Dr. Christiane Haeffele earned her medical degree from the Duke University School of Medicine in Durham, North Carolina. She completed a combined medicine and pediatric residency at Harvard University in Boston, Massachusetts at the Brigham and Women's Hospital and Boston Children's Hospital. She also completed the Doris and Howard Hiatt Residency in Global Health Equity and Internal Medicine program, working on oncology infrastructure in Rwanda in partnership with the Rwandan Ministry of Health and Partners in Health. She completed her fellowship in adult cardiology and sub-specialized training in adult congenital heart disease and echocardiography at Stanford Hospital. She served one year as a chief fellow for the general cardiology fellowship at Stanford.

Dr. Haeffele is the Director of the Structural ECHO program at Stanford Hospital. She is the Program Director for the Advanced ECHO Imaging fellowship. Dr. Haeffele is also appointed Clinical Assistant Professor of Medicine and Pediatrics at Stanford University School of Medicine.

Dr. Haeffele is a board certified cardiology physician. Her research interests include management of adults with congenital heart disease, echocardiography, structural heart disease and catheter based interventions, and the complexities of heart transplant in the adult with congenital heart disease.

Clinical Focus

  • Echocardiography
  • Interventional ECHO
  • Pregnancy
  • Fontan physiology
  • Structural Heart Disease
  • Cardiovascular Disease

Academic Appointments

Administrative Appointments

  • Program Director of Advanced ECHO Imaging Fellowship, Stanford University (2020 - Present)
  • Director of Structural ECHO, Stanford CV Medicine (2019 - Present)
  • Assistant Program Director, General Cardiology Fellowship, Stanford University (2017 - 2021)
  • Affinity Chief, General Cardiology Inpatient Service, Stanford Hospital (2016 - 2019)

Honors & Awards

  • Department of Medicine Teaching Award, Stanford Medicine (2020)
  • Timothy F. Beckett, Jr. Award, Best Clinical Teaching by a Medicine Fellow, Stanford University (2014)
  • Excellence in Cardiology Fellowship, American College of Cardiology (2015)
  • Outstanding Clinical Fellow Award, Stanford University, Division of Cardiology (2014)
  • Andrew C. Puckett Humanism in Medicine, Duke University (2007)
  • Outstanding Researcher, Department of Molecular and Cellular Biology, UC-Berkeley (2001)
  • Graduated with Honors, Molecular and Cellular Biology, UC-Berkeley (1997)

Boards, Advisory Committees, Professional Organizations

  • Member, American Heart Association (2012 - Present)
  • Member, International Society of Adult Congenital Heart Disease (2015 - Present)
  • Fellow, Society for Cardiovascular Angiography and Interventions (SCAI) (2021 - Present)
  • Fellow, American College of Cardiology (2023 - Present)
  • Fellow, American Society of Echocardiography (2023 - Present)

Professional Education

  • Board Certification: National Board of Echocardiography, Adult Echocardiography (2016)
  • Board Certification: American Board of Internal Medicine, Adult Congenital Heart Disease (2019)
  • Fellowship: Stanford University Cardiovascular Medicine Fellowship (2016) CA
  • Residency: Brigham and Women's Hospital Internal Medicine Residency (2012) MA
  • Board Certification: American Board of Internal Medicine, Cardiovascular Disease (2016)
  • Fellowship: Stanford University Cardiovascular Medicine Fellowship (2015) CA
  • Board Certification: American Board of Internal Medicine, Internal Medicine (2014)
  • Medical Education: Duke University School of Medicine (2007) NC

Current Research and Scholarly Interests

Adult Congenital Heart Disease

Clinical Trials

  • 2019-06 TRISCEND Study Recruiting

    Prospective, multi-center study to assess safety and performance of the Edwards EVOQUE Tricuspid Valve Replacement System

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  • ACURATE IDE: Safety and Effectiveness Study of ACURATE Valve for Transcatheter Aortic Valve Replacement Recruiting

    To evaluate safety and effectiveness of the ACURATE Transfemoral Aortic Valve System for transcatheter aortic valve replacement (TAVR) in subjects with severe native aortic stenosis who are indicated for TAVR.

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  • ALLIANCE: Safety and Effectiveness of the SAPIEN X4 Transcatheter Heart Valve Recruiting

    The objective of this study is to establish the safety and effectiveness of the Edwards SAPIEN X4 Transcatheter Heart Valve (THV) in subjects with symptomatic, severe, calcific aortic stenosis (AS).

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  • Beating Heart Mitral Valve Repair With the HARPOON™ System Recruiting

    The objective of the study is to evaluate the safety and effectiveness of the HARPOON™ System in the treatment of patients with severe degenerative mitral regurgitation (DMR).

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  • Clinical Trial to Evaluate the Safety and Effectiveness of Using the Tendyne Transcatheter Mitral Valve System for the Treatment of Symptomatic Mitral Regurgitation Recruiting

    Prospective, controlled, multicenter clinical investigation with four trial cohorts: Randomized, Non-repairable, Severe Mitral Annular Calcification (MAC) and Severe Mitral Annular Calcification Continued Access Protocol (MAC CAP). Subjects in the Randomized cohort will be randomized in a 1:1 ratio to the trial device or to the MitraClip system. Subjects in the Non-repairable, Severe MAC, and Severe MAC CAP cohorts will receive the trial device. The objective of the Clinical Trial to Evaluate the Safety and Effectiveness of Using the Tendyne Transcatheter Mitral Valve System for the Treatment of Symptomatic Mitral Regurgitation (SUMMIT) is to evaluate the safety and effectiveness of the Tendyne Transcatheter Mitral Valve System for the treatment of patients with symptomatic, moderate-to-severe or severe mitral regurgitation or for patients with symptomatic mitral valve disease due to severe mitral annular calcification. This randomized controlled trial will provide the opportunity to evaluate the safety and clinical benefits of the Tendyne Transcatheter Mitral Valve System compared to the MitraClip System in patients with symptomatic, moderate-to-severe or severe mitral regurgitation, within approved MitraClip indications. In addition, the safety and effectiveness of the Tendyne Transcatheter Mitral Valve System will be evaluated in patients with severe mitral annular calcification who are at prohibitive risk for mitral valve surgery. Patients who are not suitable for mitral valve surgery for reasons other than severe mitral annular calcification and are also not suitable for transcatheter repair with MitraClip, will be enrolled in the Non-repairable cohort. Subjects will be seen at screening, pre- and post-procedure, discharge, 30 days, 3 months, 6 months, and annually through 5 years.

