Pulmonary arterial hypertension in adults with systemic right ventricles referred for cardiac transplantation.

BACKGROUND: Systemic right ventricular (RV) failure may progress necessitating referral for orthotropic heart transplantation (OHT). Pulmonary hypertension (PH) frequently coexists in adult congenital heart disease and can complicate the assessment for OHT. METHODS: Single-center case series of six patients (median age 34.9 years [IQR, 31.9-42.4]) with systemic RV physiology with PH referred for OHT evaluation from 2008 to 2017. RESULTS: One-third (n = 6) of 18 patients with systemic RV physiology referred for OHT evaluation had pulmonary arterial hypertension (PAH) defined as mean pulmonary artery pressure (mPAP) > 25 mm Hg and pulmonary vascular resistance (PVR) > 3 Wood Units. Two of the six patients were considered OHT-ineligible due to PH and comorbidities. Of the remaining four, two had pre-capillary PH and underwent heart-lung transplant (HLTx). The other two demonstrated reversibility of PVR with vasodilator testing and underwent OHT alone, one of whom died post-transplant from PH crisis. CONCLUSIONS: Pulmonary arterial hypertension is common in systemic RV patients referred for OHT. Systemic RV dysfunction places these patients at risk for post-capillary PH but pre-capillary PH can exist. Despite management with selective pulmonary vasodilators and afterload reduction, criteria for listing patients for HLTx vs OHT are not known and need further elucidation.

Psychological distress in adults with congenital heart disease: focus beyond depression.

BACKGROUND: Adults with congenital heart disease face psychological challenges although an understanding of depression vs. anxiety symptoms is unclear. We analyzed the prevalence of elevated symptoms of anxiety and depression and explored associations with demographic and medical factors as well as quality of life. METHODS: Adults with congenital heart disease enrolled from an outpatient clinic completed the Hospital Anxiety and Depression Scale and two measures of quality of life: the Linear Analogue Scale and the Satisfaction with Life Scale. Medical data were obtained by chart review. RESULTS: Of 130 patients (median age = 32 years; 55% female), 55 (42%) had elevated anxiety symptoms and 16 (12%) had elevated depression symptoms on subscales of the Hospital Anxiety and Depression Scale. Most patients with elevated depression symptoms also had elevated anxiety symptoms (15/16; 94%). Of 56 patients with at least one elevated subscale, 37 (66%) were not receiving mental health treatment. Compared to patients with 0 or 1 elevated subscales, patients with elevations in both (n=15) were less likely to be studying or working (47% vs. 81%; p=0.016) and reported lower scores on the Linear Analogue Scale (60 vs. 81, p<0.001) and the Satisfaction with Life Scale (14 vs. 28, p<0.001). CONCLUSIONS: Among adults with congenital heart disease, elevated anxiety symptoms are common and typically accompany elevated depressive symptoms. The combination is associated with unemployment and lower quality of life. Improved strategies to provide psychosocial care and support appropriate engagement in employment are required.

Longer-term issues for young adults with hypoplastic left heart syndrome: contraception, pregnancy, transition, transfer, counselling, and re-operation.

Hypoplastic left heart syndrome remained a largely untreated lesion until the 1980s. In the current era, 75-80% of patients who are managed at "centres of excellence" can be expected to survive into young adulthood after staged palliation. This improved survival has led to an emerging population of patients now entering adulthood with a new set of concerns. We discuss the realised and potential issues that will be faced by these patients, including family planning, transition, and re-operation.

Accuracy of risk prediction scores in pregnant women with congenital heart disease.

