Progressive arterial occlusive disease (PAOD) and pulmonary arterial hypertension (PAH) as new adverse events of second generation TKIs in CML treatment: Who's afraid of the big bad wolf?

2012 ◽  
Vol 36 (7) ◽  
pp. 813-814 ◽  
Author(s):  
Massimo Breccia ◽  
Fabio Efficace ◽  
Giuliana Alimena
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Adverse Events ◽  
Arterial Hypertension ◽  
Second Generation ◽  
Arterial Occlusive Disease ◽  
Occlusive Disease ◽  
Pulmonary Arterial

scholarly journals Occult catheter rupture causing episodic symptoms in a patient treated with epoprostenol

2017 ◽  
Vol 8 (1) ◽  
pp. 204589321774805 ◽  
Author(s):  
Barbara L. LeVarge ◽  
Anica C. Law ◽  
Blanche Murphy
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Adverse Events ◽  
Arterial Hypertension ◽  
Long Term Outcomes ◽  
Pulmonary Arterial

Infection, thrombosis, and catheter dislodgment are well-recognized potential complications of chronic intravenous prostanoid therapy for pulmonary arterial hypertension. As long-term outcomes of pulmonary hypertension patients improve, novel adverse events are likely to arise. We describe the sudden development of unexplained hypotension and lightheadedness in a patient receiving intravenous epoprostenol for several years, ultimately determined to be due to an unusual catheter complication, not previously described in this population.

scholarly journals Long-term study of oral treprostinil to treat pulmonary arterial hypertension: dosing, tolerability, and pharmacokinetics

2020 ◽  
Vol 10 (4) ◽  
pp. 204589401986633 ◽  
Author(s):  
R. James White ◽  
Keyur Parikh ◽  
Roblee Allen ◽  
Jeremy Feldman ◽  
Carlos Jerjez-Sanchez ◽  
...  
Keyword(s):  
Pulmonary Arterial Hypertension ◽  
Adverse Events ◽  
Arterial Hypertension ◽  
Total Daily Dose ◽  
Walk Distance ◽  
Long Term Study ◽  
Pulmonary Arterial ◽  
One Year ◽  
Minute Walk Distance ◽  
Minute Walk

Oral treprostinil may be an option for low- and intermediate-risk patients with pulmonary arterial hypertension, a rare lung vascular disease. This open-label extension study collected data on participants who completed previously reported, placebo-controlled oral treprostinil studies. Eligible participants had completed the prospective parent studies and took increasing doses of oral treprostinil twice daily; some later transitioned to three times daily dosing. Investigators measured 6-minute walk distance at Month 12 as the sole efficacy measure but collected adverse events throughout the study. A single center measured pharmacokinetics in 13 subjects who changed dosing from twice daily to three times daily. Eight hundred and ninety-four participants enrolled and 71% completed one year of therapy, with a median total daily dose of 7 mg and a median 6-minute walk distance increase of 22 m (interquartile range, −14 to 67 m). Subjects achieving higher doses had larger increases in 6-minute walk distance; 42% of participants completed three years of therapy. Adverse events were typical for prostacyclin class therapy, but prostacyclin-type adverse events may have been better tolerated with three times daily dosing in 105 participants. In 13 participants transitioned to three times daily dosing with pharmacokinetic measurements before and after, trough drug levels were higher with three times daily dosing. Oral treprostinil is associated with modest but durable, dose-responsive effects on exercise tolerance for those who remained on therapy at one year in this prospective, uncontrolled study. Three times daily dosing was associated with higher trough levels and better tolerability. The recently completed Freedom-EV study will provide further insights into the utility of oral treprostinil ( https://clinicaltrials.gov/show/NCT01560624 ).

scholarly journals Multidisciplinary Team Managements and Clinical Outcomes in Patients With Pulmonary Arterial Hypertension During the Perinatal Period

2021 ◽  
Vol 8 ◽  
Author(s):  
Tingting Shu ◽  
Panpan Feng ◽  
Xiaozhu Liu ◽  
Li Wen ◽  
Huaqiao Chen ◽  
...  
Keyword(s):  
Blood Pressure ◽  
Survival Rate ◽  
Pulmonary Arterial Hypertension ◽  
Adverse Events ◽  
Arterial Hypertension ◽  
Clinical Outcomes ◽  
Multidisciplinary Team ◽  
Systolic Pressure ◽  
Perinatal Period ◽  
Pulmonary Arterial

