George Lui, MD

All Publications

• Echocardiographic Evaluation of Patients Undergoing Transcatheter Tricuspid Valve-In-Valve Replacement. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography Tompkins, R., Kelle, A. M., Cabalka, A. K., Lui, G. K., Aboulhosn, J., Dvir, D., McElhinney, D. B., VIVID Registry 2019

  Abstract

  BACKGROUND: Transcatheter tricuspid valve-in-valve replacement (TVIV) is an emerging therapy for dysfunctional surgical valves in patients with congenital and acquired TV disease. The present study was performed to establish baseline quantitative data for echocardiographic and invasive parameters obtained pre- and immediately post-TVIV.METHODS: Patients were drawn from the VIVID Registry. This study included two cohorts. The registry cohort included all patients entered in the VIVID registry through February 2017 who had both echocardiographic and invasively measured gradients across the TV. The focused cohort comprised a subset of patients from a single institution who had both pre- and post-TVIV echocardiogram images reviewed offline by a single investigator. The echocardiographic variables measured were based on published guidelines from the American Society of Echocardiography.RESULTS: Assessment of paired pre- and/or postimplant echocardiographic and invasive pressure measurements (n=199) showed reasonable correlation between mean TV gradient measured invasively with cardiac catheterization and noninvasively both pre- and post-TVIV (R=0.72, P<.001), although there was a bias toward the echocardiographic gradient being higher than the invasively measured gradient and sizable discrepancies were reported in several patients. In the focused cohort (n=42), the mean TV inflow gradient was 9.3±5.0mm Hg pre- and 5.6±2.3mm Hg post-TVIV (P<.001). The TV pressure halftime and TV:left ventricular outflow tract Doppler velocity index were 215±94 msec and 3.4±1.2, respectively, at baseline, and 170±44 msec and 2.4±0.6 post-TVIV. Both the Doppler velocity index and the TVE velocity correlated with the mean TV inflow gradient.CONCLUSIONS: This study provides benchmark data for the echocardiographic assessment of valve function after TVIV. In this population, the significance of an inflow gradient after TVIV should be interpreted in the clinical context. The appropriate threshold for defining dysfunction may differ from the levels proposed for assessment of native or newly placed surgical valves.

  View details for DOI 10.1016/j.echo.2018.12.008

  View details for PubMedID 30773424

• Extended cardiac ambulatory rhythm monitoring in adults with congenital heart disease: Arrhythmia detection and impact of extended monitoring. Congenital heart disease Schultz, K. E., Lui, G. K., McElhinney, D. B., Long, J., Balasubramanian, V., Sakarovitch, C., Fernandes, S. M., Dubin, A. M., Rogers, I. S., Romfh, A. W., Motonaga, K. S., Viswanathan, M. N., Ceresnak, S. R. 2019

  Abstract

  BACKGROUND: Arrhythmias are a leading cause of death in adults with congenital heart disease (ACHD). While 24-48-hour monitors are often used to assess arrhythmia burden, extended continuous ambulatory rhythm monitors (ECAM) can record 2 weeks of data. The utility of this device and the arrhythmia burden identified beyond 48-hour monitoring have not been evaluated in the ACHD population. Additionally, the impact of ECAM has not been studied to determine management recommendations.OBJECTIVE: To address the preliminary question, we hypothesized that clinically significant arrhythmias would be detected on ECAM beyond 48hours and this would lead to clinical management changes.METHODS: A single center retrospective cohort study of ACHD patients undergoing ECAM from June 2013 to May 2016 was performed. The number and type of arrhythmias detected within and beyond the first 48hours of monitoring were compared using Kaplan-Meier curves and Cox proportional hazard models.RESULTS: Three hundred fourteen patients had monitors performed [median age 31 (IQR 25-41) years, 61% female). Significant arrhythmias were identified in 156 patients (50%), of which 46% were noted within 48 hours. A management change based on an arrhythmia was made in 49 patients (16%).CONCLUSIONS: ECAM detects more clinically significant arrhythmias than standard 48-hour monitoring in ACHD patients. Management changes, including medication changes, further testing or imaging, and procedures, were made based on results of ECAM. Recommendations and guidelines have been made based on arrhythmias on 48-hour monitoring; the predictive ability and clinical consequence of arrhythmias found on ECAM are not yet known.

  View details for DOI 10.1111/chd.12736

  View details for PubMedID 30604934

• Transcatheter redirection of hepatic venous blood to treat unilateral pulmonary arteriovenous malformations in a Fontan circulation by short-term total exclusion of the unaffected lung. Catheterization and cardiovascular interventions : official journal of the Society for Cardiac Angiography & Interventions Adamson, G. T., Peng, L. F., Lui, G. K., Perry, S. B. 2019

  Abstract

  Clinically significant unilateral pulmonary arteriovenous malformations (PAVM) can develop in patients with a Fontan circulation when there is unbalanced distribution of hepatic venous (HV) blood flow to the lungs. There are reported surgical and transcatheter techniques to treat PAVMs by rerouting HV return, with promising short-term results. We report a case of a novel, technically simple transcatheter approach to redirect HV blood flow in an adult Fontan patient with polysplenia syndrome and severe unilateral PAVMs. Our patient had a two-stage procedure, the first to redirect all HV blood flow to the affected lung with a single covered stent, and a second to confirm resolution of PAVMs and to reintroduce HV effluent to the unaffected lung. At 10-month follow-up, her oxygen saturations had increased from 75% to 93% with a marked improvement in her functional status.

  View details for DOI 10.1002/ccd.28073

  View details for PubMedID 30629315

• Risk of cardiac tachyarrhythmia in patients with repaired tetralogy of Fallot: a multicenter cardiac MRI based study INTERNATIONAL JOURNAL OF CARDIOVASCULAR IMAGING Beurskens, N. G., Hagdorn, Q. J., Gorter, T. M., Berger, R. F., Vermeulen, K. M., van Melle, J. P., Ebels, T. E., Lui, G. K., Ceresnak, S. R., Chan, F. P., Willems, T. P. 2019; 35 (1): 143–51

