S100B and its relation to cerebral oxygenation in neonates and infants undergoing surgery for congenital heart disease

Objectives: Neonates and infants undergoing surgery for congenital heart disease are at risk for developmental impairment. Hypoxic‐ischemic brain injury might be one contributing factor. We aimed to investigate the perioperative release of the astro‐ cyte protein S100B and its relation to cerebral oxygenation.
Methods: Serum S100B was measured before and 0, 12, 24, and 48 hours after sur‐ gery. Cerebral oxygen saturation was derived by near‐infrared spectroscopy. S100B reference values based on preoperative samples; concentrations above the 75th per‐ centile were defined as elevated. Patients with elevated S100B at 24 or 48 hours were compared to cases with S100B in the normal range. Neonates (≤28 days) and infants (>28 and ≤365 days) were analyzed separately due to age‐dependent release of S100B.
Results: Seventy‐four patients underwent 94 surgical procedures (neonates, n = 38; infants, n = 56). S100B concentrations were higher in neonates before and after sur‐ gery at all time points (P ≤ .015). Highest values were noticed immediately after sur‐ gery. Postoperative S100B was elevated after 15 (40.5%) surgeries in neonates. There was no difference in pre‐, intra‐, or postoperative cerebral oxygenation. In in‐ fants, postoperative S100B was elevated after 23 (41.8%) procedures. Preoperative cerebral oxygen saturations tended to be lower (53 ± 12% vs 59 ± 12%, P = .069) and arterial‐cerebral oxygen saturation difference was higher (35 ± 11% vs 28 ± 11%, P = .018) in infants with elevated postoperative S100B. In the early postoperative course, cerebral oxygen saturation was lower (54 ± 13% vs 63 ± 12%, P = .011) and arterial‐cerebral oxygen saturation difference was wider (38 ± 11% vs 30 ± 10%, P = .008). Cerebral oxygen saturation was also lower for the entire postoperative course (62 ± 18% vs 67 ± 9%, P = .047).
Conclusions: Postoperative S100B was elevated in about 40% of neonates and in‐ fants undergoing cardiac surgery. Infants with elevated postoperative S100B had impaired perioperative cerebral tissue oxygenation. No relation between S100B and cerebral oxygenation could be demonstrated in neonates.     

Ancillary referral patterns in infants after initial assessment in a cardiac developmental outcomes clinic

Objective: Neurodevelopmental impairment is common after surgery for congeni‐ tal heart disease (CHD) in infancy. While neurodevelopmental follow‐up of high‐risk patients has increased, the referral patterns for ancillary services following initial evaluation have not been reported. The aim of this study is to describe the rates and patterns of referral at the initial visit to our outcomes clinic of patients who under‐ went surgery for CHD during infancy.
Outcomes Measures: The Cardiac Developmental Outcomes Program clinic at Texas Children’s Hospital provides routine longitudinal follow‐up with developmental pedi‐ atricians and child psychologists for children who required surgery for CHD within the first 3 months of life. Demographic, diagnostic, and clinical data, including prior receipt of intervention and referral patterns at initial presentation, were abstracted from our database.
Results: Between April 2013 and May 2017, 244 infants under 12 months of age presented for initial evaluation at a mean age of 7 ± 1.3 months. At presentation, 31% (76/244) were referred for either therapeutic intervention (early intervention or pri‐ vate therapies), ancillary medical services, or both. Referral rates for low‐risk (STAT 1‐3) and high‐risk (STAT 4‐5) infants were similar (28 vs. 33%, P = .48). Referrals were more common in: Hispanic white infants (P = .012), infants with non‐cardiac congeni‐ tal anomalies (P = .001), history of gastrostomy tube placement (P < .001), and infants with prior therapy (P = .043). Infants of non‐English speaking parents were three times more likely to be referred (95% CI = 1.5, 6.4; P = .002).
Conclusion: At the time of presentation, nearly 1 in 3 infants required referral. Referral patterns did not vary by traditional risk stratification. Sociodemographic fac‐ tors and co‐morbid medical conditions increased the likelihood of referral. This sup‐ ports the need for routine follow‐up for all post‐surgical infants regardless of level of surgical complexity. Further research into the completion of referrals and long‐term referral patterns is needed.     

