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Pulmonary Hypertension: Comorbidities and Novel Therapeutics
References
  1. Cullivan S, Gaine S, Sitbon O. New trends in pulmonary hypertension. Eur Respir Rev. 2023;32(167):220211. doi:10.1183/16000617.0211-2022
  2. Mocumbi A, Humbert M, Saxena A, et al. Pulmonary hypertension [published correction appears in Nat Rev Dis Primers. 2024;10(1):5]. Nat Rev Dis Primers. 2024;10(1):1. doi:10.1038/s41572-023-00486-7
  3. Lang IM, Palazzini M. The burden of comorbidities in pulmonary arterial hypertension. Eur Heart J Suppl. 2019;21(suppl K):K21-K28. doi:10.1093/ eurheartj/suz205
  4. Yan L, Zhao Z, Zhao Q, et al. The clinical characteristics of patients with pulmonary hypertension combined with obstructive sleep apnoea. BMC Pulm Med. 2021;21(1):378. doi:10.1186/s12890-021-01755-5
  5. Hoeper MM, Badesch DB, Ghofrani HA, et al; for the STELLAR Trial Investigators. Phase 3 trial of sotatercept for treatment of pulmonary arterial hypertension. N Engl J Med. 2023;388(16):1478-1490. doi:10.1056/NEJMoa2213558
  6. Grünig E, Jansa P, Fan F, et al. Randomized trial of macitentan/tadalafil single-tablet combination therapy for pulmonary arterial hypertension. J Am Coll Cardiol. 2024;83(4):473-484. doi:10.1016/j.jacc.2023.10.045
  7. Higuchi S, Horinouchi H, Aoki T, et al. Balloon pulmonary angioplasty in the management of chronic thromboembolic pulmonary hypertension. Radiographics. 2022;42(6):1881-1896. doi:10.1148/rg.210102
Author and Disclosure Information

Mary Jo S. Farmer, MD, PhD

Associate Professor
Department of Medicine
UMASS CHAN Medical School
Worcester, MA

Physician
Pulmonary & Critical Care Division
Baystate Health
Springfield, MA


Mary Jo S. Farmer, MD, PhD, has disclosed no relevant financial relationships.

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Author and Disclosure Information

Mary Jo S. Farmer, MD, PhD

Associate Professor
Department of Medicine
UMASS CHAN Medical School
Worcester, MA

Physician
Pulmonary & Critical Care Division
Baystate Health
Springfield, MA


Mary Jo S. Farmer, MD, PhD, has disclosed no relevant financial relationships.

Author and Disclosure Information

Mary Jo S. Farmer, MD, PhD

Associate Professor
Department of Medicine
UMASS CHAN Medical School
Worcester, MA

Physician
Pulmonary & Critical Care Division
Baystate Health
Springfield, MA


Mary Jo S. Farmer, MD, PhD, has disclosed no relevant financial relationships.

