Specializing In
- Hereditary Hemorrhagic Telangiectasia (HHT)
- Pulmonary Hypertension
Professional Summary
Education & Certifications
- Fellowship: Stanford University Pulmonary and Critical Care Fellowship (2009) CA
- Fellowship: Stanford University (2008) CA
- Fellowship: Lucile Packard Children's Hospital (2006) CA
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- Residency: Medizinische Hochschule Hannover (2002) Germany
- Medical Education: University Hospital Freiburg (1995) Germany
Honors & Awards
- Helmholtz International Research Group Award, Helmholtz Zentrum Muenchen, Germany (2013-2016)
- High-Potential Women Leaders Program, Stanford University Graduate School of Business (Jan-Feb 2022)
- K08 Career development award, NIH (2011-2016)
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- Manuscript Award, Cardiovascular Institute at Stanford (Feb 2014)
- Mid-Career Women Faculty Leadership Seminar (MidWIMS), Association of American Medical Colleges (Dec 2022)
- Peer Reviewed Medical Investigator-Initiated Research Award: Arteriovenous Malformations, Department of Defense (2019 -2022)
- Peer Reviewed Medical Investigator-Initiated Research Award: BMPR2 and RV Function in CHD, Department of Defense (2017 - 2020)
- Postdoctoral Research Fellowship, Pulmonary Hypertension Association (2003-2005)
- Poster Award, Excellence Cluster Cardio-Pulmonary System (ECCPS) and Pulmonary Vascular Research Institute (PVRI) (Jan 2014)
- R01 - Targeting Novel BMPR2 modifiers in Pulmonary Hypertension with Repurposed Drugs, NIH / NHLBI (2016 - 2021)
- R01 - Understanding and targeting right ventricular adaptation and failure, NIH / NHLBI (2021 - 2025)
- Seed Grant - Phase II Clinical Trial, SPARK and Spectrum Stanford (2012-2014)
- Seed Grant - Phase II Clinical Trial, Wall Center of Pulmonary Vascular Disease (2012-2014)
- Seed Grant - Small Molecule High Throughout Screen, Wall Center of Pulmonary Vascular Disease (2012)
- Seed Grant- BMP signaling in the RV, Cardiovascular Institute Stanford (2013-2014)
- Supplemental award of the Pulmonary Hypertension Association (PHA), Pulmonary Hypertension Association (2011 - 2016)
- Winner of Poster competition, Cardiovascular Institute Stanford (Sept 2012)
- Young Physician Scientist Award, American Society of Clinical Investigation (April 2015)
Memberships
- Chair, 3CPR Early Career Committee, American Heart Association (2021 - 2023)
- Member of Cure HHT Research Network _ CZI Rare as ONE, Cure HHT.org (2022 - Present)
- Nominating Committee, American Thoracic Society (2020 - 2021)
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- Pulmonary Circulation Program Committee, American Thoracic Society (2013 - 2019)
- Member, Pulmonary Vascular Research Institute (2014 - Present)
- Member at large, European Respiratory Society (2000 - Present)
- Member at large, American Thoracic Society (1998 - Present)
Administrative Appointments
- Director, HHT Center of Excellence, Stanford University (2019 - Present)
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Publications
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Long-term treatment of primary pulmonary hypertension with aerosolized iloprost, a prostacyclin analogue.
Hoeper, M. M., Schwarze, M., Ehlerding, S., Adler-Schuermeyer, A., Spiekerkoetter, E., Niedermeyer, J., … Fabel, H. (2000). Long-term treatment of primary pulmonary hypertension with aerosolized iloprost, a prostacyclin analogue. NEW ENGLAND JOURNAL OF MEDICINE, 342(25), 1866–1870. -
[Long-term treatment of primary pulmonary hypertension with inhaled iloprost].
Hoeper, M. M., Schwarze, M., Ehlerding, S., Adler-Schuermeyer, A., Spiekerkoetter, E., Niedermeyer, J., … Fabel, H. (2001). [Long-term treatment of primary pulmonary hypertension with inhaled iloprost]. Pneumologie (Stuttgart, Germany), 55(1), 38–43. -
Intravenous iloprost for treatment failure of aerosolised iloprost in pulmonary arterial hypertension
Hoeper, M. M., Spiekerkoetter, E., Westerkamp, V., Gatzke, R., & Fabel, H. (2002). Intravenous iloprost for treatment failure of aerosolised iloprost in pulmonary arterial hypertension. EUROPEAN RESPIRATORY JOURNAL, 20(2), 339–343. -
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Effects of inhaled salbutamol in primary pulmonary hypertension
Spiekerkoetter, E., Fabel, H., & Hoeper, M. M. (2002). Effects of inhaled salbutamol in primary pulmonary hypertension. EUROPEAN RESPIRATORY JOURNAL, 20(3), 524–528. -