Heart murmurs and echocardiography findings in the normal newborn nursery

Objective: To determine the clinical findings and management implications of echocardiograms performed on infants with murmurs in the nursery.
Design: Retrospective cohort study conducted from January 2008 through December 2015. Patients in the study were followed by chart review for up to 5 years. In addition, a survey of nursery providers was conducted in February 2016.
Setting: A single community hospital associated with a university.
Patients: All 26 573 infants who received care in the normal newborn nursery were eligible for inclusion in the study. Infants with echocardiograms were analyzed. The survey was sent by e‐mail to all 135 physicians who work in the nursery.
Outcome Measures: The primary outcomes include the specific findings on echocar‐ diogram and whether the findings required an acute change in management, outpa‐ tient follow up, or were incidental findings. The primary survey question was how physicians would manage an otherwise asymptomatic newborn with a heart murmur.
Results: Four hundred ninety‐nine infants had echocardiograms, and over the study period the utilization of echocardiography increased from 1.02% to 2.56% (P < .001) of all infants. Three hundred fifty‐four babies had echocardiography performed be‐ cause of a heart murmur. One hundred sixty‐three (46.0%) of these echocardiograms were normal and 160 (45.2%) had findings that did not require additional care. Twenty‐three neonates (6.5%) had echocardiographic findings that necessitated out‐ patient follow‐up and 8 neonates (2.3%) required neonatal intensive care due to the findings on their echocardiogram. In total, 14 infants (4%) would go on to require heart surgery or interventional cardiac catheterization. 63/135 (47%) physicians completed the survey, with wide variations in the management of newborns with heart murmurs.
Conclusions: The use of echocardiography in the normal newborn nursery has in‐ creased with time despite improved prenatal detection of heart disease and the use of pulse oximetry screening, and identifies significant heart disease in a small but important number of infants.     

Midterm outcomes of right ventricular outflow tract reconstruction using the Freestyle xenograft

Objective: Various options exist for right ventricular outflow tract (RVOT) recon‐ struction in congenital heart disease. The Freestyle porcine aortic root may be used but its longevity is not well defined.
Design: We performed a retrospective review of all non‐Ross RVOT reconstructions using the Freestyle root in our institution. Survival and reintervention, either by sur‐ gery, transcatheter valve implantation, balloon valvuloplasty, or bare metal stent placement, were recorded. Factors associated with reintervention were assessed using Cox regression.
Results: Between January 2002 and December 2015, there were 182 patients identi‐ fied. Sixteen patients were lost to follow‐up and 3 patients died, unrelated to cardiac surgery. Of the remaining 163 patients, the median age was 12.2 years (interquartile range 6.4‐16.4), median weight was 39.0 kg (interquartile range 19.9‐59.3), and the median body surface area was 1.23 m² (interquartile range 0.79‐1.64). Ninety‐three (57%) patients had tetralogy of Fallot. The median follow‐up was 5.4 years (inter‐ quartile range 2.9‐8 years). There were no operative or cardiac‐related deaths. Thirty‐eight patients (23%) required reintervention. The rate of freedom from rein‐ tervention was 93.2% (95% CI 86.7%‐96.6%) at 5 years and 48.4% (95% CI 34.9%‐60.6%) at 10 years. Age < 10 years, weight < 39 kg, and body surface area <1.2 m2 at the time of valve placement, as well as valve size ≤25 mm were signifi‐ cantly associated with need for earlier reintervention.
Conclusions: The Freestyle root in the RVOT is associated with excellent survival and low midterm need for reintervention. Its longevity is comparable to published data on homografts and other bioprosthetic valves.     