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  • Edwards CLASP TR EFS Recruiting

    Early feasibility study to assess the safety and performance of the Edwards PASCAL Transcatheter Valve Repair System in tricuspid regurgitation

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  • Edwards PASCAL CLASP IID/IIF Pivotal Clinical Trial Recruiting

    To establish the safety and effectiveness of the Edwards PASCAL Transcatheter Valve Repair System in patients with degenerative mitral regurgitation (DMR) who have been determined to be at prohibitive risk for mitral valve surgery by the Heart Team, and in patients with functional mitral regurgitation (FMR) on guideline directed medical therapy (GDMT)

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  • MitraClip REPAIR MR Study Recruiting

    The objective of this randomized controlled trial (RCT) is to compare the clinical outcome of MitraClip™ device versus surgical repair in patients with severe primary MR who are at moderate surgical risk and whose mitral valve has been determined to be suitable for correction by MV repair surgery by the cardiac surgeon on the local site heart team.

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  • Percutaneous or Surgical Repair In Mitral Prolapse And Regurgitation for ≥60 Year-olds (PRIMARY) Recruiting

    This is a prospective, multicenter, open-label, randomized trial comparing mitral valve (MV) transcatheter edge-to-edge repair (TEER) to surgical repair (1:1 ratio) in patients with primary, degenerative mitral regurgitation (MR). The trial will be conducted in the U.S., Canada, Germany and the United Kingdom, and is designed as a strategy trial. Thus, all devices legally marketed for TEER of primary degenerative MR in a particular country are eligible to be used in this trial.

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  • PROGRESS: Management of Moderate Aortic Stenosis by Clinical Surveillance or TAVR Recruiting

    This study objective is to establish the safety and effectiveness of the Edwards SAPIEN 3 / SAPIEN 3 Ultra / SAPIEN 3 Ultra RESILIA Transcatheter Heart Valve systems in subjects with moderate, calcific aortic stenosis. Following completion of enrollment, subjects will be eligible for enrollment in the continued access phase of the trial.

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  • Reducing Lung CongestIon Symptoms in Advanced Heart Failure Recruiting

    The objective of the RELIEVE-HF study is to provide reasonable assurance of safety and effectiveness of the V-Wave Interatrial Shunt System by improving meaningful clinical outcomes in patients with New York Heart Association (NYHA) functional Class II, Class III, or ambulatory Class IV heart failure (HF), irrespective of left ventricular ejection fraction, who at baseline are treated with guideline-directed drug and device therapies.

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  • Transcatheter Mitral Valve Replacement With the Medtronic Intrepid™ TMVR System in Patients With Severe Symptomatic Mitral Regurgitation. Recruiting

    Multi-center, global, prospective, non-randomized, interventional, pre-market trial. All subjects enrolled with receive the study device.

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  • CE Mark Study for the Harpoon Medical Device Not Recruiting

    The purpose of this study is to evaluate the safety and performance of the Harpoon Medical device. It is anticipated, that the Harpoon Medical device will provide advantages over current surgical interventions including: 1) a small minimally invasive incision, 2) no sternotomy, 3) no cardiopulmonary bypass, 4) no aortic manipulation, 5) a direct path to the valve plane, 6) performed on a beating heart, 7) real-time TOE-guided chordal length adjustment and 8) less complicated procedure that is teachable and adoptable.

    Stanford is currently not accepting patients for this trial.

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  • TRISCEND II Pivotal Trial Not Recruiting

    Pivotal trial to evaluate the safety and effectiveness of the Edwards EVOQUE tricuspid valve replacement system

    Stanford is currently not accepting patients for this trial.

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Graduate and Fellowship Programs

All Publications

  • Mortality and morbidity after combined heart and liver transplantation in the failing Fontan: An updated dual center retrospective study. Clinical transplantation Vaikunth, S. S., Ortega-Legaspi, J. M., Conrad, D. R., Chen, S., Daugherty, T., Haeffele, C. L., Teuteberg, J., Mclean, R., MacArthur, J. W., Woo, Y. J., Maeda, K., Ma, M., Nasirov, T., Hoteit, M., Hilscher, M. B., Wald, J., Mandelbaum, T., Olthoff, K. M., Abt, P. L., Atluri, P., Cevasco, M., Mavroudis, C. D., Fuller, S., Lui, G. K., Kim, Y. Y. 2024; 38 (4): e15302


    As the adult Fontan population with Fontan associated liver disease continues to increase, more patients are being referred for transplantation, including combined heart and liver transplantation.We report updated mortality and morbidity outcomes after combined heart and liver transplant in a retrospective cohort series of 40 patients (age 14 to 49 years) with Fontan circulation across two centers from 2006-2022.The 30-day, 1-year, 5-year and 10-year survival rate was 90%, 80%, 73% and 73% respectively. Sixty percent of patients met a composite comorbidity of needing either post-transplant mechanical circulatory support, renal replacement therapy or tracheostomy. Cardiopulmonary bypass time > 283 min (4.7 h) and meeting the composite comorbidity were associated with mortality by Kaplan Meier analysis.Further study to mitigate early mortality and the above comorbidities as well as the high risk of bleeding and vasoplegia in this patient population is warranted.

