Residence at moderately high altitude and its relationship with WHO Group 1 pulmonary arterial hypertension symptom severity and clinical characteristics: the Pulmonary Hypertension Association Registry

Shoaib Fakhri; Kelly Hannon; Kelly Moulden; Ryan Peterson; Peter Hountras; Todd Bull; James Maloney; Teresa De Marco; Dunbar Ivy; Thenappan Thenappan; Jeffrey S. Sager; John J. Ryan; Sula Mazimba; Russel Hirsch; Murali Chakinala; Oksana Shlobin; Matthew Lammi; Dianne Zwicke; Jeffrey Robinson; Raymond L. Benza; James Klinger; Daniel Grinnan; Stephen Mathai; David Badesch

doi:10.1177/2045894020964342

Residence at moderately high altitude and its relationship with WHO Group 1 pulmonary arterial hypertension symptom severity and clinical characteristics: the Pulmonary Hypertension Association Registry

Shoaib Fakhri, Kelly Hannon, Kelly Moulden, Ryan Peterson, Peter Hountras, Todd Bull, James Maloney, Teresa De Marco, Dunbar Ivy, Thenappan Thenappan, Jeffrey S. Sager, John J. Ryan, Sula Mazimba, Russel Hirsch, Murali Chakinala, Oksana Shlobin, Matthew Lammi, Dianne Zwicke, Jeffrey Robinson, Raymond L. BenzaJames Klinger, Daniel Grinnan, Stephen Mathai, David Badesch

Medicine - Cardiology Division

Research output: Contribution to journal › Article › peer-review

3 Scopus citations

Abstract

Background: WHO Group 1 pulmonary arterial hypertension is a progressive and potentially fatal disease. Individuals living at higher altitude are exposed to lower barometric pressure and hypobaric hypoxemia. This may result in pulmonary vasoconstriction and contribute to disease progression. We sought to examine the relationship between living at moderately high altitude and pulmonary arterial hypertension characteristics. Methods: Forty-two US centers participating in the Pulmonary Hypertension Association Registry enrolled patients who met the definition of WHO Group 1 pulmonary arterial hypertension. We utilized baseline data and patient questionnaire responses. Patients were divided into two groups: moderately high altitude residence (home ≥4000 ft) and low altitude residence (home <4000 ft) based on zip-code. Clinical characteristics, hemodynamic data, patient demographics, and patient reported quality of life metrics were compared. Results: Controlling for potential confounders (age, sex at birth, body mass index, supplemental oxygen use, race, 100-day cigarette use, alcohol use, and pulmonary arterial hypertension medication use), subjects residing at moderately high altitude had a 6-min walk distance 32 m greater than those at low altitude, despite having a pulmonary vascular resistance that was 2.2 Wood units higher. Additionally, those residing at moderately high altitude had 3.7 times greater odds of using supplemental oxygen. Conclusion: Patients with pulmonary arterial hypertension who live at moderately high altitude have a higher pulmonary vascular resistance and are more likely to need supplemental oxygen. Despite these findings, moderately high altitude Pulmonary Hypertension Association Registry patients have better functional tolerance as measured by 6-min walk distance. It is possible that a “high-altitude phenotype” of pulmonary arterial hypertension may exist. These findings warrant further study.

Original language	English (US)
Journal	Pulmonary Circulation
Volume	10
Issue number	4
DOIs	https://doi.org/10.1177/2045894020964342
State	Published - Nov 27 2020

Bibliographical note

Funding Information:
We would like to acknowledge the Pulmonary Hypertension Association (PHA) for providing the data used in this study through their registry (PHAR). The PHAR is supported by Pulmonary Hypertension Care Centers, Inc., a supporting organization of the Pulmonary Hypertension Association. The authors thank the other investigators, the staff, and particularly participants of the PHAR for their valuable contributions. A full list of participating PHAR sites and institutions can be found at www.PHAssociation.org/PHAR. Additional PHAR Investigators: Roblee Allen, MD, Sahil Bakshi, DO, Sonja Bartolome, MD, Charles D. Burger, MD, Linda M. Cadaret, MD, Michael Duncan, MD, Michael Eggert, MD, Jean M. Elwing, MD, Grant Farr, DO, Jeremy Feldman, MD, Jeff Fineman, MD, Raymond J. Foley, DO, Hubert James Ford, MD, Robert P. Frantz, MD, Anna R. Hemnes, MD, Evelyn M. Horn, MD, Steven M. Kawut, MD, MS, Peter J. Leary, MD, PhD, John W. McConnell, MD, Kishan S. Parikh, MD, Kenneth W. Presberg, MD, Amresh Raina, MD, Gautam Ramani, MD, Erika B. Rosenzweig, MD, James R. Runo, MD, Marc A. Simon, MD, MS, John W. Swisher, MD, PhD, Nidhy P. Varghese, MD, Corey E. Ventetuolo, MD, R. James White, MD, PhD, Timothy Williamson, MD, Delphine Yung, MD, Roham T. Zamanian, MD. This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Funding Information:
We would like to acknowledge the Pulmonary Hypertension Association (PHA) for providing the data used in this study through their registry (PHAR). The PHAR is supported by Pulmonary Hypertension Care Centers, Inc., a supporting organization of the Pulmonary Hypertension Association. The authors thank the other investigators, the staff, and particularly participants of the PHAR for their valuable contributions. A full list of participating PHAR sites and institutions can be found at www.PHAssociation.org/PHAR .

