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Anesthetic management of a child with Cornelia de Lange Syndrome undergoing open heart surgery:A case report

World Journal of Cardiology 2022年第1期14卷 54-63页

作者： Oguzhan Arun Bahar Oc Esma Nur Metin Ahmet Sert Resul Yilmaz Mehmet Oc Department of Anesthesiology and Reanimation Selcuk University Faculty of MedicineKonya 42130Turkey Department of Pediatric Cardiology Selcuk University Faculty of MedicineKonya 42130Turkey Pediatric Intensive Care Selcuk University Faculty of MedicineKonya 42130Turkey Department of Cardiovascular Surgery Selcuk University Faculty of MedicineKonya 42130Turkey

BACKGROUND Cornelia de Lange syndrome(CdLS)is a congenital multisystemic genetic *** expected lifespan of children with this disorder has been prolonged in parallel with the advances in medicine in recent ***,they still more frequently undergo cardiac *** are some challenges for clinicians when faced with CdLS *** present the perioperative management of a child with CdLS undergoing open-heart *** SUMMARY Severe pulmonic and subpulmonic valvular stenosis,enlargement of the right side of the heart,mild tricuspid regurgitation,atrial septal defect,and patent ductus arteriosus were diagnosed in a 14-month-old boy with manifested cyanosis,developmental delay,and *** balloon valvuloplasty was unsuccessful due to a severe stenotic pulmonary valve,therefore it was decided to perform an open surgical *** a successful and uncomplicated intraoperative course,the patient was extubated on postoperative day 5,and adrenalin and dopamine infusions were gradually decreased and stopped on postoperative days 6 and 10,*** laryngomalacia and suboptimal vocal cord movements were diagnosed,and tracheotomy and percutaneous endoscopic gastrostomy were performed under general anesthesia in the same session at postoperative day *** patient was discharged on postoperative day 85 after a challenging postoperative period with additional airway and nutritional *** This is the first report of the perioperative anesthetic and clinical management of a CdLS patient undergoing open-heart surgery.

关键词： Cornelia de Lange Syndrome Brachmann de Lange Syndrome Pulmonary valve stenosis valvular heart disease Cardiac surgery Anesthesia Case report

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Paradoxical Low Flow Low Gradient Severe Aortic Stenosis—An Under-Recognized Entity

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Case Reports in Clinical Medicine 2022年第2期11卷 31-36页

作者： Phillip Crane Mandeep Singh Kalsi Matias Yudi Naveen Sharma Department of Cardiology The Northern Hospital Melbourne Australia Department of Cardiology Austin Health Melbourne Australia

Some patients with severe aortic stenosis (AS), due to restrictive cardiac physiology, paradoxically have relatively low flow and low gradients across stenotic aortic valves despite preserved left ventricular (LV) systolic function. It results in symptoms and reduced quality of life and carries a high mortality. Whilst this form of severe AS, termed paradoxical low flow low gradient (pLFLG), is well reported, patients with this diagnosis experience inappropriate barriers to aortic valve replacement (AVR), the only efficacious treatment. We present the case of an 88-year-old female with 12 months of exertional dyspnoea on a background of hypothyroidism and hypercholesterolemia. Transthoracic echocardiogram (TTE) revealed LV hypertrophy, with a small LV cavity size and reduced stroke volume, yet normal systolic function. A heavily calcified aortic valve was identified with severe aortic stenosis, based on valve area, yet with incongruous mean transvalvular gradient of 25 mmHg (severe ≥ 50 mmHg). Following exclusion of other differential diagnoses, her symptoms were attributed to paradoxical LFLG severe AS. She was however declined definitive transcatheter aortic valve implantation (TAVI) due to her paradoxically low mean aortic gradient. Following further deterioration in her symptoms and supportive quantification of poor exercise performance, she was ultimately re-referred, accepted, and underwent TAVI. Following her AVR, the patient experiences significant improvement in both symptoms and quality of life after only one month. Paradoxical LFLG severe AS remains a well-documented yet under recognized disease. It carries high morbidity and mortality if untreated, yet is significantly less likely to be referred and accepted for intervention. With its prevalence expected to rise with an ageing population, this case serves as a timely reminder for clinicians to address the under recognition of important pathology.