References
  1. Cullivan S, Gaine S, Sitbon O. New trends in pulmonary hypertension. Eur Respir Rev. 2023;32(167):220211. doi:10.1183/16000617.0211-2022
  2. Mocumbi A, Humbert M, Saxena A, et al. Pulmonary hypertension [published correction appears in Nat Rev Dis Primers. 2024;10(1):5]. Nat Rev Dis Primers. 2024;10(1):1. doi:10.1038/s41572-023-00486-7
  3. Lang IM, Palazzini M. The burden of comorbidities in pulmonary arterial hypertension. Eur Heart J Suppl. 2019;21(suppl K):K21-K28. doi:10.1093/ eurheartj/suz205
  4. Yan L, Zhao Z, Zhao Q, et al. The clinical characteristics of patients with pulmonary hypertension combined with obstructive sleep apnoea. BMC Pulm Med. 2021;21(1):378. doi:10.1186/s12890-021-01755-5
  5. Hoeper MM, Badesch DB, Ghofrani HA, et al; for the STELLAR Trial Investigators. Phase 3 trial of sotatercept for treatment of pulmonary arterial hypertension. N Engl J Med. 2023;388(16):1478-1490. doi:10.1056/NEJMoa2213558
  6. Grünig E, Jansa P, Fan F, et al. Randomized trial of macitentan/tadalafil single-tablet combination therapy for pulmonary arterial hypertension. J Am Coll Cardiol. 2024;83(4):473-484. doi:10.1016/j.jacc.2023.10.045
  7. Higuchi S, Horinouchi H, Aoki T, et al. Balloon pulmonary angioplasty in the management of chronic thromboembolic pulmonary hypertension. Radiographics. 2022;42(6):1881-1896. doi:10.1148/rg.210102
References
  1. Cullivan S, Gaine S, Sitbon O. New trends in pulmonary hypertension. Eur Respir Rev. 2023;32(167):220211. doi:10.1183/16000617.0211-2022
  2. Mocumbi A, Humbert M, Saxena A, et al. Pulmonary hypertension [published correction appears in Nat Rev Dis Primers. 2024;10(1):5]. Nat Rev Dis Primers. 2024;10(1):1. doi:10.1038/s41572-023-00486-7
  3. Lang IM, Palazzini M. The burden of comorbidities in pulmonary arterial hypertension. Eur Heart J Suppl. 2019;21(suppl K):K21-K28. doi:10.1093/ eurheartj/suz205
  4. Yan L, Zhao Z, Zhao Q, et al. The clinical characteristics of patients with pulmonary hypertension combined with obstructive sleep apnoea. BMC Pulm Med. 2021;21(1):378. doi:10.1186/s12890-021-01755-5
  5. Hoeper MM, Badesch DB, Ghofrani HA, et al; for the STELLAR Trial Investigators. Phase 3 trial of sotatercept for treatment of pulmonary arterial hypertension. N Engl J Med. 2023;388(16):1478-1490. doi:10.1056/NEJMoa2213558
  6. Grünig E, Jansa P, Fan F, et al. Randomized trial of macitentan/tadalafil single-tablet combination therapy for pulmonary arterial hypertension. J Am Coll Cardiol. 2024;83(4):473-484. doi:10.1016/j.jacc.2023.10.045
  7. Higuchi S, Horinouchi H, Aoki T, et al. Balloon pulmonary angioplasty in the management of chronic thromboembolic pulmonary hypertension. Radiographics. 2022;42(6):1881-1896. doi:10.1148/rg.210102
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Pulmonary hypertension (PH), a disease of the pulmonary vasculature characterized by mean arterial pressure of > 20 mmHg, has high mortality and morbidity and encompasses a series of conditions.1,2 The 5 groups of PH as defined by WHO include pulmonary arterial hypertension (PAH; group 1), pulmonary hypertension with left-sided heart disease (PH-LHD; group 2), PH with chronic lung disease (group 3), PH associated with blood clots and scarring as a complication of long-term pulmonary embolism or thromboembolic disease (CTEPH; group 4), and multifactorial or unclear mechanism PH (group 5).2 Many comorbidities predispose patients to PH and interact with disease features, such as congenital heart disease, chronic lung disease, interstitial lung disease, COPD, endemic infections, systemic hypertension, diabetes, coronary artery disease, and sleep apnea.2,3 For example, features of sleep apnea interact with PH, changing clinical sleep breathing measures.4

Efforts in recent years have focused on improving disease management and developing treatment options that target novel pathways, especially so in PAH.1,5,6 A recently approved treatment, sotatercept, is a novel fusion protein that attempts to restore balance between growth-promoting and growth-inhibiting signaling pathways in PAH. Sotatercept has been shown to improve 6-minute walk distance, a measure of aerobic capacity.5 Established PAH oral therapies are now available in single-tablet combinations (macitentan/tadalafil), which have demonstrated improvements in pulmonary vascular resistance compared with either medicine alone.6 For chronic thromboembolic disease, treatment approaches such as balloon pulmonary angioplasty are being used to improve cardiopulmonary outcomes and are constantly advancing.7 These new treatments provide additional options in management of the various manifestations of this chronic disease.

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