Publisher Copyright:
© The Author(s) 2020.

Keywords

6-min walk distance
elevation
pulmonary arterial hypertension
pulmonary vascular resistance
vascular

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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10.1177/2045894020964342

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Fakhri, S., Hannon, K., Moulden, K., Peterson, R., Hountras, P., Bull, T., Maloney, J., De Marco, T., Ivy, D., Thenappan, T., Sager, J. S., Ryan, J. J., Mazimba, S., Hirsch, R., Chakinala, M., Shlobin, O., Lammi, M., Zwicke, D., Robinson, J., ... Badesch, D. (2020). Residence at moderately high altitude and its relationship with WHO Group 1 pulmonary arterial hypertension symptom severity and clinical characteristics: the Pulmonary Hypertension Association Registry. Pulmonary Circulation, 10(4). https://doi.org/10.1177/2045894020964342

Residence at moderately high altitude and its relationship with WHO Group 1 pulmonary arterial hypertension symptom severity and clinical characteristics : the Pulmonary Hypertension Association Registry. / Fakhri, Shoaib; Hannon, Kelly; Moulden, Kelly et al.

In: Pulmonary Circulation, Vol. 10, No. 4, 27.11.2020.

Research output: Contribution to journal › Article › peer-review

Fakhri, S, Hannon, K, Moulden, K, Peterson, R, Hountras, P, Bull, T, Maloney, J, De Marco, T, Ivy, D, Thenappan, T, Sager, JS, Ryan, JJ, Mazimba, S, Hirsch, R, Chakinala, M, Shlobin, O, Lammi, M, Zwicke, D, Robinson, J, Benza, RL, Klinger, J, Grinnan, D, Mathai, S & Badesch, D 2020, 'Residence at moderately high altitude and its relationship with WHO Group 1 pulmonary arterial hypertension symptom severity and clinical characteristics: the Pulmonary Hypertension Association Registry', Pulmonary Circulation, vol. 10, no. 4. https://doi.org/10.1177/2045894020964342

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title = "Residence at moderately high altitude and its relationship with WHO Group 1 pulmonary arterial hypertension symptom severity and clinical characteristics: the Pulmonary Hypertension Association Registry",

abstract = "Background: WHO Group 1 pulmonary arterial hypertension is a progressive and potentially fatal disease. Individuals living at higher altitude are exposed to lower barometric pressure and hypobaric hypoxemia. This may result in pulmonary vasoconstriction and contribute to disease progression. We sought to examine the relationship between living at moderately high altitude and pulmonary arterial hypertension characteristics. Methods: Forty-two US centers participating in the Pulmonary Hypertension Association Registry enrolled patients who met the definition of WHO Group 1 pulmonary arterial hypertension. We utilized baseline data and patient questionnaire responses. Patients were divided into two groups: moderately high altitude residence (home ≥4000 ft) and low altitude residence (home <4000 ft) based on zip-code. Clinical characteristics, hemodynamic data, patient demographics, and patient reported quality of life metrics were compared. Results: Controlling for potential confounders (age, sex at birth, body mass index, supplemental oxygen use, race, 100-day cigarette use, alcohol use, and pulmonary arterial hypertension medication use), subjects residing at moderately high altitude had a 6-min walk distance 32 m greater than those at low altitude, despite having a pulmonary vascular resistance that was 2.2 Wood units higher. Additionally, those residing at moderately high altitude had 3.7 times greater odds of using supplemental oxygen. Conclusion: Patients with pulmonary arterial hypertension who live at moderately high altitude have a higher pulmonary vascular resistance and are more likely to need supplemental oxygen. Despite these findings, moderately high altitude Pulmonary Hypertension Association Registry patients have better functional tolerance as measured by 6-min walk distance. It is possible that a “high-altitude phenotype” of pulmonary arterial hypertension may exist. These findings warrant further study.",

keywords = "6-min walk distance, elevation, pulmonary arterial hypertension, pulmonary vascular resistance, vascular",