Can transcatheter PDA closure be performed in neonates ≤1000 grams? The Memphis experience

Objective: Advancements in transcatheter technology have now made it possible to safely close patent ductus arteriosus (PDA) in extremely low birth weight (ELBW) infants. The objective of this article is to describe our technique for transcatheter PDA closure (TCPC) in ELBW infants.
Design: The techniques employed are very specific to this population and are drasti‐ cally different when compared to the procedure performed in patients weighing >5 kg.
Setting: A multidisciplinary team approach should be taken to evaluate and manage ELBW infants in order to achieve success. It is important that specific techniques with venous‐only approach outlined in this article be followed to achieve optimal results with low risk of complications.
Patients: To date, in Memphis, 55 ELBW infants have had successful TCPC at a weight of ≤1000 g with minimal procedure‐related complications.
Interventions: It is important that specific techniques with venous‐only approach outlined in this article be followed to achieve optimal results with low risk of complications.
Outcome measures: This procedure entails a steep learning curve and should be lim‐ ited to specialized centers with expertise in these thanscatheter procedures.
Results: There has been 100% procedural success of performing TCPC in children ≤1000 g. There have been only two procedure‐related complications which hap‐ pened to the first two patients, ≤1000 g, that we performed TCPC on.
Conclusions: It is feasible and probably safe to perform TCPC in children ≤1000 g. The techniques described in this article represent our institutional experience and have helped us improve clinical outcomes in ELBW infants.     

Surveillance and screening practices of New England congenital cardiologists for patients after the Fontan operation

Introduction: Surveillance and management guidelines for Fontan patients are lacking due to the paucity of evidence in the literature of screening efficacy on outcome measures.
Methods: The Fontan Working Group within the New England Congenital Cardiology Association designed an electronic survey to assess surveillance practices for pa‐ tients with Fontan procedures among New England congenital cardiologists and to explore variability in screening low‐risk vs high‐risk Fontan patients across regional programs.
Results: Fifty‐six cardiologists representing 12 regional programs responded to the survey, comprising ~40% of the total New England congenital cardiac physicians. The majority of desired testing and consultation was available within 50 miles of the pa‐ tient's home institution with some limitations of cardiac catheterization and cardiac magnetic resonance imaging availability. Surveillance and screening were less fre‐ quent in low‐risk Fontan patients compared to high‐risk Fontan patients. Counseling practices were similar for both low‐risk and high‐risk Fontan patients. Aspirin mono‐ therapy was recommended by 82% of providers for low‐risk Fontan patients, while anticoagulation regimens were more varied for the high‐risk population. Practitioners with ≤15 years of experience were more likely to provide quality of life testing in both low‐risk and high‐risk Fontan patients. There were no other major differences in test‐ ing frequencies by years of practice, quaternary vs nonquaternary care facility, or the number of Fontan patients in a practice.
Conclusion: This survey provides insight into regional practices of screening and surveillance of Fontan patients. These data may be used to design future research studies and evidence‐based guidelines to streamline the approach to manage these complex patients.     

Lost in the system? Transfer to adult congenital heart disease care-Challenges and solutions

Objective: Transfer of congenital heart disease care from the pediatric to adult set‐ ting has been identified as a priority and is associated with better outcomes. Our objective is to determine what percentage of patients with congenital heart disease transferred to adult congenital cardiac care.
Design: A retrospective cohort study.
Setting: Referrals to a tertiary referral center for adult congenital heart disease pa‐ tients from its pediatric referral base.
Patients: This resulted in 1514 patients age 16‐30, seen at least once in three pediat‐ ric Georgia health care systems during 2008‐2010.
Interventions: We analyzed for protective factors associated with age‐appropriate care, including distance from referral center, age, timing of transfer, gender, severity of adult congenital heart disease, and comorbidities.
Outcome Measures: We analyzed initial care by age among patients under pediatric care from 2008 to 2010 and if patients under pediatric care subsequently transferred to an adult congenital cardiologist in this separate pediatric and adult health system during 2008‐2015.
Results: Among 1514 initial patients (39% severe complexity), 24% were beyond the recommended transfer age of 21 years. Overall, only 12.1% transferred care to the referral affiliated adult hospital. 90% of these adults that successfully transferred were seen by an adult congenital cardiologist, with an average of 33.9 months be‐ tween last pediatric visit and first adult visit. Distance to referral center contributed to delayed transfer to adult care. Those with severe congenital heart disease were more likely to transfer (18.7% vs 6.2% for not severe).
Conclusion: Patients with severe disease are more likely to transfer to adult congeni‐ tal heart disease care than nonsevere disease. Most congenital heart disease patients do not transfer to adult congenital cardiology care with distance to referral center being a contributing factor. Both pediatric and adult care providers need to under‐ stand and address barriers in order to improve successful transfer.     

