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Relationships among pulmonary hypertension (PHTN), mitral valve intervention (MVI), and death in patients with Shone's syndrome (SS) are unclear. This study's recent data suggested temporal progression of mitral valve disease and an early survival benefit with MVI at initial operation. In this expanded cohort, the study characterized the trajectory and impact of PHTN on survival, as well as its relationship with MVIs. Study investigators conducted a record review of 219 patients with SS who underwent operation at a quaternary center between 1956 and 2022; 111 of these patients were pediatric (aged <18 years). Patients were determined to have PHTN by using a time-defined hierarchic algorithm consisting of cardiac catheterization data, echocardiographic variables, pulmonary vasodilator therapy, and a record-verified PHTN diagnosis. Analysis included risk-adjusted predicted survival and freedom from PHTN curves stratified by MVI timing. Among 219 patients, 92 (43%) had mitral stenosis on presentation, with a mean gradient of 3.5 mm Hg (interquartile range, 4.7 mm Hg). Sixty patients had undergone at least 1 MVI; 23 (9%) patients had 3 or more MVIs. The median follow-up was 19 years (interquartile range, 29 years), and 18 (8%) deaths occurred. Ninety-three (42%) patients had PHTN, with 50% and 25% freedom from PHTN at 37 and 51 years from presentation, respectively. PHTN was strongly associated with death (P <.001). In unadjusted analyses, MVI, even if successive, delayed the onset of PHTN at 10 years and improved survival (P <.001). However, this protective effect was lost after risk adjustment. PHTN development in SS is progressive and mirrors the survival curve, findings suggesting that PHTN is a major mortality risk factor. Salutary effects of early or successive MVI are reduced after risk adjustment. [Display omitted] [ABSTRACT FROM AUTHOR]

Compression of the neurovascular structures of the upper extremity as they pass through the thoracic outlet result in thoracic outlet syndrome. The myriad of symptoms associated with the syndrome vary based on the structure(s) compressed: the subclavian artery/vein or the inferior trunk of the brachial plexus. This is a common site of compression especially in the presence of upper extremity injury, overuse or anatomical abnormalities. Majority of patients present with neurogenic pain and weakness; herein, we present the case of a patient with symptoms of both arterial and neurogenic compression caused by aberrant anterior scalene anatomy. These patients are excellent surgical candidates for first rib resection and anterior scalenectomy. A transaxillary approach offers the clinician an adequate window to identify anatomical abnormalities intraoperatively and safely excise the first rib and anterior scalene muscle. [ABSTRACT FROM AUTHOR]

Shone's syndrome (SS) has a varied anatomic spectrum without consensus on need and timing for mitral valve intervention (MVI). We sought to (1) characterize the anatomic spectrum and treatment pathways; (2) describe long-term outcomes and their determinants; and (3) define the impact of MVI timing on survival. In total, 121 patients with SS who underwent operation at Cleveland Clinic between 1956 and 2021 were reviewed. Multivariable parametric hazard analyses including time-varying covariables, and modulated renewal to account for repeated events, were performed. End points included time-related survival and reintervention. Median follow-up was 9.9 years. Mitral stenosis (MS) (98%), coarctation (80%), and aortic stenosis (70%) predominated. The most common combination was MS + aortic stenosis + coarctation (26%). Median initial mean mitral and aortic gradients were 3.6 (15th/85th percentiles: 2.0/6.8) and 9.0 (2.1/46) mm Hg, respectively. Median initial surgery age was 0.041 (0.011/3.2) years. Initial surgeries included coarctation repair (43%), arch repair (18%), and staged biventricular repair (18%). Overall survival was 92% at 20 years. Freedom from reoperation was 66% and 24% at 1 and 20 years. Patients with no MVI or initial MVI (N = 7) tended to be associated with better early survival compared with those with MVI at subsequent operation (N = 29) (P =.06). Risk factors for early reintervention included initial Norwood operation, with younger age and arch hypoplasia increasing later reintervention. Despite excellent long-term survival, reoperation in SS is frequent and occurs most commonly on left ventricular outflow tract and mitral valve. Although MS is present in most, few require MVI. Delaying MVI may compromise early survival. [Display omitted] [ABSTRACT FROM AUTHOR]

The breakdown of plant material fuels soil functioning and biodiversity. Currently, process understanding of global decomposition patterns and the drivers of such patterns are hampered by the lack of coherent large-scale datasets. We buried 36,000 individual litterbags (tea bags) worldwide and found an overall negative correlation between initial mass-loss rates and stabilization factors of plant-derived carbon, using the Tea Bag Index (TBI). The stabilization factor quantifies the degree to which easy-to-degrade components accumulate during early-stage decomposition (e.g. by environmental limitations). However, agriculture and an interaction between moisture and temperature led to a decoupling between initial mass-loss rates and stabilization, notably in colder locations. Using TBI improved mass-loss estimates of natural litter compared to models that ignored stabilization. Ignoring the transformation of dead plant material to more recalcitrant substances during early-stage decomposition, and the environmental control of this transformation, could overestimate carbon losses during early decomposition in carbon cycle models., (© 2024 The Authors. Ecology Letters published by John Wiley & Sons Ltd.)