Williams-Beuren Syndrome

2018 ◽  
Author(s):  
Yonmee Chang ◽  
Andrew J. Matisoff
Keyword(s):  
Cardiac Arrest ◽  
Multidisciplinary Approach ◽  
Perioperative Management ◽  
Coronary Artery Stenosis ◽  
Genetic Disorder ◽  
Anesthetic Management ◽  
Extracorporeal Cardiopulmonary Resuscitation ◽  
Vascular Walls ◽  
Supravalvar Aortic Stenosis ◽  
Elastin Gene

Williams-Beuren syndrome, also known as Williams syndrome (WS) is a genetic disorder involving the elastin gene on chromosome 7q11.23. Elastin is important for elasticity of vascular walls, and its deficiency can lead to widespread arteriopathy, most notably supravalvar aortic stenosis of the ascending aorta and coronary artery stenosis. Because of these cardiac defects, patients with WS are at high risk for cardiac arrest under anesthesia with a documented incidence around 5%. Appropriate perioperative management of all anesthetics includes a multidisciplinary approach to risk stratification, precise anesthetic management, and disaster planning. This chapter reviews the etiology, pathogenesis, diagnosis, and manifestations of WS. The authors also discuss the preoperative workup and anesthetic implications of WS, as well as issues related to cardiac arrest and extracorporeal cardiopulmonary resuscitation.

scholarly journals Epidural Anesthesia for Cesarean Section in a Pregnant Woman with Marfan Syndrome and Dural Ectasia

2017 ◽  
Vol 2017 ◽  
pp. 1-5 ◽  
Author(s):  
Franco Pepe ◽  
Mariagrazia Stracquadanio ◽  
Francesco De Luca ◽  
Agata Privitera ◽  
Elisabetta Sanalitro ◽  
...  
Keyword(s):  
Cesarean Section ◽  
Cesarean Delivery ◽  
Marfan Syndrome ◽  
Epidural Anesthesia ◽  
Perioperative Management ◽  
Perioperative Complications ◽  
Genetic Disorder ◽  
Anesthetic Management ◽  
Previous Pregnancy ◽  
Dural Ectasia

Marfan syndrome (MFS) is a genetic disorder of connective tissue, characterized by variable clinical features and multisystem complications. The anesthetic management during delivery is debated. Regional anesthesia has been used with success during cesarean delivery, but in some MFS patients there is a probability of erratic and inadequate spread of intrathecal local anesthetics as a result of dural ectasia. In these cases, epidural anesthesia may be a particularly useful technique during cesarean delivery because it allows an adequate spread and action of local anesthetic with a controlled onset of anesthesia, analgesia, and sympathetic block and a low risk of perioperative complications. We report the perioperative management of a patient with MFS and dural ectasia who successfully underwent cesarean section using epidural technique anesthesia. The previous pregnancy of this woman ended with cesarean section with a failed spinal anesthesia that was converted to general anesthesia due to unknown dural ectasia at that time.

Management of out-of hospital cardiac arrest patients with extracorporeal cardiopulmonary resuscitation in 2021

2021 ◽  
pp. 1-10
Author(s):  
Christopher Gaisendrees ◽  
Matias Vollmer ◽  
Sebastian G Walter ◽  
Ilija Djordjevic ◽  
Kaveh Eghbalzadeh ◽  
...  
Keyword(s):  
Cardiac Arrest ◽  
Cardiopulmonary Resuscitation ◽  
Extracorporeal Cardiopulmonary Resuscitation ◽  
Hospital Cardiac Arrest

Does Targeted Temperature Management Improve Neurological Outcome in Extracorporeal Cardiopulmonary Resuscitation (ECPR)?

2021 ◽  
pp. 088506662110189
Author(s):  
Merry Huang ◽  
Aaron Shoskes ◽  
Migdady Ibrahim ◽  
Moein Amin ◽  
Leen Hasan ◽  
...  
Keyword(s):  
Cardiac Arrest ◽  
Cardiopulmonary Resuscitation ◽  
Neurological Outcome ◽  
Targeted Temperature Management ◽  
Temperature Management ◽  
Survival Outcomes ◽  
Pool Data ◽  
Good Neurological Outcome ◽  
Extracorporeal Cardiopulmonary Resuscitation ◽  
Over Time

Purpose: Targeted temperature management (TTM) is a standard of care in patients after cardiac arrest for neuroprotection. Currently, the effectiveness and efficacy of TTM after extracorporeal cardiopulmonary resuscitation (ECPR) is unknown. We aimed to compare neurological and survival outcomes between TTM vs non-TTM in patients undergoing ECPR for refractory cardiac arrest. Methods: We searched PubMed and 5 other databases for randomized controlled trials and observational studies reporting neurological outcomes or survival in adult patients undergoing ECPR with or without TTM. Good neurological outcome was defined as cerebral performance category <3. Two independent reviewers extracted the data. Random-effects meta-analyses were used to pool data. Results: We included 35 studies (n = 2,643) with the median age of 56 years (interquartile range [IQR]: 52-59). The median time from collapse to ECMO cannulation was 58 minutes (IQR: 49-82) and the median ECMO duration was 3 days (IQR: 2.0-4.1). Of 2,643, 1,329 (50.3%) patients received TTM and 1,314 (49.7%) did not. There was no difference in the frequency of good neurological outcome at any time between TTM (29%, 95% confidence interval [CI]: 23%-36%) vs. without TTM (19%, 95% CI: 9%-31%) in patients with ECPR ( P = 0.09). Similarly, there was no difference in overall survival between patients with TTM (30%, 95% CI: 22%-39%) vs. without TTM (24%, 95% CI: 14%-34%) ( P = 0.31). A cumulative meta-analysis by publication year showed improved neurological and survival outcomes over time. Conclusions: Among ECPR patients, survival and neurological outcome were not different between those with TTM vs. without TTM. Our study suggests that neurological and survival outcome are improving over time as ECPR therapy is more widely used. Our results were limited by the heterogeneity of included studies and further research with granular temperature data is necessary to assess the benefit and risk of TTM in ECPR population.

Beyond Extracorporeal Cardiopulmonary Resuscitation: Systems of Care Supporting Cardiac Arrest Patients

2021 ◽  
pp. 1-6
Author(s):  
Nicholas George ◽  
Alexandra Lawler ◽  
Ian Leong ◽  
Ankur A. Doshi ◽  
Francis X. Guyette ◽  
...  
Keyword(s):  
Cardiac Arrest ◽  
Cardiopulmonary Resuscitation ◽  
Systems Of Care ◽  
Extracorporeal Cardiopulmonary Resuscitation
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