Vasopressor magnitude predicts poor outcome in adults with congenital heart disease after cardiac surgery

Background: High levels of vasoactive inotrope support (VIS) after congenital heart surgery are predictive of morbidity in pediatric patients. We sought to discern if this relationship applies to adults with congenital heart disease (ACHD).
Methods: We retrospectively studied adult patients (≥18 years old) admitted to the intensive care unit after cardiac surgery for congenital heart disease from 2002 to 2013 at Mayo Clinic. Vasoactive medication dose values within 96 hours of admis‐ sion were examined to determine the relationship between VIS score and poor out‐ come of early mortality, early morbidity, or complication related morbidity.
Results: Overall, 1040 ACHD patients had cardiac surgery during the study time frame; 243 (23.4%) met study inclusion criteria. Sixty‐two patients (25%), experi‐ enced composite poor outcome [including eight deaths within 90 days of hospital discharge (3%)]. Thirty‐eight patients (15%) endured complication related early mor‐ bidity. The maximum VIS (maxVIS) score area under the curve was 0.92 (95% CI: 0.86‐0.98) for in‐hospital mortality; and 0.82 (95% CI: 0.76‐0.89) for combined poor clinical outcome. On univariate analysis, maxVIS score ≥3 was predictive of compos‐ ite adverse outcome (OR: 14.2, 95% CI: 7.2‐28.2; P < 0.001), prolonged ICU LOS ICU LOS (OR: 19.2; 95% CI: 8.7‐42.1; P < 0.0001), prolonged mechanical ventilation (OR: 13.6; 95% CI: 4.4‐41.8; P < 0.0001) and complication related morbidity (OR: 7.3; 95% CI: 3.4‐15.5; P < 0.0001).
Conclusions: MaxVIS score strongly predicted adverse outcomes and can be used as a risk prediction tool to facilitate early intervention that may improve outcome and assist with clinical decision making for ACHD patients after cardiac surgery.     

Coronary artery disease screening in adults with congenital heart disease prior to cardiac surgery

Objective: As adults with congenital heart disease (CHD) grow older, preoperative screening for coronary artery disease (CAD) may be indicated prior to CHD surgery. Data regarding the indications for preoperative CAD screening in this population are limited. Current practice is to follow guidelines for patients with valvular heart dis‐ ease; however, the risk for CAD in certain congenital heart diagnoses may be higher than the general population. This study aimed to assess the results of preoperative CAD screening in patients prior to CHD surgery.
Design: Retrospective study.
Setting: Single tertiary center.
Patients: Patients ≥35 years that had CHD surgery from 1/1/2007 to 5/1/2017.
Outcome Measures: Data regarding CAD risk factors and preoperative CAD screen‐ ing results were obtained. Prevalence and risk factors for CAD were analyzed, along with their relationship to perioperative outcomes.
Results: A total of 73 patients underwent CAD screening with either cardiac catheteri‐ zation (56%) or computed tomography angiography (34%) prior to CHD surgery. Overall 16 (22%) patients were found to have CAD. Only two patients had severe coronary stenosis and underwent coronary bypass grafting at time of CHD surgery. Patients with CAD were more likely to be older and have history of hypertension, dyslipidemia, and tobacco smoking. CHD diagnosis was not significantly associated with presence of CAD.
Conclusion: CAD is common in asymptomatic older patients referred for screening prior to CHD surgery; however, severe CAD requiring concomitant coronary inter‐ vention is uncommon. Preoperative CAD screening should be based on age and tra‐ ditional CAD risk factors, rather than underlying CHD.     