    View details for DOI 10.1111/ctr.15302

    View details for PubMedID 38567883

  • Hepatocellular carcinoma in survivors after Fontan operation: a case-control study. European heart journal Kim, Y. Y., Lluri, G., Haeffele, C., Daugherty, T., Krasuski, R. A., Serfas, J. D., de Freitas, R. A., Porlier, A., Lubert, A. M., Wu, F. M., Valente, A. M., Krieger, E. V., Buber, J., Rodriguez, F. H., Gaignard, S., Saraf, A., Hindes, M., Earing, M. G., Lewis, M. J., Rosenbaum, M. S., Zaidi, A. N., Hopkins, K., Bradley, E. A., Cedars, A. M., Ko, J. L., Franklin, W. J., Frederickson, A., Ginde, S., Grewal, J., Nyman, A., Min, J., Schluger, C., Rand, E., Rosenthal, B. E., Hilscher, M., Rychik, J., Hoteit, M. A. 2023

    View details for DOI 10.1093/eurheartj/ehad788

    View details for PubMedID 38085593

  • Echocardiographic Outcomes With Transcatheter Edge-to-Edge Repair for Degenerative Mitral Regurgitation in Prohibitive Surgical Risk Patients. JACC. Cardiovascular imaging Marcoff, L., Koulogiannis, K., Aldaia, L., Mediratta, A., Chadderdon, S. M., Makar, M. M., Ruf, T. F., Gößler, T., Zaroff, J. G., Leung, G. K., Ku, I. A., Nabauer, M., Grayburn, P. A., Wang, Z., Hawthorne, K. M., Fowler, D. E., Dal-Bianco, J. P., Vannan, M. A., Bevilacqua, C., Meineri, M., Ender, J., Forner, A. F., Puthumana, J. J., Mansoor, A. H., Lloyd, D. J., Voskanian, S. J., Ghobrial, A., Hahn, R. T., Mahmood, F., Haeffele, C., Ong, G., Schneider, L. M., Wang, D. D., Sekaran, N. K., Koss, E., Mehla, P., Harb, S., Miyasaka, R., Ivannikova, M., Stewart-Dehner, T., Mitchel, L., Raissi, S. R., Kalbacher, D., Biswas, S., Ho, E. C., Goldberg, Y., Smith, R. L., Hausleiter, J., Lim, D. S., Gillam, L. D. 2023


    The CLASP IID randomized trial (Edwards PASCAL TrAnScatheter Valve RePair System Pivotal Clinical Trial; NCT03706833) demonstrated the safety and effectiveness of the PASCAL system for mitral transcatheter edge-to-edge repair (M-TEER) in patients at prohibitive surgical risk with significant symptomatic degenerative mitral regurgitation (DMR).This study describes the echocardiographic methods and outcomes from the CLASP IID trial and analyzes baseline variables associated with residual mitral regurgitation (MR) ≤1+.An independent echocardiographic core laboratory assessed echocardiographic parameters based on American Society of Echocardiography guidelines focusing on MR mechanism, severity, and feasibility of M-TEER. Factors associated with residual MR ≤1+ were identified using logistic regression.In 180 randomized patients, baseline echocardiographic parameters were well matched between the PASCAL (n = 117) and MitraClip (n = 63) groups, with flail leaflets present in 79.2% of patients. Baseline MR was 4+ in 76.4% and 3+ in 23.6% of patients. All patients achieved MR ≤2+ at discharge. The proportion of patients with MR ≤1+ was similar in both groups at discharge but diverged at 6 months, favoring PASCAL (83.7% vs 71.2%). Overall, patients with a smaller flail gap were significantly more likely to achieve MR ≤1+ at discharge (adjusted OR: 0.70; 95% CI: 0.50-0.99). Patients treated with PASCAL and those with a smaller flail gap were significantly more likely to sustain MR ≤1+ to 6 months (adjusted OR: 2.72 and 0.76; 95% CI: 1.08-6.89 and 0.60-0.98, respectively).The study used DMR-specific echocardiographic methodology for M-TEER reflecting current guidelines and advances in 3-dimensional echocardiography. Treatment with PASCAL and a smaller flail gap were significant factors in sustaining MR ≤1+ to 6 months. Results demonstrate that MR ≤1+ is an achievable benchmark for successful M-TEER. (Edwards PASCAL TrAnScatheter Valve RePair System Pivotal Clinical Trial [CLASP IID]; NCT03706833).

    View details for DOI 10.1016/j.jcmg.2023.09.015

    View details for PubMedID 38099912

  • Transfemoral tricuspid valve replacement and one-year outcomes: the TRISCEND study. European heart journal Kodali, S., Hahn, R. T., Makkar, R., Makar, M., Davidson, C. J., Puthumana, J. J., Zahr, F., Chadderdon, S., Fam, N., Ong, G., Yadav, P., Thourani, V., Vannan, M. A., O'Neill, W. W., Wang, D. D., Tchétché, D., Dumonteil, N., Bonfils, L., Lepage, L., Smith, R., Grayburn, P. A., Sharma, R. P., Haeffele, C., Babaliaros, V., Gleason, P. T., Elmariah, S., Inglessis-Azuaje, I., Passeri, J., Herrmann, H. C., Silvestry, F. E., Lim, S., Fowler, D., Webb, J. G., Moss, R., Modine, T., Lafitte, S., Latib, A., Ho, E., Goldberg, Y., Shah, P., Nyman, C., Rodés-Cabau, J., Bédard, E., Brugger, N., Sannino, A., Mack, M. J., Leon, M. B., Windecker, S. 2023


    For patients with symptomatic, severe tricuspid regurgitation (TR), early results of transcatheter tricuspid valve (TV) intervention studies have shown significant improvements in functional status and quality of life associated with right-heart reverse remodelling. Longer-term follow-up is needed to confirm sustained improvements in these outcomes.The prospective, single-arm, multicentre TRISCEND study enrolled 176 patients to evaluate the safety and performance of transcatheter TV replacement in patients with ≥moderate, symptomatic TR despite medical therapy. Major adverse events, reduction in TR grade and haemodynamic outcomes by echocardiography, and clinical, functional, and quality-of-life parameters are reported to one year.Enrolled patients were 71.0% female, mean age 78.7 years, 88.0% ≥ severe TR, and 75.4% New York Heart Association classes III-IV. Tricuspid regurgitation was reduced to ≤mild in 97.6% (P < .001), with increases in stroke volume (10.5 ± 16.8 mL, P < .001) and cardiac output (0.6 ± 1.2 L/min, P < .001). New York Heart Association class I or II was achieved in 93.3% (P < .001), Kansas City Cardiomyopathy Questionnaire score increased by 25.7 points (P < .001), and six-minute walk distance increased by 56.2 m (P < .001). All-cause mortality was 9.1%, and 10.2% of patients were hospitalized for heart failure.In an elderly, highly comorbid population with ≥moderate TR, patients receiving transfemoral EVOQUE transcatheter TV replacement had sustained TR reduction, significant increases in stroke volume and cardiac output, and high survival and low hospitalization rates with improved clinical, functional, and quality-of-life outcomes to one year. Funded by Edwards Lifesciences, TRISCEND number, NCT04221490.

