Long-Term outcomes in adult patients with congenital heart disease considered for transplantation: A single center study.

BACKGROUND: Adult congenital heart disease (ACHD) patients pose unique challenges in identifying the time for transplantation and factors influencing outcomes. OBJECTIVE: To identify hemodynamic, functional, and laboratory parameters that correlate with 1- and 10-year outcomes in ACHD patients considered for transplantation. METHODS: A retrospective chart review of long-term outcomes in adult patients with congenital heart disease (CHD) evaluated for heart or heart + additional organ transplant between 2004 and 2014 at our center was performed. A machine learning decision tree model was used to evaluate multiple clinical parameters correlating with 1- and 10-year survival. RESULTS: We identified 58 patients meeting criteria. D-transposition of the great arteries (D-TGA) with atrial switch operation (20.7%), tetralogy of Fallot/pulmonary atresia (15.5%), and tricuspid atresia (13.8%) were the most common diagnosis for transplant. Single ventricle patients were most likely to be listed for transplantation (39.8% of evaluated patients). Among a comprehensive list of clinical factors, invasive hemodynamic parameters (pulmonary capillary wedge pressure (PCWP), systemic vascular pressure (SVP), and end diastolic pressures (EDP) most correlated with 1- and 10-year outcomes. Transplanted patients with SVP < 14 and non- transplanted patients with PCWP < 15 had 100% survival 1-year post-transplantation. CONCLUSION: For the first time, our study identifies that hemodynamic parameters most strongly correlate with 1- and 10-year outcomes in ACHD patients considered for transplantation, using a data-driven machine learning model.

Baseline tubular biomarkers in young adults with congenital heart disease as compared to healthy young adults: Detecting subclinical kidney injury.

BACKGROUND: There are significant implications for kidney disease in young adults with congenital heart disease. Prior investigations have not focused on the use of urinary tubular biomarkers for the early identification of kidney disease in this growing patient group. OBJECTIVE: Determine if young adults with congenital heart disease have differences in the baseline concentration of urinary tubular biomarkers when compared to healthy young adults. DESIGN/METHODS: In a pilot case control study, 30 patients from 18 to 35 years of age with congenital heart disease and a normal serum creatinine were recruited during a routine follow-up visit. In the same age group, 30 control subjects without history of heart or kidney disease were recruited. Urine samples were obtained to measure beta 2-microglobin, alpha 1-microglobin, N-acetyl-B-D-glucosaminidase, liver fatty acid binding protein, kidney injury molecule-1, insulin-like growth factor binding protein 7, and tissue inhibitor of metalloproteinases-2. Comparisons were done using Wilcoxon rank-sum or Fisher's exact test. RESULTS: No study participants had proteinuria on urine dipstick. Median concentrations of kidney injury molecule-1 were higher (P = .01) and concentrations of insulin-like growth factor binding protein 7 (P = .001) and tissue inhibitor of metalloproteinases-2 (P = .009) were lower in the subjects with congenital heart disease when compared to the control subjects. There were no significant differences between the groups with respect to the other biomarkers. CONCLUSION: Our data suggest that young adults with congenital heart disease may have subclinical kidney dysfunction. Lower levels of insulin-like growth factor binding protein 7 and tissue inhibitor of metalloproteinases-2 may indicate an impaired ability to respond to injury, while higher levels of kidney injury molecule-1 may reflect early tubular injury.

Residents' understanding of adult congenital heart disease.