author = "Shoaib Fakhri and Kelly Hannon and Kelly Moulden and Ryan Peterson and Peter Hountras and Todd Bull and James Maloney and {De Marco}, Teresa and Dunbar Ivy and Thenappan Thenappan and Sager, {Jeffrey S.} and Ryan, {John J.} and Sula Mazimba and Russel Hirsch and Murali Chakinala and Oksana Shlobin and Matthew Lammi and Dianne Zwicke and Jeffrey Robinson and Benza, {Raymond L.} and James Klinger and Daniel Grinnan and Stephen Mathai and David Badesch",

note = "Funding Information: We would like to acknowledge the Pulmonary Hypertension Association (PHA) for providing the data used in this study through their registry (PHAR). The PHAR is supported by Pulmonary Hypertension Care Centers, Inc., a supporting organization of the Pulmonary Hypertension Association. The authors thank the other investigators, the staff, and particularly participants of the PHAR for their valuable contributions. A full list of participating PHAR sites and institutions can be found at www.PHAssociation.org/PHAR. Additional PHAR Investigators: Roblee Allen, MD, Sahil Bakshi, DO, Sonja Bartolome, MD, Charles D. Burger, MD, Linda M. Cadaret, MD, Michael Duncan, MD, Michael Eggert, MD, Jean M. Elwing, MD, Grant Farr, DO, Jeremy Feldman, MD, Jeff Fineman, MD, Raymond J. Foley, DO, Hubert James Ford, MD, Robert P. Frantz, MD, Anna R. Hemnes, MD, Evelyn M. Horn, MD, Steven M. Kawut, MD, MS, Peter J. Leary, MD, PhD, John W. McConnell, MD, Kishan S. Parikh, MD, Kenneth W. Presberg, MD, Amresh Raina, MD, Gautam Ramani, MD, Erika B. Rosenzweig, MD, James R. Runo, MD, Marc A. Simon, MD, MS, John W. Swisher, MD, PhD, Nidhy P. Varghese, MD, Corey E. Ventetuolo, MD, R. James White, MD, PhD, Timothy Williamson, MD, Delphine Yung, MD, Roham T. Zamanian, MD. This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors. Funding Information: We would like to acknowledge the Pulmonary Hypertension Association (PHA) for providing the data used in this study through their registry (PHAR). The PHAR is supported by Pulmonary Hypertension Care Centers, Inc., a supporting organization of the Pulmonary Hypertension Association. The authors thank the other investigators, the staff, and particularly participants of the PHAR for their valuable contributions. A full list of participating PHAR sites and institutions can be found at www.PHAssociation.org/PHAR . Publisher Copyright: {\textcopyright} The Author(s) 2020.",

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doi = "10.1177/2045894020964342",

language = "English (US)",

volume = "10",

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TY - JOUR

T1 - Residence at moderately high altitude and its relationship with WHO Group 1 pulmonary arterial hypertension symptom severity and clinical characteristics

T2 - the Pulmonary Hypertension Association Registry

AU - Fakhri, Shoaib

AU - Hannon, Kelly

AU - Moulden, Kelly

AU - Peterson, Ryan

AU - Hountras, Peter

AU - Bull, Todd

AU - Maloney, James

AU - De Marco, Teresa

AU - Ivy, Dunbar

AU - Thenappan, Thenappan

AU - Sager, Jeffrey S.

AU - Ryan, John J.

AU - Mazimba, Sula

AU - Hirsch, Russel

AU - Chakinala, Murali

AU - Shlobin, Oksana

AU - Lammi, Matthew

AU - Zwicke, Dianne

AU - Robinson, Jeffrey

AU - Benza, Raymond L.

AU - Klinger, James

AU - Grinnan, Daniel

AU - Mathai, Stephen

AU - Badesch, David

N1 - Funding Information: We would like to acknowledge the Pulmonary Hypertension Association (PHA) for providing the data used in this study through their registry (PHAR). The PHAR is supported by Pulmonary Hypertension Care Centers, Inc., a supporting organization of the Pulmonary Hypertension Association. The authors thank the other investigators, the staff, and particularly participants of the PHAR for their valuable contributions. A full list of participating PHAR sites and institutions can be found at www.PHAssociation.org/PHAR. Additional PHAR Investigators: Roblee Allen, MD, Sahil Bakshi, DO, Sonja Bartolome, MD, Charles D. Burger, MD, Linda M. Cadaret, MD, Michael Duncan, MD, Michael Eggert, MD, Jean M. Elwing, MD, Grant Farr, DO, Jeremy Feldman, MD, Jeff Fineman, MD, Raymond J. Foley, DO, Hubert James Ford, MD, Robert P. Frantz, MD, Anna R. Hemnes, MD, Evelyn M. Horn, MD, Steven M. Kawut, MD, MS, Peter J. Leary, MD, PhD, John W. McConnell, MD, Kishan S. Parikh, MD, Kenneth W. Presberg, MD, Amresh Raina, MD, Gautam Ramani, MD, Erika B. Rosenzweig, MD, James R. Runo, MD, Marc A. Simon, MD, MS, John W. Swisher, MD, PhD, Nidhy P. Varghese, MD, Corey E. Ventetuolo, MD, R. James White, MD, PhD, Timothy Williamson, MD, Delphine Yung, MD, Roham T. Zamanian, MD. This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors. Funding Information: We would like to acknowledge the Pulmonary Hypertension Association (PHA) for providing the data used in this study through their registry (PHAR). The PHAR is supported by Pulmonary Hypertension Care Centers, Inc., a supporting organization of the Pulmonary Hypertension Association. The authors thank the other investigators, the staff, and particularly participants of the PHAR for their valuable contributions. A full list of participating PHAR sites and institutions can be found at www.PHAssociation.org/PHAR . Publisher Copyright: © The Author(s) 2020.