    View details for DOI 10.1093/eurheartj/ehad667

    View details for PubMedID 37930776

  • Patient-Reported Outcomes in Tricuspid Valve Intervention: Patient Preference Results From the TRISCEND II Trial Brener, M., Barker, C., Davidson, C., Narang, A., Sharma, R., Haeffele, C., Yadav, P., Thourani, V., Kodali, S., Hahn, R., Makkar, R., Makar, M., Hausleiter, J., Nabauer, M., O'Neill, W., Wang, D., Lindman, B., Zahr, F., Chadderdon, S., Eleid, M., Pislaru, S., Mancilla, M., Kemp, L., Mollenkopf, S., Ryan, M., Cohen, D. ELSEVIER SCIENCE INC. 2023: B289-B290
  • Emergency Transcatheter Repair for Anterior Leaflet Tear Following Percutaneous Balloon Mitral Valvuloplasty. JACC. Case reports Dowling, C., Haeffele, C., Yeung, A. C., Sharma, R. P. 2023; 23: 101980


    We present the case of a 66-year-old woman who developed severe mitral regurgitation from rupture of the anterior mitral valve leaflet following percutaneous balloon mitral valvuloplasty. Emergency transcatheter mitral valve repair was used to reduce the severity of mitral regurgitation and facilitate definitive surgical treatment. (Level of Difficulty: Advanced.).

    View details for DOI 10.1016/j.jaccas.2023.101980

    View details for PubMedID 37954949

    View details for PubMedCentralID PMC10635879

  • CHARACTERISTICS AND OUTCOMES OF HEPATOCELLULAR CARCINOMA IN THE FONTAN SURVIVOR: A MULTICENTER RETROSPECTIVE STUDY EXAMINING LIVER OUTCOMES Rosenthal, B. E., Kim, Y. Y., Nyman, A., Lluri, G., Haeffele, C., De Freitas, R. A., Lubert, A., Krasuski, R., Wu, F., Krieger, E., Saraf, A., Earing, M., Lewis, M. J., Rodriguez, F. H., Zaidi, A., Bradley, E., Cedars, A. M., Franklin, W. J., Ginde, S., Grewal, J., Schluger, C., Min, J., Hilscher, M., Rychik, J., Rand, E., Hoteit, M. A. LIPPINCOTT WILLIAMS & WILKINS. 2023: S1810-S1811
  • Fontan Associated Liver Disease: Canary in the Coal Mine or Silent Killer? Journal of the American Heart Association Haeffele, C., McElhinney, D. B. 2023: e031597

    View details for DOI 10.1161/JAHA.123.031597

    View details for PubMedID 37776191

  • The Resilient Heart: Exploring Post-Traumatic Stress in Adult Patients With Congenital Heart Disease. The American journal of cardiology Haeffele, C., Sillman, C. 2023

    View details for DOI 10.1016/j.amjcard.2023.07.147

    View details for PubMedID 37612217

  • Percutaneous Treatment of an Aorto-Right Ventricular Fistula FollowingBalloon-Expandable Transcatheter Aortic Valve Replacement. JACC. Case reports Dowling, C., Haeffele, C., Pogatchnik, B. P., Sharma, R. P. 2023; 18: 101906


    We present the case of a 71-year-old man who experienced congestive cardiac failure after transcatheter aortic valve replacement with a balloon-expandable transcatheter heart valve. Echocardiography and cardiac computed tomography demonstrated an aorto-right ventricular fistula, and successful percutaneous closure was performed with a vascular plug. (Level of Difficulty: Advanced.).

    View details for DOI 10.1016/j.jaccas.2023.101906

    View details for PubMedID 37545680

  • Morbidity and Mortality in Adult Fontan Patients After Heart or Combined Heart-Liver Transplantation. Journal of the American College of Cardiology Lewis, M. J., Reardon, L. C., Aboulhosn, J., Haeffele, C., Chen, S., Kim, Y., Fuller, S., Forbess, L., Alshawabkeh, L., Urey, M. A., Book, W. M., Rodriguez, F., Menachem, J. N., Clark, D. E., Valente, A. M., Carazo, M., Egbe, A., Connolly, H. M., Krieger, E. V., Angiulo, J., Cedars, A., Ko, J., Jacobsen, R. M., Earing, M. G., Cramer, J. W., Ermis, P., Broda, C., Nugaeva, N., Ross, H., Awerbach, J. D., Krasuski, R. A., Rosenbaum, M. 2023; 81 (22): 2161-2171


