Microbiota Transplant Therapy Is Safe and Feasible in Pulmonary Arterial Hypertension.

Pulmonary arterial hypertension (PAH) is a complex inflammatory disease that the gut microbiome likely contributes to and may be a potential therapeutic avenue for nontoxically improving outcomes. Here, we show that microbiota transplant therapy (MTT) is safe and feasible. The MTT regimen achieves only modest levels of donor microbiota engraftment but is accompanied by a transient reduction in circulating pro-inflammatory cytokines.

Hemodynamics and Phosphodiesterase-5 Inhibitor Treatment Associated with Survival in ILD-PH: A PVRI GoDeep Meta-Registry Analysis.

Background: Pulmonary hypertension (PH) in interstitial lung disease (ILD) lacks approved therapies. The PVRI GoDeep meta-registry collects real-world data of PH patients from international PH referral centers.

Methods: ILD-PH patients and relevant subgroups (IIP, IPF) were stratified by pulmonary vascular resistance (PVR).

Gut Microbiome in Pulmonary Arterial Hypertension-An Emerging Frontier.

Pulmonary arterial hypertension (PAH) is an irreversible disease characterized by vascular and systemic inflammation, ultimately leading to right ventricular failure. There is a great need for adjunctive therapies to extend survival for PAH patients. The gut microbiome influences the host immune system and is a potential novel target for PAH treatment.

From Normal to Zero Flow in 30 Seconds on HeartMate 3 LVAD: What Happened?

Background: We present a patient who acutely developed a zero flow alarm without warning signs almost 4 months after a HeartMate 3 (HM3) left ventricular assist device (LVAD) implantation.

Case Summary: Patient was doing well as an outpatient when suddenly the zero flow alarm of his HM3 sounded without change in pump parameters. Patient progressed to cardiac arrest and did not survive.

Restructures the Micro/Mycobiome to Combat Inflammation-Mediated Right Ventricular Dysfunction in Pulmonary Arterial Hypertension.

Background: Inflammation suppresses right ventricular (RV) function in pulmonary arterial hypertension (PAH). In particular, we showed GP130 (glycoprotein-130) signaling promotes pathological microtubule remodeling and RV dysfunction in rodent PAH. Emerging data demonstrate the intestinal microbiome regulates systemic inflammation, but the impact of modulating the gut microbiome on the GP130-microtubule axis in RV failure is unknown.

The Frequency and Predictors of Pulmonary Rehabilitation Referrals Among Patients With Pulmonary Arterial Hypertension: An Analysis of the Pulmonary Hypertension Association Registry.

Background: Pulmonary arterial hypertension (PAH) is a complex cardiopulmonary disease associated with exertional dyspnea and impaired health-related quality of life (HRQOL) despite medical therapy. Pulmonary rehabilitation (PR), a supervised exercise program for patients with chronic lung disease, improves symptoms, HRQOL, and exercise capacity. Despite these benefits, there is a paucity of data regarding PR in PAH.

Comparison of the prognostic value of right atrial echocardiographic parameters in pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) guidelines advocate measures of area for right atrial (RA) dimensions, while echocardiographic guidelines recommend RA volume. We compared the prognostic value of RA echocardiographic parameters to predict transplant-free survival in 332 adult patients with PAH. RA area correlated strongly with volume (r = 0.

Medication Nonadherence in Patients with Pulmonary Arterial Hypertension: The Pulmonary Hypertension Association Registry (PHAR).

Pulmonary arterial hypertension (PAH) is associated with significant morbidity and mortality. The extent of medication nonadherence in PAH is uncertain and may be linked to adverse outcomes. There has been a lack of multicenter, registry-based studies assessing medication nonadherence and patient-centered outcomes in PAH.

Restructures the Micro/Mycobiome to Combat Glycoprotein-130 Associated Microtubule Remodeling and Right Ventricular Dysfunction in Pulmonary Arterial Hypertension.

Emerging data demonstrate systemic and local inflammation regulate right ventricular (RV) adaption in preclinical and human pulmonary arterial hypertension (PAH). Pathological RV inflammation is targetable as antagonism of glycoprotein-130 (GP130) signaling counteracts pathological microtubule remodeling and improves RV function in rodents. Microtubules control several aspects of cardiomyocyte biology including cellular and nuclear size/structure, t-tubule homeostasis, and the proper localization of connexin-43.

Inhaled treprostinil in pulmonary hypertension associated with COPD: PERFECT study results.

Background: Pulmonary hypertension (PH) accompanying COPD (PH-COPD) is associated with worse outcomes than COPD alone. There are currently no approved therapies to treat PH-COPD. The PERFECT study (ClinicalTrials.

Extrapulmonary manifestations of pulmonary arterial hypertension.

Introduction: Extrapulmonary manifestations of pulmonary arterial hypertension (PAH) may play a critical pathobiological role and a deeper understanding will advance insight into mechanisms and novel therapeutic targets. This manuscript reviews our understanding of extrapulmonary manifestations of PAH.

Areas Covered: A group of experts was assembled and a complimentary PubMed search performed (October 2023 - March 2024).

Ketone bodies in right ventricular failure: A unique therapeutic opportunity.

Background: Ketone bodies are pleotropic metabolites that play important roles in multiple biological processes ranging from bioenergetics to inflammation regulation via suppression of the NLRP3 inflammasome, and epigenetic modifications. Ketone bodies are elevated in left ventricular failure (LVF) and multiple approaches that increase ketone concentrations exert advantageous cardiac effects in rodents and humans. However, the relationships between ketone bodies and right ventricular failure (RVF) are relatively unexplored.

Sex differences in right ventricular function between Groups 1 and 3 pulmonary hypertension.

Group 3 pulmonary hypertension (PH) patients have disproportionate right ventricular dysfunction (RVD) compared to pulmonary arterial hypertension. We evaluated how sex and PH etiology modulated RVD. Strain echocardiography showed no intrasex differences between PH types.

Pulmonary tumor thrombotic microangiopathy: Exploration into current diagnostic aids and therapeutics.

Pulmonary tumor thrombotic microangiopathy (PTTM) is an under-recognized cause of pulmonary hypertension and fulminant right ventricle failure. It is associated with a high mortality due to delay in diagnosis. We present two cases of PTTM, both diagnosed postmortem, highlighting the importance of timely identification and initiation of treatment for this near-fatal condition.

Effect of Combination of Balloon Pulmonary Angioplasty and Medical Therapy on Reverse Right Ventricular Remodeling and Hemodynamics in Chronic Thromboembolic Pulmonary Hypertension.

Introduction: Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive and debilitating disorder that results from incomplete resolution of vascular obstructions resulting in pulmonary hypertension. Surgical pulmonary thromboendarterectomy (PTE) is the treatment of choice for CTEPH. Unfortunately, many CTEPH patients are ineligible for PTE or do not have access to an expert surgical center.

Ketone Bodies in Right Ventricular Failure: A Unique Therapeutic Opportunity.

Ketone bodies are pleotropic metabolites that play important roles in multiple biological processes ranging from bioenergetics to inflammation regulation via suppression of the NLRP3 inflammasome, and epigenetic modifications. Ketone bodies are elevated in left ventricular failure (LVF) and multiple approaches that increase ketone concentrations exert advantageous cardiac effects in rodents and humans. However, the relationships between ketone bodies and right ventricular failure (RVF) are relatively unexplored.