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, NinthRevision, 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. K E Y W O R D Sadult congenital heart disease, outcomes, pregnancy, risk stratification | 471 KIM et al. | INTRODUC TI ONAs survival of those born with congenital heart disease (CHD) shifts toward adulthood, there are a growing number of females with CHD who reach childbearing age. From 1998 to 2007, the proportion of hospitalizations for delivery increased by 34.9% in women with CHD compared to 21.3% in the female general population. 1 Pregnancy outcomes for women with CHD can be highly variable and pose special challenges for congenital cardiovascular specialists and high-risk obstetricians who are tasked with counseling these women and managing them throughout pregnancy, delivery, and postpartum period. Risk stratification schemes predicting adverse outcomes in pregnant women with CHD have been published 2-6 with variable accuracy. 7-12 The objectives of this study were (1) to assess the performance of CARPREG I, ZAHARA, and the modified World Health Organization (WHO) classification in predicting adverse cardiovascular outcomes in women with CHD at a single tertiary care center with an established adult CHD program in an urban North American setting and (2) to compare the...

Objective To determine the prevalence and predictors of nonattendance in an ACHD outpatient clinic, and to examine the relationship between nonattendance and emergency department (ED) visits, hospitalizations, and death. Methods Patients ≥ 18 years who had scheduled appointments at an ACHD outpatient clinic between August 1, 2014 and December 31, 2014 were included. The primary outcome of interest was nonattendance of the first scheduled appointment of the study period, defined as “no‐show” or “same‐day cancellation.” Secondary outcomes of interest were ED visits, hospitalizations, and death until December 2017. Results Of 527 scheduled visits, 55 (10.4%) were nonattended. Demographic and socioeconomic characteristics such as race, income, and insurance type were associated with non‐attendance (all P values < .05), whereas age, gender, and disease complexity were not. On multivariable analysis, predictors of nonattendance were black race (adjusted odds ratio [AOR] 4.95; P < .001), other race (AOR 3.54; P = .003), and history of no‐show in the past (AOR 4.95; P < .001). Compared to patients who attended clinic, patients with a nonattended visit had a threefold increased odds of multiple ED visits and a significantly lower rate of ED‐free survival over time. There were no significant differences in hospitalizations or death by attendance. Conclusions ACHD clinic nonattendance is associated with race and prior history of no‐show, and may serve as a marker of higher ED utilization for patients with ACHD.

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.

OBJECTIVES Controversy exists in ascertaining the ideal location for adults with congenital heart disease requiring surgical intervention. In this study, we sought to compare the perioperative management between our paediatric and adult hospitals and to determine how clinical factors and the location affect the length of stay after pulmonary valve replacement. METHODS A retrospective analysis of patients, ≥18 years of age, undergoing pulmonary valve replacement was conducted at our paediatric and adult hospitals between 1 January 2000 and 30 October 2014. Patients with previous Ross or concomitant left heart procedures were excluded. Descriptive statistics were used to assess demographics and clinical characteristics. Inverse probability weight-adjusted models were used to determine differences in the number of surgical complications, duration of mechanical ventilation and postoperative length of stay between paediatric and adult hospitals. Additional models were calculated to identify factors associated with prolonged length of stay. RESULTS There were altogether 98 patients in the adult (48 patients) and paediatric (50 patients) hospitals. Patients in the adult hospital were older with more comorbidities (arrhythmia, hypertension, depression and a history of cardiac arrest, all P < 0.05). Those at the paediatric hospital had better preoperative right ventricular function and less tricuspid regurgitation. The cardiopulmonary bypass time, the length of intubation and the length of stay were higher at the adult hospital, despite no difference in the number of complications between locations. Factors contributing to the increased length of stay include patient characteristics and postoperative management strategies. There were no deaths. CONCLUSIONS Pulmonary valve replacement may be performed safely with no deaths and with a comparable complication rate at both hospitals. Patients undergoing surgery at the adult hospital have longer intubation times and length of stay. Opportunities exist to streamline management strategies.

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.

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