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To determine if patients with congenital heart disease are undergoing laparoscopic surgery requiring abdominal insufflation and to compare the outcomes of these procedures with those who underwent an open surgical approach.This was a retrospective study using the National Inpatient Sample from 2006 to 2014. Individuals with congenital heart disease who underwent at least one of six selected surgical procedures (laparoscopic or open) were included in the study. Subgroup analysis was performed on patients with Fontan palliation.The primary outcome was to determine the frequency with which congenital heart disease patients undergo laparoscopic surgery requiring abdominal insufflation compared with open surgery. Secondary outcomes included all-cause in-hospital mortality and in-hospital length of stay. Of the 5,527 patients included, nearly half underwent laparoscopic surgery (46.3%), and 128 (2.3%) had single-ventricle circulation. All-cause mortality was significantly higher for those who underwent open surgery compared with the laparoscopic approach (3.6% v 0.9%; odds ratio [OR], 4.0 [2.6-6.3]; p0.0001). Subgroup analysis of patients with Fontal palliation older than five years showed 30 (42%) underwent laparoscopic surgery and there was no mortality difference between the laparoscopic and open approaches (OR, 1.4 [0.2-21.3], p = 0.8). Length of stay was significantly shorter for patients undergoing laparoscopic compared with open surgery (median three days [interquartile range, two-five] v six days [three-13], p0.0001).Individuals with congenital heart disease are being offered laparoscopic surgery that requires abdominal insufflation. All-cause mortality and length of stay were higher for patients who underwent open surgical operations.

Background Adults with congenital heart disease (CHD) have been considered potentially high risk for novel coronavirus disease-19 (COVID-19) mortality or other complications. Objectives This study sought to define the impact of COVID-19 in adults with CHD and to identify risk factors associated with adverse outcomes. Methods Adults (age 18 years or older) with CHD and with confirmed or clinically suspected COVID-19 were included from CHD centers worldwide. Data collection included anatomic diagnosis and subsequent interventions, comorbidities, medications, echocardiographic findings, presenting symptoms, course of illness, and outcomes. Predictors of death or severe infection were determined. Results From 58 adult CHD centers, the study included 1,044 infected patients (age: 35.1 ± 13.0 years; range 18 to 86 years; 51% women), 87% of whom had laboratory-confirmed coronavirus infection. The cohort included 118 (11%) patients with single ventricle and/or Fontan physiology, 87 (8%) patients with cyanosis, and 73 (7%) patients with pulmonary hypertension. There were 24 COVID-related deaths (case/fatality: 2.3%; 95% confidence interval: 1.4% to 3.2%). Factors associated with death included male sex, diabetes, cyanosis, pulmonary hypertension, renal insufficiency, and previous hospital admission for heart failure. Worse physiological stage was associated with mortality (p = 0.001), whereas anatomic complexity or defect group were not. Conclusions COVID-19 mortality in adults with CHD is commensurate with the general population. The most vulnerable patients are those with worse physiological stage, such as cyanosis and pulmonary hypertension, whereas anatomic complexity does not appear to predict infection severity., Central Illustration

Background Systemic atrioventricular valve regurgitation (AVVR) is frequently encountered in adults with congenital heart disease (CHD). Surgical intervention is the mainstay of therapy, but in a specific high-risk subset, percutaneous valve repair might offer a lower-risk alternative. Methods Three patients with complex CHD and severe symptomatic AVVR underwent percutaneous mitral valve repair at a single center. All were deemed to be high-risk for surgery by a multidisciplinary CHD team and provided informed consent for the compassionate use of the MitraClip (Abbott, Santa Clara, CA). Three-dimensional heart models were generated for the procedure, which was performed by an adult CHD cardiologist (who provided imaging support) and an interventional cardiologist with expertise in CHD and percutaneous mitral valve repair. Results The first case was a 39 year-old-woman with [S,L,D] dextrocardia, double outlet right ventricle, mild tricuspid hypoplasia, and a secundum atrial septal defect, who was palliated at age 35 with a right bidirectional Glenn and later developed severe, symptomatic mitral regurgitation, and underwent placement of one MitraClip XTR device. Two patients with L-loop transposition of the great arteries each successfully underwent placement of two MitraClip XTR devices; one patient had a single-leaflet detachment of one of the clips with no change in regurgitation or clip position on follow-up. All patients had significant reduction of AVVR and improvement in NYHA functional class. Conclusions Percutaneous atrioventricular valve repair in adults with CHD is feasible with the MitraClip but requires significant preprocedural planning and a multidisciplinary team that combines CHD and interventional therapeutic expertise.

