21 January 2022

This week in PVD

Obstructive sleep apnea common in pulmonary hypertension

In a new study, researchers reported obstructive sleep apnea is common in patients with pulmonary hypertension, and risk factors include older age, male sex and lower daytime arterial oxygen pressure.

This single-center, observational study enrolled 140 patients with pulmonary hypertension (mean age, 39.5 years; 76.4% women) diagnosed via right heart catheterization who underwent overnight cardiorespiratory monitoring from December 2018 to December 2020. OSA was defined by an apnea-hypopnea index of 5 or more hours with 50% or more obstructive apneic events. Researchers then compared patients with pulmonary hypertension with and without OSA.

Twenty-five percent of patients with pulmonary hypertension had OSA. The incidence of OSA was 76% among patients with pulmonary hypertension associated with pulmonary disease or low oxygen, 50% among those with chronic thromboembolic pulmonary hypertension, 15.4% among those with connective tissue disease-associated PAH, 11.8% among those with unknown mechanisms of pulmonary hypertension and 8% among those with congenital heart disease-associated PAH.

Gene activity analysis of whole blood finds 3 major PAH subgroups

Gene activity analysis of whole blood collected from people with pulmonary arterial hypertension (PAH) identified three patient subgroups associated with distinct clinical features and outcomes, a study concluded.

These findings may provide molecular insights into the development of PAH, improve disease risk assessments, and potentially guide individual treatment strategies, the scientists noted.

The study, “Biological heterogeneity in idiopathic pulmonary arterial hypertension identified through unsupervised transcriptomic profiling of whole blood,” was published in the journal Nature Communications.

In PAH, the pulmonary arteries that transport blood through the lungs narrow, restricting blood flow and increasing blood pressure (hypertension).

The cause of idiopathic PAH (IPAH) is currently unknown, and diagnosis is usually confirmed after other forms of PAH have been ruled out, such as infections, the use of certain medications, or other diseases. As a result, IPAH patients show a wide range of differences in disease progression, treatment responses, and survival.

Several studies have found mutations in genes associated with both IPAH and inherited forms of PAH (HPAH), as well as disease-related signatures in protein production and gene activity that have identified biomarkers to aid in diagnosis and prognosis (outcomes).

Pulmonary arterial hypertension associated with CHD: developments in treatments, remaining gaps according to experts

Due to substantial progress in the early diagnosis and treatment of children born with congenital heart disease (CHD), mortality rates in this population have declined significantly. It is estimated that more than 90% of pediatric patients with CHD now survive into adulthood.1 Among the reported 1.4 million adults with CHD in the United States, pulmonary arterial hypertension (PAH) occurs in approximately 10% of cases.2,3

PAH associated with CHD (PAH-CHD) is linked to significant reductions in survival and quality of life, especially for patients progressing to Eisenmenger syndrome, the most severe phenotype of PAH-CHD. Treatment requires lifelong management in multidisciplinary specialty centers with expertise in PAH-CHD. Advances in treatment for pediatric CHD have led to a reduction in the number of patients who ultimately develop Eisenmenger syndrome, and the management of remaining cases has improved with the use of disease-targeting therapies.3

However, there are numerous remaining gaps in PAH-CHD research and treatment overall, particularly in the area of PAH associated with systemic to pulmonary shunts.3 With greater numbers of CHD patients reaching adulthood and thus facing an increased risk of developing PAH, there is a critical need for effective treatment options for the various forms of PAH-CHD.1

RV dysfunction index predicts outcomes in patients with HFrEF, PH post discharge

The use of right ventricular tissue Doppler imaging systolic velocity (RV-TDI s’) to estimate RV dysfunction could predict postdischarge clinical outcomes among individuals hospitalized for heart failure with reduced ejection fraction (HFrEF) and pulmonary hypertension (PH), according to a study published in the Journal of Cardiology.

Researchers sourced data on 573 patients with HFrEF admitted to Tokyo Women’s Medical University Hospital in Japan between July 2013 and November 2017. Study participants were stratified by RV dysfunction, with reduced RV function defined as RV-TDI s’ less than or equal to 9.5 cm/s. Primary endpoints included composite cardiovascular (CV) death and rehospitalization due to heart failure (HF). Kaplan-Meier curves were used to evaluate CV death, HF-related rehospitalization, and their composite, with the log-rank test used to assess intergroup differences.

The patient population had a median age of 66.4±15.8 years, and 72% were men.

Study identifies two distinct sub-phenotypes of ARDS

Researchers have identified two new distinct subphenotypes of adults with acute respiratory distress syndrome, according to results published in BMJ Open.

“In current clinical practice, risk stratification for patients with ARDS solely depends on the PaO2/FiO2 clinical measure,” Abhijit Duggal, MD, physician in the department of critical care medicine at the Respiratory Institute at Cleveland Clinic, said in a press release issued by Endpoint Health. “The application of this single assessment often results in bedside challenges in evaluating optimal treatment options for patients. It can also lead to challenges in assessing the impact of interventions in clinical trials. While our findings warrant further study before they can be generalized, this analysis demonstrates that it may be possible to enhance risk stratification strategies by using a few additional clinical variables that are collected during routine ARDS care.”

Heart imaging useful in PAH patients with connective tissue disease

The use of imaging to evaluate the right side of the heart could be useful for non-invasively diagnosing and monitoring pulmonary arterial hypertension (PAH) in people with connective tissue diseases, according to a new study.

The study, “Evaluation of pulmonary arterial pressure in patients with connective tissue disease-associated pulmonary arterial hypertension by myocardial perfusion imaging,” was published in the Annals of Noninvasive Electrocardiology.

Connective tissue diseases, or CTDs, are a group of inflammatory conditions that, as the name suggests, affect the body’s connective tissue. Common CTDs include scleroderma, lupus, and rheumatoid arthritis. PAH, when the pressure in the lung’s blood vessels is abnormally high, can be a serious complication of CTDs.

High blood pressure in the lungs, as occurs in PAH, puts strain on the right ventricle — the part of the heart that pumps blood to the lungs to pick up oxygen. Once it picks up oxygen, the blood circles back to the heart’s left ventricle, which pumps it out to the rest of the body.


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