Disruption of cardio-pulmonary coupling in myopathies: Pathophysiological and mechanistic characterization with special emphasis on nemaline myopathy



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Emphysema Percent Linked To Pulmonary Microvascular Blood Volume

Lower pulmonary microvascular blood volume (PMBV) is associated with greater percent emphysema in those with a diffuse emphysema subtype, according to study findings published in Thorax.

Recent research has identified 6 emphysema subtypes through quantitative computed tomography (CT); of those, the diffuse subtype is the most common in the general population and among individuals with smoking-related chronic obstructive pulmonary disease (COPD). Investigators used contrast-enhanced dual-energy CT (DECT) to measure PMBV in patients with emphysema, in order to determine the association between PMBV and percent emphysema as well as the association between PMBV and emphysema subtype.

The investigators recruited participants without renal impairment from the Multi-Ethnic Study of Atherosclerosis (MESA), a prospective community-based cohort study that enrolled individuals age 45 to 84 years from 6 US communities between 2000 to 2002. Individuals weighing over 300 pounds and those with cancer or cardiovascular disease were among those excluded from the current study.

Participants with an estimated glomerular filtration rate (eGFR) of greater than 60mL/min/1.73m2 attending the sixth MESA examination in 2017/2018 were invited to undergo contrast-enhanced DECT of the lungs for the current study. The contrast-enhanced DECT was used to assess patients' novel CT emphysema subtypes and percent emphysema. PMBV was defined as blood volume in the peripheral 2 cm of the lung. Material decomposition was used to calculate pulmonary blood volume.

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Lower pulmonary microvascular blood volume was associated with greater percent emphysema.

The analysis included 495 participants (mean age, 71 years; 44% women; 35% White, 31% Black, 15% Hispanic, 18% Asian [all self-reported]). Of those, 53% had never smoked, and 72% did not have airflow limitation on spirometry.

Mean (SD) PMBV was 352 (120) mL and mean percent emphysema was 4.95% (4.75%). The investigators found lower PMBV was associated with greater percent emphysema (-0.90% per 100 mL PMBV; 95% CI, -1.29 to -0.51) in fully adjusted models. The association was present among those with no airflow limitation or smoking history, although participants with airflow obstruction and a smoking history 10-pack-years or more had a greater association. This association was specific to the diffuse CT emphysema subtype (-1.48% per 100 mL PMBV; 95% CI, -2.31 to -0.55). No association was found between PMBV and percent emphysema in unadjusted analyses.

Study limitations include the lack of a gold standard for assessing PMBV and potential methodological confounders in site-specific protocol differences.

"Lower pulmonary microvascular blood volume was associated with greater percent emphysema," the investigators concluded, adding, "The association was of greater magnitude among those with airflow limitation and a heavy smoking history but was also significant among participants with no airflow limitation or smoking history and was specific to the diffuse CT emphysema subtype."

Disclosure: Some study authors declared affiliations with biotech, pharmaceutical, and/or device companies. Please see the original reference for a full list of authors' disclosures.


What People Living With COPD Or Emphysema Need To Know About Available Treatments

StatePoint

More than 11 million people in the United States are living with chronic obstructive pulmonary disease (COPD), including more than 3 million people with emphysema, a type of COPD. This chronic lung disease limits air flow and causes breathing-related symptoms. While there is no cure, an innovative treatment option is helping some patients with severe COPD or emphysema breathe easier.

Endobronchial Valve Therapy (EBV) is a minimally invasive treatment option that aims to improve lung function, activity level and your quality of life.

"I noticed the first day after receiving EBV that I was able to take a very deep breath. Before my treatment, my breathing was very shallow," says Karrie E., who is now able to walk longer distances before taking rests, as well as go to the grocery store to do her own shopping.

Carolyn P., who also received EBV therapy after living with COPD for years agrees, "I had the valves put in and it changed the quality of my life. I can walk without feeling like my chest is going to burst." Carolyn is now a regular walker at the malls in her hometown.

So, what are EBVs? According to the American Lung Association, which has partnered with Zephyr by Pulmonx to create awareness for managing advanced COPD with EBV, these one-way valves reduce lung hyperinflation by allowing trapped air to escape. As a result, lung function may improve when healthier areas of the lung provide the necessary oxygen exchange. They may help with feeling less short of breath and being more active. This safe, effective treatment option for eligible patients is backed by research-based evidence, and currently, there are two FDA-approved types of valves.

If you have been diagnosed with advanced COPD or emphysema and do not smoke or are willing to quit, EBV treatment might be the right option for you. This relatively quick, non-surgical procedure takes place in a hospital, and has a short recovery time, generally requiring about a three-day hospital stay.

Those who have found success with the procedure — less shortness of breath and a greater quality of life — encourage others not to delay treatment: "If it's right for you, don't wait to have it done," says Bryan C.

The American Lung Association encourages you to speak with your healthcare provider about this treatment option. You can learn more and take an online questionnaire to determine if you qualify for this procedure by visiting Lung.Org/ebv.

If you are one of the millions of Americans living with COPD, you can breathe easier knowing that advances in treatments are helping people with your condition live a better quality of life.

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