The prognosis and therapeutic responses are worse for pulmonary arterial hypertension associated with systemic sclerosis (SSc-PAH) compared with idiopathic pulmonary arterial hypertension (IPAH). This discrepancy could be driven by divergence in underlying metabolic determinants of disease.Are circulating bioactive metabolites differentially altered in SSc-PAH vs IPAH, and can this alteration explain clinical disparity between these PAH subgroups?Plasma biosamples from 400 patients with SSc-PAH and 1,082 patients with IPAH were included in the study. Another cohort of 100 patients with scleroderma with no PH and 44 patients with scleroderma with PH was included for external validation. More than 700 bioactive lipid metabolites, representing a range of vasoactive and immune-inflammatory pathways, were assayed in plasma samples from independent discovery and validation cohorts using liquid chromatography/high-resolution mass spectrometry-based approaches. Regression analyses were used to identify metabolites that exhibited differential levels between SSc-PAH and IPAH and associated with disease severity.From hundreds of circulating bioactive lipid molecules, five metabolites were found to distinguish between SSc-PAH and IPAH, as well as associate with markers of disease severity. Relative to IPAH, patients with SSc-PAH carried increased levels of fatty acid metabolites, including lignoceric acid and nervonic acid, as well as eicosanoids/oxylipins and sex hormone metabolites.Patients with SSc-PAH are characterized by an unfavorable bioactive metabolic profile that may explain the poor and limited response to therapy. These data provide important metabolic insights into the molecular heterogeneity underlying differences between subgroups of PAH.

Data on lead management in patients with congenital complete heart block (CCHB) with cardiac implantable electronic devices are lacking.The purpose of this study was to describe the natural history and outcomes in patients with CCHB with cardiac implantable electronic devices undergoing transvenous lead extraction (TLE).Data on all attempted TLE procedures in patients with CCHB at 2 institutions between 2011 and 2021 were collected from a retrospective registry.Overall, 16 patients (mean age at transvenous device implant 13.8 ± 4.7 years) were included. Before TLE, patients underwent an average of 2.25 ± 1.3 generator changes, 3 (19%) underwent cardiac resynchronization therapy upgrade, and 7 (44%) underwent a lead revision with subsequently abandoned leads. Mean patient age at TLE was 34.4 ± 9.4 years with a mean duration of lead implant of 19.2 ± 6.9 years. Lead malfunction (n = 11 [69%]) and infection (n = 5 [31%]) were the most common indications for TLE. A total of 38 leads were removed, with complete procedural success achieved in 14 of 16 (87.5%). Two (12.5%) major complications occurred, including right ventricular laceration and superior vena cava tear requiring sternotomies. All patients survived at 1-year follow-up.Patients with CCHB represent a unique cohort highlighted by several generator changes, lead revisions, and abandoned leads at a young age, along with a long duration of lead dwelling time and a high prevalence of lead malfunction requiring TLE. There may be a high risk of major complications during TLE, suggesting TLE should be performed only in experienced centers. Larger studies are needed to confirm these findings.

PURPOSE: To assess the ability of four-dimensional (4D) flow MRI to quantify flow volume of the Fontan circuit, including the frequency and hemodynamic contribution of systemic-to-pulmonary venovenous collateral vessels. MATERIALS AND METHODS: In this retrospective study, patients with Fontan circulation were included from three institutions (2017–2021). Flow measurements were performed at several locations along the circuit by two readers, and collateral shunt volumes were quantified. The frequency of venovenous collaterals and structural defects were tabulated from concurrent MR angiography, contemporaneous CT, or catheter angiography and related to Fontan clinical status. Statistical analysis included Pearson and Spearman correlation and Bland-Altman analysis. RESULTS: Seventy-five patients (mean age, 20 years; range, 5–58 years; 46 female and 29 male patients) were included. Interobserver agreement was high for aortic output, pulmonary arteries, pulmonary veins, superior vena cava (Glenn shunt), and inferior vena cava (Fontan conduit) (range, ρ = 0.913–0.975). Calculated shunt volume also showed strong agreement, on the basis of the difference between aortic and pulmonary flow (ρ = 0.935). A total of 37 of 75 (49%) of the patients exhibited shunts exceeding 1.00 L/min, 81% (30 of 37) of whom had pulmonary venous or atrial flow volume step-ups and corresponding venovenous collaterals. A total of 12% of patients (nine of 75) exhibited a high-output state (>4 L/min/m(2)), most of whom had venovenous shunts exceeding 30% of cardiac output. CONCLUSION: Fontan flow and venovenous shunting can be reliably quantified at 4D flow MRI; high-output states were found in a higher proportion of patients than expected, among whom venovenous collaterals were common and constituted a substantial proportion of cardiac output. Keywords: Pediatrics, MR Angiography, Cardiac, Technology Assessment, Hemodynamics/Flow Dynamics, Congenital Supplemental material is available for this article. © RSNA, 2021