OBJECTIVE: Medical residents are exposed to increasing numbers of adults with congenital heart disease (ACHD). While inadequate ACHD knowledge may lead to inappropriate practice, this educational deficit has not been investigated. Our aim was to analyze residents' attitudes, perceived ability, and knowledge of ACHD medicine. DESIGN, METHODS, OUTCOME MEASURES: A single center, multiprogram cross-sectional study was conducted in 2015 using an electronic survey to assess 472 medical residents' perceived knowledge and self-assessed skills related to ACHD medicine. Demographic data obtained included age, gender, level of training and program type. RESULTS: The survey was completed by 25% of surveyed residents (N = 116, median age 29.5 years, 39% male). Responses were received from Family Physician (FP; 8.5%), Internal Medicine (43%), Pediatrics (34%), Internal Medicine-Pediatrics (IM-P; 7%), and Transitional residents (4%). There was no difference between ACHD knowledge and year of residency (P = NS). IM-P residents were more confident in their knowledge and assessment of ACHD patients (P < .05). Those with prior cardiology elective during residency (59%) demonstrated a significant correlation with ACHD knowledge (P < .05) and confidence in ability to assess ACHD patients (P < .05). Overall, knowledge and confidence in ACHD assessment trended towards a positive correlation (P = .061, gamma statistic = 0.8). Residents' learning preferences included ACHD lectures (81.6%) and web sites (60.2%). CONCLUSION: Most residents in this study lacked ACHD knowledge or comfort level required to care for adults with complex defects. Still, residents remain interested in teaching venues to improve examination skills. Residency programs should include routine cardiology electives to prepare residents to care for this complex group of patients.

Patient Preference and Perception of Care Provided by Advance Nurse Practitioners and Physicians in Outpatient Adult Congenital Clinics.

OBJECTIVE: Nurse practitioners (NPs) have an established role for delivering competent care to patients in the primary care setting. The aim of this study was to compare satisfaction of patients managed by NPs vs. physicians in the outpatient adult congenital heart disease (CHD) clinic. DESIGN: A prospective study conducted in two outpatient adult CHD clinics to assess patient satisfaction through standardized surveys. Demographic data included CHD diagnosis, age, and New York Heart Association functional class. All patients completed a Short-Form-12 to assess health status. RESULTS: Of the 371 patients recruited (52% men; median age 29 years, severe CHD complexity 31.5%) and seen by NP (n = 187) or physician (n = 184), physician-managed practices had higher perceived: overall experience, courtesy of provider (P < 0.05) and confidence (trust) in provider (P < 0.1). Overall, patients reported satisfaction with an NP providing care (98%), the NP was able to effectively deal with illness/CHD (95%), and an increased chance (94%) or willingness to see an NP at a future visit. Only 73% reported an understanding of NP training and how an NP differed from a registered nurse. There was a stronger perception of how an NP differed from a physician (83%). CONCLUSION: Patient satisfaction was high regardless of whether care was provided by physicians or NPs. However, patients appear to make distinctions in what they believe the type of care each practitioner is best at providing. Patient education regarding competence of the different health care providers may continue to improve patient satisfaction.

Current and future status of adult congenital training in North America.

Adult congenital heart disease (ACHD) presents a unique and growing patient population, but there are no data to suggest there are sufficient training programs available to train specialists to meet the needs of this population. The objective of this national survey was to determine the current status of ACHD fellowship curricula and training. An online questionnaire was provided to adult and pediatric cardiology fellowship programs to determine program demographics, status, duration, faculty teaching responsibilities, volume or patient exposure, and institutional obstacles. Of the 225 programs surveyed, 81 responded (36%). Nearly all were university affiliated (93%) with <1/3 (29%) reporting an ACHD fellowship program. Most offered either a 1- (42%) or a 2-year (47%) fellowship. ACHD fellowship funding was provided by hospital budget (68%). Half reported an increase in applicants to their fellowship. Applicants were more likely to have had previous training in adult cardiovascular medicine (56%). Respondents ranked "Volume of Outpatients/Year" and "Lack of Institutional Support" as the most important aspect of an ACHD program to applicants and greatest obstacle to ACHD training, respectively. After training, ACHD fellows obtained adult cardiology/ACHD positions (47%) and were within an academic center (88%). In conclusion, the results demonstrate a deficiency in the number of currently available ACHD fellowship programs. Measures should be taken to strengthen and standardize ACHD training to meet the increasing workforce requirements of this population.