Parental anxiety before invasive cardiac procedure in children with congenital heart disease: Contributing factors and consequences

Objective: Medical information provided to parents of a child with a congenital heart disease can induce major stress. Visual analog scales have been validated to assess anxiety in the adult population. The aim of this study was to analyze parental anxiety using a visual analog scale and to explore the influencing factors.
Design: This prospective cross‐sectional study.
Setting: Tertiary care regional referral center for congenital heart disease of Marseille-La Timone university hospital.
Patients: Parents of children with a congenital heart disease, as defined by the ACC‐ CHD classification, referred for cardiac surgery or interventional cardiac catheterization, were offered to participate.
Intervention and outcome measure: The parental level of anxiety was assessed using a visual analog scale (0‐10) before intervention and after complete information given by the cardiologist, the surgeon or the anesthetists.
Results: Seventy‐three children [7 days‐13 years], represented by 49 fathers and 71 mothers, were included in the study. A total of 42 children required cardiac surgery and 31 children underwent interventional cardiac catheterization. The mean score of maternal anxiety was significantly higher than the paternal anxiety (8.2 vs 6.3, P < .01). A high level of maternal anxiety (visual analog scale > 8) was associated with paternal anxiety (P = .02), the child's comorbidity (P = .03), the distance between home and referral center (P = .04), and the level of risk adjustment for congenital heart surgery (P = .01). In multivariate analysis, maternal anxiety was associated with paternal anxiety (OR = 4.9; 95% confidence interval [1.1‐19.2]), and the level of risk adjustment for congenital heart surgery (OR = 11.4; 95% confidence interval [1.2‐116.2]). No significant association was found between parental anxiety and prenatal diagnosis.
Conclusion: This study highlighted several factors associated with the parental anxiety. Identifying the parents at risk of high stress can be useful to set up psychological support during hospitalization.     

Contributors to disease‐specific health knowledge in adults with congenital heart disease: A correlational study

Objective: Growth in the adults with congenital heart disease (ACHD) population represents a challenge to the health care infrastructure. As patients with chronic disease are increasingly held accountable for their own care, contributors to disease‐ specific health knowledge, which are known to correlate with patients’ participation in care, merit investigation to design patient‐focused interventions.
Design: We conducted a single‐site, cross‐sectional study of ACHD patients. Investigators retrospectively gathered clinical data as well as psychometric and health status assessments completed at the time of enrollment.
Outcome Measures: We investigated the impact of clinical and psychological varia‐ bles on Leuven Knowledge Questionnaire for Congenital Heart Diseases health knowledge composite scores (HKCS). Variables with significant associations were considered in a stepwise multivariable regression model to determine which combi‐ nation of variables jointly explained variability in HKCS.
Results: Overall HKCS was associated with the number of prior cardiac surgeries (r = 0.273; 95% CI: 0.050‐0.467; P = .016), perceived stress (r = 0.260; 95% CI: 0.033‐0.458; P = .024), SF‐36 emotional well‐being (r = −0.251; 95% CI: −0.451, −0.024; P = .030), history of noncardiac surgery (P = .037), cirrhosis (P = .048), and presence of implantable cardioverter‐defibrillator (P = .028). On multivariable mod‐ eling, only the number of cardiac surgeries was found to correlate with HKCS.
Conclusions: While univariate correlations were found between HCKS and several other clinical and psychological variables, only number of prior cardiac surgeries inde‐ pendently correlated with disease‐specific health knowledge in ACHD patients. These results suggest that clinical and psychological variables are not impediments to disease‐specific health knowledge.     

Feeding methods for infants with single ventricle physiology are associated with length of stay during stage 2 surgery hospitalization