Guidelines for management of Melody transcatheter pulmonary valve (TPV) infective endocarditis (IE) are lacking. We aimed to identify factors associated with surgical valve removal versus antimicrobial therapy in Melody TPV IE. Multicenter retrospective analysis of all patients receiving Melody TPV from 10/2010 to 3/2019 was performed to identify cases of IE. Surgical explants versus non-surgical cases were compared. Of the 663 Melody TPV implants, there were 66 cases of IE in 59 patients (59/663, 8.8%). 39/66 (59%) were treated with IV antimicrobials and 27/66(41%) underwent valve explantation. 26/59 patients (44%) were treated medically without explantation or recurrence with average follow-up time of 3.5 years (range:1–9). 32% of Streptococcus cases, 53% of MSSA, and all MRSA cases were explanted. 2 of the 4 deaths had MSSA. CART analysis demonstrated two important parameters associated with explantation: a peak echo gradient ≥ 47 mmHg at IE diagnosis(OR 10.6, p 24 mmHg compared to baseline (OR 6.7, p = 0.01). Rates of explantation varied by institution (27 to 64%). In our multicenter experience, 44% of patients with Melody IE were successfully medically treated without valve explantation or recurrence. The degree of valve stenosis at time of IE diagnosis was strongly associated with explantation. Rates of explantation varied significantly among the institutions.

Single ventricle physiology and palliation via the Fontan operation lead to a series of cardiovascular changes. In addition, organs such as the kidneys and liver have been shown to experience insults and subsequent injury. This has led to routine surveillance of patients. We present findings from a small cohort of patients that was deeply phenotyped to illustrate the need for comprehensive evaluation. A cohort of four Fontan patients with fairly high cardiovascular function was recruited 5–10 years post-Fontan. Patients underwent a rigorous clinical work-up after which a research MRI scan was performed during which (I) data were obtained during exercise to evaluate changes in stroke volume during supine exercise and (II) magnetic resonance angiograms with phase-contrast images were obtained for computational modeling of flows through the Fontan circulation at rest. Clinical measures were consistent with a fairly homogeneous high function cohort (peak oxygen consumption >20 mL/kg/min, robust response to exercise, peak ventilatory efficiency below levels associated with heart failure, MR-derived ejection fraction >50%). Liver evaluation did not reveal clear signs of cirrhosis or extensive fibrosis. However, we observed considerable variability (27–162%) in the increase in stroke index with exercise [100%±64% increase, 53.9±17.4 mL/beat m(2) (rest), 101.1±20.7 mL/beat m(2), (exercise)]. Computational flow modeling at rest in two patients also showed marked differences in flow distribution and shear stress. We report marked differences in both changes in stroke index during an exercise MRI protocol as well as computational flow patterns at rest suggesting different compensation strategies may be associated with high functioning Fontan patients. The observed heterogeneity illustrates the need for deep phenotyping to capture patient-specific adaptive mechanisms.

Our multi-institutional university consortium implemented a gastroschisis pathway in 2015 to standardize and improve care by promoting avoidance of routine intubation and paralysis during silo placement, expeditious abdominal wall closure, discontinuation of antibiotics/narcotics within 48 h of closure, and early initiation/advancement of feeds.Adherence to the gastroschisis pathway was prospectively monitored. Outcomes for the contemporary cohort (2015-2018) were compared with a historical cohort (2007-2012).Good adherence to the pathway was observed for 70 cases of inborn uncomplicated gastroschisis. The contemporary cohort had significantly lower median mechanical ventilator days (2 versus 5; p 0.01) and antibiotic days (5.5 versus 9; p 0.01) as well as earlier days to initiation of feeds (12 versus 15; p 0.01). However, no differences were observed in length of stay (28 versus 29 days; p = 0.70). A skin closure technique was performed in 66% of the patients, of which 46% were performed at bedside without intubation, the assistance of an operating-room team, or general anesthesia.In this study, adherence to a clinical pathway for gastroschisis across different facilities was feasible and led to reduction in exposure to mechanical ventilation and antibiotics. The adoption of a bedside skin closure technique appears to facilitate compliance with the pathway.Level II/III TYPE OF STUDY: Prospective comparative study with historical cohort.

Introduction Liver fibrosis and cirrhosis are late complications in Fontan palliation. Liver biopsy is the gold standard. The goal of this study is to correlate transjugular liver biopsy (TJLB) in the setting of Fontan palliation with noninvasive testing and hemodynamics. Methods Between August 2014 and July 2017, 49 Fontan patients underwent TJLB. All the patients had hemodynamic evaluation, 28 patients had MRE (magnetic resonance elastography) and 40 patients had cardiopulmonary exercise test. Histologic liver fibrosis was quantitated using traditional histologic scoring systems and a modified Ishak congestive hepatic fibrosis score. Results Median age 17.8 years, median time since Fontan 15.2 years. Primary diagnosis and Fontan type were variables, but predominantly LV morphology (30/49), lateral tunnel Fontan (29/49), originally fenestrated (37/49), and 11/49 had a pacemaker. Histologic fibrosis correlated with MRE (R = 0.62, P ≤ .001). Histologic fibrosis and MRE correlated with Fontan pressure (R = 0.38, P = .008 & R = 0.59, P ≤ .001). Morphology of the single ventricle did not correlate with liver fibrosis. The presence of a fenestration resulted in a higher cardiac index (P = .026) but did not resulted in lower liver fibrosis (P = .64). Conclusion Noninvasive tests, such as MRE, may be suitable for longitudinal follow-up in patients with single ventricle physiology. Our data suggest that there is reasonable correlation of MRE liver stiffness with biopsy scoring systems and Fontan pressures. We demonstrated the feasibility of TJLB in the setting of Fontan palliation and demonstrated its correlation with noninvasive measures particularly MRE. We recommend selective use of TJLB when MRE score is >5 KPa or when there are other clinical signs of cirrhosis.

