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]
See Update Below: the larger RELAX trial (enrolling patients with less severe pulmonary hypertension) did not show a benefit of sildenafil. 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 [… read more]
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.
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]
Rich et al from U. of Chicago add to the “we said, they said” record on echocardiography’s accuracy in measuring PA pressures (and by implication, whether it can be used to justify oral therapy for PAH without invasive testing). They measured 160 people’s PAP by right heart catheterization and ECHO, both tests within one month; they then [… read more]
Osteopontin is a cytokine; Lorenzen et al measured levels in 95 people with idiopathic pulmonary arterial hypertension, 25 of whom were followed prospectively for 3 months after therapy initiation. Elevated osteopontin was more common in people with IPAH than in healthy controls, predicted mortality, and correlated with 6-minute-walk distance and NYHA class in the retrospective cohort. CHEST 2011;139:1010-1017.
Pulmonary hypertension is often “diagnosed” with a tricuspid regurgitant jet velocity > 2.5 m/s on echocardiogram. Bossone et al found that 76 of 615 (12%) trained athletes (strength or endurance) exceeded this number, and suggest the upper limit of normal should be 40 mm Hg in endurance athletes. CHEST 2011;139:788-794.
Fang et al prospectively observed 126 patients undergoing lung transplant at 9 U.S. centers. PA pressure was obtained invasively in the OR just after transplant. Those who developed grade 3 primary graft dysfunction at 72 hours (1′ endpoint) had mPAP of 38.5 mm Hg, compared to 29.6 for those who did not; the relationship persisted [… read more]
Aduen et al performed simultaneous echocardiograms and right heart catheterizations on 117 patients, concluding echo is more accurate and precise than others previously found. They also promote a method of calculating and reporting mean PA pressure on echo, rather than systolic PA pressure. Right heart catheterization remains indispensable as the gold standard. CHEST 2011;139:347-352
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