Background: Tube feedings are often needed to achieve the growth and nutrition goals associated with decreased morbidity and mortality in patients with single ventricle anat‐ omy. Variability in feeding method through the interstage period has been previously described, however, comparable information following stage 2 palliation is lacking.
Objectives: To identify types of feeding methods following stage 2 palliation and their influence on length of stay.
Design: Secondary analysis of the National Pediatric Cardiology Quality Improvement Collaborative registry was performed on 932 patients. Demographic data, medical characteristics, postoperative complications, type of feeding method, and length of stay for stage 2 palliation were analyzed.
Results: Type of feeding method remained relatively unchanged during hospitalization for stage 2 palliation. Gastrostomy tube fed only patients were the oldest at time of surgery (182.7 ± 57.7 days, P < .001) and had the lowest weight‐for‐age z scores at ad‐ mission (−1.6 ± 1.4, P < .001). Oral + gastrostomy tube groups had the longest median bypass times (172.5 minutes, P = .001) and longest length of stay (median 12 days, P < .001). Multivariable modeling revealed that feeding by tube only (P < .001), oral + tube feeding (P ≤ .001), reintubation (P < .001), and prolonged intubation (P < .001) were associated with increased length of stay. Neither age (P = .156) nor weight‐for‐age z score at admission (P = .066) was predictive of length of stay.
Conclusions: Feeding methods established at admission for stage 2 palliation are not likely to change by discharge. Length of stay is more likely to be impacted by tube feeding and intubation history than age or weight‐for‐age z score at admission. Better understanding for selection of feeding methods and their impact on patient out‐ comes is needed to develop evidence‐based guidelines to decrease variability in clini‐ cal practice patterns and provide appropriate counseling to caregivers.     

Identifying self‐reported neurocognitive deficits in the adult with congenital heart disease using a simple screening tool

Objective: Children with congenital heart disease (CHD) and adults with acquired heart disease are at an increased risk of neurocognitive impairment. The objective of this study was to determine the prevalence of self‐reported neurocognitive impair‐ ment and its risk factors in the adult congenital heart disease (ACHD) population.
Design: The Wisconsin Adult Congenital Heart Disease Program recently began screening ACHD patients to identify those with significant self‐perceived neurocog‐ nitive impairments. Screening consists of using a validated neuro‐oncology screening instrument that has been modified for the ACHD population. Patients who answer this survey in a predetermined fashion consistent with significant self‐perceived neu‐ rocognitive deficits are referred for a formal neurocognitive evaluation. Demographic and clinical information are obtained by chart review.
Results: Three hundred ten patients (49% males) completed the screening process. The average age was 30 years (range: 17–69 years). For the cohort, 57 (18%) patients had no prior cardiac surgeries, 85 (28%) one surgery, 77 (25%) two, and 91 (29%) at least three surgeries. Of those screened, 106 (34%) met criteria for a formal neuro‐ cognitive evaluation. Patients who were referred had undergone a greater number of prior cardiac surgeries (2.2 vs 1.7, P = .008) and were more likely to have severe com‐ plexity CHD (P = .006). Of those patients who were referred, the worst perceived functioning was in math and attention.
Conclusion: There is a high prevalence of ACHD patients with significant self‐per‐ ceived neurocognitive deficits. Simple screening questionnaires may help identify those patients at high risk and allow for timely and appropriate referral for formal neurocognitive evaluation, diagnosis, and therapy.     

Health care‐associated infections are associated with increased length of stay and cost but not mortality in children undergoing cardiac surgery

Introduction: Health care‐associated infections (HAIs) increase mortality, length of stay, and cost in hospitalized patients. The incidence of and risk factors for developing HAIs in the pediatric population after cardiac surgery have been studied. This study evaluates the impact of HAIs on length of stay, inpatient mortality, and cost of hospitalization in the pediatric population after cardiac surgery.
Methods: TheKids’InpatientDatabasewasqueriedforanalysis.Patientsunder18years of age who underwent cardiac surgery from 1997 to 2012 were included. HAIs were defined as central line‐associated blood stream infections, catheter‐associated urinary tract infections, ventilator‐associated pneumonias, and surgical wound infections. Univariate analysis compared admissions with and without a HAI. Next, regression analysis was done to determine patient factors independently associated with a HAI, and to determine what specific HAIs were independently associated with our primary outcomes.
Results: In total 46 169 admissions were included, 773 (1.6%) of which had a HAI. Regression analysis showed younger age (P < .001), heart failure (OR 1.2, 95% CI 1.1‐1.4, P = .03), and acute kidney injury (AKI; 2.7, 2.0‐3.6, P < .001), among others were all independently associated with a HAI. The presence of HAI was associated with increased length of stay (median 29 vs 6 days, P < .001), total cost (median $271 884 vs $88 385, P < .001), and inpatient mortality (6.1% vs 2.5%, P < .001) by univariate analysis. Regression analysis demonstrated that each HAI were independently associated with increased length of stay and increased total charges for the hospital stay. However, HAI, was not associated with increased mortality after regression analysis.
Conclusions: The incidence of HAIs in this analysis was low (1.6%) but contributed significantly to length of stay and cost. No individual HAI was associated with increased mortality. Potential modifiable risk factors include age and prevention of AKI.     