Background Although methamphetamine abuse is associated with the development of heart failure (HF), nationwide data on methamphetamine‐associated HF (MethHF) hospitalizations are limited. This study evaluates nationwide HF hospitalizations associated with substance abuse to better understand MethHF prevalence trends and the clinical characteristics of those patients. Methods and Results This cross‐sectional period‐prevalence study used hospital discharge data from the National Inpatient Sample to identify adult primary HF hospitalizations with a secondary diagnosis of abuse of methamphetamines, cocaine, or alcohol in the United States from 2002 to 2014. All 2014 MethHF admissions were separated by regional census division to evaluate geographical distribution. Demographics, payer information, and clinical characteristics of MethHF hospitalizations were compared with all other HF hospitalizations. Total nationwide MethHF hospitalizations increased from 547 in 2002 to 6625 in 2014 with a predominance on the West Coast. Methamphetamine abuse was slightly more common among primary HF hospitalizations compared with all‐cause hospitalizations (7.4 versus 6.4 per 1000; Cohen h= 0.012; P< 0.001). Among HF hospitalizations, patients with MethHF were younger (mean age, 48.9 versus 72.4 years; Cohen d= 1.93; P< 0.001), more likely to be on Medicaid (59.4% versus 8.8%; Cohen h= 1.16; P< 0.001) or uninsured (12.0% versus 2.6%; Cohen h= 0.36; P< 0.001), and more likely to present to urban hospitals (43.8% versus 28.3%; Cohen h= 0.32 ; P< 0.001) than patients with non‐methamphetamine associated HF. Patients with MethHF had higher rates of psychiatric comorbidities and were more likely to leave the hospital against medical advice. Conclusions MethHF hospitalizations have significantly increased in the United States, particularly on the West Coast. Coordinated public health policies and systems of care are needed to address this rising epidemic.

PURPOSE: To compare invasive right heart catheterization with four-dimensional (4D) flow MRI for estimating shunt fraction in patients with intracardiac and extracardiac shunts. MATERIALS AND METHODS: In this retrospective study, patients who underwent 4D flow MRI and invasive right heart catheterization with a shunt run between August 2015 and November 2018 were included. The primary objective was comparison of estimated shunt fraction (ratio of pulmonary-to-systemic flow, Q(p)/Q(s)) at 4D flow and catheterization. Secondary objectives included comparison of the right ventricular–to–left ventricular stroke volume ratio (RVSV/LVSV) to shunt fraction (for those with applicable shunts) and comparison of cardiac output between 4D flow and catheterization. Statistical analysis included Pearson correlation and Bland-Altman plots. RESULTS: A total of 33 patients met inclusion criteria (mean age, 49 years ± 16 [standard deviation]; 24 women). 4D flow measurements of Q(p)/Q(s) strongly correlated with those at catheterization (r = 0.938), and there was no bias. RVSV/LVSV correlated strongly with Q(p)/Q(s) from 4D flow (r = 0.852) and catheterization (r = 0.842). Measurements of left ventricle (Q(s)) and right ventricle (Q(P)) cardiac output from 4D flow and catheterization (Fick) correlated moderately overall (r = 0.673 [Q(p)] and r = 0.750 [Q(s)]). CONCLUSION: Shunt fraction measurement using 4D flow MRI compares well with that using invasive cardiac catheterization. Supplemental material is available for this article. © RSNA, 2021

Introduction:Many newborns with pulmonary atresia/intact ventricular septum require intervention to establish pulmonary flow and sufficient cardiac output. The resulting haemodynamic changes are not well characterised and may have unintended consequences.Methods:This is a 30-year (1988–2018) retrospective study of patients with pulmonary atresia intact ventricular septum.Results:Eighty-nine patients were included, and median follow-up was 8 years. Fifty-five per cent had coronary sinusoids and 27% had right ventricular-dependent coronary circulation. Most patients were managed with surgical aortopulmonary or modified Blalock–Taussig shunt (73%), and 12 patients underwent balloon atrial septostomy before surgical intervention. The remaining patients (27%) underwent only transcatheter interventions; 7 required an atrial septostomy and 17 required ductal stentings. All-cause mortality was 10%, most deaths (89%) occurred before 18 months of age. Of these early deaths, 87% required a balloon atrial septostomy and 85% had right ventricular-dependent coronary sinusoids. Eighteen-month mortality was significantly higher for patients who required a balloon atrial septostomy compared to those who did not (36% versus 1.4% p < 0.0001).Discussion:Patients with pulmonary atresia/intact ventricular septum who require balloon atrial septostomy in the newborn period have significantly higher 18-month mortality. Quantifying the mortality difference may help guide prognostication and expectation setting. Infants who had septostomy and a surgical shunt in the newborn period fared better than those who only underwent septostomy (even when accompanied by ductal stenting). For infants with right ventricular-dependent circulation, atrial septostomy should only be performed on an urgent or emergent basis and these patients should be considered for early surgical intervention and neonatal transplant.