  Abstract

  Cardiac tachyarrhythmias are the leading cause of morbidity and mortality in patients with repaired tetralogy of Fallot (TOF). We evaluated risk factors for sustained ventricular tachyarrhythmia (VT) and atrial tachyarrhythmia (ATA) in these patients. Patients (n = 319) who underwent cardiac magnetic resonance (CMR) imaging at two tertiary centers between 2007 and 2016 were assessed. Potential risk markers, based on history, cardiac magnetic resonance imaging (CMR), electrocardiography (ECG) and echocardiography, were analyzed for prediction of the primary endpoint of VT, and the secondary endpoint of ATA. During a follow-up of 3.5 (0.9-6.1) years, 20 (6.3%) patients reached the primary endpoint, and 30 (9.4%) the secondary endpoint. Multivariable cox hazards regression identified right ventricular (RV) end-diastolic volume (Hazard ratio [HR] 2.03, per 10 ml/m2 increase; p = 0.02), RV end-systolic volume (HR 3.04, per 10 ml/m2 increase; p = 0.04), RV mass (HR 1.88, per 10 g/m2 increase; p = 0.02), and RV ejection fraction (HR 6.06, per 10% decrease; p = 0.02) derived from CMR to be independent risk factors of VT. In addition, QRS-duration (HR 1.70, per 10 ms increase; p = 0.001) and body mass index (BMI: HR 1.8, per 5 kg/m2 increase; p = 0.02) were independent markers of VT. Older age at TOF repair (HR 1.33, per 2 months increase; p = 0.03) and BMI (HR 1.76, per 5 kg/m2 increase; p < 0.001) independently predicted ATA. RV systolic dysfunction, hypertrophy and dilatation on CMR, together with QRS prolongation, and obesity are predictive of VT in TOF patients. Older age at TOF repair and obesity were associated with the occurrence of ATA.

  View details for DOI 10.1007/s10554-018-1435-9

  View details for Web of Science ID 000458270500017

  View details for PubMedID 30094564

• Guidelines for Performing a Comprehensive Transesophageal Echocardiographic: Examination in Children and All Patients with Congenital Heart Disease: Recommendations from the American Society of Echocardiography. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography Puchalski, M. D., Lui, G. K., Miller-Hance, W. C., Brook, M. M., Young, L. T., Bhat, A., Roberson, D. A., Mercer-Rosa, L., Miller, O. I., Parra, D. A., Burch, T., Carron, H. D., Wong, P. C. 2018

  View details for DOI 10.1016/j.echo.2018.08.016

  View details for PubMedID 30579694

• Repair of Untreated Older Patients with Tetralogy of Fallot with Major Aortopulmonary Collaterals. The Annals of thoracic surgery Vaikunth, S., Bauser-Heaton, H., Lui, G. K., Wise-Faberowski, L., Chan, F. P., Asija, R., Hanley, F. L., McElhinney, D. B. 2018

  Abstract

  BACKGROUND: Our programmatic approach to tetralogy of Fallot with major aortopulmonary collaterals emphasizes single-stage unifocalization with complete intracardiac repair during infancy. Little is known about suitability for complete repair in patients beyond infancy. We sought to analyze outcomes of our approach in older patients with previously untreated tetralogy of Fallot with major aortopulmonary collaterals.METHODS: Any patient with this lesion not treated prior to 2 years of age referred to our center from 2002-17 met inclusion criteria.RESULTS: Of 33 patients, 32 were out-of-state (64% international) referrals, and 33% (n=11) were >9 years old, had polycythemia, and/or at least 1 high pressure collateral (>25 mm Hg). Complete repair was achieved in 94% (n=31) of patients, 82% (n=27) in one stage and 12% (n=4) after unifocalization to a central shunt. The median right ventricular to aortic pressure ratio was 0.31 after surgery and 0.37 at follow-up. At a median of 4.8 years after repair, 9 patients (19%) underwent reintervention, including 5 conduit and 7 branch pulmonary artery interventions. Three patients also underwent aortic valve replacement.CONCLUSIONS: In this selected cohort of older patients with previously unoperated tetralogy of Fallot with major aortopulmonary collaterals, outcomes were comparable to infants undergoing treatment according to our approach. These findings support the notion that patients who are either born in low-resource settings or present to healthcare providers beyond infancy should be considered candidates and evaluated for complete repair.

  View details for DOI 10.1016/j.athoracsur.2018.11.016

  View details for PubMedID 30550802

• Ventricular mechanics in adolescent and adult patients with a Fontan circulation: Relation to geometry and wall stress. Echocardiography (Mount Kisco, N.Y.) Rosner, A., Khalapyan, T., Pedrosa, J., Dalen, H., McElhinney, D. B., Friedberg, M. K., Lui, G. K. 2018

  Abstract

  BACKGROUND: Patients with single ventricle physiology and Fontan circulation are at increased risk for late complications and reduced survival. The aim of the study was to investigate the correlation between ventricular geometry and systolic regional function in different underlying anatomic conditions in adolescent and adult Fontan-palliated patients.METHOD: In a retrospective cross-sectional study, we measured 2D strain, ventricular diameters, ventricular volumes, ejection fraction (EF), global and segmental wall stress, and sphericity index. The same analyses were performed in 99 age- and gender-matched healthy individuals.RESULTS: One hundred and one patients were included at a mean age of 21 (range 14-59) years. In comparison with healthy subjects, patients with Fontan circulation displayed larger ventricular volumes (153±78mL vs 116±38mL P<0.05), reduced EF (43%±15% vs 55%±8% P<0.05), reduced longitudinal (-13%±6% vs -21%±4% P<0.05) and circumferential strain values (-15%±7% vs -22%±4% P<0.05). Functionally single ventricles were more spherical (ratio of longitudinal to short-axis diameters 1.3±0.3 vs 1.7±0.2 P<0.05). Circumferential strain correlated well with global wall stress and the degree of sphericity (R2 =0.320), while segmental strain did not correlate with segmental wall stress. The percentage of segments with akinesia was relatively high (16±16% vs 0±0% P<0.05) indicating reduced segmental contractile function.CONCLUSION: Functionally single ventricles after Fontan palliation have significantly reduced systolic regional and global function with a high intersegmental inhomogeneity. The underlying pathological mechanisms might be multifactorial, including ventricular geometry, sphericity, and regional contractile properties. Future studies are needed to investigate the role of ventricular geometry for clinical performance and outcome.

  View details for DOI 10.1111/echo.14169

  View details for PubMedID 30350438

• Predicting acquired cardiovascular disease in adults with congenital heart disease is risky business. International journal of cardiology Lui, G. K., Bhatt, A. B. 2018

  View details for DOI 10.1016/j.ijcard.2018.10.061

  View details for PubMedID 30482629

• Applying current normative data to prognosis in heart failure: The Fitness Registry and the Importance of Exercise National Database (FRIEND) INTERNATIONAL JOURNAL OF CARDIOLOGY Moneghetti, K. J., Hock, J., Kaminsky, L., Arena, R., Lui, G. K., Haddad, F., Wheeler, M., Froelicher, V., Ashley, E., Myers, J., Christle, J. W. 2018; 263: 75–79