Efficiency of the home cardiac rehabilitation program for adults with complex congenital heart disease

Objective: We aimed to study the efficiency and safety of once‐a‐week outpatient rehabilitation followed by home program with tele‐monitoring in patients with com‐ plex cyanotic congenital heart disease.
Design: Prospective nonrandomized study.
Method: Patients who have been diagnosed either Eisenmenger’s syndrome or inop‐ erable complex cyanotic heart disease and able to attend 12‐week cardiac rehabilita‐ tion program were included. Training with treadmill walking and bicycling under supervision at cardiac rehabilitation unit once‐a‐week in the first 6 weeks followed by home‐based exercise program (bicycle and walking) with a target at 40%‐70% of maximum heart rate (HRmax) at pretraining peak exercise for another 6 weeks was performed in the intervention group. Video and telephone calls were scheduled for evaluation of compliance and complication. Data from cardiopulmonary exercise testing (CPET) on cycle ergometry including peak oxygen consumption (peakVO2), oxygen pulse (O2 pulse), ventilatory equivalent for carbon dioxide (VE/CO2 at an‐ aerobic threshold), constant work‐rate endurance time (CWRET) at 75% of peak VO2, and 6‐minute walk distance (6MWD) were compared between baseline and after training by paired t test.
Result: Of the 400 patients in our adult congenital heart disease clinic, 60 patients met the inclusion criteria. Eleven patients who could follow program regularly were assigned home program. There was a statistically significant improvement of CWRET, O₂ pulse, and 6MWD after finishing the program (P = .003, .039, and .001, respec‐ tively). The mean difference of 6MWD change in the home‐program group was sig‐ nificantly higher than in the control group (69.3 ± 47.9 meters vs. 4.1 ± 43.4 meters, P = .003). No serious adverse outcomes were reported during home training.
Conclusion: Once‐a‐week outpatient hospital‐based exercise program followed by supervised home‐based exercise program showed a significant benefit in improve‐ ment of exercise capacity in adults with complex cyanotic congenital heart disease without serious adverse outcomes.     

Hospital resource utilization and presence of advance directives at the end of life for adults with congenital heart disease

Objective: Overall health care resource utilization by adults with congenital heart disease has increased dramatically in the past two decades, yet little is known about utilization patterns at the end of life. The objective of this study is to better under‐ stand the patterns and influences on end‐of‐life care intensity for adults with con‐ genital heart disease.
Methods: We identified a sample of adults with congenital heart disease (n = 65), cancer (n = 10 784), or heart failure (n = 3809) who died between January 2010 and December 2015, cared for in one multi‐hospital health care system. We used multi‐ variate analysis to evaluate markers of resource utilization, location of death, and documentation of advance care planning among patients with congenital heart dis‐ ease versus those with cancer and those with heart failure.
Results: Approximately 40% of adults with congenital heart disease experienced in‐ patient and intensive care unit (ICU) hospitalizations in the last 30 days of life; 64% died in the hospital. Compared to patients with cancer, patients with adult congenital heart disease (ACHD) were more likely to have inpatient (adjusted risk ratio 1.57; 95% CI 1.12‐2.18) and ICU admissions in the last 30 days of life (adjusted risk ratio 2.56; 95% CI 1.83‐3.61), more likely to die in the hospital (adjusted risk ratio 1.75; 95% CI 1.43‐2.13), and more likely to have documentation of advance care planning (adjusted risk ratio 1.46; 95% CI 1.09‐1.96). Compared to patients with heart failure (HF), patients with ACHD were less likely to have an ICU admission in the last 30 days of life (adjusted risk ratio 0.73; 95% CI 0.54‐0.99).
Conclusions: Adults with congenital heart disease have significant hospital resource utilization near the end of life compared to patients with cancer, notable for more hospitalizations and a higher likelihood of death in the hospital. This population rep‐ resents an important opportunity for the application of palliative and supportive care.     