Objectives: Long-term survival in patients with truncus arteriosus (TA) is favorable, but there remains significant morbidity associated with need for ongoing re-interventions. The purpose of this study was to understand the long-term outcomes of the truncal valve (TV) and identify risk factors associated with the need for TV intervention. Methods: We retrospectively reviewed 170 patients who underwent initial TA repair at our institution from 1985-2015. Analysis of long-term outcomes was performed on the 148 patients who survived greater than 30 days post-operatively and to hospital discharge using multivariable competing risks Cox regression modeling. Results: Median follow up time was 12.6 years (IQR 5.0, 22.1 years) after full repair. Freedom from death or transplant at 1, 5, 10, and 20 years was 93.1 ± 2.1%, 88.0 ± 2.7%, 86.2 ± 3.0% and 78.3 ± 4.1%. Thirty patients (20%) underwent at least one intervention on the TV (22 repairs, 21 replacements). Freedom from any TV intervention at 1, 5, 10 and 20 years was 99 ± 1%, 94 ± 8%, 82 ± 9%, and 70 ± 5%. Of those with TV repair, 59% subsequently underwent TV replacement. Independent risk factors for need for TV intervention included ≥moderate TV regurgitation (TVR) (HR 4.77, p Conclusion: Long-term need for TV intervention remains significant. Moderate or worse initial TVR or stenosis, residual TVR after initial TA repair, and single coronary ostium are risk factors associated with need for subsequent TV intervention.

Background Care delivery for the growing population of adults living with congenital heart disease (CHD) has been met with challenges due to a shortage of physicians trained to care for this population. To meet this urgent need, restructuring and standardization of the training programs were implemented in 2015. The consequences of such a system on the graduating fellows have not been examined. Methods A 25-question electronic survey was distributed to early career physicians who graduated following training in adult CHD (ACHD) care between 2015 and 2017 and are currently practicing in the United States. The survey results were anonymous. Results Of the 30 physicians who trained in ACHD between 2015 and 2017 in the U.S., 21 (70%) responded to the survey. The majority completed a 2-year ACHD program, practice at an adult hospital, are happy with their current job, spend most of their time in ACHD-related activities, make on average around 250,000 USD for entry level positions, and prioritize supportive leadership and colleagues. Their training was adequate for their job requirements. However, the acquisition of an additional skill, in addition to clinical ACHD care, allowed them to secure a more ideal job. A sizeable number of jobs required program building or expansion with only 9.5% of trainees comfortable doing so immediately after graduation. Conclusions The new ACHD training curriculum successfully meets most of the needs for ACHD jobs. Integration of specialty tracks, ensuring uniformity in the quality of training between programs, and promoting leadership skills may improve career prospects.

AIMS: Despite the well-defined association of high-sensitivity hsCRP with cardiovascular outcomes in apparently healthy adults and those with acquired heart disease, the relevance of this inflammatory marker in adults with congenital heart disease (ACHD) remains unclear. We aimed to examine the clinical correlates and prognostic value of high-sensitivity C-reactive protein levels in ACHD. METHODS AND RESULTS: We conducted a prospective cohort study of (n = 707) outpatient ACHD (age 39 ± 14 years, 49% women), enrolled mainly at a referral centre, who had serum hsCRP measured in conjunction with a clinical assessment between 2012 and 2016. We analysed clinical correlates of hsCRP and its association with adverse events including the primary combined outcome of all-cause mortality or non-elective cardiovascular hospitalization. Higher hsCRP was strongly associated with measures of functional status including New York Heart Association class and peak V̇O(2), and with comorbidities such as atrial arrhythmia. During average follow-up of 815 ± 536 days, 114 patients (16%) experienced the primary outcome, including 29 deaths. Having elevated hsCRP, in the highest (≥2.98 mg/L) compared with the lower three quartiles, conferred increased risk for the primary outcome [30.5% vs. 11.3%, adjusted hazard ratio (HR) = 2.00, 95% confidence interval (CI) 1.35–2.97; P = 0.0006] and all-cause mortality (11.9% vs. 1.5%, adjusted HR = 4.23, 95% CI 1.87–9.59; P = 0.0006). Elevated hsCRP was associated with adverse outcomes across ACHD subgroups and other patient characteristics. CONCLUSION: Adults with congenital heart disease with elevated hsCRP have not only worse functional status and exercise capacity, but also greater risk for death or non-elective cardiovascular hospitalization. Further study is warranted to characterize the role of inflammation in the pathophysiology of ACHD.