  Abstract

  Percent of predicted peak VO2 (ppVO2) is considered a standard measure for establishing disease severity, however, there are known limitations to traditional normative values. This study sought to compare ppVO2 from the newly derived "Fitness Registry and the Importance of Exercise: a National Database" (FRIEND) registry equation to conventional prediction equations in a clinical cohort of patients undergoing cardiopulmonary exercise testing (CPX).We selected 1094 patients referred for evaluation of heart failure (HF) symptoms who underwent CPX. ppVO2 was calculated using the FRIEND, Wasserman/Hansen and Jones equations. Participants were followed for a median of 4.5 years [Interquartile range 3.5-6.0] for the composite endpoint of death, advanced HF therapy, or acute decompensated HF requiring hospital admission. Mean age was 48 ± 15 years and 62% were female. The FRIEND registry equation predicted the lowest ppVO2 (measured/predicted; 71 ± 31%), compared to the Wasserman/Hansen (74 ± 29%) and Jones equations (83 ± 33%) (p < 0.001). All expressions of peak VO2 were significant as univariate predictors of outcome with no significant differences between equations on pairwise analysis of receiver operating characteristic curves. When compared at a similar threshold of ppVO2 the event rate was significantly lower in the FRIEND registry equation versus the currently used Wasserman and Jones equations.The use of the newly derived FRIEND registry equation predicts HF outcomes; however, it appears to predict a higher predicted VO2; the clinical implication being a lower threshold of percent predicted peak VO2 should be considered when risk stratifying patients with HF.

  View details for DOI 10.1016/j.ijcard.2018.02.102

  View details for Web of Science ID 000432918000017

  View details for PubMedID 29525067

• OUTCOME OF EN-BLOC COMBINED HEART AND LIVER TRANSPLANTATION IN THE ADULT FAILING FONTAN Vaikunth, S., Concepcion, W., Daugherty, T., Fowler, M., Lutchman, G., Maeda, K., Rosenthal, D., Teuteberg, J., Woo, Y., Lui, G. K. ELSEVIER SCIENCE INC. 2018: 539

  View details for DOI 10.1016/S0735-1097(18)31080-5

  View details for Web of Science ID 000429659701389

• Population-based surveillance of congenital heart defects among adolescents and adults: surveillance methodology. Birth defects research Glidewell, J., Book, W., Raskind-Hood, C., Hogue, C., Dunn, J. E., Gurvitz, M., Ozonoff, A., McGarry, C., Van Zutphen, A., Lui, G., Downing, K., Riehle-Colarusso, T. 2018

  Abstract

  Improved treatment of congenital heart defects (CHDs) has increased survival of persons with CHDs; however, no U.S. population-based systems exist to assess prevalence, healthcare utilization, or longer-term outcomes among adolescents and adults with CHDs.Novel approaches identified individuals aged 11-64 years who received healthcare with ICD-9-CM codes for CHDs at three sites: Emory University in Atlanta, Georgia (EU), Massachusetts Department of Public Health (MA), New York State Department of Health (NY) between January 1, 2008 (2009 for MA) and December 31, 2010. Case-finding sources included outpatient clinics; Medicaid and other claims data; and hospital inpatient, outpatient, and emergency visit data. Supplemental information came from state vital records (EU, MA), and birth defects registries (EU, NY). Demographics and diagnostic and procedural codes were linked, de-duplicated, and shared in a de-identified dataset. Cases were categorized into one of five mutually exclusive CHD severity groups; non-cardiac comorbidity codes were grouped into broad categories.73,112 individuals with CHD codes in healthcare encounters were identified. Primary data source type varied: clinics (EU, NY for adolescents), claims (MA), hospital (NY for adults). There was a high rate of missing data for some variables and data varied in format and quality. Some diagnostic codes had poor specificity for CHD ascertainment.To our knowledge, this is the first population-based, multi-site CHD surveillance among adolescents and adults in the U.S. Identification of people living with CHDs through healthcare encounters using multiple data sources was feasible, though data quality varied and linkage/de-duplication was labor-intensive.

  View details for DOI 10.1002/bdr2.1400

  View details for PubMedID 30394691

• Classic-Pattern Dyssynchrony in Adolescents and Adults With a Fontan Circulation. Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography Rösner, A., Khalapyan, T., Dalen, H., McElhinney, D. B., Friedberg, M. K., Lui, G. K. 2018; 31 (2): 211–19

  Abstract

  Previous studies have suggested the presence of dyssynchrony in the functionally single ventricle. The aim of this study was to investigate the presence of classic-pattern dyssynchrony (CPD), characterized by typical early and late deformation of opposite walls, and its relation to QRS duration and myocardial function in patients with single-ventricle physiology after Fontan palliation.In a retrospective cross-sectional study, 101 adolescent and adult patients with single-ventricle physiology after the Fontan procedure were investigated. Strain curves were visually assessed for the presence of CPD. Systolic and diastolic function were assessed using echocardiography.One hundred one patients were included, with varying anatomic morphology: two sizable ventricular components (n = 21), right dominant (n = 21), left dominant (n = 49), and undefined anatomy (n = 10). Fifteen of 101 Fontan patients had CPD. Forty-three percent of patients with two sizable ventricular masses displayed CPD, mostly with prolonged QRS, while the number of patients with CPD with right-dominant (9%) and left-dominant (6%) morphology was significantly lower (P = .016). Those with CPD displayed significantly (P < .05) larger QRS widths (142 ± 22 vs 112 ± 24 msec), lower ejection fractions (31 ± 14% vs 45 ± 14%), lower global early diastolic strain rates (0.7 ± 0.5 vs 1.2 ± 0.8 sec-1), and global systolic circumferential (-10 ± 5% vs -16 ± 7%) and longitudinal (-9 ± 5% vs -14 ± 5%) strain, respectively.CPD is present in a proportion of adolescent and adult patients after Fontan palliation. The presence of CPD is associated with reduced systolic and diastolic function compared with Fontan patients without CPD. Because the presence of CPD appears to be a promising predictor for response to cardiac resynchronization therapy in patients with biventricular circulation, these findings may have important potential for prospective evaluation of cardiac resynchronization therapy in patients with univentricular circulation.