Bosentan treatment in patients with primary pulmonary hypertension receiving nonparenteral prostanoids
Hoeper, M. M., Taha, N., Bekjarova, A., Gatzke, R., & Spiekerkoetter, E. (2003). Bosentan treatment in patients with primary pulmonary hypertension receiving nonparenteral prostanoids. EUROPEAN RESPIRATORY JOURNAL, 22(2), 330–334. -
Interdependent serotonin transporter and receptor pathways regulate S100A4/Mts1, a gene associated with pulmonary vascular disease
Lawrie, A., Spiekerkoetter, E., Martinez, E. C., Ambartsumian, N., Sheward, W. J., MacLean, M. R., … Rabinovitch, M. (2005). Interdependent serotonin transporter and receptor pathways regulate S100A4/Mts1, a gene associated with pulmonary vascular disease. CIRCULATION RESEARCH, 97(3), 227–35. -
Increased fibulin-5 and elastin in S100A4/Mts1 mice with pulmonary hypertension
Merklinger, S. L., Wagner, R. A., Spiekerkoetter, E., Hinek, A., Knutsen, R. H., Kabir, M. G., … Rabinovitch, M. (2005). Increased fibulin-5 and elastin in S100A4/Mts1 mice with pulmonary hypertension. CIRCULATION RESEARCH, 97(6), 596–604. -
Goal-oriented treatment and combination therapy for pulmonary arterial hypertension
Hoeper, M. M., Markevych, I., Spiekerkoetter, E., Welte, T., & Niedermeyer, J. (2005). Goal-oriented treatment and combination therapy for pulmonary arterial hypertension. EUROPEAN RESPIRATORY JOURNAL, 26(5), 858–863. -
Mts1/S100A4 stimulates human pulmonary artery smooth muscle cell migration through multiple signaling pathways
Spiekerkoetter, E., Lawrie, A., Merklinger, S., Ambartsumian, N., Lukanidin, D., Schmidt, A. A., & Rabbiovitch, M. (2005). Mts1/S100A4 stimulates human pulmonary artery smooth muscle cell migration through multiple signaling pathways. CHEST, 128(6), 577S–577S. -
Experience with inhaled iloprost and bosentan in portopulmonary hypertension
Hoeper, M. M., Seyfarth, H. J., Hoefflken, G., Wirtz, H., Spielkerkoetter, E., Pletz, M. W., … Halank, M. (2007). Experience with inhaled iloprost and bosentan in portopulmonary hypertension. EUROPEAN RESPIRATORY JOURNAL, 30(6), 1096–1102. -
Reactivation of gamma HV68 induces neointimal lesions in pulmonary arteries of S100A4/Mts1-overexpressing mice in association with degradation of elastin
Spiekerkoetter, E., Alvira, C. M., Kim, Y.-M., Bruneau, A., Pricola, K. L., Wang, L., … Rabinovitch, M. (2008). Reactivation of gamma HV68 induces neointimal lesions in pulmonary arteries of S100A4/Mts1-overexpressing mice in association with degradation of elastin. AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY, 294(2), L276–L289. -
S100A4 and Bone Morphogenetic Protein-2 Codependently Induce Vascular Smooth Muscle Cell Migration via Phospho-Extracellular Signal-Regulated Kinase and Chloride Intracellular Channel 4
Spiekerkoetter, E., Guignabert, C., Perez, V. D. J., Alastalo, T.-P., Powers, J. M., Wang, L., … Rabinovitch, M. (2009). S100A4 and Bone Morphogenetic Protein-2 Codependently Induce Vascular Smooth Muscle Cell Migration via Phospho-Extracellular Signal-Regulated Kinase and Chloride Intracellular Channel 4. CIRCULATION RESEARCH, 105(7), 639–U37. -
BMP promotes motility and represses growth of smooth muscle cells by activation of tandem Wnt pathways
Perez, V. A. J., Ali, Z., Alastalo, T.-P., Ikeno, F., Sawada, H., Lai, Y.-J., … Rabinovitch, M. (2011). BMP promotes motility and represses growth of smooth muscle cells by activation of tandem Wnt pathways. JOURNAL OF CELL BIOLOGY, 192(1), 171–88. -
Neutrophil Elastase Is Produced by Pulmonary Artery Smooth Muscle Cells and Is Linked to Neointimal Lesions
Kim, Y.-M., Haghighat, L., Spiekerkoetter, E., Sawada, H., Alvira, C. M., Wang, L., … Rabinovitch, M. (2011). Neutrophil Elastase Is Produced by Pulmonary Artery Smooth Muscle Cells and Is Linked to Neointimal Lesions. AMERICAN JOURNAL OF PATHOLOGY, 179(3), 1560–72. -
Safety and efficacy of transition from systemic prostanoids to inhaled treprostinil in pulmonary arterial hypertension.
de Jesus Perez, V. A., Rosenzweig, E., Rubin, L. J., Poch, D., Bajwa, A., Park, M., … Zamanian, R. T. (2012). Safety and efficacy of transition from systemic prostanoids to inhaled treprostinil in pulmonary arterial hypertension. American Journal of Cardiology, 110(10), 1546–50. -
Loss of adenomatous poliposis coli-a3 integrin interaction promotes endothelial apoptosis in mice and humans.