    An increasing number of adult Fontan patients require heart transplantation (HT) or combined heart-liver transplant (CHLT); however, data regarding outcomes and optimal referral time remain limited.The purpose of this study was to define survivorship post-HT/CHLT and predictors of post-transplant mortality, including timing of referral, in the adult Fontan population.A retrospective cohort study of adult Fontan patients who underwent HT or CHLT across 15 centers in the United States and Canada was performed. Inclusion criteria included the following: 1) Fontan; 2) HT/CHLT referral; and 3) age ≥16 years at the time of referral. Date of "failing" Fontan was defined as the earliest of the following: worsening fluid retention, new ascites, refractory arrhythmia, "failing Fontan" diagnosis by treating cardiologist, or admission for heart failure.A total of 131 patients underwent transplant, including 40 CHLT, from 1995 to 2021 with a median post-transplant follow-up time of 1.6 years (Q1 0.35 years, Q3 4.3 years). Survival was 79% at 1 year and 66% at 5 years. Survival differed by decade of transplantation and was 87% at 1 year and 76% at 5 years after 2010. Time from Fontan failure to evaluation (HR/year: 1.23 [95% CI: 1.11-1.36]; P < 0.001) and markers of failure, including NYHA functional class IV (HR: 2.29 [95% CI: 1.10-5.28]; P = 0.050), lower extremity varicosities (HR: 3.92 [95% CI: 1.68-9.14]; P = 0.002), and venovenous collaterals (HR: 2.70 [95% CI: 1.17-6.20]; P = 0.019), were associated with decreased post-transplant survival at 1 year in a bivariate model that included transplant decade.In our multicenter cohort, post-transplant survival improved over time. Late referral after Fontan failure and markers of failing Fontan physiology, including worse functional status, lower extremity varicosities, and venovenous collaterals, were associated with post-transplant mortality.

    View details for DOI 10.1016/j.jacc.2023.03.422

    View details for PubMedID 37257951

  • Clinical Outcomes of Adult Fontan-Associated Liver Disease and Combined Heart-Liver Transplantation. Journal of the American College of Cardiology Lewis, M. J., Reardon, L. C., Aboulhosn, J., Haeffele, C., Chen, S., Kim, Y., Fuller, S., Forbess, L., Alshawabkeh, L., Urey, M. A., Book, W. M., Rodriguez, F., Menachem, J. N., Clark, D. E., Valente, A. M., Carazo, M., Egbe, A., Connolly, H. M., Krieger, E. V., Angiulo, J., Cedars, A., Ko, J., Jacobsen, R. M., Earing, M. G., Cramer, J. W., Ermis, P., Broda, C., Nugaeva, N., Ross, H., Awerbach, J. D., Krasuski, R. A., Rosenbaum, M. 2023; 81 (22): 2149-2160


    The impact of Fontan-associated liver disease (FALD) on post-transplant mortality and indications for combined heart-liver transplant (CHLT) in adult Fontan patients remains unknown.The purpose of this study was to assess the impact of FALD on post-transplant outcomes and compare HT vs CHLT in adult Fontan patients.We performed a retrospective-cohort study of adult Fontan patients who underwent HT or CHLT across 15 centers. Inclusion criteria were as follows: 1) Fontan; 2) HT/CHLT referral; and 3) age ≥16 years at referral. Pretransplant FALD score was calculated using the following: 1) cirrhosis; 2) varices; 3) splenomegaly; or 4) ≥2 paracenteses.A total of 131 patients (91 HT and 40 CHLT) were included. CHLT recipients were more likely to be older (P = 0.016), have a lower hemoglobin (P = 0.025), require ≥2 diuretic agents pretransplant (P = 0.051), or be transplanted in more recent decades (P = 0.001). Postmatching, CHLT demonstrated a trend toward improved survival at 1 year (93% vs 74%; P = 0.097) and improved survival at 5 years (86% vs 52%; P = 0.041) compared with HT alone. In patients with a FALD score ≥2, CHLT was associated with improved survival (1 year: 85% vs 62%; P = 0.044; 5 years: 77% vs 42%; P = 0.019). In a model with transplant decade and FALD score, CHLT was associated with improved survival (HR: 0.33; P = 0.044) and increasing FALD score was associated with worse survival (FALD score: 2 [HR: 14.6; P = 0.015], 3 [HR: 22.2; P = 0.007], and 4 [HR: 27.8; P = 0.011]).Higher FALD scores were associated with post-transplant mortality. Although prospective confirmation of our findings is necessary, compared with HT alone, CHLT recipients were older with higher FALD scores, but had similar survival overall and superior survival in patients with a FALD score ≥2.

    View details for DOI 10.1016/j.jacc.2023.03.421

    View details for PubMedID 37257950

  • Outcomes After Development of Ventricular Arrhythmias in Single Ventricular Heart Disease Patients With Fontan Palliation. Circulation. Arrhythmia and electrophysiology Giacone, H. M., Chubb, H., Dubin, A. M., Motonaga, K. S., Ceresnak, S. R., Goodyer, W. R., Hanish, D., Trela, A. V., Boramanand, N., Lencioni, E., Boothroyd, D., Graber-Naidich, A., Wright, G., Haeffele, C., Hollander, S. A., McElhinney, D. B., Ma, M., Hanley, F. L., Chen, S. 2023: e011143


    With the advent of more intensive rhythm monitoring strategies, ventricular arrhythmias (VAs) are increasingly detected in Fontan patients. However, the prognostic implications of VA are poorly understood. We assessed the incidence of VA in Fontan patients and the implications on transplant-free survival.Medical records of Fontan patients seen at a single center between 2002 and 2019 were reviewed to identify post-Fontan VA (nonsustained ventricular tachycardia >4 beats or sustained >30 seconds). Patients with preFontan VA were excluded. Hemodynamically unstable VA was defined as malignant VA. The primary outcome was death or heart transplantation. Death with censoring at transplant was a secondary outcome.Of 431 Fontan patients, transplant-free survival was 82% at 15 years post-Fontan with 64 (15%) meeting primary outcome of either death (n=16, 3.7%), at a median 4.6 (0.4-10.2) years post-Fontan, or transplant (n=48, 11%), at a median of 11.1 (5.9-16.2) years post-Fontan. Forty-eight (11%) patients were diagnosed with VA (90% nonsustained ventricular tachycardia, 10% sustained ventricular tachycardia). Malignant VA (n=9, 2.0%) was associated with younger age, worse systolic function, and valvular regurgitation. Risk for VA increased with time from Fontan, 2.4% at 10 years to 19% at 20 years. History of Stage 1 surgery with right ventricular to pulmonary artery conduit and older age at Fontan were significant risk factors for VA. VA was strongly associated with an increased risk of transplant or death (HR, 9.2 [95% CI, 4.5-18.7]; P<0.001), with a transplant-free survival of 48% at 5-year post-VA diagnosis.Ventricular arrhythmias occurred in 11% of Fontan patients and was highly associated with transplant or death, with a transplant-free survival of <50% at 5-year post-VA diagnosis. Risk factors for VA included older age at Fontan and history of right ventricular to pulmonary artery conduit. A diagnosis of VA in Fontan patients should prompt increased clinical surveillance.