  View details for DOI 10.1016/j.echo.2017.10.018

  View details for PubMedID 29229494

• Risk Estimates for Atherosclerotic Cardiovascular Disease in Adults With Congenital Heart Disease AMERICAN JOURNAL OF CARDIOLOGY Lui, G. K., Rogers, I. S., Ding, V. Y., Hedlin, H. K., MacMillen, K., Maron, D. J., Sillman, C., Romfh, A., Dade, T. C., Haeffele, C., Grady, S. R., McElhinney, D. B., Murphy, D. J., Fernandes, S. M. 2017; 119 (1): 112-118

  Abstract

  The adult with congenital heart disease (CHD) is at risk of developing atherosclerotic cardiovascular disease (ASCVD). We performed a cross-sectional study to describe established ASCVD risk factors and estimate 10-year and lifetime risk of ASCVD in adults over age 18 with CHD of moderate or great complexity using 3 validated risk assessment tools-the Framingham Study Cardiovascular Disease Risk Assessment, the Reynolds Risk Score, and the ASCVD Risk Estimator. We obtained extensive clinical and survey data on 178 enrolled patients, with average age 37.1 ± 12.6 years, 51% men. At least 1 modifiable ASCVD risk factor was present in 70%; the 2 most common were overweight/obesity (53%) and systemic hypertension (24%). Laboratory data were available in 103 of the 178 patients. Abnormal levels of glycated hemoglobin, high-sensitivity C-reactive protein, and high-density lipoprotein were each found in around 30% of patients. The 10-year ASCVD predicted risk using all 3 tools was relatively low (i.e., at least 90% of patients <10% risk), yet the median estimated lifetime risk was 36%. In conclusion, ASCVD risk factors are prevalent in adults with CHD. The risk estimation tools suggest that this population is particularly vulnerable to ASCVD with aging and should undergo guideline-based screening and management of modifiable risk factors.

  View details for DOI 10.1016/j.amjcard.2016.09.023

  View details for Web of Science ID 000391246900018

  View details for PubMedID 28247847

  View details for PubMedCentralID PMC5334785

• Proximity to Pediatric Cardiac Surgical Care among Adolescents with Congenital Heart Defects in 11 New York Counties. Birth defects research Sommerhalter, K. M., Insaf, T. Z., Akkaya-Hocagil, T., McGarry, C. E., Farr, S. L., Downing, K. F., Lui, G. K., Zaidi, A. N., Van Zutphen, A. R. 2017; 109 (18): 1494–1503

  Abstract

  Many individuals with congenital heart defects (CHDs) discontinue cardiac care in adolescence, putting them at risk of adverse health outcomes. Because geographic barriers may contribute to cessation of care, we sought to characterize geographic access to comprehensive cardiac care among adolescents with CHDs.Using a population-based, 11-county surveillance system of CHDs in New York, we characterized proximity to the nearest pediatric cardiac surgical care center among adolescents aged 11 to 19 years with CHDs. Residential addresses were extracted from surveillance records documenting 2008 to 2010 healthcare encounters. Addresses were geocoded using ArcGIS and the New York State Street and Address Maintenance Program, a statewide address point database. One-way drive and public transit time from residence to nearest center were calculated using R packages gmapsdistance and rgeos with the Google Maps Distance Matrix application programming interface. A marginal model was constructed to identify predictors associated with one-way travel time.We identified 2522 adolescents with 3058 corresponding residential addresses and 12 pediatric cardiac surgical care centers. The median drive time from residence to nearest center was 18.3 min, and drive time was 30 min or less for 2475 (80.9%) addresses. Predicted drive time was longest for rural western addresses in high poverty census tracts (68.7 min). Public transit was available for most residences in urban areas but for few in rural areas.We identified areas with geographic barriers to surgical care. Future research is needed to determine how these barriers influence continuity of care among adolescents with CHDs. Birth Defects Research 109:1494-1503, 2017.© 2017 Wiley Periodicals, Inc.

  View details for DOI 10.1002/bdr2.1129

  View details for PubMedID 29152921

• An Analysis of Left Ventricular Retraining in Patients With Dextro- and Levo-Transposition of the Great Arteries. The Annals of thoracic surgery Mainwaring, R. D., Patrick, W. L., Ibrahimiye, A. N., Watanabe, N., Lui, G. K., Hanley, F. L. 2017

  Abstract

  Patients with dextro (D)-transposition of the great arteries (TGA) who have undergone a previous atrial switch and for some patients with levo (L)-TGA (ie, no ventricular septal defect or outflow tract obstruction), the left ventricle (LV) may require retraining before late arterial switch. The purpose of this study was to analyze the results of LV retraining for these two entities.This was a retrospective review of 51 patients enrolled in an LV retraining program. There were 25 patients with D-TGA and 26 with L-TGA. The median age of the D-TGA patients was 15 years, and 22 of 25 were in New York Heart Association class III or IV. The median age of the L-TGA patients was 12 months, and 3 of 26 patients were in New York Heart Association class III or IV.LV retraining was successful in 13 of the 25 patients (52%) with D-TGA, and 10 these 13 patients (77%) underwent successful late arterial switch. Of the 26 patients with L-TGA, LV retraining was successful in 24 (92%), and a double-switch operation was successful in 19 of 19 (100%) who subsequently underwent that procedure. A mathematical formula based on the incremental gain in left-to-right ventricular pressure ratio correctly predicted the success or failure in 94% of the patients.The data demonstrate differences in the success of LV retraining and late arterial switch for D-TGA and L-TGA. We would propose that the LV retraining ratio may be useful in objectively selecting patients eligible for late arterial switch.

  View details for DOI 10.1016/j.athoracsur.2017.11.047

  View details for PubMedID 29274314

• Diagnosis and Management of Noncardiac Complications in Adults With Congenital Heart Disease: A Scientific Statement From the American Heart Association. Circulation Lui, G. K., Saidi, A., Bhatt, A. B., Burchill, L. J., Deen, J. F., Earing, M. G., Gewitz, M., Ginns, J., Kay, J. D., Kim, Y. Y., Kovacs, A. H., Krieger, E. V., Wu, F. M., Yoo, S. J. 2017

  Abstract

  Life expectancy and quality of life for those born with congenital heart disease (CHD) have greatly improved over the past 3 decades. While representing a great advance for these patients, who have been able to move from childhood to successful adult lives in increasing numbers, this development has resulted in an epidemiological shift and a generation of patients who are at risk of developing chronic multisystem disease in adulthood. Noncardiac complications significantly contribute to the morbidity and mortality of adults with CHD. Reduced survival has been documented in patients with CHD with renal dysfunction, restrictive lung disease, anemia, and cirrhosis. Furthermore, as this population ages, atherosclerotic cardiovascular disease and its risk factors are becoming increasingly prevalent. Disorders of psychosocial and cognitive development are key factors affecting the quality of life of these individuals. It is incumbent on physicians who care for patients with CHD to be mindful of the effects that disease of organs other than the heart may have on the well-being of adults with CHD. Further research is needed to understand how these noncardiac complications may affect the long-term outcome in these patients and what modifiable factors can be targeted for preventive intervention.

  View details for DOI 10.1161/CIR.0000000000000535

  View details for PubMedID 28993401

• Dextro-Transposition of the Great Arteries Long-term Sequelae of Atrial and Arterial Switch CARDIOLOGY CLINICS Haeffele, C., Lui, G. K. 2015; 33 (4): 543-?