de Jesus Perez, V. A., Yuan, K., Orcholski, M. E., Sawada, H., Zhao, M., Li, C. G., … Rabinovitch, M. (2012). Loss of adenomatous poliposis coli-a3 integrin interaction promotes endothelial apoptosis in mice and humans. Circulation Research, 111(12), 1551–64. -
Prevalence of malignancies after lung transplantation
Spiekerkoetter, E., Krug, N., Hoeper, M., Wiebe, K., Hamm, M., Harringer, W., … Fabel, H. (1998). Prevalence of malignancies after lung transplantation. TRANSPLANTATION PROCEEDINGS, 30(4), 1523–1524. -
Reduced BMPR2 Increases GM-CSF mRNA Translation by Inhibiting eIF2 alpha Mediated Stress Granule Formation and Propensity to Pulmonary Vascular Disease
Sawada, H., Alastalo, T.-P., Glotzbach, J. P., Chan, R., Fuchs, G., Januszyk, M., … Rabinovitch, M. (2011). Reduced BMPR2 Increases GM-CSF mRNA Translation by Inhibiting eIF2 alpha Mediated Stress Granule Formation and Propensity to Pulmonary Vascular Disease. CIRCULATION. ORLANDO,FL,FL,FL: LIPPINCOTT WILLIAMS & WILKINS. -
Chloride intracellular channel 4 (CLIC4), a novel downstream target of bone morphogenetic protein receptor II (BMPR-II) in human pulmonary artery smooth muscle cell (PASMC) migration
Spiekerkoetter, E., Wang, L. L., Zamanian, R., Ambartsumian, N., Schmidt, A. M., Lukanidin, E., & RABINOVITCH, M. (2005). Chloride intracellular channel 4 (CLIC4), a novel downstream target of bone morphogenetic protein receptor II (BMPR-II) in human pulmonary artery smooth muscle cell (PASMC) migration. CIRCULATION. DALLAS,TX,TX,TX,TX: LIPPINCOTT WILLIAMS & WILKINS. -
FK506 activates BMPR2, rescues endothelial dysfunction, and reverses pulmonary hypertension.
Spiekerkoetter, E., Tian, X., Cai, J., Hopper, R. K., Sudheendra, D., Li, C. G., … Rabinovitch, M. (2013). FK506 activates BMPR2, rescues endothelial dysfunction, and reverses pulmonary hypertension. Journal of Clinical Investigation, 123(8), 3600–3613. -
A case of recurrent pericardial constriction presenting with severe pulmonary hypertension.
Brunner, N. W., Ramachandran, K., Kudelko, K. T., Sung, Y. K., Spiekerkoetter, E., Yang, P. C., … Perez, V. deJ. (2013). A case of recurrent pericardial constriction presenting with severe pulmonary hypertension. Pulmonary Circulation, 3(2), 436–39. -
Reduced BMPR2 expression induces GM-CSF translation and macrophage recruitment in humans and mice to exacerbate pulmonary hypertension.
Sawada, H., Saito, T., Nickel, N. P., Alastalo, T.-P., Glotzbach, J. P., Chan, R., … Rabinovitch, M. (2014). Reduced BMPR2 expression induces GM-CSF translation and macrophage recruitment in humans and mice to exacerbate pulmonary hypertension. Journal of Experimental Medicine, 211(2), 263–80. -
Targeting the Wnt signaling pathways in pulmonary arterial hypertension.
de Jesus Perez, V., Yuan, K., Alastalo, T.-P., Spiekerkoetter, E., & Rabinovitch, M. (2014). Targeting the Wnt signaling pathways in pulmonary arterial hypertension. Drug Discovery Today, 19(8), 1270–76. -
Current Clinical Management of Pulmonary Arterial Hypertension
Zamanian, R. T., Kudelko, K. T., Sung, Y. K., Perez, V. D. J., Liu, J., & Spiekerkoetter, E. (2014). Current Clinical Management of Pulmonary Arterial Hypertension. CIRCULATION RESEARCH, 115(1), 131–47. -
Hedgehog Signaling Restrains Bladder Cancer Progression by Eliciting Stromal Production of Urothelial Differentiation Factors
Shin, K., Lim, A., Zhao, C., Sahoo, D., Pan, Y., Spiekerkoetter, E., … Beachy, P. A. (2014). Hedgehog Signaling Restrains Bladder Cancer Progression by Eliciting Stromal Production of Urothelial Differentiation Factors. CANCER CELL, 26(4), 521–33. -
Elafin Reverses Pulmonary Hypertension via Caveolin-1-Dependent Bone Morphogenetic Protein Signaling
Nickel, N. P., Spiekerkoetter, E., Gu, M., Li, C. G., Li, H., Kaschwich, M., … Rabinovitch, M. (2015). Elafin Reverses Pulmonary Hypertension via Caveolin-1-Dependent Bone Morphogenetic Protein Signaling. AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE, 191(11), 1273–86. -
RNA Sequencing Analysis Detection of a Novel Pathway of Endothelial Dysfunction in Pulmonary Arterial Hypertension
Rhodes, C. J., Im, H., Cao, A., Hennigs, J. K., Wang, L., Sa, S., … Rabinovitch, M. (2015). RNA Sequencing Analysis Detection of a Novel Pathway of Endothelial Dysfunction in Pulmonary Arterial Hypertension. AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE, 192(3), 356–66. -
Low-Dose FK506 (Tacrolimus) in End-Stage Pulmonary Arterial Hypertension.
Spiekerkoetter, E., Sung, Y. K., Sudheendra, D., Bill, M., Aldred, M. A., van de Veerdonk, M. C., … Zamanian, R. T. (2015). Low-Dose FK506 (Tacrolimus) in End-Stage Pulmonary Arterial Hypertension. American Journal of Respiratory and Critical Care Medicine, 192(2), 254–57. -
Safety and Efficacy of Transition from Systemic Prostanoids to Inhaled Treprostinil in Pulmonary Arterial Hypertension
Perez, V. A. J., Rosenzweig, E., Rubin, L. J., Poch, D., Bajwa, A., Park, M., … Zamanian, R. T. (2012). Safety and Efficacy of Transition from Systemic Prostanoids to Inhaled Treprostinil in Pulmonary Arterial Hypertension. AMERICAN JOURNAL OF CARDIOLOGY, 110(10), 1546–50. -
A case of recurrent pericardial constriction presenting with severe pulmonary hypertension
Brunner, N. W., Ramachandran, K., Kudelko, K. T., Sung, Y. K., Spiekerkoetter, E., Yang, P. C., … Perez, V. de J. (2013). A case of recurrent pericardial constriction presenting with severe pulmonary hypertension. PULMONARY CIRCULATION, 3(2), 436–39. -
Career Development of Young Physician-Scientists in the Cardiovascular Sciences: Perspective and Advice From the Early Career Committee of the Cardiopulmonary, Critical Care, and Resuscitation Council of the American Heart Association.