Pregnancy is associated with a hypercoagulable state. Women requiring anticoagulation need careful attention throughout pregnancy and the post-partum period. The choice of anticoagulant therapy, the degree of monitoring, and the therapeutic target should be modulated by balancing the risks and the benefits to the mother and fetus. Many of the available anticoagulant agents may be used safely in pregnancy, but they are disadvantaged by competing efficacy and risks to the mother and fetus. For example, vitamin K antagonists are the most efficacious for preventing mechanical valve thrombosis, but they pose risks to the fetus. Collaborative research that collects patient-level data will help clinicians navigate the intricate process of anticoagulation in pregnancy. Development of anticoagulant agents that are homogeneous, efficacious, safe to the fetus, and not affected by physiological perturbations of pregnancy will have tremendous effect on the outcomes of pregnancy in women who require anticoagulation.

Patients born with congenital heart disease (CHD) have benefited from remarkable advances in surgical and catheter-based interventions. As a result, the majority of children born with even the most complex forms of CHD live into adulthood. The specialized field of adult CHD (ACHD) was born out of the necessity to care for this new population of survivors of childhood CHD and their distinctive features. In this review, relevant aspects of ACHD that lead to, and are affected by, heart failure will be examined along with the increasing prevalence of HF in the burgeoning population of ACHD. We also highlight the challenges in defining HF in this particular group of patients.

Red cell distribution width (RDW), a measure of variability in red cell size, predicts adverse outcomes in acquired causes of heart failure. We examined the relation of RDW and outcomes in adults with congenital heart disease. We performed a prospective cohort study on 696 ambulatory patients ≥18years old enrolled in the Boston Adult Congenital Heart Disease Biobank between 2012 and 2016 (mean age 38.7 ± 13.5 years; 49.9% women). The combined outcome was all-cause mortality or nonelective cardiovascular hospitalization. Most patients had moderately or severely complex congenital heart disease (42.5% and 38.5%, respectively). Mean RDW was 14.0 ± 1.3%. RDW15% was present in 81 patients (11.6%). After median 767days of follow-up, 115 patients sustained the primary combined outcome, including 31 who died. Higher RDW predicted both the combined outcome (hazard ratio [HR] for RDW15% = 4.5, 95% confidence interval [CI] 3.0 to 6.6; HR per + 1SD RDW = 1.8, 95% CI 1.6 to 2.0, both p0.0001) and death alone (HR for RDW15% = 7.1, 95% CI 3.5 to 14.4; HR per + 1SD RDW = 1.8, 95% CI 1.6 to 2.0, both p0.0001). RDW remained an independent predictor of the combined outcome after adjusting for age, cyanosis, congenital heart disease complexity, ventricular systolic function, New York Heart Association functional class, hemoglobin concentration, mean corpuscular volume, high-sensitivity C-reactive protein and estimated glomerular filtration rate (HR per + 1SD RDW = 1.5, 95% CI 1.2 to 1.9, p0.0001). RDW also remained an independent predictor of mortality alone after adjustment for age plus each variable individually. In conclusion, elevated RDW is an independent predictor of all-cause mortality or nonelective cardiovascular hospitalization in adults with congenital heart disease. This simple clinical biomarker identifies increased risk for adverse events even among patients with preserved functional status.

We present a case of a 20-year-old man with tetralogy of Fallot status post-repair with a transannular patch and left pulmonary artery reconstruction at age 1 and subsequent pulmonary valve replacement with bioprosthetic valve 3 years ago. A transthoracic echocardiogram revealed severe bioprosthetic valve insufficiency. Cardiac magnetic resonance with 4-dimensional flow revealed a paravalvular leak. (Level of Difficulty: Intermediate.), The authors present a case of a 20-year-old man with tetralogy of Fallot status post-repair with a transannular patch and left pulmonary artery…

There is an increasing number of adult patients with congenital heart disease (CHD). While several biomarkers have been validated and integrated into general cardiology clinical practice, these tests are often applied to adults with CHD in the absence of disease-specific validation. Although these patients are often grouped into a single population, there is heterogeneous pathophysiology, variable disease chronicity, extensive multisystem involvement, and a low event rate relative to acquired heart disease. These stand as challenges to systematic investigation and clinical application of biomarkers for adults with CHD. This paper reviews recent studies investigating the use of biomarkers in this population, with emphasis on biomarkers applied in clinical adult CHD care. A handful of biomarkers have been integrated into adult CHD practice, such as iron studies in cyanotic heart disease and stool alpha-1 antitrypsin for diagnosis of protein losing enteropathy in the Fontan circulation. Use of kidney and liver tests has been studied in prognostication of adult CHD patients. A few other biomarkers like natriuretic peptides and troponins seem likely to provide useful information in other ACHD situations based on limited disease-specific data and extrapolation from acquired heart disease. More research is needed to support the robust validity of most existing clinical biomarkers in adult congenital cardiology practice. Until data from larger, prospectively enrolled cohorts are available, clinical use of biomarkers in these patients will require careful interpretation with attention to underlying pathophysiology, as well as detailed understanding of potential pitfalls of specific assays and clinical contexts.