  View details for DOI 10.1016/j.ccl.2015.07.012

  View details for Web of Science ID 000364729200006

  View details for PubMedID 26471819

• A Rapid, High-Quality, Cost-Effective, Comprehensive and Expandable Targeted Next-Generation Sequencing Assay for Inherited Heart Diseases. Circulation research Wilson, K. D., Shen, P., Fung, E., Karakikes, I., Zhang, A., Inanloorahatloo, K., Odegaard, J., Sallam, K., Davis, R. W., Lui, G. K., Ashley, E. A., Scharfe, C., Wu, J. C. 2015; 117 (7): 603-611

  Abstract

  Thousands of mutations across >50 genes have been implicated in inherited cardiomyopathies. However, options for sequencing this rapidly evolving gene set are limited because many sequencing services and off-the-shelf kits suffer from slow turnaround, inefficient capture of genomic DNA, and high cost. Furthermore, customization of these assays to cover emerging targets that suit individual needs is often expensive and time consuming.We sought to develop a custom high throughput, clinical-grade next-generation sequencing assay for detecting cardiac disease gene mutations with improved accuracy, flexibility, turnaround, and cost.We used double-stranded probes (complementary long padlock probes), an inexpensive and customizable capture technology, to efficiently capture and amplify the entire coding region and flanking intronic and regulatory sequences of 88 genes and 40 microRNAs associated with inherited cardiomyopathies, congenital heart disease, and cardiac development. Multiplexing 11 samples per sequencing run resulted in a mean base pair coverage of 420, of which 97% had >20× coverage and >99% were concordant with known heterozygous single nucleotide polymorphisms. The assay correctly detected germline variants in 24 individuals and revealed several polymorphic regions in miR-499. Total run time was 3 days at an approximate cost of $100 per sample.Accurate, high-throughput detection of mutations across numerous cardiac genes is achievable with complementary long padlock probe technology. Moreover, this format allows facile insertion of additional probes as more cardiomyopathy and congenital heart disease genes are discovered, giving researchers a powerful new tool for DNA mutation detection and discovery.

  View details for DOI 10.1161/CIRCRESAHA.115.306723

  View details for PubMedID 26265630

  View details for PubMedCentralID PMC4568077

• Predicting Outcomes Using the Heart Failure Survival Score in Adults with Moderate or Complex Congenital Heart Disease. Congenital heart disease Lin, E. Y., Cohen, H. W., Bhatt, A. B., Stefanescu, A., Dudzinski, D., DeFaria Yeh, D., Johnson, J., Lui, G. K. 2015; 10 (5): 387-395

  Abstract

  Adults with congenital heart disease (CHD) face increased risk for morbidity and mortality with age, but few prognostic models exist.This study aims to assess whether the Heart Failure Survival Score (HFSS), which risk stratifies patients for heart transplantation, predicts outcomes in adults with moderate or complex CHD.This was a multicenter, retrospective study which identified 441 patients with moderate or complex CHD between 2005 and 2013, of whom 169 had all the HFSS parameters required to calculate the risk score. Because all study patients were deemed low risk by the HFSS, the score was dichotomized at the median (10.4). Outcomes included death, transplant or ventricular assist device (VAD), arrhythmia requiring treatment, nonelective cardiovascular (CV) hospitalizations, and the composite. Associations of mean HFSS and HFSS <10.4 with each outcome were assessed.The cohort had mean ± standard deviation age of 33.6 ± 12.6 years, peak VO2 21.8 ± 7.5 mL/kg/min, HFSS of 10.45 ± 0.88, and median years follow-up of 2.7 (1.1, 5.2). There were five deaths (2.8%), no transplants or VADs, 25 arrhythmias (14.8%), 22 CV hospitalizations (13%), and 39 composites (23.1%). Lower mean HFSS was observed for patients who died (9.6 ± 0.83 vs. 10.5 ± 0.87, P = .02), arrhythmia requiring treatment (10.0 ± 0.70 vs. 10.5 ± 0.89, P = .005), CV hospitalizations (9.9 ± 0.73 vs. 10.5 ± 0.88, P = .002), and the composite (10.0 ± 0.70 vs. 10.6 ± 0.89, P < .001). The positive and negative predictive values of HFSS <10.4 for the composite were 34% and 88% respectively, with sensitivity and specificity 74% and 56%.Although a low HFSS was significantly associated with outcomes, it did not adequately risk stratify adults with CHD, whose heterogeneous pathophysiology differs from that of the acquired heart failure population. Further studies are warranted to provide a more accurate prognosis.

  View details for DOI 10.1111/chd.12229

  View details for PubMedID 25358483

  View details for PubMedCentralID PMC4417083

• Left Ventricular Retraining and Late Arterial Switch for D-Transposition of the Great Arteries ANNALS OF THORACIC SURGERY Watanabe, N., Mainwaring, R. D., Carrillo, S. A., Lui, G. K., Reddy, V. M., Hanley, F. L. 2015; 99 (5): 1655-1663

  Abstract

  For many decades, patients with d-transposition of the great arteries underwent an atrial switch procedure. Although many of these patients have continued to do well, a subset experience profound right ventricular failure. Some may be candidates for left ventricular (LV) retraining and late arterial switch. The purpose of this study was to review our experience with LV retraining and late arterial switch.This was a retrospective review of 32 patients with d-transposition. Thirty patients underwent a previous atrial switch and subsequently experienced right ventricular failure, whereas 2 presented late (8 months and 6 years) without previous intervention. The median age at the time of enrollment in this program was 15 years. Seven patients proceeded directly to late arterial switch owing to systemic LV pressures. The remaining 25 underwent a pulmonary artery band for LV retraining.Twenty of the 32 (63%) patients enrolled in this program were able to undergo a late arterial switch. There were 2 operative mortalities (10%). Two additional patients survived surgery but died in the early outpatient time period. There has been no late mortality after the arterial switch with a median follow-up of 5 years. Twelve patients underwent one or more pulmonary artery band procedures without evidence of effective LV retraining. There have been 2 early and 3 late (42%) deaths in this subgroup.The outcomes after arterial switch are encouraging and suggest that LV retraining and late arterial switch provide a viable option for this complex group of patients.