Agarwal, S., Spiekerkoetter, E., Austin, E. D., de Jesus Perez, V., Dezfulian, C., Maron, B. A., … Perman, S. M. (2018). Career Development of Young Physician-Scientists in the Cardiovascular Sciences: Perspective and Advice From the Early Career Committee of the Cardiopulmonary, Critical Care, and Resuscitation Council of the American Heart Association. Circulation Research, 122(10), 1330–33. -
Randomised placebo-controlled safety and tolerability trial of FK506 (tacrolimus) for pulmonary arterial hypertension
Spiekerkoetter, E., Sung, Y. K., Sudheendra, D., Scott, V., Del Rosario, P., Bill, M., … Zamanian, R. T. (2017). Randomised placebo-controlled safety and tolerability trial of FK506 (tacrolimus) for pulmonary arterial hypertension. EUROPEAN RESPIRATORY JOURNAL, 50(3). -
A Pro - Con debate: Current Controversies in PAH Pathogenesis at the American Thoracic Society International Meeting in 2017.
Kuebler, W. M., Nicolls, M. R., Olschewski, A., Abe, K., Rabinovitch, M., Stewart, D. J., … Spiekerkoetter, E. (2018). A Pro - Con debate: Current Controversies in PAH Pathogenesis at the American Thoracic Society International Meeting in 2017. American Journal of Physiology. Lung Cellular and Molecular Physiology. -
Update in Pulmonary Vascular Disease 2016 and 2017
Brittain, E. L., Thennapan, T., Maron, B. A., Chan, S. Y., Austin, E. D., Spiekerkoetter, E., … Yu, P. B. (2018). Update in Pulmonary Vascular Disease 2016 and 2017. AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE, 198(1), 13–23. -
Fragile Histidine Triad (FHIT), a Novel Modifier Gene in Pulmonary Arterial Hypertension.
Dannewitz Prosseda, S., Tian, X., Kuramoto, K., Boehm, M., Sudheendra, D., Miyagawa, K., … Spiekerkoetter, E. (2018). Fragile Histidine Triad (FHIT), a Novel Modifier Gene in Pulmonary Arterial Hypertension. American Journal of Respiratory and Critical Care Medicine. -
Consequences of BMPR2 Deficiency in the Pulmonary Vasculature and Beyond: Contributions to Pulmonary Arterial Hypertension.
Andruska, A., & Spiekerkoetter, E. (2018). Consequences of BMPR2 Deficiency in the Pulmonary Vasculature and Beyond: Contributions to Pulmonary Arterial Hypertension. International Journal of Molecular Sciences, 19(9). -
New and Emerging Therapies for Pulmonary Arterial Hypertension
Spiekerkoetter, E., Kawut, S., & de Jesus Perez, V. (2018). New and Emerging Therapies for Pulmonary Arterial Hypertension. Annual Reviews in Medicine. -
Exome data clouds the pathogenicity of genetic variants in Pulmonary Arterial Hypertension
Abbasi, Y., Jabbari, J., Jabbari, R., Glinge, C., Izadyar, S. B., Spiekerkoetter, E., … Tfelt-Hansen, J. (2018). Exome data clouds the pathogenicity of genetic variants in Pulmonary Arterial Hypertension. MOLECULAR GENETICS & GENOMIC MEDICINE, 6(5), 835–44. -
EXPRESS: Drug Repositioning in Pulmonary Arterial Hypertension: Challenges and Opportunities.
Grinnan, D., Trankle, C., Andruska, A., Bloom, B., & Spiekerkoetter, E. F. (2019). EXPRESS: Drug Repositioning in Pulmonary Arterial Hypertension: Challenges and Opportunities. Pulmonary Circulation, 2045894019832226. -
Current clinical management of pulmonary arterial hypertension.
Zamanian, R. T., Kudelko, K. T., Sung, Y. K., de Jesus Perez, V., Liu, J., & Spiekerkoetter, E. (2014). Current clinical management of pulmonary arterial hypertension. Circulation Research, 115(1), 131–47. -
New and Emerging Therapies for Pulmonary Arterial Hypertension.
Spiekerkoetter, E., Kawut, S. M., & de Jesus Perez, V. A. (2019). New and Emerging Therapies for Pulmonary Arterial Hypertension. Annual Review of Medicine, 70, 45–59. -
EXPRESS: Myocardial Bridge - An Unrecognized Cause of Chest Pain in Pulmonary Arterial Hypertension.
Rajmohan, D., Sung, Y. K., Kudelko, K., Perez, V. inicio A., Haddad, F., Tremmel, J., … Spiekerkoetter, E. F. (2019). EXPRESS: Myocardial Bridge - An Unrecognized Cause of Chest Pain in Pulmonary Arterial Hypertension. Pulmonary Circulation, 2045894019860738. -
Delineating the molecular and histological events that govern right ventricular recovery using a novel mouse model of PA de-banding.
