call for standardization of DNR decisions at specific care points (before/during/after TAVR) to ensure consistent alignment with patients' health-related goals and values.

COVID-19 impact on adults with congenital heart disease self-perceived vulnerability and coping.

The COVID-19 pandemic poses risk for worsened quality of life in patients with adult congenital heart disease. In a qualitative thematic analysis of semi-structured interviews conducted July 2020 to August 2021, we examined the pandemic's impact on participants' (N = 25) experiences with self-perception and coping. All had moderate or complex disease; median age 32 years. The pandemic altered some participants' self-perception, including increased vulnerability beyond heart-attributed risk. Restrictions frequently prevented participants from using their usual coping strategies, forcing use of alternative methods. For an already at-risk population, these findings suggest the need for increased mental health awareness, assessment, and support.

Cardiac and Pregnancy Outcomes of Pregnant Patients With Congenital Heart Disease According to Risk Classification System.

Pregnancy risk assessment for patients with adult congenital heart disease (ACHD) must include physiologic and anatomic impacts. We aimed to determine whether maternal cardiac and pregnancy outcomes vary by disease severity defined according to the following 3 different classifications: ACHD anatomic severity, ACHD physiologic class, and modified World Health Organization (mWHO) class. Cardiac outcomes included a composite of arrhythmia, heart failure, stroke, and thromboembolism. Pregnancy outcomes included a composite of intrauterine growth restriction, preterm birth, preeclampsia, or postpartum hemorrhage. We employed generalized estimating equations to account for multiple pregnancies. Of the 245 pregnancies, 17.1% were preterm and 45.7% were cesarean deliveries. Cardiac hospitalizations occurred in 22.0% and arrhythmias in 12.7%. Cardiac outcomes tended to be more prevalent in people with more severe heart disease. Pregnancy outcomes were U-shaped or less prevalent in people with more severe disease. There was a 2.9-fold increased risk for the composite cardiac outcome for complex anatomy (adjusted incidence rate ratio 2.90, 95% confidence interval 1.08 to 7.81, p = 0.04), a 9.4-fold increased risk for physiologic class C or D (9.37, 1.28 to 68.79, p = 0.03), and a fourfold increased risk for mWHO class III or IV (3.99, 1.53 to 10.40, p = 0.005). There was a lower risk for the composite pregnancy outcome for mWHO class II or II to III (0.54, 0.36 to 0.79, p = 0.002) but no association with anatomy or physiology. In conclusion, physiologic class may be most accurately associated with adverse outcomes and therefore efforts to optimize hemodynamics before pregnancy may help to mitigate the risk.

From Theory to Patient Care: A Model for the Development, Adaptation, and Testing of Psychosocial Interventions for Patients With Serious Illness.

Psychosocial and supportive care interventions are a cornerstone of palliative care science, yet there is little published guidance regarding how to develop, test, adapt, and ultimately disseminate evidence-based interventions. Our objective was to describe the application of a single intervention-development model in multiple populations of patients with serious illness. Specifically, we use the "Promoting Resilience in Stress Management" (PRISM) intervention as an exemplar for how the Obesity Related Behavioral Intervention Trials (ORBIT) intervention-development model may be applied to: 1) create an initial palliative care intervention; 2) adapt an existing intervention for a new patient-population; 3) expand an existing intervention to include new content; and, 4) consider dissemination and implementation of a research-proven intervention. We began by identifying key psychological and social science theories and translating them a testable clinical hypothesis. Next, we conducted observational studies and randomized trials to design, refine, and standardize PRISM within unique patient-populations. We moved backwards in the ORBIT model when necessary to adapt or expand PRISM content and delivery-strategies to meet patient-reported needs. Finally, we began to explore PRISM's effectiveness using Dissemination and Implementation research methods. Key lessons include the need to ground intervention-development in evidence-based theory; involve patient, clinician, and other stakeholders at every phase of development; "meet patients where they are at" with flexible delivery strategies; invest in the time to find the right scientific premise and the right intervention content; and, perhaps most importantly, involve an interdisciplinary research team.