OBJECTIVE: To assess performance of risk stratification schemes in predicting adverse cardiac outcomes in pregnant women with congenital heart disease (CHD) and to compare these schemes to clinical factors alone. DESIGN: Single-center retrospective study. SETTING: Tertiary care academic hospital. PATIENTS: Women ≥18 years with International Classification of Diseases, Ninth Revision, Clinical Modification codes indicating CHD who delivered between 1998 and 2014. CARPREG I and ZAHARA risk scores and modified World Health Organization (WHO) criteria were applied to each woman. OUTCOME MEASURES: The primary outcome was defined by ≥1 of the following: arrhythmia, heart failure/pulmonary edema, transient ischemic attack, stroke, dissection, myocardial infarction, cardiac arrest, death during gestation and up to 6 months postpartum. RESULTS: Of 178 women, the most common CHD lesions were congenital aortic stenosis (15.2%), ventricular septal defect (13.5%), atrial septal defect (12.9%), and tetralogy of Fallot (12.9%). Thirty-five women (19.7%) sustained 39 cardiac events. Observed vs expected event rates were 9.9% vs 5% (P = .02) for CARPREG I score 0 and 26.1% vs 7.5% (P < .001) for ZAHARA scores 0.51-1.5. ZAHARA outperformed CARPREG I at predicting adverse cardiovascular outcomes (AUC 0.80 vs 0.72, P = .03) but was not significantly better than modified WHO. Clinical predictors of adverse cardiac event were symptoms (P = .002), systemic ventricular dysfunction (P < .001), and subpulmonary ventricular dysfunction (P = .03) with an AUC 0.83 comparable to ZAHARA (P = .66). CONCLUSIONS: CARPREG I and ZAHARA scores underestimate cardiac risk for lower risk pregnancies in these women. Of the three risk schemes, CARPREG I performed least well in predictive capacity. Clinical factors specific to the population studied are comparable to stratification schemes.

Too little too late? Communication with patients with congenital heart disease about challenges of adult life.

OBJECTIVE: To investigate the experiences and communication preferences of adult patients with congenital heart disease (CHD) in the domains of employment, insurance, and family planning. DESIGN: Patients ≥ 18 years of age completed a questionnaire about experiences and communication preferences regarding employment, health insurance, and family planning. RESULTS: Of 152 patients (median age = 33 years, 50% female, 35% with CHD of great complexity), one in four reported work-related problems due to CHD and a quarter also recalled a previous gap in health insurance. Of females, 29% experienced an unplanned pregnancy. The median importance of discussion ratings (on a 0-10 scale) were 3.5 (employment), 6.0 (insurance), and 8.0 (family planning). Few patients recalled discussions about employment (19%) or health insurance (20%). Over half recalled discussions about family planning, although males were less likely to have had these discussions than females (24% vs 86%, P < .001). Across the three domains, patients identified 16-18 years as the most appropriate age to initiate discussion, although for patients who recalled discussions, they typically occurred between 20 and 25 years. CONCLUSIONS: Adults with CHD commonly face employment, health insurance, and family planning challenges. However, discussions about these matters occur with less frequency than recommended and at older ages than patients would prefer. Communication about such issues should be incorporated into a comprehensive educational curriculum for adolescents during the process of transition to adult care.

Pulmonary hypertension: Barrier or just a bump in the road in transplanting adults with congenital heart disease.

BACKGROUND: Heart failure (HF) is the leading cause of death in adults with congenital heart disease (ACHD). Identification of disease progression and timing of referral for advanced therapies is often delayed. However, increased awareness and understanding of ACHD and improvements in the approach to treatment have led to improved outcomes. Pulmonary hypertension (PH) is a common barrier to HT. In ACHD, the approach to PH and HT is quite complicated, given the anatomic heterogeneity and lower prevalence and experience. However, in some cases, PH is a result of elevated systemic filling pressures and low output. METHODS: We describe the approach used to successfully transplant an ACHD patient with severe pre-HT PH performing HT alone. We review the literature and describe the one patient's journey from primarily palliative, to a combined heart-lung transplant candidate, to successful HT patient. RESULTS: We discuss the methodology used to successfully transplant a patient, with significantly elevated pulmonary pressures and an initial pulmonary vascular resistance (PVR) > 13 Wood units. CONCLUSIONS: There are a number of complexities associated with the ACHD population and it is of utmost importance to carefully identify the underlying hemodynamic milieu and inform the appropriate treatment course in order to have successful transplant outcomes.