Background: Pulmonary arterial hypertension (PAH) patients with pregnancy have high maternal mortality. This study aimed to provide clinical evidence with multidisciplinary team (MDT) management and to evaluate the clinical outcomes in PAH patients during the perinatal period.Methods: We conducted a retrospective evaluation of PAH patients pregnant at the First Affiliated Hospital of Chongqing Medical University between May 2015 and May 2021.Results: Twenty-two patients (24 pregnancies) were included in this study and received MDT management, and 21 pregnancies chose to continue pregnancy with cesarean section. Nine (37.5%) were first-time pregnancies at 27.78 ± 6.16 years old, and 15 (62.5%) were multiple pregnancies at 30.73 ± 3.71 years old. The average gestational week at hospitalization and delivery were 29.38 ± 8.63 weeks and 32.37 ± 7.20 weeks, individually. Twenty-one (87.5%) pregnancies received single or combined pulmonary vasodilators. The maternal survival rate of PAH patients reached 91.7%. Fifteen (62.5%) pregnancies were complicated with severe adverse events. Patients with complicated adverse events showed lower percutaneous oxygen saturation (SpO2), lower albumin, lower fibrinogen, higher pulmonary artery systolic pressure (PASP), higher blood pressure, longer activated partial thromboplastin time, and longer coagulation time. Fourteen (66.7%) pregnancies with cesarean sections were prematurely delivered and 85.7% newborns who survived after the operation remained alive.Conclusion: The survival rate of parturients with PAH was improved in relation to MDT and pulmonary vasodilator therapy during the perinatal period compared with previous studies. SpO2, albumin, PASP, blood pressure, and coagulation function should be monitored carefully in PAH patients during pregnancy.

scholarly journals Pulmonary Veno-Occlusive Disease: A Rare Cause of Pulmonary Hypertension

2019 ◽  
Vol 7 ◽  
pp. 232470961984037 ◽  
Author(s):  
Said Hajouli ◽  
Muhamad Alhaj Moustafa ◽  
Jessica S. Wang Memoli
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Occlusive Disease ◽  
Class I ◽  
Rare Entity ◽  
Pulmonary Arterial

Pulmonary veno-occlusive disease (PVOD) is a rare entity that is usually mistaken with pulmonary arterial hypertension (PAH) but is considered class I′ of PAH. It is important to subclassify PVOD and distinguish it from PAH as treatment with vasodilators in PVOD patients is controversial and may be fatal. In this article, we describe a case of PVOD and how we diagnosed it.

scholarly journals Pulmonary veno-occlusive disease in a patient with recently diagnosed systemic sclerosis

2019 ◽  
Vol 5 (2) ◽  
pp. NP1-NP4
Author(s):  
Nina M van Leeuwen ◽  
Sofia Ramiro ◽  
Maarten K Ninaber ◽  
Esther Nossent ◽  
Jeska K de Vries-Bouwstra
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Occlusive Disease ◽  
Endothelin Receptor Antagonists ◽  
Endothelin Receptor ◽  
Phosphodiesterase Type 5 Inhibitors ◽  
Pulmonary Arterial ◽  
Phosphodiesterase Type ◽  
Worse Prognosis

Pulmonary veno-occlusive disease is a rare cause of pulmonary hypertension in patients with systemic sclerosis that can be misclassified as pulmonary arterial hypertension. Differentiation between pulmonary veno-occlusive disease and pulmonary arterial hypertension is challenging because of the similar clinical picture. Nevertheless, discrimination is important because pulmonary veno-occlusive disease has a worse prognosis. Vasodilators including phosphodiesterase type 5 inhibitors and endothelin receptor antagonists should be started with caution and often in combination with diuretics to prevent pulmonary edema.

scholarly journals Anti-synthetase syndrome-associated pulmonary veno-occlusive disease

2020 ◽  
Vol 10 (3) ◽  
pp. 204589402093528 ◽  
Author(s):  
Dana Kay ◽  
Ferdous Kadri ◽  
Garrett Fitzpatrick ◽  
Hassan Alnuaimat ◽  
Raju Reddy ◽  
...  
Keyword(s):  
Pulmonary Hypertension ◽  
Pulmonary Arterial Hypertension ◽  
Arterial Hypertension ◽  
Right Heart Failure ◽  
Right Heart Catheterization ◽  
Occlusive Disease ◽  
Heart Catheterization ◽  
Right Heart ◽  
Pulmonary Arterial ◽  
Pulmonary Arterial Hypertension Therapy

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.

Sign in / Sign up

Export Citation Format

Share Document