  View details for DOI 10.1016/j.athoracsur.2014.12.084

  View details for Web of Science ID 000353877900040

  View details for PubMedID 25817887

• Heart Murmur and Physical Examination in Athletes with a Focus on Congenital Heart Disease CURRENT SPORTS MEDICINE REPORTS Lui, G. K., Al Sindi, F., Bhatt, A. B. 2015; 14 (3): 263-265

  View details for Web of Science ID 000354551400031

  View details for PubMedID 25968870

• Management of Cardiovascular Risk Factors in Adults With Congenital Heart Disease JOURNAL OF THE AMERICAN HEART ASSOCIATION Lui, G. K., Fernandes, S., McElhinney, D. B. 2014; 3 (6)

  View details for DOI 10.1161/JAHA.114.001076

  View details for Web of Science ID 000345067600011

  View details for PubMedCentralID PMC4338694

• Usefulness of the Seattle Heart Failure Model to Identify Adults With Congenital Heart Disease at High Risk of Poor Outcome AMERICAN JOURNAL OF CARDIOLOGY Stefanescu, A., Macklin, E. A., Lin, E., Dudzinski, D. M., Johnson, J., Kennedy, K. F., Jacoby, D., Yeh, D. D., Lewis, G. D., Yeh, R. W., Liberthson, R., Lui, G., Bhatt, A. B. 2014; 113 (5): 865-870

  Abstract

  Our objective was to determine whether the Seattle Heart Failure Model (SHFM) differentiates patients with adult congenital heart disease (ACHD) at high versus low risk for cardiovascular outcomes and poor exercise capacity. The ACHD population is growing and presents increasingly for care in the community and at tertiary centers. Few strategies exist to identify the patients with ACHD at high risk for heart failure and mortality.We studied 153 adults with transposition of the great arteries, Ebstein anomaly, tetralogy of Fallot, double outlet right ventricle, and single ventricle from 2 ACHD centers. The primary outcome was cardiovascular death, with a secondary composite outcome of death, transplant, ventricular assist device, cardiovascular admission, and treatment for arrhythmia. We defined risk groups based on SHFM 5-year predicted survival: high (predicted survival <70%), intermediate (70% to 85%), and low risk (>85%). Ten patients had the primary outcome of death, and 46 the combined end point. The hazard of death in the SHFM high- versus the intermediate-risk group was 7.09 (95% confidence interval 1.5 to 33.4, p = 0.01; no deaths in the low-risk group) and the hazard of the composite outcome between the high- versus low-risk group was 6.64 (95% confidence interval 2.5 to 17.6, p = 0.0001). Kaplan-Meier survival analysis showed greater probability of all-cause mortality (p = 0.003) in the high-risk group. In conclusion, the SHFM can help identify subjects with ACHD at risk for adverse outcome and poor cardiopulmonary efficiency. This may add to the care of patients with ACHD in the community and streamline care at tertiary centers.

  View details for DOI 10.1016/j.amjcard.2013.11.043

  View details for Web of Science ID 000332142200019

• Anti-thrombotic strategies in the third trimester of pregnancy: A case of spontaneous coronary dissection requiring emergent PCI INTERNATIONAL JOURNAL OF CARDIOLOGY Paruchuri, V., Hafiz, A. M., Gubernikoff, G., De Leon, J., Lui, G., Naidu, S. S., Di Biase, L. 2013; 167 (1): E22-E25

  View details for DOI 10.1016/j.ijcard.2013.03.017

  View details for Web of Science ID 000320603100010

  View details for PubMedID 23582436

• Caring for the Adult with Congenital Heart Disease in an Adult Catheterization Laboratory by Pediatric InterventionalistsSafety and Efficacy CONGENITAL HEART DISEASE Sutton, N. J., Greenberg, M. A., Menegus, M. A., Lui, G., Pass, R. H. 2013; 8 (2): 111-116

  Abstract

  The purpose of this study is to describe the outcomes of cardiac catheterizations performed by pediatric interventional cardiologists in an adult catheterization laboratory on adult patients with congenital heart disease (CHD).With improved survival rates, the number of adults with CHD increases by ∼5%/year; this population often requires cardiac catheterization.From January 2005 to December 2009, two groups of patients were identified, an adult group (>21 years) and an adolescent group (13-21 years), who had catheterizations performed by pediatric interventional staff.Fifty-seven catheterizations were performed in 53 adults, while 59 were performed in 47 adolescents. The male to female ratio differed significantly between groups; only 15/53 (28%) of adults were male vs. 26/47 (55%) of adolescents (P =.006). Among adults, 27 had previously corrected CHD, 16 with atrial septal defect (ASD), and six with patent foramen ovale (PFO). This differed significantly from the adolescents, where only 30 had previously corrected CHD, seven with ASD, and one with PFO (P =.012). Among adults who were catheterized, interventions were performed on 28/53 (53%). All interventions were successful and included ASD/PFO closure, patent ductus arteriosus occlusion, coarctation dilation, pulmonary artery dilations, and one saphenous vein graft aneurysm closure. Nineteen adults had coronary angiography performed by adult interventionalists in consult with pediatric interventionalists. Two complications occurred among adults (3.8%) vs. one complication (2%; P = 1) among adolescents. No femoral vessel complications or catheterization-associated mortality occurred.Cardiac catheterizations can be performed effectively and safely in adults with CHD by pediatric interventional cardiologists in an adult catheterization laboratory.

  View details for DOI 10.1111/chd.12004

  View details for Web of Science ID 000317140100013

  View details for PubMedID 23006871

• Aortic Root Dilatation in Adults with Surgically Repaired Tetralogy of Fallot A Multicenter Cross-Sectional Study CIRCULATION Mongeon, F., Gurvitz, M. Z., Broberg, C. S., Aboulhosn, J., Opotowsky, A. R., Kay, J. D., Valente, A. M., Earing, M. G., Lui, G. K., Fernandes, S. M., Gersony, D. R., Cook, S. C., Ting, J. G., Nickolaus, M. J., Landzberg, M. J., Khairy, P. 2013; 127 (2): 172-?

  Abstract

  Although aortic root pathology has been described in patients with tetralogy of Fallot, the scope of the problem remains poorly defined. We sought to determine the prevalence and predictors of aortic root dilatation in adults with repaired tetralogy of Fallot.A multicenter cross-sectional study was conducted with standardized reassessment of echocardiographic parameters in 474 adults (≥18 years) with surgically repaired tetralogy of Fallot or pulmonary atresia with ventricular septal defect. The aortic root was measured in a parasternal long-axis view, in diastole, at the level of the sinus of Valsalva. Prevalence and predictors of an absolute diameter ≥40 mm and of an observed-to-expected ratio >1.5 were assessed. The aortic root dimension was ≥40 mm in 28.9% (95% confidence interval, 26.9%-30.9%). In multivariate analyses, the only independently associated factor was male sex (odds ratio, 4.48; 95% confidence interval, 1.55-12.89; P=0.006). The prevalence of an observed-to-expected aortic root dimension ratio >1.5 was 6.6% (95% confidence interval, 5.3%-7.9%). It was associated with pulmonary atresia and moderate or severe aortic regurgitation in univariate analyses, but no independent predictor was identified. The side of the aortic arch was not associated with aortic root dimension. The prevalence of moderate or severe aortic regurgitation was 3.5% (95% confidence interval, 2.7%-4.2%).Although nearly one third of adults with repaired tetralogy of Fallot have an aortic root diameter ≥40 mm, the prevalence of a dilated aortic root, when defined by an indexed ratio of observed-to-expected values, is low. Similarly, moderate or severe aortic regurgitation is uncommon.

