Choosing Wisely, the initiative for medical cost-effectiveness (don’t call it rationing!) of the American Board of Internal Medicine, included the use of vasodilators for pulmonary hypertension owing to left heart disease or hypoxemic lung disease (WHO Groups II and III) as #2 on its top five “no-no’s” in its new pulmonology section. The ”Five Things Physicians and [... read more]
A large phase III randomized trial testing Ambrisentan (Letairis) for early idiopathic pulmonary fibrosis was stopped early after an interim analysis of the data showed possible harm. The results were reported in Annals of Internal Medicine. Patients being treated with Letairis had more evidence of progression of idiopathic pulmonary fibrosis and were hospitalized more often, including [... read more]
New Pulmonary Hypertension Drug Riociguat Proves Effective Pulmonary arterial hypertension is a rare but life-threatening illness affecting around 10,000 people in the U.S., in which ongoing remodeling of the pulmonary vasculature leads to narrowed pulmonary arteries with increased pulmonary artery pressure (pulmonary hypertension), and if untreated, eventual right heart failure and death. (The overwhelming majority [... read more]
Pulmonary Hypertension 2013 Update/Review Part 2 of 2: Treatment of PH by Brett Ley, MD There are 3 classes of pulmonary vasodilator drugs: phosphodiesterase-5 inhibitors (PDE-5 inhibitors, e.g. sildenafil, tadalafil), endothelin receptor antagonists (ERAs, e.g. bosentan, ambrisentan), and prostacyclins (epoprostenol, iloprost, treprostinil). Because the large trials have focused on PAH, currently only WHO group 1 [... read more]
Pulmonary Hypertension 2013 Update/Review Part 1 of 2: Classification and Diagnosis by Brett Ley, MD Pulmonary hypertension (PH) is defined simply as a mean pulmonary artery pressure of 25 mmHg or greater. However, this entity encompasses a heterogeneous group of patients and underlying etiologies where accurate diagnosis, correct physiologic classification, and careful evaluation for right heart dysfunction are essential [... read more]
In Most Patients with Pulmonary Embolism, Central Clot is Worse than Peripheral by Brett Ley, MD Pulmonary embolism (PE) presents with a wide range of clinical severity and course. Management decisions (level of care, length of observation, and aggressive therapies such as thrombolysis) are generally based on a patient’s risk of a poor outcome. Guidelines recommend risk [... read more]
Xarelto (rivaroxaban): New FDA Indication for DVT, PE The U.S. Food and Drug Administration (FDA) approved Bayer’s Xarelto (rivaroxaban) for a new indication for treating deep vein thrombosis (DVT) or pulmonary embolism (PE), and for long-term prophylaxis of recurrent DVT and PE. Xarelto already had FDA approval for prevention of DVTs and PEs after knee or [... read more]
Idiopathic Pulmonary Fibrosis (IPF) 2012 Review (More PulmCCM Topic Updates) This document is updated periodically as new research findings in IPF are published. What Causes IPF? (Etiology/Pathogenesis of IPF) Since it’s still called “idiopathic,” you probably figured out already that the cause(s) of IPF remain unknown. Researchers use a paradigm for studying idiopathic pulmonary fibrosis called [... read more]
When people with pulmonary arterial hypertension and progressive right heart failure suffer an acute insult or decompensation severe enough to land them in the ICU, it’s usually not pretty. Hemodynamic management is complex and difficult,with low cardiac output resulting both from right ventricular weakness and high right sided filling pressures reducing stroke volume from the left ventricle (ventricular interdependence). [... read more]
See Update Below: the larger RELAX trial refuted the results in this small pilot study. Guazzi et al randomized 44 people with heart failure with preserved ejection fraction and associated pulmonary hypertension (confirmed by right heart catheterization) to receive the phosphodiesterase-5 inhibitor sildenafil or placebo for one year, along with standard therapy. Those in the sildenafil [... read more]
Chronic thromboembolic pulmonary hypertension: concise clinical review. Fedullo P et al. AJRCCM 2011;183:1605-1613. CTEPH review.
A 44-page guideline from the American Heart Association on the management of massive & submassive pulmonary embolism; proximal deep venous thrombosis, and chronic thromboembolic pulmonary hypertension. Circulation 2011;123:1788-1830. FREE FULL TEXT
Sildenafil looks to be reasonably safe and efficacious for long-term treatment of pulmonary arterial hypertension with NYHA class II or III symptoms, according to results of the SUPER-2 trial. This was an extension of SUPER-1, reported in NEJM 2005, which was a 12-week randomized trial in people with PAH and NYHA II/III symptoms, in which those taking [... read more]
Chronic thromboembolic pulmonary hypertension is a “new” condition (in terms of our recognition and understanding of it), whose true prevalence, natural history, and response to therapies continue to be elucidated. Meanwhile, specialized surgical centers continually make thromboendarterectomy safer, providing definitive cures that are nothing short of miraculous for those affected by this otherwise usually fatal [... read more]
Brown et al report that 21% of patients in the REVEAL registry were diagnosed with IPAH >2 years after the onset of symptoms. Younger patients, and those picking up a diagnosis of obstructive lung disease or sleep apnea along the way, were more likely to have a delay in diagnosis. CHEST 2011;140:19-26.
Update on Pulmonary Vascular Diseases 2010. Dweik RA, Erzurum SC. AJRCCM 2011;184:26-31.
Lore has it that pure-right sided heart disease in pulmonary arterial hypertension doesn’t cause pleural effusions. Luo et al report here that it does, in 35 of 89 people (39%) with connective-tissue disease-related PAH. (29 of the 35 had no other discernible cause for the effusion.) CHEST 2011;140:42-47. Their group previously reported that people with [... read more]
Ruiter et al showed that 30 of 70 (43%) Dutch patients with idiopathic pulmonary arterial hypertension were iron deficient, and had lower 6-minute walk distances (390 vs. 460 m), regardless of whether anemia was present. Eur Resp J 2011;37:1386-1391.
Actuarial survival in PAH has been predicted mainly by functional class and echocardiographic evidence of right heart failure. Observing 484 patients with WHO group I PAH for 5 years after diagnosis, Kane et al showed that using their own multivariate model (incorporating clinical data including renal function, BNP, DLCO, BP & HR, etc.) generated better [... read more]
The erection-producing drug is a more potent PDE-5 inhibitor than sildenafil or tadalafil. Jing et al enrolled 66 people with pulmonary arterial hypertension (IPAH and other types; NYHA class II and III functional class) and randomized them to vardenafil or placebo in a 2:1 ratio. After 12 weeks, vardenafil-takers walked 69 m farther in 6 [... read more]