    View details for DOI 10.1161/CIRCEP.122.011143

    View details for PubMedID 37254747

  • First described mitral clip in an adult extracardiac Fontan patient: a case report. European heart journal. Case reports Haeffele, C. L., Lui, G. K., Peng, L., Chan, F., Sharma, R. P. 2023; 7 (1): ytac479


    The use of transcatheter edge-to-edge repair (TEER) in patients with advanced heart failure has been shown to reduce hospitalizations and increase survival. As patients with Fontan circulations grow older, a significant proportion of them will develop severe atrioventricular (AV) valve regurgitation in the systemic ventricle. Conventional surgical repair and transplant carry high mortality risk for the adult Fontan patient with progressive heart failure.A 51-year-old female extracardiac Fontan patient developed severe AV valve regurgitation and progressive functional decline. Based on her operative risk for conventional surgical intervention or transplant, TEER using the Abbott MitraClip device was performed. The degree of mitral regurgitation was decreased from severe to moderate regurgitation.This is the first known case describing the use of a successful TEER in an adult patient with an extracardiac Fontan. Given the increasing numbers of patients surviving into adulthood with a Fontan circulation, transcatheter interventions may provide an alternative treatment option to conventional surgeries and medical therapies.

    View details for DOI 10.1093/ehjcr/ytac479

    View details for PubMedID 36733686

    View details for PubMedCentralID PMC9887705

  • Arrhythmias in Repaired Tetralogy of Fallot: A Scientific Statement From the American Heart Association. Circulation. Arrhythmia and electrophysiology Krieger, E. V., Zeppenfeld, K., DeWitt, E. S., Duarte, V. E., Egbe, A. C., Haeffele, C., Lin, K. Y., Robinson, M. R., Sillman, C., Upadhyay, S. 2022: e000084


    Arrhythmia and sudden cardiac death remain common in repaired tetralogy of Fallot and affect even those with excellent anatomic repairs. Atrial arrhythmia often has mechanisms different from those in acquired heart disease. Ventricular arrhythmia remains a major source of mortality in repaired tetralogy of Fallot. Noninvasive risk stratification is important to identify patients who may benefit from ablation or primary prevention implantable cardioverter defibrillators. Multiple noninvasive risk factors are associated with ventricular arrhythmia, but no universally accepted risk stratification algorithm exists. The mechanism of ventricular arrhythmia is usually attributable to a consistent and discrete set of slowly conducting anatomic isthmuses related to both the native anatomy and the consequences of the surgical repair, which interact with ventricular remodeling to provide arrhythmic substrate. This substrate can be identified during electroanatomic mapping and prophylactically ablated in appropriate patients. This scientific statement discusses the mechanisms and treatment of arrhythmia in repaired tetralogy of Fallot.

    View details for DOI 10.1161/HAE.0000000000000084

    View details for PubMedID 36263773

  • Extracorporeal membrane oxygenator as a bridge to heart-liver en bloc transplant in a patient with Fontan circulation. JTCVS techniques Kim, J. L., Vaikunth, S. S., Haeffele, C., MacArthur, J. W. 2022; 12: 171-174

    View details for DOI 10.1016/j.xjtc.2022.01.018

    View details for PubMedID 35403048

  • The atrial switch patient with a dilated subpulmonic left ventricle (vol 4,100153, 2021) INTERNATIONAL JOURNAL OF CARDIOLOGY CONGENITAL HEART DISEASE Vaikunth, S. S., Haeffele, C., Mcelhinney, D. B., Lui, G. K., Romfh, A. W. 2021; 5
  • A 40-Year-Old Man With Tricuspid Atresia, Status Post-Fontan, With Severe COVID-19 Pneumonia and Pneumothorax. JACC. Case reports Vaikunth, S. S., Bykhovsky, M. R., Romfh, A. W., Haeffele, C. L., Rogers, I. S., Dong, E., Scribner, C., Lui, G. K. 2021


    We report a case of COVID-19 in an adult single-ventricle patient post-Fontan-to our knowledge, the first report in this population documenting the use of the latest management recommendations for this novel disease. Additionally, this patient had significant pre-existing ventricular dysfunction, valvular disease, and comorbidities including HIV. (Level of Difficulty: Advanced.).

    View details for DOI 10.1016/j.jaccas.2020.10.032

    View details for PubMedID 33558861

  • The atrial switch patient with a dilated subpulmonic left ventricle International Journal of Cardiology Congenital Heart Disease Vaikunth, S. S., Haeffele, C., McElhinney, D. B., Lui, G. K., Romfh, A. 2021; Volume 4 (100153)
  • Adult congenital heart disease: left-sided obstructive lesions. Heart (British Cardiac Society) Haeffele, C. 2021

    View details for DOI 10.1136/heartjnl-2019-316364

    View details for PubMedID 34815332

  • Clinical Outcomes, Echocardiographic Findings, and Care Quality Metrics for People Living with HIV and Rheumatic Heart Disease in Uganda. Clinical infectious diseases : an official publication of the Infectious Diseases Society of America Chang, A. Y., Rwebembera, J., Bendavid, E., Okello, E., Barry, M., Beaton, A. Z., Haeffele, C., Webel, A. R., Kityo, C., Longenecker, C. T. 2021