  View details for DOI 10.1161/CIRCULATIONAHA.112.129585

  View details for Web of Science ID 000313637200013

  View details for PubMedID 23224208

• Left and Right Ventricular Diastolic Function in Adults With Surgically Repaired Tetralogy of Fallot: A Multi-Institutional Study. The Canadian journal of cardiology Aboulhosn, J. A., Lluri, G., Gurvitz, M. Z., Khairy, P., Mongeon, F. P., Kay, J., Valente, A. M., Earing, M. G., Opotowsky, A. R., Lui, G., Gersony, D. R., Cook, S., Child, J., Ting, J., Webb, G., Landzberg, M., Broberg, C. S. 2013

  Abstract

  BACKGROUND: Our purpose was to assess the prevalence and clinical implications of left ventricular (LV) and right ventricular (RV) diastolic dysfunction (DD) in patients with repaired tetralogy of Fallot (TOF). METHODS: Adults with repaired TOF at 11 North American congenital heart disease centres were included. Of 556 patients analyzed, 325 had sufficient Doppler data to evaluate for LV DD, defined as mitral lateral e' < 10 cm/s and E/e' ratio > 9. Abnormal RV diastolic function was defined as tricuspid E/A ratio of 0.8 to 2.1 with E/e' ratio > 6 or a tricuspid ratio E/A > 2.1 with a deceleration time < 120 milliseconds, and 105 patients were found to have sufficient Doppler data. RESULTS: Abnormal LV diastolic Doppler indices were prevalent in 13.8% and associated with hypertension, dyslipidemia, 3 or more cardiac operations, and a history of ventricular tachycardia. Abnormal RV indices were prevalent in 52.4% and associated with hypertension, dyslipidemia, diabetes mellitus, and 3 or more cardiac operations. Ventricular tachycardia was more common in those with abnormal RV diastolic function. CONCLUSION: Further prospective studies are needed to evaluate these associations with DD and the impact of treatment risk factors on diastolic function and arrhythmia burden.

  View details for DOI 10.1016/j.cjca.2012.11.003

  View details for PubMedID 23369488

• Not All Obstructive Cardiac Lesions Are Created Equal: Double-Chamber Right Ventricle In Pregnancy ECHOCARDIOGRAPHY-A JOURNAL OF CARDIOVASCULAR ULTRASOUND AND ALLIED TECHNIQUES Murthy, S., Lui, G., Raiszadeh, F., Boxt, L., Taub, C. 2012; 29 (8): E197-E200

  Abstract

  Double-chambered right ventricle (DCRV) is a rare form of right ventricular outflow tract (RVOT) obstruction accounting for approximately 1% of patients with congenital heart disease. It consists of an anomalous muscle bundle that divides the right ventricle usually between the sinus (inlet) and the infundibulum (outlet). This division creates a proximal chamber with high pressure and a distal chamber with low pressure. The hemodynamic obstruction of the RVOT is usually an acquired phenomenon, however the substrate for the anomalous muscle bundle is likely congenital. The diagnosis of DCRV should be considered in the young patient with an elevated right ventricular systolic pressure and intracavitary gradient. Echocardiography and cardiac MRI are the principal diagnostic tools for the assessment of DCRV. This entity is often misdiagnosed as pulmonary hypertension in the young patient, and can often go overlooked and untreated for many years. Definitive therapy involves surgical resection of the muscle bundle. This can often be curative and if done in a timely fashion, may prevent right ventricular remodeling. We describe the unique diagnostic dilemma, the course and management of a young adult with DCRV during pregnancy.

  View details for DOI 10.1111/j.1540-8175.2012.01721.x

  View details for Web of Science ID 000308466700006

  View details for PubMedID 22639818

• Making Complex Adult Congenital Heart Disease a Little Simpler SEMINARS IN ROENTGENOLOGY Zwany, S. K., Lui, G. K., Scheinfeld, M. H., Levsky, J. M. 2012; 47 (3): 289-301

  View details for DOI 10.1053/j.ro.2012.01.004

  View details for Web of Science ID 000305048900011

  View details for PubMedID 22657118

• Prevalence of Left Ventricular Systolic Dysfunction in Adults With Repaired Tetralogy of Fallot AMERICAN JOURNAL OF CARDIOLOGY Broberg, C. S., Aboulhosn, J., Mongeon, F., Kay, J., Valente, A. M., Khairy, P., Earing, M. G., Opotowsky, A. R., Lui, G., Gersony, D. R., Cook, S., Ting, J. G., Webb, G., Gurvitz, M. Z. 2011; 107 (8): 1215-1220

  Abstract

  Left ventricular (LV) systolic dysfunction has been observed in patients with repaired tetralogy of Fallot (TOF), although its clinical associations are unknown. Adults with repaired TOF were identified from 11 adult congenital heart disease centers. Clinical history was reviewed. Patients with pulmonary atresia were excluded. Echocardiograms were reanalyzed to estimate LV ejection fraction. LV function was defined as normal (LV ejection fraction ≥ 55%) or mildly (45% to 54%), moderately (35% to 44%), or severely (< 35%) decreased. Right ventricular (RV) and LV dimensions and Doppler parameters were remeasured. Function of all valves was qualitatively scored. Of 511 patients studied, LV systolic dysfunction was present in 107 (20.9%, 95% confidence interval 17.4 to 24.5). Specifically, 74 (14.4%) had mildly decreased and 33 (6.3%) had moderately to severely decreased systolic function. Presence of moderate to severe LV dysfunction was associated with male gender, LV enlargement, duration of shunt before repair, history of arrhythmia, QRS duration, implanted cardioverter-defibrillator, and moderate to severe RV dysfunction. Severity or duration of pulmonary regurgitation was not different. In conclusion, LV systolic dysfunction was found in 21% of adult patients with TOF and was associated with shunt duration, RV dysfunction, and arrhythmia.