Boehm, M., Tian, X., Mao, Y., Ichimura, K., Dufva, M. J., Ali, K., … Spiekerkoetter, E. (2019). Delineating the molecular and histological events that govern right ventricular recovery using a novel mouse model of PA de-banding. Cardiovascular Research. -
Hot topics in the mechanisms of pulmonary arterial hypertension disease: cancer-like pathobiology, the role of the adventitia, systemic involvement, and right ventricular failure.
Spiekerkoetter, E., Goncharova, E. A., Guignabert, C., Stenmark, K., Kwapiszewska, G., Rabinovitch, M., … Kuebler, W. M. (2019). Hot topics in the mechanisms of pulmonary arterial hypertension disease: cancer-like pathobiology, the role of the adventitia, systemic involvement, and right ventricular failure. Pulmonary Circulation, 9(4), 2045894019889775. -
The Hallmarks of Severe Pulmonary Arterial Hypertension: The Cancer Hypothesis - Ten years later.
Cool, C. D., Kuebler, W. M., Bogaard, H. J., Spiekerkoetter, E., Nicolls, M. R., & Voelkel, N. F. (2020). The Hallmarks of Severe Pulmonary Arterial Hypertension: The Cancer Hypothesis - Ten years later. American Journal of Physiology. Lung Cellular and Molecular Physiology. -
Targeting BMPR2 Trafficking with Chaperones - An Important Step Towards Precision Medicine in Pulmonary Arterial Hypertension.
Andruska, A., Ali, K., & Spiekerkoetter, E. (2020). Targeting BMPR2 Trafficking with Chaperones - An Important Step Towards Precision Medicine in Pulmonary Arterial Hypertension. American Journal of Respiratory Cell and Molecular Biology. -
The left ventricle undergoes biomechanical and gene expression changes in response to increased right ventricular pressure overload.
Kheyfets, V. O., Dufva, M. J., Boehm, M., Tian, X., Qin, X., Tabakh, J. E., … Spiekerkoetter, E. (2020). The left ventricle undergoes biomechanical and gene expression changes in response to increased right ventricular pressure overload. Physiological Reports, 8(9), e14347. -
A Notch3-Marked Subpopulation of Vascular Smooth Muscle Cells is the Cell of Origin for Occlusive Pulmonary Vascular Lesions.
Steffes, L. C., Froistad, A. A., Andruska, A., Boehm, M., McGlynn, M., Zhang, F., … Kumar, M. E. (2020). A Notch3-Marked Subpopulation of Vascular Smooth Muscle Cells is the Cell of Origin for Occlusive Pulmonary Vascular Lesions. Circulation. -
Repurposing of medications for pulmonary arterial hypertension.
Toshner, M., Spiekerkoetter, E., Bogaard, H., Hansmann, G., Nikkho, S., & Prins, K. W. (2020). Repurposing of medications for pulmonary arterial hypertension. Pulmonary Circulation, 10(4), 2045894020941494. -
Targeted Proteomics of Right Heart Adaptation to Pulmonary Arterial Hypertension.
Amsallem, M., Sweatt, A. J., Arthur Ataam, J., Guihaire, J., Lecerf, F., Lambert, M., … Zamanian, R. T. (2020). Targeted Proteomics of Right Heart Adaptation to Pulmonary Arterial Hypertension. The European Respiratory Journal. -
Novel Advances in Modifying BMPR2 Signaling in PAH.
Dannewitz Prosseda, S., Ali, M. K., & Spiekerkoetter, E. (2020). Novel Advances in Modifying BMPR2 Signaling in PAH. Genes, 12(1). -
Comparative analysis on the anti-inflammatory/immune effect of mesenchymal stem cell therapy for the treatment of pulmonary arterial hypertension.
Oh, S., Jang, A. Y., Chae, S., Choi, S., Moon, J., Kim, M., … Chung, W.-J. J. (2021). Comparative analysis on the anti-inflammatory/immune effect of mesenchymal stem cell therapy for the treatment of pulmonary arterial hypertension. Scientific Reports, 11(1), 2012. -
Improving Right Ventricular Function by Increasing BMP Signaling with FK506.
Boehm, M., Tian, X., Ali, M. K., Mao, Y., Ichimura, K., Zhao, M., … Spiekerkoetter, E. (2021). Improving Right Ventricular Function by Increasing BMP Signaling with FK506. American Journal of Respiratory Cell and Molecular Biology. -
FHIT, a Novel Modifier Gene in Pulmonary Arterial Hypertension
Prosseda, S. D., Tian, X., Kuramoto, K., Boehm, M., Sudheendra, D., Miyagawa, K., … Spiekerkoetter, E. (2019). FHIT, a Novel Modifier Gene in Pulmonary Arterial Hypertension. AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE, 199(1), 83–98. -
Pulmonary arterial banding in mice may be a suitable model for studies on ventricular mechanics in pediatric pulmonary arterial hypertension.
Dufva, M. J., Boehm, M., Ichimura, K., Truong, U., Qin, X., Tabakh, J., … Kheyfets, V. O. (2021). Pulmonary arterial banding in mice may be a suitable model for studies on ventricular mechanics in pediatric pulmonary arterial hypertension. Journal of Cardiovascular Magnetic Resonance : Official Journal of the Society for Cardiovascular Magnetic Resonance, 23(1), 66. -
Severe Pulmonary Arterial Hypertension is Characterized by Increased Neutrophil Elastase and Relative Elafin Deficiency.
