Group V Pulmonary Hypertension
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Group V pulmonary hypertension includes sarcoidosis-associated pulmonary hypertension (SAPH), which can develop as a complication of sarcoidosis and result in lung and heart involvement. Screening for SAPH should be considered in sarcoidosis patients experiencing worsening symptoms despite treatment. While medications and procedures have shown benefits in some cases, further clinical trials are still needed to better understand how to identify and treat SAPH.
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Group V Pulmonary Hypertension
- 1. Group V Pulmonary Hypertension Talal Dahhan, MD, MMEL, CLM, cHCM, cHQA, FACP, FCCP Associate Professor of Medicine Critical Care Medicine Program Director Pulmonary Vascular Disease Center Division of Pulmonary, Allergy and Critical Care Medicine Department of Medicine Duke University
- 2. Disclosure Nothing – I wish I do
- 3. Objectives Review the new ESC Guidelines Discuss Updates in relation to SAPH Recent Updates in Myeloproliferative Diseases
- 6. Sarcoidosis … Systemic disorder Multi-organ involvement Lungs Pulmonary Hypertension Savale et al, ERJ 2022
- 7. SAPH mechanisms SAPH Savale et al, Clin Chest Med 2023
- 8. Thru RHC … Savale et al, Clin Chest Med 2023
- 9. Screening of SAPH TTE Should be considered in particular Sarcoidosis patients What clinical features ? Dyspnea despite maximum anti-inflammatory medications Right heart failure symptoms and signs Worsening symptoms overtime despite stable PFTs (FVC) 6MWD decline NT-ProBNP rise FVC/DLCO ratio > 1.6 Crouser et al, AJRCCM 2020
- 10. Echocardiography Estimated RVSP that counts a lot on TR jet velocity Probabilities: Low: TR jet < 2.8 m/s + no other signs of RV failure Intermediate: = 2.8 m/s + other signs or > 2.9 – 3.4 m/s with no other signs of RV failure High = > 3.4 m/s or > 2.9 m/s with other RV failure signs LV dysfunction signs TR jet is preferred to look at over RVSP in SAPH due to RA underestimation ! Humbert et al, ERJ 2022 Huitema et al, Sarcoidosis Vasc Diff Lung Dis 2022
- 11. RHC Must … Resting RHC numbers may not be enough What to do: Fluid challenge if LV dysfunction is suspected Vasodilatory challenge Exercise RHC Grouping and Severity Savale et al, Clin Chest Med 2023
- 12. Management Oxygenation and NIV Diuretics Smoking cessation Contraception Medications Angioplasty and Stenting Savale et al, Clin Chest Med 2023
- 13. Management Oxygenation and NIV Diuretics Smoking cessation Contraception Medications Angioplasty and Stenting Savale et al, Clin Chest Med 2023
- 15. Riociguat in SAPH Baughman et al, CHEST 2022
- 16. Bosentan in SAPH Baughman et al, CHEST 2014
- 18. Do Medications work ? 1990 – 2010 6MWD and NT-ProBNP change 95 patients FC III/IV and Stage IV Sarcoidosis Following up Pro-BNP might help reducing hospitalization Mortality 33% Parikh, Dahhan et al, Sci Rep 2019
- 19. French Data 2004 - 2015 6MWD and NT-ProBNP change 126 patients FC III/IV and Stage IV Sarcoidosis Decrease in PVR and 6MWD Mortality slowed down over last five years Boucly et al, ERJ 2017
- 20. Immunsuppressants When ? Vascular Compression by lymph nodes Fibrosing Mediastinitis PET scan seems to help identify active disease Results TBD Shloben et al, ERJ 2020 Baughman et al, Resp Med 2018
- 21. PA Angioplasty / Stenting Few Case Series Sustained Improvement in PA pressures Improved 6MWD Multidisciplinary approach in determining which stenosis Gradient across obstruction helps to determine daSilva-deAbreu A et al, Curr Probl Cardiol. 2021
- 22. Summary SAPH exists Careful identification of the group is needed Clinical trials are needed to understand more whom should benefit from treatment Thank you! :@drdahhan Email: talal.dahhan@duke.edu