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Compared to conventional full sternotomy (FS) approaches, minimally invasive mitral valve surgery (MIMVS) offers improved cosmesis, decreased pain and bleeding, and faster recovery, without compromising repair or survival rates. However, little is known about outcomes in patients with pulmonary hypertension (PH), an independent risk factor for morbidity and mortality.

Retrospective review was performed between 2002 and 2019 for all adult patients undergoing isolated mitral valve (MV) surgery. Patients with PH (mean pulmonary artery pressure ≥ 25 mmHg) were stratified by FS or MIMVS and nearest-neighbor propensity score matching was performed to adjust for differences in baseline characteristics.

Overall, 591 surgeries (317 MIMVS, 274 FS) met inclusion criteria during the study period. Nearest neighbor propensity matching generated 112 well-matched pairs. Cardiopulmonary bypass (137 vs 89.5 min, P < 0.001), cross clamp (102 vs 63 min, P < 0.001), and total operative times (241 vs. 178.5 min, p < 0.001) were longer for the MIMVS group. Postoperatively, MIMVS was associated with shorter initial ventilator times (6 vs 9.6 hrs, P < 0.001) and hospital lengths of stay (7 vs. 8 days, p = 0.049), as well as blood product utilization rates (26.8% vs. 41.1%, p = 0.03). Thirty-day (0.0% vs 2.7%, P = 0.12) and 10-year survival (Log-rank, P = 0.661) were similar between groups.

MIMVS is safe in patients with PH and provides traditional benefits of minimally invasive surgery including shorter initial ventilator times and hospital LOS, without compromising on long-term survival.

MIMVS is safe in patients with PH and provides traditional benefits of minimally invasive surgery including shorter initial ventilator times and hospital LOS, without compromising on long-term survival.

Bronchoscopic valve placement constitutes an effective endoscopic lung volume reduction (ELVR) therapy in patients with severe emphysema and low collateral ventilation. After occluding the most destroyed lobe with valves, significant target lobe volume reduction leads to improvements in lung function, exercise capacity, and quality of life. In some patients, the effects are not consistent leading to long-term therapy failure. We hypothesized that surgical lung volume reduction (LVRS) would re-establish ELVR short-term clinical improvements following ELVR long-term failure.

In this retrospective single-center analysis, we included all patients who underwent consolidating lung volume reduction surgery (LVRS) by lobectomy after long-term failure of valve therapy between 2010 and 2015. Changes in forced expiratory volume in 1 second (FEV1), residual volume (RV), 6-min walking distance (6MWD), and dyspnea score (mMRC) 90 days after ELVR and LVRS were analyzed, and the outcomes of both procedures were compared.

A total of 20 patients underwent LVRS after ELVR failure. In the majority (90%), a lower lobectomy was performed. The 30-day mortality of the cohort was 0% and 90-day mortality 5% (1/20). The remaining 19 patients showed a significant increase in FEV1 (+27.5 ± 19.4%) and reduction in RV (-21.0 ± 17.4%) and TLC (-11.1 ± 11.1%). This resulted in significant improvements in exercise tolerance (6MWD +56 ± 60 m) and relief of dyspnea (ΔmMRC -1.8 ± 1.4 pts.).

Consolidating LVRS by lobectomy after failure of previously successful ELVR is feasible and results in significant symptom relief and improvement of lung function.

Consolidating LVRS by lobectomy after failure of previously successful ELVR is feasible and results in significant symptom relief and improvement of lung function.Non-coding mutations at the far end of a large gene desert surrounding the SOX9 gene result in a human craniofacial disorder called Pierre Robin sequence (PRS). Leveraging a human stem cell differentiation model, we identify two clusters of enhancers within the PRS-associated region that regulate SOX9 expression during a restricted window of facial progenitor development at distances up to 1.45 Mb. Enhancers within the 1.45 Mb cluster exhibit highly synergistic activity that is dependent on the Coordinator motif. Using mouse models, we demonstrate that PRS phenotypic specificity arises from the convergence of two mechanisms confinement of Sox9 dosage perturbation to developing facial structures through context-specific enhancer activity and heightened sensitivity of the lower jaw to Sox9 expression reduction. Overall, we characterize the longest-range human enhancers involved in congenital malformations, directly demonstrate that PRS is an enhanceropathy, and illustrate how small changes in gene expression can lead to morphological variation.

To evaluate the prognostic performance of the Pediatric Index of Mortality 2 (PIM2), ferritin, lactate, C-reactive protein (CRP), and leukocytes, alone and in combination, in pediatric patients with sepsis admitted to the pediatric intensive care unit (PICU).

A retrospective study was conducted in a PICU in Brazil. All patients aged 6 months to 18 years admitted with a diagnosis of sepsis were eligible for inclusion. Those with ferritin and C-reactive protein measured within 48 h and lactate and leukocytes within 24 h of admission were included in the prognostic performance analysis.

Of 350 eligible patients with sepsis, 294 had undergone all measurements required for analysis and were included in the study. PIM2, ferritin, lactate, and CRP had good discriminatory power for mortality, with PIM2 and ferritin being superior to CRP. The cutoff values for PIM2 (> 14%), ferritin (> 135 ng/mL), lactate (> 1.7 mmol/L), and CRP (> 6.7 mg/mL) were associated with mortality. The combination of ferritin, lactate, and CRP had a positive predictive value of 43% for mortality, similar to that of PIM2 alone (38.6%). The combined use of the three biomarkers plus PIM2 increased the positive predictive value to 76% and accuracy to 0.945.

PIM2, ferritin, lactate, and CRP alone showed good prognostic performance for mortality in pediatric patients older than 6 months with sepsis. When combined, they were able to predict death in three-fourths of the patients with sepsis. Total leukocyte count was not useful as a prognostic marker.

PIM2, ferritin, lactate, and CRP alone showed good prognostic performance for mortality in pediatric patients older than 6 months with sepsis. When combined, they were able to predict death in three-fourths of the patients with sepsis. learn more Total leukocyte count was not useful as a prognostic marker.