Sweatt, A. J., Miyagawa, K., Rhodes, C. J., Taylor, S., Del Rosario, P. A., Hsi, A., … Zamanian, R. T. (2021). Severe Pulmonary Arterial Hypertension is Characterized by Increased Neutrophil Elastase and Relative Elafin Deficiency. Chest. -
Promising therapeutic approaches in pulmonary arterial hypertension.
Ali, M. K., Ichimura, K., & Spiekerkoetter, E. (2021). Promising therapeutic approaches in pulmonary arterial hypertension. Current Opinion in Pharmacology, 59, 127–139. -
Arteriovenous Malformations-Current Understanding of the Pathogenesis with Implications for Treatment.
Schimmel, K., Ali, M. K., Tan, S. Y., Teng, J., Do, H. M., Steinberg, G. K., … Spiekerkoetter, E. (2021). Arteriovenous Malformations-Current Understanding of the Pathogenesis with Implications for Treatment. International Journal of Molecular Sciences, 22(16). -
Role of Long Non-Coding RNAs in Pulmonary Arterial Hypertension.
Han, Y., Ali, M. K., Dua, K., Spiekerkoetter, E., & Mao, Y. (2021). Role of Long Non-Coding RNAs in Pulmonary Arterial Hypertension. Cells, 10(8). -
Bone morphogenetic protein 2 (BMP 2) and wingless 3a (Wnt 3a) stimulate human pulmonary artery smooth muscle cell (hPASMC) motility via activation of a Rho and Rac dependent non-canonical Wnt pathway
de Jesus Perez, V. A., Hansmann, G., Spiekerkoetter, E., & Rabinovitch, M. (2006). Bone morphogenetic protein 2 (BMP 2) and wingless 3a (Wnt 3a) stimulate human pulmonary artery smooth muscle cell (hPASMC) motility via activation of a Rho and Rac dependent non-canonical Wnt pathway. CIRCULATION. LIPPINCOTT WILLIAMS & WILKINS. -
Shunt-type plexiform lesions identified in the Sugen5416/Hypoxia rat model of pulmonary arterial hypertension using SPCT.
van der Have, O., Westoo, C., Ahrne, F., Tian, X., Ichimura, K., Dreier, T., … Tran-Lundmark, K. (2022). Shunt-type plexiform lesions identified in the Sugen5416/Hypoxia rat model of pulmonary arterial hypertension using SPCT. The European Respiratory Journal. -
Relationship between impaired BMP signalling and clinical risk factors at early-stage vascular injury in the preterm infant.
Heydarian, M., Oak, P., Zhang, X., Kamgari, N., Kindt, A., Koschlig, M., … Hilgendorff, A. (2022). Relationship between impaired BMP signalling and clinical risk factors at early-stage vascular injury in the preterm infant. Thorax. -
Cardiac Fibrosis in the Pressure Overloaded Left and Right Ventricle as a Therapeutic Target.
Schimmel, K., Ichimura, K., Reddy, S., Haddad, F., & Spiekerkoetter, E. (2022). Cardiac Fibrosis in the Pressure Overloaded Left and Right Ventricle as a Therapeutic Target. Frontiers in Cardiovascular Medicine, 9, 886553. -
The role of circular RNAs in pulmonary hypertension.
Ali, M. K., Schimmel, K., Zhao, L., Chen, C.-K. K., Dua, K., Nicolls, M. R., & Spiekerkoetter, E. (2022). The role of circular RNAs in pulmonary hypertension. The European Respiratory Journal. -
The Human Respiratory Microbiome: Current Understandings and Future Directions.
Zhao, L., Luo, J.-L. L., Ali, M. K., Spiekerkoetter, E., & Nicolls, M. R. (2022). The Human Respiratory Microbiome: Current Understandings and Future Directions. American Journal of Respiratory Cell and Molecular Biology. -
Combined environmental stimuli, d-fenfluramine and hypoxia, induce severe pulmonary hypertension in S100A4/Mts1-overexpressing mice that are prone to develop pulmonary vascular obliterative disease (PVOD)
Spiekerkoetter, E., Merklinger, S. L., Lawrie, A., Ambartsumian, N., Lukanidin, E., & Rabinovitch, M. (2004). Combined environmental stimuli, d-fenfluramine and hypoxia, induce severe pulmonary hypertension in S100A4/Mts1-overexpressing mice that are prone to develop pulmonary vascular obliterative disease (PVOD). CIRCULATION. LIPPINCOTT WILLIAMS & WILKINS. -
Drug repositioning in pulmonary arterial hypertension: challenges and opportunities
Grinnan, D., Trankle, C., Andruska, A., Bloom, B., & Spiekerkoetter, E. (2019). Drug repositioning in pulmonary arterial hypertension: challenges and opportunities. PULMONARY CIRCULATION, 9(1). -
Consequences of BMPR2 Deficiency in the Pulmonary Vasculature and Beyond: Contributions to Pulmonary Arterial Hypertension
Andruska, A., & Spiekerkoetter, E. (2018). Consequences of BMPR2 Deficiency in the Pulmonary Vasculature and Beyond: Contributions to Pulmonary Arterial Hypertension. INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES, 19(9). -
PTPN1 Deficiency Modulates BMPR2 Signaling and Induces Endothelial Dysfunction in Pulmonary Arterial Hypertension.