Transcatheter repair of a regurgitant mitral valve may be achieved with the MitraClip (MC), mimicking a surgical Alfieri stitch. Here we report the use of MC for severe tricuspid regurgitation (systemic atrio-ventricular valve [SAVV]) in a patient with L-loop transposition of the great arteries (L

Atrial fibrillation/flutter (AF) is frequently encountered in patients undergoing bone-marrow transplant (BMT). The epidemiology of inpatients admitted for BMT that develop AF is poorly defined. Using the National Inpatient Sample, we reviewed hospital admissions in the U.S. between 2002 and 2014

Background The burden of substance abuse among patients with heart failure and its association with subsequent emergency department visits and hospital admissions are poorly characterized. Methods We evaluated the medical records of patients with a diagnosis of heart failure treated at the University of California–San Diego from 2005 to 2016. We identified substance abuse via diagnosis codes or urine drug screens. We used Poisson regression to evaluate the incidence rate ratios (IRR) of substance abuse for emergency department visits or hospitalizations with a primary diagnosis of heart failure, adjusted for age, sex, race, medical insurance status, and medical diagnoses. Results We identified 11,268 patients with heart failure and 15,909 hospital encounters for heart failure over 49,712 person-years of follow-up. Substance abuse was diagnosed in 15.2% of patients. Disorders such as methamphetamine abuse (prevalence 5.2%, IRR 1.96, 95% confidence interval [CI] 1.85-2.07), opioid use and abuse (8.2%, IRR 1.54, 95% CI 1.47-1.61), and alcohol abuse (4.5%, IRR 1.51, 95% CI 1.42-1.60) were associated with a greater number of hospital encounters for heart failure, with associations that were comparable to diagnoses such as atrial fibrillation (37%, IRR 1.78, 95% CI 1.73-1.84), ischemic heart disease (24%, IRR 1.67, 95% CI 1.62-1.73), and chronic kidney disease (26%, IRR 1.57, 95% CI 1.51-1.62). Conclusions Although less prevalent than common medical comorbidities in patients with heart failure, substance-abuse disorders are significant sources of morbidity that are independently associated with emergency department visits and hospitalizations for heart failure. Greater recognition and treatment of substance abuse may improve outcomes among patients with heart failure.

Surgery after drug-eluting stent (DES) implantation may be associated with increased risk for perioperative stent thrombosis (ST).We evaluated the outcomes of 67 patients who underwent non-cardiac (n=51) or cardiac (n=16) surgery after DES implantation at our institution between 2008 and 2010 and who underwent preoperative "bridging" with a glycoprotein IIb/IIIa inhibitor. Surgery occurred after a mean time of 13.9 ± 1.7 and 8.7 ± 2 months post stenting for non-cardiac (NCS) and cardiac surgery, respectively. Glycoprotein IIb/IIIa inhibitors were administered preoperatively for a mean of 7.1 ± 0.4 and 7.8 ± 0.7 days, respectively, then discontinued four to six hours before surgery. Most patients received aspirin through the perioperative period (33 NCS patients and 15 cardiac surgery patients). Clopidogrel was restarted as early as possible in the postoperative period. In the non-cardiac surgery group, two patients (3.9%, 95% confidence intervals 0.5% to 13.5%) suffered acute ST in the immediate postoperative period and four patients suffered major bleeding by the Global Utilisation of Streptokinase and Tissue Plasminogen Activator for Occluded Coronary Arteries (GUSTO) criteria. One cardiac surgery patient had probable ST one hour postoperatively.In spite of preoperative "bridging" with a glycoprotein IIb/IIIa inhibitor, postoperative stent thrombosis can still occur in patients with prior DES undergoing surgery requiring antiplatelet medication interruption.

Pregnancy is associated with a hypercoagulable state. Women requiring anticoagulation need careful attention throughout pregnancy and the post-partum period. The choice of anticoagulant therapy, the degree of monitoring, and the therapeutic target should be modulated by balancing the risks and the benefits to the mother and fetus. Many of the available anticoagulant agents may be used safely in pregnancy, but they are disadvantaged by competing efficacy and risks to the mother and fetus. For example, vitamin K antagonists are the most efficacious for preventing mechanical valve thrombosis, but they pose risks to the fetus. Collaborative research that collects patient-level data will help clinicians navigate the intricate process of anticoagulation in pregnancy. Development of anticoagulant agents that are homogeneous, efficacious, safe to the fetus, and not affected by physiological perturbations of pregnancy will have tremendous effect on the outcomes of pregnancy in women who require anticoagulation.

