IMPACT OF TARGETED PULMONARY ARTERIAL HYPERTENSION THERAPY IN PATIENTS WITH COMBINED POST- AND PRE-CAPILLARY PULMONARY HYPERTENSION

Author(s):  
Nima Moghaddam ◽  
John R Swiston ◽  
Michael YC Tsang ◽  
Robert Levy ◽  
Lisa Lee ◽  
...  
2020 ◽  
Vol 10 (2) ◽  
pp. 204589402092015 ◽  
Author(s):  
John J. Ryan ◽  
Lana Melendres-Groves ◽  
Roham T. Zamanian ◽  
Ronald J. Oudiz ◽  
Murali Chakinala ◽  
...  

The COVID-19 pandemic presents many unique challenges when caring for patients with pulmonary hypertension. The COVID-19 pandemic has altered routine standard of care practice and the acute management particularly for those patients with pulmonary arterial hypertension, where pulmonary arterial hypertension-specific treatments are used. It is important to balance the ongoing care and evaluation of pulmonary arterial hypertension patients with “exposure risk” to COVID-19 for patients coming to clinic or the hospital. If there is a morbidity and mortality benefit from starting pulmonary arterial hypertension therapies, for example in a patient with high-likelihood of pulmonary arterial hypertension, then it remains important to complete the thorough evaluation. However, the COVID-19 outbreak may also represent a unique time when pulmonary hypertension experts have to weigh the risks and benefits of the diagnostic work-up including potential exposure to COVID-19 versus initiating targeted pulmonary arterial hypertension therapy in a select high-risk, high likelihood World Symposium Pulmonary Hypertension Group 1 pulmonary arterial hypertension patients. This document will highlight some of the issues facing providers, patients, and the pulmonary arterial hypertension community in real-time as the COVID-19 pandemic is evolving and is intended to share expected common clinical scenarios and best clinical practices to help the community at-large.


2017 ◽  
Vol 7 (4) ◽  
pp. 803-807 ◽  
Author(s):  
Arun Jose ◽  
Hind Rafei ◽  
Jalil Ahari

Dasatinib is a small-molecule tyrosine kinase inhibitor used in the treatment of hematological malignancies. Pulmonary arterial hypertension (PAH) is a rare but known complication. The mainstay of treatment is cessation of Dasatinib, and while clinical improvement is rapid, complete hemodynamic resolution of pulmonary hypertension (PH) still remains exceedingly uncommon. We present a case of Dasatinib-induced PAH in a woman with chronic myeloid leukemia, who demonstrated rapid and complete clinical and hemodynamic resolution following treatment with combination pulmonary vasodilator therapy using an endothelin receptor antagonist and a phosphodiesterase-5 inhibitor. This case suggests there may be an association between the use of targeted PH medication in combination and the complete resolution of dasatinib-associated PAH, but further investigation is required.


2020 ◽  
Vol 10 (3) ◽  
pp. 204589402093528 ◽  
Author(s):  
Dana Kay ◽  
Ferdous Kadri ◽  
Garrett Fitzpatrick ◽  
Hassan Alnuaimat ◽  
Raju Reddy ◽  
...  

Pulmonary arterial hypertension has been reported with a prevalence of 7.9% in patients with anti-synthetase syndrome; however, anti-synthetase syndrome associated with pulmonary veno-occlusive disease (PVOD) has never before been described in the literature. We present a novel case of anti-synthetase syndrome-associated PVOD in a patient who presented with hypoxic respiratory failure associated with right heart failure and was diagnosed with anti-synthetase syndrome based on his autoimmune serology and pre-capillary pulmonary hypertension on right heart catheterization. He was initiated on pulmonary arterial hypertension therapy, but with escalating dose of parenteral epoprostenol, experienced acute clinical worsening with chest imaging concerning for PVOD that was confirmed on autopsy. Anti-synthetase syndrome can be associated with PVOD, and it should be suspected in patients who have evidence of pre-capillary pulmonary hypertension and who deteriorate with the initiation of pulmonary hypertension-specific therapy.


2017 ◽  
Vol 47 (10) ◽  
pp. 1124-1128
Author(s):  
Avalon Moonen ◽  
Roger Garsia ◽  
Peter Youssef ◽  
Paul Torzillo ◽  
Tamera Corte ◽  
...  

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