Ali, M. K., Tian, X., Zhao, L., Schimmel, K., Rhodes, C. J., Wilkins, M. R., … Spiekerkoetter, E. F. (2023). PTPN1 Deficiency Modulates BMPR2 Signaling and Induces Endothelial Dysfunction in Pulmonary Arterial Hypertension. Cells, 12(2). -
Decreasing ELK3 expression improves Bone Morphogenetic Protein Receptor 2 signaling and pulmonary vascular cell function in PAH.
Ali, M. K., Zhao, L., Perez, V. de J., Nicolls, M. R., & Spiekerkoetter, E. F. (2023). Decreasing ELK3 expression improves Bone Morphogenetic Protein Receptor 2 signaling and pulmonary vascular cell function in PAH. BioRxiv : the Preprint Server for Biology. -
Repetitive Schistosoma Exposure Causes Perivascular Lung Fibrosis and Persistent Pulmonary Hypertension.
Kumar, R., Lee, M. H., Kassa, B., Fonseca Balladares, D. C., Mickael, C., Sanders, L., … Graham, B. B. (2023). Repetitive Schistosoma Exposure Causes Perivascular Lung Fibrosis and Persistent Pulmonary Hypertension. Clinical Science (London, England : 1979). -
Novel left ventricular mechanical index in pulmonary arterial hypertension.
Ichimura, K., Santana, E. J., Kuznetsova, T., Cauwenberghs, N., Sabovcik, F., Chun, L., … Haddad, F. (2023). Novel left ventricular mechanical index in pulmonary arterial hypertension. Pulmonary Circulation, 13(2), e12216. -
Characterizing the Spatiotemporal Transcriptomic Response of the Right Ventricle to Acute Pressure Overload.
Kheyfets, V. O., Kumar, S., Heerdt, P. M., Ichimura, K., Brown, R. D., Lucero, M., … Spiekerkoetter, E. (2023). Characterizing the Spatiotemporal Transcriptomic Response of the Right Ventricle to Acute Pressure Overload. International Journal of Molecular Sciences, 24(11). -
MicroRNA-34a-Dependent Attenuation of Angiogenesis in Right Ventricular Failure.
Reddy, S., Hu, D.-Q. Q., Zhao, M., Ichimura, S., Barnes, E. A., Cornfield, D. N., … Bernstein, D. (2024). MicroRNA-34a-Dependent Attenuation of Angiogenesis in Right Ventricular Failure. Journal of the American Heart Association, e029427. -
Rat microbial biogeography and age-dependent lactic acid bacteria in healthy lungs.
Zhao, L., Cunningham, C. M., Andruska, A. M., Schimmel, K., Ali, M. K., Kim, D., … Nicolls, M. R. (2024). Rat microbial biogeography and age-dependent lactic acid bacteria in healthy lungs. Lab Animal, 53(2), 43–55. -
3D Imaging Reveals Complex Microvascular Remodeling in the Right Ventricle in Pulmonary Hypertension.
Ichimura, K., Boehm, M., Andruska, A. M., Zhang, F., Schimmel, K., Bonham, S., … Spiekerkoetter, E. (2024). 3D Imaging Reveals Complex Microvascular Remodeling in the Right Ventricle in Pulmonary Hypertension. Circulation Research. -
Cardiorenal Syndrome in Right Heart Failure Due to Pulmonary Arterial Hypertension-The Right Ventricle as a Therapeutic Target to Improve Renal Function.
Ichimura, K., Gross, A., Mathew, R. O., Salman, L., Reddy, S., Spiekerkoetter, E., & Sidhu, M. S. (2024). Cardiorenal Syndrome in Right Heart Failure Due to Pulmonary Arterial Hypertension-The Right Ventricle as a Therapeutic Target to Improve Renal Function. Cardiovascular Drugs and Therapy. -
Tricuspid annular plane systolic excursion in pulmonary hypertension-Moving beyond the sector plane.
Ichimura, K., Celestin, B. E., Bagherzadeh, S. P., Zamanian, R. T., Salerno, M., Spiekerkoetter, E., & Haddad, F. (2024). Tricuspid annular plane systolic excursion in pulmonary hypertension-Moving beyond the sector plane. Pulmonary Circulation, 14(3), e12416. -
Somatic mutations in arteriovenous malformations in hereditary hemorrhagic telangiectasia support a bi-allelic two-hit mutation mechanism of pathogenesis.
DeBose-Scarlett, E., Ressler, A. K., Gallione, C. J., Sapisochin Cantis, G., Friday, C., Weinsheimer, S., … Marchuk, D. A. (2024). Somatic mutations in arteriovenous malformations in hereditary hemorrhagic telangiectasia support a bi-allelic two-hit mutation mechanism of pathogenesis. American Journal of Human Genetics. -
Increased BMP-Responsive Transcription Factors in Distinct Endothelial and Mesenchymal Cells in PAH.
Andruska, A. M., Cantu Valadez, R., Ichimura, K., Chu, P., Zhang, T., Schimmel, K., … Spiekerkoetter, E. (2024). Increased BMP-Responsive Transcription Factors in Distinct Endothelial and Mesenchymal Cells in PAH. American Journal of Respiratory and Critical Care Medicine. -
Abnormalities in Coronary Microvasculature in Pulmonary Atresia With Intact Ventricular Septum.
Vincent, A. G., Ichimura, K., Ichimura, S., Alden, A. O., Price, E., Salmi, D., … Reddy, S. (2024). Abnormalities in Coronary Microvasculature in Pulmonary Atresia With Intact Ventricular Septum. Circulation. Cardiovascular Imaging, e017415. -
Case report: A finding of PVOD and PAH in first degree relatives suggests shared heritable risk and overlapping features of both pulmonary vascular diseases.