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: arrhyth‐ mia, heart failure/pulmonary edema, transient ischemic attack, stroke, dissection, myo‐ cardial infarction, cardiac arrest, death during gestation and up to 6 months postpartum.
Results: Of 178 women, the most common CHD lesions were congenital aortic ste‐ nosis (15.2%), ventricular septal defect (13.5%), atrial septal defect (12.9%), and te‐ tralogy 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.     

Utilization of echocardiography in Ehlers‐Danlos syndrome

Objectives: This study assessed the frequency and utility of echocardiographic examination in patients with all forms of Ehlers‐Danlos syndrome and sought to iden‐ tify clinical variables associated with an abnormal echocardiogram.
Design/setting: This was a retrospective study of all patients carrying a diagnosis of Ehlers‐Danlos syndrome of any type who were evaluated by a pediatrician or pediatric subspecialist at a single tertiary medical center with an affiliated children’s hospital during the period January 2013 to December 2018.
Patients: Chart review was performed on all patients carrying a diagnosis of Ehlers‐ Danlos syndrome in the electronic medical record.
Outcome Measures: Data from genetics examination, cardiovascular examination where applicable, genetic test results when available, and echocardiography were recorded.
Results: Of 262 patients identified, echocardiography and cardiac evaluation were common occurring in 90% and 50% of patients with any form of Ehlers‐Danlos syndrome. Cardiovascular complications occurred in 50% of patients with vascular Ehlers‐Danlos syndrome but echocardiography was normal in all. Aortic dilation was common in classic Ehlers‐Danlos syndrome but absent in hypermobile Ehlers‐Danlos syndrome. Mitral valve prolapse and bicuspid aortic valve occurred at the same inci‐ dence as the general population. Cardiac symptoms were present in 12% but did not correlate with abnormal cardiac structure. Presentation with symptoms of musculo‐ skeletal pain was inversely related to the presence of cardiac pathology.
Conclusions: In light of the absence of cardiac pathology in patients with hypermo‐ bile Ehlers‐Danlos syndrome, routine cardiac evaluation and echocardiography are not required for patients with hypermobile Ehlers‐Danlos syndrome.     

Long‐term follow‐up of adult patients with congenital heart disease and an implantable cardioverter defibrillator

Objective: Sudden cardiac death is common in the adult congenital heart disease (ACHD) population. Knowledge and experience about the use of implantable cardio‐ verter defibrillators (ICD) in ACHD patients is very limited. We aimed to characterize a cohort of patients with ACHD and ICDs.
Design: Thirty consecutive ACHD patients submitted to an ICD implantation in a single tertiary center were evaluated. Data on baseline clinical features, heart defect, indication for ICD, type of device, appropriate therapies, ICD‐related complication, and mortality during follow‐up were collected.
Results: Of the 30 patients, 56.7% received appropriate therapies due to ventricular tachycardia (VT) or ventricular fibrillation (VF). The rate of inappropriate therapies and device‐related complications was 33.3%. Secondary prevention and primary pre‐ vention patients with class I indications for ICD had more appropriate therapies than complication, but this relationship was reversed for patients with class II indications. Remote monitoring played an important role in diagnosing new atrial arrhythmias be‐ fore scheduled visits in 46.2% of patients, leading to a change in medication. VT/VF episodes were associated with a composite of death, cardiac transplantation, and hos‐ pital admission (OR 13.0; 95% CI: 2.1‐81.5).
Conclusion: ICDs are not only useful in preventing SCD, but also have a major role in diagnosing atrial tachyarrhythmias ahead of scheduled visits. Although improve‐ ments in ICD technology might reduce complications and inappropriate therapies, adequate selection of candidates for primary prevention still remains difficult be‐ cause of the lack of clear indications.