Learning and research

Recent international guidelines recommend a multidisciplinary evaluation and care model for patients with chronic thromboembolic pulmonary hypertension (CTEPH), but there is a paucity of supporting data. The aim of this study was to describe the outcomes of a multidisciplinary team approach to the comprehensive care of CTEPH patients. 

Pulmonary hypertension (PH) is defined hemodynamically as a mean pulmonary arterial pressure (mPAP) ≥ 20 mmHg, measured at right heart catheterization (RHC). Pulmonary arterial hypertension (PAH) is defined as a mPAP ≥ 20 mmHg with a pulmonary capillary wedge pressure (PCWP) or left ventricular end-diastolic pressure (LVEDP) of ≤ 15 mmHg and a pulmonary vascular resistance (PVR) > 2 Woods Units (WU). 

Pulmonary arterial hypertension (PAH) is a heterogenic diagnosis including idiopathic and hereditary PAH (IPAH/HPAH) and groups associated to connective tissue disease (APAH-CTD) and congenital heart disease (APAH-CHD). Pre- and post-diagnosis societal costs in PAH subgroups are not well known.

Several disease related factors of pulmonary hypertension (PH) can negatively impact the nutritional status, leading to an increased risk of malnutrition. However, there are no studies on the best method for nutritional screening in PH patients.

Prognostic markers of Group 3 pulmonary hypertension (PH) remain largely unknown. In this study, we evaluate clinical data to provide a comprehensive profile of patients with Group 3 PH and evaluate the potential use of vasoreactivity testing as a prognostic tool within this population.

A meaningful number of patients with PAH die suddenly, and there is little data to understand the events surrounding sudden death in PAH. We tested the hypothesis that sudden death is associated with pre-mortem ECG or hemodynamics changes compared to those who died of RHF.

ACE2 has shown effectiveness in treating pulmonary hypertension in multiple animal models and has some promise in early human trials. The key barrier to translation is that enzymatically active ACE2 is difficult to manufacture and exhibits a short half-life in humans, making chronic administration challenging.

Reduced functional capacity and poor sleep quality are common in pulmonary arterial hypertension (PAH). Wearable devices are an emerging, user-friendly tool to capture activity and sleep information. We aimed to determine whether Fitbit-derived activity and sleep trends provide clinically meaningful information in patients with PAH. 

Part of the Hypoxia in pulmonary vascular research – altitude and beyond webinar series

Kholdani and colleagues describe patient characteristics, completeness of diagnostic testing, treatment patterns, risk characteristics, and outcomes of a large cohort of patients with pulmonary arterial hypertension seen at an academic pulmonary hypertension center over a 20-year period.