Advance Care Planning in Adults with Congenital Heart Disease: A Patient Priority.

BACKGROUND: Adult congenital heart disease (ACHD) patients with moderate or great defect complexity are at risk for premature death. Although early engagement in advance care planning (ACP) is recommended, previous research suggests that it seldom occurs. METHODS: This study investigated ACHD patient preferences for ACP and factors that impact preferences. ACHD patients completed an ACP preferences questionnaire, the Hospital Anxiety and Depression Scale and a measure of attachment styles. RESULTS: Of 152 ACHD patients (median age 33years, 50% female), 13% reported previous ACP discussions with providers and 21% had completed advance directives. On a 0-10 scale, the median rating for the importance of discussing ACP with providers was 7; 18years was identified as the most appropriate age to initiate this dialogue. Higher ratings for the importance of discussing ACP with providers was observed in patients who were female (p=0.03), had lower disease complexity (p=0.03), and had elevated anxiety symptoms (p=0.001); elevated anxiety remained significant in a multivariable model. Interest in receiving information about life expectancy (61% overall) was greater among patients with lower disease complexity (p=0.04) and a history of ≥2 cardiac surgeries (p=0.01); disease complexity remained significant in a multivariable model. CONCLUSIONS: As a group, ACHD patients value the opportunity for ACP discussions and prefer earlier communication. Although some clinicians might avoid ACP discussions in patients who are generally more anxious or have less complex CHD, such avoidance does not appear to be warranted.

Prevalence and Correlates of Post-traumatic Stress Disorder in Adults With Congenital Heart Disease.

Post-traumatic stress disorder (PTSD) is associated with adverse outcomes and increased mortality in cardiac patients. No studies have examined PTSD in the adult congenital heart disease (ACHD) population. The objectives of this study were to assess the prevalence of self-reported symptoms of PTSD in patients with ACHD and explore potential associated factors. Patients were enrolled from an outpatient ACHD clinic and completed several validated measures including the Impact of Event Scale-Revised, PTSD Checklist-Civilian Version, and the Hospital Anxiety and Depression Scale. Clinical data were abstracted through medical data review. A total of 134 participants (mean age 34.6 ± 10.6; 46% men) were enrolled. Of the 127 participants who completed the Impact of Event Scale-Revised, 14 (11%) met criteria for elevated PTSD symptoms specifically related to their congenital heart disease or treatment. Of the 134 patients who completed PTSD Checklist-Civilian Version, 27 (21%) met criteria for global PTSD symptoms. In univariate analyses, patients with congenital heart disease-specific PTSD had their most recent cardiac surgery at an earlier year (p = 0.008), were less likely to have attended college (p = 0.04), had higher rates of stroke or transient ischemic attack (p = 0.03), and reported greater depressive symptoms on the Hospital Anxiety and Depression Scale (7 vs 2, p <0.001). In multivariable analysis, the 2 factors most strongly associated with PTSD were depressive symptoms (p <0.001) and year of most recent cardiac surgery (p <0.03). In conclusion, PTSD is present in 11% to 21% of subjects seen at a tertiary referral center for ACHD. The high prevalence of PTSD in this complex group of patients has important implications for the medical and psychosocial management of this growing population.

Establishment of Pediatric Cardiac Intensive Care Advanced Practice Provider Services.

The addition of advanced practice providers (APPs; nurse practitioners and physician assistants) to a pediatric cardiac intensive care unit (PCICU) team is a health care innovation that addresses medical provider shortages while allowing PCICUs to deliver high-quality, cost-effective patient care. APPs, through their consistent clinical presence, effective communication, and facilitation of interdisciplinary collaboration, provide a sustainable solution for the highly specialized needs of PCICU patients. In addition, APPs provide leadership, patient and staff education, facilitate implementation of evidence-based practice and quality improvement initiatives, and the performance of clinical research in the PCICU. This article reviews mechanisms for developing, implementing, and sustaining advance practice services in PCICUs.