Learning and research

Congenital heart defects (CHDs) represent one of the most prevalent categories of neonatal defects, and maternal dietary patterns have been linked to the risk of these conditions. Branched-chain amino acids (BCAAs), particularly leucine, are essential for various metabolic and physiological processes involved in heart development. In this study, we examined the molecular mechanisms through which elevated levels of leucine induce defects in cardiac microvascular endothelial cells.

To the editor,
Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare disease characterized by alveolar surfactant accumulation, progressive dyspnea, and hypoxemic respiratory insufficiency, and in some patients, secondary infections, pulmonary fibrosis, respiratory failure, and death.1 GM-CSF (granulocyte/macrophage colony-stimulating factor) autoantibodies cause aPAP by blocking GM-CSF signaling, which impairs alveolar macrophage functions including surfactant clearance.

In this Community Call, we discussed:

• An electrocardiogram-based AI algorithm for early detection of pulmonary hypertension
• Branched-chain α-ketoacids aerobically activate HIF1α signalling in vascular cells

Right ventricular (RV) (dys)function determines outcomes in pulmonary hypertension (PH). We previously found that asymmetric RV myocardial work (MW) corresponds with inefficient RV function in experimental PH models. We therefore aimed to investigate regional distribution of RV MW and its correlation with catheter hemodynamics in children with PH.

Activation of the sympathetic nervous system is observed in pulmonary arterial hypertension patients. This study investigates whether inhibiting the conversion of dopamine into noradrenaline by dopamine β-hydroxylase (DβH) inhibition with BIA 21-5337 improved right ventricular (RV) function or remodeling in pressure overload-induced RV failure. RV failure was induced in male Wistar rats by pulmonary trunk banding (PTB). 

Data from invasive CPET (iCPET) revealed long COVID patients have impaired systemic oxygen extraction (EO2), suggesting impaired mitochondrial ATP production. However, it remains uncertain whether the initial severity of SARS-CoV-2 infection has implications on EO2 and exercise capacity (VO2) nor has there been assessment of anerobic ATP generation in long COVID patients.

Our prospective study investigates the 3-year trajectory of disease-specific quality of life (QoL) using the PEmb-QoL questionnaire, functional performance via 6-min walk tests, and the 5-year survival following acute pulmonary embolism (PE) and explores their association with patient demographics and clinical characteristics. We highlight that PE-specific QoL improves over time despite no significant changes in cardiopulmonary performance.

Dear Editor,
We thank Drs. Perros, Chaveroux, and Montani for their interest in our articles describing the activation of the integrated stress response (ISR) pathway as a mechanism underlying the pathogenesis of pulmonary veno-occlusive disease (PVOD), based on the rat mitomycin C (MMC) model of PVOD.

To the Editor,
We read with great interest the recent paper by Prabhakar et al., “Mechanisms underlying age-associated exacerbation of pulmonary veno-occlusive disease,”1 which investigates the role of the integrated stress response (ISR) in pulmonary veno-occlusive disease (PVOD).