  View details for DOI 10.1016/j.amjcard.2010.12.026

  View details for Web of Science ID 000289702400019

  View details for PubMedID 21349477

• Heart Rate Response During Exercise and Pregnancy Outcome in Women With Congenital Heart Disease CIRCULATION Lui, G. K., Silversides, C. K., Khairy, P., Fernandes, S. M., Valente, A. M., Nickolaus, M. J., Earing, M. G., AboulHosn, J. A., Rosenbaum, M. S., Cook, S., Kay, J. D., Jin, Z., Gersony, D. R. 2011; 123 (3): 242-248

  Abstract

  Cardiopulmonary exercise testing is often used to evaluate exercise capacity in adults with congenital heart disease including women who are considering pregnancy. The relationship between cardiopulmonary exercise testing parameters and pregnancy outcome has not been defined.We conducted a multicenter retrospective observational study of women with congenital heart disease who had undergone cardiopulmonary exercise testing within 2 years of pregnancy or during the first trimester. Cardiopulmonary exercise testing variables included peak oxygen consumption and measures of chronotropic response: peak heart rate, percentage of maximum age predicted heart rate, heart rate reserve (peak heart rate-resting heart rate), and chronotropic index [(peak heart rate-resting heart rate)/(220-age-resting heart rate)]. We identified 89 pregnancies in 83 women. There were 4 spontaneous abortions and 1 termination. One or more adverse cardiac events occurred in 18%; congestive heart failure in 14%, and sustained arrhythmia in 7%. Peak heart rate (odds ratio [OR] 0.71; 95% confidence interval [CI] [0.53, 0.94]; P=0.02), percentage of maximum age predicted heart rate (OR 0.93; 95% CI [0.88, 0.98]; P=0.01), and chronotropic index (OR 0.65; 95% CI [0.47, 0.90]; P=0.01) were associated with a cardiac event. Neonatal events occurred in 20%. Peak heart rate (OR 0.75; 95% CI [0.58, 0.98]; P=0.04), percentage of maximum age predicted heart rate (OR 0.94; 95% CI [0.89, 0.99]; P=0.02), heart rate reserve (OR 0.8; 95% CI [0.64, 0.99]; P=0.04), and chronotropic index (OR 0.73; 95% CI [0.54, 0.98]; P=0.04) correlated with a neonatal event. Peak oxygen consumption was not associated with an adverse pregnancy outcome.Abnormal chronotropic response correlates with adverse pregnancy outcomes in women with congenital heart disease and should be considered in refining risk stratification schemes.

  View details for DOI 10.1161/CIRCULATIONAHA.110.953380

  View details for Web of Science ID 000286507800010

  View details for PubMedID 21220738

• Arrhythmia Burden in Adults With Surgically Repaired Tetralogy of Fallot A Multi-Institutional Study CIRCULATION Khairy, P., Aboulhosn, J., Gurvitz, M. Z., Opotowsky, A. R., Mongeon, F., Kay, J., Valente, A. M., Earing, M. G., Lui, G., Gersony, D. R., Cook, S., Ting, J. G., Nickolaus, M. J., Webb, G., Landzberg, M. J., Broberg, C. S. 2010; 122 (9): 868-875

  Abstract

  The arrhythmia burden in tetralogy of Fallot, types of arrhythmias encountered, and risk profile may change as the population ages.The Alliance for Adult Research in Congenital Cardiology (AARCC) conducted a multicenter cross-sectional study to quantify the arrhythmia burden in tetralogy of Fallot, to characterize age-related trends, and to identify associated factors. A total of 556 patients, 54.0% female, 36.8+/-12.0 years of age were recruited from 11 centers. Overall, 43.3% had a sustained arrhythmia or arrhythmia intervention. Prevalence of atrial tachyarrhythmias was 20.1%. Factors associated with intraatrial reentrant tachycardia in multivariable analyses were right atrial enlargement (odds ratio [OR], 6.2; 95% confidence interval [CI], 2.8 to 13.6), hypertension (OR, 2.3; 95% CI, 1.1 to 4.6), and number of cardiac surgeries (OR, 1.4; 95% CI, 1.2 to 1.6). Older age (OR, 1.09 per year; 95% CI, 1.05 to 1.12), lower left ventricular ejection fraction (OR, 0.93 per unit; 95% CI, 0.89 to 0.96), left atrial dilation (OR, 3.2; 95% CI, 1.5 to 6.8), and number of cardiac surgeries (OR, 1.5; 95% CI, 1.2 to 1.9) were jointly associated with atrial fibrillation. Ventricular arrhythmias were prevalent in 14.6% and jointly associated with number of cardiac surgeries (OR, 1.3; 95% CI, 1.1 to 1.6), QRS duration (OR, 1.02 per 1 ms; 95% CI, 1.01 to 1.03), and left ventricular diastolic dysfunction (OR, 3.3; 95% CI, 1.5 to 7.1). Prevalence of atrial fibrillation and ventricular arrhythmias markedly increased after 45 years of age.The arrhythmia burden in adults with tetralogy of Fallot is considerable, with various subtypes characterized by different profiles. Atrial fibrillation and ventricular arrhythmias appear to be influenced more by left- than right-sided heart disease.

  View details for DOI 10.1161/CIRCULATIONAHA.109.928481

  View details for Web of Science ID 000281502900004

  View details for PubMedID 20713900

• The CALF (Congenital Heart Disease in Adults Lower Extremity Systemic Venous Health in Fontan Patients) Study JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY Valente, A. M., Bhatt, A. B., Cook, S., Earing, M. G., Gersony, D. R., Aboulhosn, J., Opotowsky, A. R., Lui, G., Gurvitz, M., Graham, D., Fernandes, S. M., Khairy, P., Webb, G., Gerhard-Herman, M., Landzberg, M. J. 2010; 56 (2): 144-150

  Abstract

  The objective of this study was to document the prevalence of chronic venous insufficiency (CVI) and its associated factors in adults with Fontan physiology.As the population of adults with complex congenital heart disease and Fontan physiology increases, so does the occurrence of highly morbid and mortal outcomes, including heart failure and thromboembolism. The presence of abnormal peripheral hemodynamic conditions in this population and their potential contribution to adverse outcomes is not well known. The primary objective of this study was to document the prevalence of CVI in adults with Fontan physiology.A total of 159 adults with Fontan physiology from 7 adult congenital heart centers were prospectively assessed for lower extremity CVI, with the assignment of clinical, etiological, anatomical, and pathophysiological classification grades, and compared with age-matched and sex-matched controls. Leg photographs were independently reassessed to confirm interobserver reliability.The prevalence of CVI was significantly greater in the Fontan population (60%; 95% confidence interval [CI]: 52% to 68%) compared with healthy controls (32%; 95% CI: 15% to 54%) (p = 0.008). Strikingly, the prevalence of severe CVI (clinical, etiological, anatomical, and pathophysiological grade > or = 4) was significantly higher in the Fontan group (22%; 95% CI: 16% to 29%) versus the healthy controls (0%; 95% CI: 0% to 14%) (p = 0.005). In a multivariate analysis, several factors were independently associated with severe CVI, including increased numbers of catheterizations with groin venous access, lower extremity itching, and deep venous thrombosis.CVI is common in adult patients with congenital heart disease with Fontan physiology. The contribution of abnormal peripheral hemodynamic conditions to comorbidities, including thromboembolism and heart failure, and interventions to improve peripheral hemodynamic conditions require further exploration.

  View details for DOI 10.1016/j.jacc.2010.02.048

  View details for Web of Science ID 000279313600009

  View details for PubMedID 20620728