    Rheumatic Heart Disease (RHD) affects 41 million people worldwide, mostly in low- and middle-income countries, where it is co-endemic with HIV. HIV is also a chronic inflammatory disorder associated with cardiovascular complications, yet the epidemiology of patients affected by both diseases is poorly understood.Utilizing the Uganda National RHD Registry, we described the echocardiographic findings, clinical characteristics, medication prescription rates, and outcomes of all 73 people carrying concurrent diagnoses of HIV and RHD between 2009 and 2018. These individuals were compared to an age- and sex-matched cohort of 365 subjects with RHD only.The median age of the HIV-RHD group was 36 years (IQR 15) and 86% were women. The HIV-RHD cohort had higher rates of prior stroke/transient ischemic attack (12% vs 5%, p=0.02) than the RHD-only group, with this association persisting following multivariable adjustment (OR 3.08, p=0.03). Prevalence of other comorbidities, echocardiographic findings, prophylactic penicillin prescription rates, retention in clinical care, and mortality were similar between the two groups.Patients living with RHD and HIV in Uganda are a relatively young, predominantly female group. Although RHD-HIV comorbid individuals have higher rates of stroke, their similar all-cause mortality and RHD care quality metrics (such as retention in care) compared to those with RHD alone suggest rheumatic heart disease defines their clinical outcome more than HIV does. We believe this study to be one of the first reports of the epidemiologic profile and longitudinal outcomes of patients who carry diagnoses of both conditions.

    View details for DOI 10.1093/cid/ciab681

    View details for PubMedID 34382644

  • Comparison of combined heart‒liver vs heart-only transplantation in pediatric and young adult Fontan recipients. The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation Sganga, D., Hollander, S. A., Vaikunth, S., Haeffele, C., Bensen, R., Navaratnam, M., McDonald, N., Profita, E., Maeda, K., Concepcion, W., Bernstein, D., Chen, S. 2020


    BACKGROUND: Indications for a heart‒liver transplantation (HLT) for Fontan recipients are not well defined. We compared listing characteristics, post-operative complications, and post-transplant outcomes of Fontan recipients who underwent HLT with those of patients who underwent heart-only transplantation (HT). We hypothesized that patients who underwent HLT have increased post-operative complications but superior survival outcomes compared with patients who underwent HT.METHODS: We performed a retrospective review of Fontan recipients who underwent HLT or HT at a single institution. Characteristics at the time of listing, including the extent of liver disease determined by laboratory, imaging, and biopsy data, were compared. Post-operative complications were assessed, and the Kaplan‒Meier survival method was used to compare post-transplant survival. Univariate regression analyses were performed to identify the risk factors for increased mortality and morbidity among patients who underwent HT.RESULTS: A total of 47 patients (9 for HLT, 38 for HT) were included. Patients who underwent HLT were older, were more likely to be on dual inotrope therapy, and had evidence of worse liver disease. Whereas ischemic time was longer for the group who underwent HLT, post-operative complications were similar. Over a median post-transplant follow-up of 17 (interquartile range: 5-52) months, overall mortality for the cohort was 17%; only 1 patient who underwent HLT died (11%) vs 7 patients who underwent HT (18%) (p = 0.64). Among patients who underwent HT, cirrhosis on pre-transplant imaging was associated with worse outcomes.CONCLUSIONS: Despite greater inotrope need and more severe liver disease at the time of listing, Fontan recipients undergoing HLT have post-transplant outcomes comparable with those of patients undergoing HT. HLT may offer a survival benefit for Fontan recipients with liver disease.

    View details for DOI 10.1016/j.healun.2020.12.008

    View details for PubMedID 33485775

  • Does liver biopsy accurately measure fibrosis in Fontan associated liver disease? A comparison of liver biopsy pre-combined heart and liver transplant and liver explant post-transplant. Clinical transplantation Vaikunth, S. S., Higgins, J. P., Concepcion, W., Haeffele, C., Wright, G. E., Chen, S., Lui, G. K., Daugherty, T. 2020: e14120


    The accuracy of liver biopsy to stage fibrosis due to Fontan associated liver disease (FALD) remains unclear. We compared results of biopsy pre-combined heart and liver transplantation (CHLT) to results of whole liver explant. Liver biopsy and explants from 15 Fontan patients (ages 16 - 49, median 28 years) were retrospectively reviewed. Staging was as follows: stage 0: no fibrosis, stage 1: pericellular fibrosis, stage 2: bridging fibrosis, stage 3: regenerative nodules. There is no stage 4. Clinical characteristics including Model of End-stage Liver Disease eXcluding INR and Varices, Ascites, Splenomegaly, and Thrombocytopenia (VAST) scores were collected, and descriptive statistics and Mann-Whitney U tests used to analyze data. All patients had biopsies with at least bridging fibrosis, and all had nodularity on explant; transjugular biopsy never overestimated fibrosis. Explant showed higher grade fibrosis (Stage 3) than pre-CHLT biopsy (Stage 2) in 6 of 15 patients and equal grade of fibrosis (Stage 3) in 9 of 15 patients. Though clinical characteristics varied significantly, VAST score was ≥ 2 in all but two patients. Transjugular liver biopsy does not overestimate and can underestimate fibrosis in Fontan patients undergoing CHLT, likely due to the patchy nature of fibrosis in FALD.

    View details for DOI 10.1111/ctr.14120

    View details for PubMedID 33053213

  • CLINICAL CHARACTERISTICS OF "FAILING" ADULT FONTAN PATIENTS ACROSS 14 CENTERS: A REPORT FROM THE FOSTER STUDY Lewis, M. J., Haeffele, C., Chen, S., Reardon, L., Aboulhosn, J. A., Nugaeva, N., Ross, H., Kim, Y., Krasuski, R. A., Valente, A., Carazo, M., Krieger, E., Angiulo, J., Book, W., Rodriguez, F., Egbe, A., Jacobson, R., Earing, M., Cramer, J. W., Cedars, A. M., Ko, J., Broda, C., Ermis, P., Rosenbaum, M. ELSEVIER SCIENCE INC. 2020: 553
  • Heart failure in the adult Ebstein patient. Heart failure reviews Schultz, K. n., Haeffele, C. L. 2020