Methamphetamine abuse is on the rise in the U.S. Much like cocaine and alcohol, methamphetamine has been implicated in the development heart failure (HF). The epidemiology of patients who abuse methamphetamine admitted with acute heart failure (MethHF) is undefined. Using the National Inpatient

Heart failure represents a common end-stage syndrome for many adults with congenital heart disease (ACHD). These patients, however, have been excluded from most heart transplantation research. It is not known how current criteria, derived from non-ACHD populations, used to determine priority at the time of transplant listing, impact the outcomes for ACHD patients listed for heart transplantation.The goal of this study was to investigate outcomes of ACHD in comparison to non-ACHD patients while listed for heart transplantation.We conducted a retrospective study using the Scientific Registry of Transplant Recipients on patients ≥18 years of age listed in the United States between 1999 and 2014. The probability of mortality or delisting due to clinical worsening was estimated using cumulative incidence functions, where transplantation was a competing event.Among 1,290 ACHD and 38,557 non-ACHD patients listed, 237 ACHD and 6,377 non-ACHD patients died or were delisted due to clinical worsening. Death or delisting for clinical worsening was more likely for ACHD patients initially listed as status 1A (24% ACHD vs. 17% non-ACHD after 180 days; p0.001). There were no significant differences between ACHD and non-ACHD patients listed as status 1B or 2. In multivariable analysis, factors associated with death or delisting due to clinical worsening within 1 year in ACHD included: estimated glomerular filtration rate60 ml/min/1.73 m(2) (hazard ratio [HR]: 1.4; 95% confidence interval [CI]: 1.0 to 1.9; p = 0.043); albumin3.2 g/dl (HR: 2.0; 95% CI: 1.3 to 2.9; p0.001); and hospitalization at the time of listing, whether in the intensive care unit (HR: 2.3; 95% CI: 1.6 to 3.5; p0.001) or not (HR: 1.9; 95% CI: 1.2 to 3.0; p = 0.006) relative to outpatients.Wait-list mortality or delisting due to worsening clinical status is disproportionately common for ACHD patients listed as status 1A. An allocation system that takes into account the distinctive aspects of ACHD patients may help better care for this growing population.

Background: Heart failure represents a final common pathway for many adult survivors with congenital heart disease (ACHD). Data assessing severity and management of heart failure are limited, and often heart transplantation is the only viable treatment option. The criteria used to determine priority status at the time of transplant listing, however, does not account for ACHD physiology. We investigated the differences in death or delisting due to clinical worsening in ACHD vs. non-ACHD candidates for heart transplantation. Methods: We conducted a retrospective study of all patients listed for heart transplantation in the United States between 1999 and 2014 using the Scientific Registry of Transplant Recipients. Patients were censored at the time of transplant or delisting due to improvement. Results: Among the 1,290 ACHD and 35,559 non-ACHD patients listed for heart transplantation, median age was 35 vs. 56 years and 62% vs. 76% were male, respectively. Waitlist outcomes for the full follow up time per initial listing status are shown in Figure 1. Of the patients who died or worsened, 57% were initially listed at the lowest priority status for ACHD compared to 40% for non-ACHD. In patients initially listed at status 1A, the 180-day actuarial probability of death or delisting due to worsening was 41% for ACHD vs. 29% for non-ACHD, p < 0.01; and for death alone was 29% vs. 21%, p = 0.05; respectively, Figure 2. Conclusion: ACHD candidates for heart transplantation in the United States are more frequently listed at the lowest priority status, and when listed at the highest priority status are more likely to die or be delisted due to clinical worsening compared to other candidates.

Background As the U.S. population grows older, there is greater need to examine physical independence. Previous studies have assessed risk factors in relation to either disability or mortality, but an outcome that combines both is still needed. Methods and Results The Cardiovascular Health Study is a population‐based, prospective study where participants underwent baseline echocardiogram, measurement of carotid intima‐media thickness ( IMT ), and various biomarkers, then followed for up to 18 years. Years of able life ( YAL ) constituted the number of years the participant was able to perform all activities of daily living. Linear regression was used to model the relationship between selected measures and outcomes, adjusted for confounding variables. Among 4902 participants, mean age was 72.6±5.4 years, median YAL for males was 8.8 (interquartile range [IQR], 4.3 to 13.8) and 10.3 (IQR, 5.8 to 15.8) for females. Reductions in YAL in the fully adjusted model for females and males, respectively, were: −1.34 (95% confidence interval [CI], −2.18, −0.49) and −1.41 (95% CI, −2.03, −0.8) for abnormal left ventricular ( LV ) ejection fraction, −0.5 (95% CI, −0.78, −0.22) and −0.62 (95% CI, −0.87, −0.36) per SD increase in LV mass, −0.5 (95% CI, −0.7, −0.29) and −0.79 (95% CI, −0.99, −0.58) for IMT , −0.5 (95% CI, −0.64, −0.37) and −0.79 (95% CI, −0.94, −0.65) for N‐terminal pro‐brain natriuretic peptide, −1.08 (95% CI, −1.34, −0.83) and −0.73 (95% CI, −0.97, −0.5) for high‐sensitivity troponin‐T, and −0.26 (95% CI, −0.42, −0.09) and −0.23 (95% CI, −0.41, −0.05) for procollagen‐ III N‐terminal propeptide. Most tested variables remained significant even after adjusting for incident cardiovascular (CV) disease. Conclusions In this population‐based cohort, variables obtained by CV imaging and biomarkers of inflammation, coagulation, atherosclerosis, myocardial injury and stress, and cardiac collagen turnover were associated with YAL , an important outcome that integrates physical ability and longevity in older persons.