George Lui, MD

  • George King-Tso Lui


Adult Congenital Heart Disease

Cardiovascular Disease

Work and Education

Professional Education

Yale School of Medicine, New Haven, CT, 2002


Brigham and Women's Hospital Harvard Medical School, Boston, MA, 2003


Brigham and Women's Hospital Harvard Medical School, Boston, MA, 2006


Columbia University Medical Center, New York, NY, 2009

Board Certifications

Adult Congenital Heart Disease, American Board of Internal Medicine

Cardiovascular Disease, American Board of Internal Medicine

All Publications

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: 7579


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 4.5years [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 4815years and 62% were female. The FRIEND registry equation predicted the lowest ppVO2 (measured/predicted; 7131%), compared to the Wasserman/Hansen (7429%) and Jones equations (8333%) (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
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 Rsner, A., Khalapyan, T., Dalen, H., McElhinney, D. B., Friedberg, M. K., Lui, G. K. 2018; 31 (2): 21119


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 (14222 vs 11224 msec), lower ejection fractions (3114% vs 4514%), lower global early diastolic strain rates (0.70.5 vs 1.20.8sec-1), and global systolic circumferential (-105% vs -167%) and longitudinal (-95% vs -145%) 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


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 12.6years, 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

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


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

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


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

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): 14941503


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

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


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


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 7.5mL/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

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

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


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

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)
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


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


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-?


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


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


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/

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


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


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


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


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