Harrisarcher5878

Late systemic outflow tract obstruction following completion of the Fontan palliation is rarely seen and is a difficult problem to treat. Absence of the main pulmonary trunk and pulmonary valve at this stage makes a conventional Damus-Kaye-Stansel connection difficult to achieve. We report the case of a 37-year-old female who underwent Fontan completion as an adult and subsequently presented with systemic outflow tract obstruction. A valved conduit was interposed between the native pulmonary annulus and the ascending aorta to create a modified Damus-Kaye-Stansel type connection.BACKGROUND Despite significant improvement in outcomes with truncus arteriosus (TA) repair, right ventricular outflow tract (RVOT) reconstruction with a right ventricular to pulmonary artery (RV-to-PA) conduit remains a source of long-term reintervention and reoperation. This study evaluated our experience with reintervention in homograft and polytetrafluoroethylene (PTFE) RV-to-PA conduits in neonates. METHODS Primary TA repairs from 2004 to 2016 at a single institution were included. Stratification was based on RVOT reconstruction with PTFE or homograft conduit. Primary outcome was operative conduit replacement. Secondary outcomes included the rates and types of catheter-based conduit interventions. RESULTS Twenty-eight patients underwent primary TA repair and 89.3% (n = 25) of them had RVOT reconstruction with a homograft (28.0%, n = 7) or PTFE (72.0%, n = 18) conduit. Rates of reoperation for conduit replacement and catheter-based interventions were similar between those with PTFE and homograft conduits (85.7% vs 72.2%, P = .49 and 57.1% vs 83.3%, P = .11, respectively). selleckchem Additionally, the median time to conduit replacement and catheter-based conduit interventions were comparable. In multivariable analysis, conduit size, but not conduit type, was a predictor of conduit revision (hazard ratio 1.66, 95% confidence interval 1.11-2.49, P = .02). At five-year and ten-year follow-up, patients with PTFE conduits had better survival than those with homograft conduits (100.0% vs 71.4%, P = .02); however, no mortalities were associated with conduit reoperations or catheter-based reinterventions. CONCLUSIONS Polytetrafluoroethylene and homograft RVOT reconstruction in neonatal TA repair demonstrate similar durability as defined by reoperation and reintervention rates. The validation of the durability of PTFE conduits in neonatal TA repair requires confirmatory studies in larger cohorts.Cavopulmonary anastomosis was first described by Carlon, Mondini, De Marchi in a canine model in 1951 and later, in the clinical practice, by Glenn in 1958. Total right heart bypass was first introduced by Fontan and Kreutzer in 1971, in each instance as treatment for tricuspid atresia. Several modifications of such a procedure followed the initial concept of the right atrium as a pumping chamber, including modifications aimed to minimize energy loss at the anastomotic level and arrhythmias. Tribute is given to our pioneers who developed such an operation aimed to treat any child with functionally univentricular hearts.Severe local acidosis causes tissue damage and pain, and is associated with many diseases, including cerebral and cardiac ischemia, cancer, infection, and inflammation. However, the molecular mechanisms of the cellular response to extracellular acidic environment are not fully understood. We recently identified a novel and evolutionarily conserved membrane protein, PAC (also known as PACC1 or TMEM206), encoding the proton-activated chloride (Cl-) channel, whose activity is widely observed in human cell lines. We demonstrated that genetic deletion of Pac abolished the proton-activated Cl- currents in mouse neurons and also attenuated the acid-induced neuronal cell death and brain damage after ischemic stroke. Here, we show that the proton-activated Cl- currents are also conserved in primary rat cortical neurons, with characteristics similar to those observed in human and mouse cells. Pac gene knockdown nearly abolished the proton-activated Cl- currents in rat neurons and reduced the neuronal cell death triggered by acid treatment. These data further support the notion that activation of the PAC channel and subsequent Cl- entry into neurons during acidosis play a pathogenic role in acidotoxicity and brain injury.OBJECTIVE The effect of pregnancy on otosclerosis is controversial. If pregnancy physiologically increases the risk of progression, females with children would be expected to receive stapedectomy earlier than childless females and males. Here, we seek to determine whether sex moderates the relationship between number of children and age at stapedectomy. STUDY DESIGN Retrospective observational study of national health care claims. SETTING 2003 to 2016 Optum Clinformatics Data Mart. SUBJECTS AND METHODS In total, 6025 privately insured US adults (3553 females, 2472 males) who received stapedectomy for otosclerosis were queried for age and number of children at the time of initial surgery. RESULTS The average age at stapedectomy was significantly lower in females than males (46.8 vs 48.1 years; t test, P less then .0001). Females with children had a significantly lower age at surgery compared to childless females (39.3 vs 49.9 years; t test, P less then .0001). Males with children similarly had a significantly lower age at surgery compared to childless males (40.5 vs 51.3 years; t test, P less then .0001). A higher number of children was correlated with lower age for both females (Pearson, r = -0.3817, P less then .0001) and males (Pearson, r = -0. 3675, P less then .0001). Linear regression showed that younger age of surgery could be predicted by female sex and number of children (F(3, 6021) = 336.93, P less then .001, R2 = 0.1437) with no significant interaction between sex and number of children (P = .186). CONCLUSION Sex does not moderate the effect of increasing number of children on decreasing age at stapedectomy. Social, rather than biological, factors surrounding parenthood such as increased overall health care utilization may explain prior associations between pregnancy and otosclerosis.