    Ebstein anomaly comprises approximately 1% of all congenital heart diseases. It occurs when the tricuspid valve fails to properly delaminate from the right ventricle, resulting in a clinical spectrum of abnormal tricuspid valve morphology and right ventricular dysfunction. Due to the anatomy of the tricuspid valve and right ventricle, as well as associated right- and left-sided pathology, patients are at risk for both right and left ventricular failure and the associated symptoms of each. Ebstein patients are also at risk for atrial arrhythmias, due to the atrial enlargement intrinsic to the anatomy, as well as the presence of potential accessory pathways. Arrhythmias are generally poorly tolerated, particularly in the setting of ventricular dysfunction. Cyanosis may also be present in Ebstein patients, due to the common occurrence of atrial communications, which can exacerbate other symptoms of heart failure. Treatment of heart failure can be through pharmacologic and procedural interventions, depending on the underlying cause of heart failure. While early heart failure symptoms may be treated with medical management, most Ebstein patients will require surgery. Various surgical and catheter-based interventions targeting the tricuspid valve and the atrialized right ventricular tissue have been developed to help treat the underlying cause of the heart failure. The optimal timing of transcatheter and surgical intervention in the Ebstein patient to prevent or treat heart failure needs further study.

    View details for DOI 10.1007/s10741-020-09930-2

    View details for PubMedID 32472521

  • Predicting 10-year mortality in adults with congenital heart disease International Journal of Cardiology Congenital Heart Disease Fernandes, S. M., Lui, G. K., Long, J., Lin, A., Rogers, I. S., Sillman, C., Romfh, A., Dade, T., Dong, E., Haeffele, C., Scribner, C., Major, M., McElhinney, D. 2020
  • The Hidden Victims of the COVID-19 Pandemic: Congenital Heart Disease Patients. JACC. Case reports El-Saiedi, S. A., Haeffele, C. n., Hanna, B. M., Lui, G. K. 2020; 2 (9): 1411–13

    View details for DOI 10.1016/j.jaccas.2020.05.081

    View details for PubMedID 32835286

    View details for PubMedCentralID PMC7294284

  • Fontan Liver Lesions: Not Always HCC JACC: Case Reports Haeffele, C., Aggarwal, A., Lutchman, G., Veldtman, G. R., Lui, G. K. 2020; 1 (12)
  • Percutaneous Pulmonary Vein Stenting to Treat Severe Pulmonary Vein Stenosis After Surgical Reconstruction. Innovations (Philadelphia, Pa.) Dalal, A. R., Markham, R. n., Haeffele, C. n., Sharma, R. n., Watkins, A. C. 2020: 1556984520933962


    A 36-year-old female underwent left lower lobectomy with left atrial and left upper pulmonary vein (LUPV) reconstruction with a bovine pericardial patch for an intrathoracic pheochromocytoma. Postoperatively, she developed shortness of breath and transesophageal echocardiography demonstrated LUPV stenosis with increased velocities. Computed tomography angiogram of the chest revealed LUPV stenosis at the left atrium ostium with an area of 39 mm2. Under angiographic and echocardiographic guidance, a 10 × 19 mm Omnilink Elite uncovered stent was deployed in the LUPV ostia. While reported following left atrial ablation, pulmonary vein stenting can be successful in a pulmonary vein surgically reconstructed with bovine pericardium.

    View details for DOI 10.1177/1556984520933962

    View details for PubMedID 32639846

  • Fontan Liver Lesions: Not Always HCC. JACC. Case reports Haeffele, C., Aggarwal, A., Lutchman, G., Veldtman, G. R., Wu, F. M., Lui, G. K. 2019; 1 (2): 175-178


    A 24-year-old Fontan procedure patient underwent surveillance liver cardiac magnetic resonance imaging. Findings were suggestive of hepatocellular carcinoma (HCC). Currently, HCC is diagnosed based on imaging alone. Given her otherwise reassuring clinical profile, she underwent liver biopsy. Pathology demonstrated focal nodular hyperplasia. This raises concern for overdiagnosis of HCC in Fontan patients without tissue confirmation. (Level of Difficulty: Advanced.).

    View details for DOI 10.1016/j.jaccas.2019.05.031

    View details for PubMedID 34316779

    View details for PubMedCentralID PMC8301494

  • 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 ± 12.6 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 PubMedID 28247847

  • 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-?


    Over the last 50 years, improved surgical techniques and progressive medical management have allowed patients with complete or dextro-transposition of the great arteries (D-TGA) to survive into adulthood. Older adult patients underwent an atrial switch procedure (Mustard or Senning operation), whereas the younger cohort of patients with TGA has undergone the arterial switch operation (ASO). The Mustard/Senning maintains the right ventricle as the systemic ventricle, whereas the more recently adopted ASO attempts to restore normal physiologic and anatomic relationships. Neither operation is without consequence. Neither is without consequence and require long term follow up.

    View details for DOI 10.1016/j.ccl.2015.07.012

    View details for Web of Science ID 000364729200006

    View details for PubMedID 26471819

  • Receptor-selective coactivators as tools to define the biology of specific receptor-coactivator pairs MOLECULAR CELL Gaillard, S., Grasfeder, L. L., Haeffele, C. L., Lobenhofer, E. K., Chu, T., Wolfinger, R., Kazmin, D., Koves, T. R., Muoio, D. M., Chang, C., McDonnell, D. P. 2006; 24 (5): 797-803


    In the absence of specific high-affinity agonists and antagonists, it has been difficult to define the target genes and biological responses attributable to many of the orphan nuclear receptors (ONRs). Indeed, it appears that many members of this receptor superfamily are not regulated by classical small molecules but rather their activity is controlled by interacting cofactors. Motivated by this finding, we have developed an approach to genetically isolate specific receptor-cofactor pairs in cells, allowing us to define the biological responses attributable to each complex. This is accomplished by using combinatorial peptide phage display to engineer the receptor interacting domain of each cofactor such that it interacts selectively with one nuclear receptor. In this study, we describe the customization of PGC-1alpha and its use to study the biology of the estrogen-related receptor alpha (ERRalpha) in cultured liver cells.

    View details for DOI 10.1016/j.molcel.2006.10.012

    View details for Web of Science ID 000242812000015

    View details for PubMedID 17157261