PY - 2020/11/27

Y1 - 2020/11/27

N2 - Background: WHO Group 1 pulmonary arterial hypertension is a progressive and potentially fatal disease. Individuals living at higher altitude are exposed to lower barometric pressure and hypobaric hypoxemia. This may result in pulmonary vasoconstriction and contribute to disease progression. We sought to examine the relationship between living at moderately high altitude and pulmonary arterial hypertension characteristics. Methods: Forty-two US centers participating in the Pulmonary Hypertension Association Registry enrolled patients who met the definition of WHO Group 1 pulmonary arterial hypertension. We utilized baseline data and patient questionnaire responses. Patients were divided into two groups: moderately high altitude residence (home ≥4000 ft) and low altitude residence (home <4000 ft) based on zip-code. Clinical characteristics, hemodynamic data, patient demographics, and patient reported quality of life metrics were compared. Results: Controlling for potential confounders (age, sex at birth, body mass index, supplemental oxygen use, race, 100-day cigarette use, alcohol use, and pulmonary arterial hypertension medication use), subjects residing at moderately high altitude had a 6-min walk distance 32 m greater than those at low altitude, despite having a pulmonary vascular resistance that was 2.2 Wood units higher. Additionally, those residing at moderately high altitude had 3.7 times greater odds of using supplemental oxygen. Conclusion: Patients with pulmonary arterial hypertension who live at moderately high altitude have a higher pulmonary vascular resistance and are more likely to need supplemental oxygen. Despite these findings, moderately high altitude Pulmonary Hypertension Association Registry patients have better functional tolerance as measured by 6-min walk distance. It is possible that a “high-altitude phenotype” of pulmonary arterial hypertension may exist. These findings warrant further study.

AB - Background: WHO Group 1 pulmonary arterial hypertension is a progressive and potentially fatal disease. Individuals living at higher altitude are exposed to lower barometric pressure and hypobaric hypoxemia. This may result in pulmonary vasoconstriction and contribute to disease progression. We sought to examine the relationship between living at moderately high altitude and pulmonary arterial hypertension characteristics. Methods: Forty-two US centers participating in the Pulmonary Hypertension Association Registry enrolled patients who met the definition of WHO Group 1 pulmonary arterial hypertension. We utilized baseline data and patient questionnaire responses. Patients were divided into two groups: moderately high altitude residence (home ≥4000 ft) and low altitude residence (home <4000 ft) based on zip-code. Clinical characteristics, hemodynamic data, patient demographics, and patient reported quality of life metrics were compared. Results: Controlling for potential confounders (age, sex at birth, body mass index, supplemental oxygen use, race, 100-day cigarette use, alcohol use, and pulmonary arterial hypertension medication use), subjects residing at moderately high altitude had a 6-min walk distance 32 m greater than those at low altitude, despite having a pulmonary vascular resistance that was 2.2 Wood units higher. Additionally, those residing at moderately high altitude had 3.7 times greater odds of using supplemental oxygen. Conclusion: Patients with pulmonary arterial hypertension who live at moderately high altitude have a higher pulmonary vascular resistance and are more likely to need supplemental oxygen. Despite these findings, moderately high altitude Pulmonary Hypertension Association Registry patients have better functional tolerance as measured by 6-min walk distance. It is possible that a “high-altitude phenotype” of pulmonary arterial hypertension may exist. These findings warrant further study.

KW - 6-min walk distance

KW - elevation

KW - pulmonary arterial hypertension

KW - pulmonary vascular resistance

KW - vascular

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DO - 10.1177/2045894020964342

M3 - Article

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JO - Pulmonary Circulation

JF - Pulmonary Circulation

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ER -

Residence at moderately high altitude and its relationship with WHO Group 1 pulmonary arterial hypertension symptom severity and clinical characteristics: the Pulmonary Hypertension Association Registry

Abstract

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Keywords

UN SDGs

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