Pulmonary arterial hypertension (PAH) frequently is associated with an imbalance in antiproliferative bone morphogenic protein-2 receptor signaling and proproliferative type-II activin receptor signaling, favoring the latter. Sotatercept is an activin ligand trap that reduces the dominant detrimental activin signaling and provides clinical benefit. We report a patient with heritable PAH in whom sotatercept had neither positive nor negative effects; we relate that fact to his PAH being caused by a previously unreported variant of unknown significance (c.1276T>C, p.[Cys426Arg]) in the GDF2 gene. GDF2 encodes bone morphogenic protein type-9, the presence of which is required for proper functioning of the pulmonary microvasculature. Low levels of functionally active bone morphogenic protein type-9 contribute to PAH. As we enter an era of precision medicine for patients with PAH with increasingly costly therapies, genetic screening may direct appropriate therapy and limit the use of expensive but likely ineffective therapies.

High-flow nasal cannula (HFNC) is a first-line therapy for patients with acute respiratory failure. Despite increased HFNC utilization over recent years-accelerated in part by the COVID-19 pandemic-high-quality evidence to guide HFNC discontinuation is lacking. Decisions about when and how quickly to reduce flow rate, Fio2, or both simultaneously are frequently left to clinicians' discretion without clear guidance on an optimal approach. Failure to de-escalate HFNC support when clinically appropriate has many potential consequences, such as prolongation of ICU/hospital length of stay, increased health care costs, and reduced availability of limited hospital resources. With the goal of improving care efficiency and resource utilization among hospitalized patients with acute respiratory failure, we propose a standardized approach for HFNC discontinuation focused on "liberation" (similar to spontaneous breathing trials for patients undergoing mechanical ventilation), using a stepwise approach guided by physiology.

Effective bidirectional communication is crucial during end-of-life decision-making, which requires clear understanding between clinicians and patients and their family members about treatment options, preferences, and goals of care. For those who have a non-English language preference or who have difficulty speaking, reading, writing, and understanding English, interpreters are essential. However, patients who speak rarer languages, known as languages of lesser diffusion (LLDs), such as Karen, spoken in Thailand and Myanmar, face unique challenges because of limited interpretation resources. In this work, we discuss the case of a Karen-speaking patient admitted to the ICU who lacked decision-making capacity, requiring the involvement of family members who also spoke Karen for a code status discussion. Despite efforts to find an interpreter, no Karen interpreter was available initially, complicating the communication and decision-making about changing the code status to do not resuscitate/do not intubate. A remote Karen interpreter was identified later, allowing for effective communication and clinician assurance that the family did understand the implications of their decision and had made it voluntarily. End-of-life decision-making is complex and challenging, requiring culturally sensitive communication. Patients who speak LLDs face unique difficulties in these discussions compared with those who speak more common languages because of the lack of interpretation resources. The purpose of this case report is to draw attention to these specific challenges and explore ethical concerns when engaging in decision-making conversations with patients and families who speak an LLD.

Pediatric pulmonary hypertension (PH) is a severe incurable disease with a poor prognosis. In pediatric PH, trial design is hampered by the absence of age-appropriate trial end points. This study evaluated physical activity (PA) measured by hip-anchored accelerometry as a potential trial end point in pediatric PH.

The cuff leak test (CLT) is an important tool to assess the risk of upper airway obstruction after extubation.

There is an ongoing debate about whether clearance of Mycobacterium tuberculosis infection occurs and at what magnitude. Recent studies quantifying "uncertainty zones" of interferon-gamma release assays (IGRAs) provide a more stringent estimate of reversion.