Winters, R., Forbes, L. M., Ivy, D., Cool, C., Park, B. D., Hountras, P., … Bull, T. M. (2024). Case report: A finding of PVOD and PAH in first degree relatives suggests shared heritable risk and overlapping features of both pulmonary vascular diseases. Respirology Case Reports, 12(11), e70064. -
Protocol for efficient generation of human artery and vein endothelial cells from pluripotent stem cells.
Loh, K. M., Zheng, S. L., Liu, K. J., Yin, Q., Amir-Ugokwe, Z. A., Jha, S. K., … Ang, L. T. (2024). Protocol for efficient generation of human artery and vein endothelial cells from pluripotent stem cells. STAR Protocols, 6(1), 103494. -
Developing Benchmarks in the Diagnosis and Treatment of Pulmonary Arterial Hypertension in a Tertiary, Academic Medical Center.
Kholdani, C. A., Lee, J. H., Swenson, K. E., Liu, J., Hsi, A., Kudelko, K. T., … Sung, Y. K. (2025). Developing Benchmarks in the Diagnosis and Treatment of Pulmonary Arterial Hypertension in a Tertiary, Academic Medical Center. Pulmonary Circulation, 15(1), e70063.
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Effects of inhaled salbutamol in primary pulmonary hypertension
Clinical Trials
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Practice Locations
Hereditary Hemorrhagic Telangiectasia Center of Excellence Palo Alto, CA
Palo Alto, CA
Hereditary Hemorrhagic Telangiectasia Center of Excellence
300 Pasteur Dr
Palo Alto , CA 94305
Make An Appointment More Clinic Information Getting HereChest Clinic in Palo Alto Palo Alto, CA
Palo Alto, CA
Chest Clinic in Palo Alto
300 Pasteur Dr, Fl 1 Rm A175
Palo Alto , CA 94305
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Stanford Health Care, Stanford Health Care Tri-Valley, and Stanford Medicine Partners are each independent nonprofit organizations that are affiliated with but separate from each other and from Stanford University. The physicians who provide care at facilities operated by Stanford Health Care, Stanford Health Care Tri-Valley, and Stanford Medicine Partners are faculty, foundation, or community physicians who are not employees, representatives, or agents of Stanford Health Care, Stanford Health Care Tri- Valley, or Stanford Medicine Partners. Stanford Health Care, Stanford Health Care Tri-Valley, and Stanford Medicine Partners do not exercise control over the care provided by such faculty, foundation, and community physicians and are not responsible for their actions.
Patient Reviews
(37 reviews)
Dr Edda saved my life
Dr. Edda Spiekerkoetter was very engaged in the video call. I could tell by her notes she had worked on my account prior to the video call. She had names and referrals ready. I appreciate Dr. Spiekerkoetter contacting others in this field to get their assessments prior to the video. I understand it is not any easier for a doctor to get time with a specialist, so I know she really worked her magic to get my case in front of other specialists. Thank you so much.
She is a great listener and someone who explains everything very well. She goes above and beyond to research medical questions for her patients to provide the best care. She’s the best!
The doctor and the technicians were so professional, knowledgeable and very kind. I so appreciate the excellent care I received.
The whole team has been excellent including Dr. Spiekerkoetter
She is the best!!!
Very thorough and extremely clear in explaining the issues.
Very helpful and friendly
she explained my concerned matters very clear and friendly.
Excellent doctor! She explained everything very well.
Thanks for your services
Edda is always very thorough, caring and knowledgeable. She is always willing to assist with referals, ect. when needed. Love her!
She is outstanding!
I continue to be impressed with Dr. Spiekerkoetter's knowledge and ease in speaking to her regarding my conditions and treatment. She is very thorough and pleasant.
I highly recommend her to any and all who need this type of expertise.
Great review and through
We had a very good conversation and explanation about my health history and future plans.
very good and very nice stuff they care about there patient
She was great
Love my Doctor
Very personable and inviting, answered all my questions and invested in the Health of my wife/patient.
All very kind.
Dr. Frauke is very knowledgeable, caring and was very thorough in going over my HHT medical history.
The care provider is excellent, very knowledgeable and concerned for her patients.
One of the things I especially like about Stanford is the amount of time & engagement everyone gives. All of my physicians watch my body language & if they even think I'm not understanding they restate what was said.
Love my doctor.
We were very impressed by the doctor's knowledge into my medical history (having obviously read my file in every detail) and the depth of questions that she asked.
My daughter accompanied me she speaks English for the reason that I didn't have problems -
Nurse practitioner was very helpful and friendly. Dr. Spiekerkoetter is excellent.
Dr. Spiekerkoetter & the entire staff are all the best medical team I have ever had. Wouldn't trade them for anybody.
Dr. Spiekerkoetter - wonderful, down to earth lady.
Good experience I had. The care provider demonstrated courtesy.
Such an excellent doctor. Loved her manner of explanations & very thorough with all tests, labs & procedures.
Dr. Spiekerkoetter is a consummate professional, I trust her implicitly.
Provider gave me hope I felt at ease and I feel I'm in good hands.
Dr.S. spent 1 hr. with my mom and I answered all of our questions. Gave us the small and big picture. Very attentive to detail.
Felt we were very well informed by the doctor seeing us -
Love my care providing team at the PH Clinic. They are like family. Everyone is happy to see me... hugs all around.
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Fax: 650-320-9443
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