关键词： Paroxysmal Low Flow Low Gradient Doppler Echocardiography Aortic Valve Stenosis Aortic Valve Implantation valvular heart disease

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Early outcomes and analysis of factors influencing in-hospital mortality after concomitant heart valve replacement and coronary artery bypass grafting

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South China Journal of Cardiology 2022年第1期23卷 95-102,F0003页

作者： ZHENG Hai-yun WU Hao HE Xiao-ling ZHU Ping Guangdong Cardiovascular Institute Guangdong Provincial People's HospitalGuangdong Academy of Medical SciencesGuangzhouChina Guangdong Key Laboratory of cardiac pathogenesis and precise prevention and treatment GuangzhouChina Sun Yat-sen Memorial Hospital Sun Yat-sen UniversityGuangzhouChina

Background Objective:To investigate the perioperative precautions,surgical methods and early clinical efficacy of patients with valvular heart disease complicated by coronary atherosclerotic heart disease treated with coronary artery bypass grafting at the same time as heart valve replacement and to analyze the relevant factors affecting the death of patients during hospitalization after ***:Between January 2020 and September 2021,a total of 103 patients who underwent simultaneous heart valve replacement and coronary artery bypass grafting for heart valve disease in conjunction with coronary atherosclerotic heart disease at the Department of cardiac surgery,Guangdong Cardiovascular Institute were retrospectively evaluated for clinical data,and a systematic review and summary of all preoperative data,surgical methods,intraoperative data,and major complications were *** variations in each cardiotocography indicator were examined before and after surgery to determine surgical effectiveness and statistical perioperative patient *** integrating the Sino SCORE risk assessment system and analyzing the case features included in this data,12 alternative risk variables for mortality were identified and submitted to univariate and multivariate analysis using logistic *** performing a univariate analysis of the alternative risk factors,the option of P<0.05 was considered a risk factor and included in the multivariate analysis,followed by a multivariate logistic regression analysis in which the factors were determined to be independent risk factors with P<0.05,and their OR values and 95 percent confidence intervals(CIs)were calculated to analyze the associated factors affecting patients’early prognosis ***:Of the 103 patients,91 were successfully discharged from the hospital,12 died,11.7%of the deaths were due to the following causes:postoperative low cardiac output syndrome,multiple organ failure,and severe infec

关键词： valvular heart disease Coronary atherosclerotic heart disease heart valve replacement Coronary artery bypass grafting Clinical efficacy Influence factor

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Long-term natural history of ascending aorta after surgical valye replacement:echocardiographic follow-up

Long-term natural history of ascending aorta after surgical ...

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中国超声医学工程学会第十三届全国超声心动图学术会议

作者： Jin-ping ZHANG Yong JIANG Wei-chun WU Ming-hui ZHANG Li ZHANG Jing-jin WANG Hao WANG

Objectives:The present study sought to identify the natural evolution of ascending aorta of rheumatic heart disease(RHD) after surgical valve replacement(SVR) in ***:In this retrospective analysis,outcomes of 1230 patients(60.2% were female,age 47.5±9.48 years,range:14-75 years) who underwent primary SVR without replacement or wrapping of the ascending aorta in Fuwai hospital between 1998 and 2010 were *** 10016 imaging studies met inclusion criteria in terms of length of *** and serial ascending aorta echocardiographic measurements(divided into 4 stages,the median echo follow-up time was 7.59 years) were ***:There was a significant increase in the mean dimension of ascending aorta in more than 3 years follow-up(31.56 mm versus 32.34 mm,31.04 mm versus 32.61 mm,31.13 mm versus 33.29 mm,P69 *** adverse aortic events were really rare and occurred in 5/1911 patients(0.3%).In 4 subsets analyzed,there were evident difference in the mean diameter of ascending aorta and all shown a trend of progressive ***:Our study showed there was a slight increase in the mean dimension of ascending *** supports that stability of ascending aortic dilatation following SVR and argues that reoperation might just think about primary valve *** conclusion,patients with SAV concomitant with or without aneurysmally dilated ascending aortas infrequently need to have the aorta replaced.

关键词： surgical valve replacement valvular heart disease rheumatic heart disease echocardiography follow-up

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