Cardiovascular abnormalities in Marfan syndrome]

During the period February to December 1990, 52 adult patients were referred to our clinic for evaluation of the presence of the Marfan syndrome. In 24 out of 52 patients the Marfan syndrome was diagnosed. Cardiac abnormalities were found in all patients: mitral insufficiency because of mitral valve prolapse (83%), aortic dilatation (67%), aortic insufficiency (38%), tricuspid valve insufficiency with or without tricuspid valve prolapse (17%) and atrial septal defect (4%). In 3 patients an aneurysm of the ascending aorta was found. Early recognition of the Marfan syndrome is relevant for prevention of the life threatening complication of aortic dissection. In patients with valve abnormalities endocarditis prophylaxis is advised. A Marfan outpatient clinic offers optimal diagnostic possibilities.     

急性主动脉壁间血肿患者血浆D-二聚体水平研究

[目的]急性主动脉壁间血肿在发病机制、解剖改变、临床表现等方面与急性主动脉夹层分离有一定差异。目前,国内对壁间血肿研究较少,且未见血浆D-二聚体水平对其诊断价值的相关报道。[方法]回顾性分析某院近4年来所有诊断急性主动脉综合症的患者,根据CT血管成像结果将其分为两组:经典急性主动脉夹层分离组(acuteaor-ticdissection,AAD组)与急性主动脉壁间血肿组(aorticintramuralhematoma,AIH组),比较两组入院时血浆D-二聚体浓度及其他临床资料。[结果]共纳入患者51例,AAD组与AIH组分别为38例(74.5%)和13例(25.5%)。入院时AAD组D-二聚体浓度为(3.50±1.72)μg/ml,AIH组则为1.54±1.50μg/ml,两组间差异有统计学意义(P﹤0.05)。以﹥0.50μg/ml为阈值,D-二聚体敏感性在AAD组为100%(38/38),而AIH组则为84.6%(11/13),但两组间差异无统计学意义(P﹥0.05)。住院期间脑卒中、急性肾功能衰竭、急性心肌梗死、急性主动脉瓣关闭不全和截瘫发生率方面,AAD组为39.5%(15/38),AIH组为30.8%(4/13),两组间差异无统计学意义(P﹥0.05)。住院期间死亡率,AAD组为18.4%(7/38),AIH组为15.4%(2/13),两组间差异无统计学意义(P﹥0.05)。[结论]AIH患者血浆D-二聚体浓度低于AAD患者。以0.5μg/ml为阈值,D-二聚体对AIH和AAD的预测敏感性类似。但部分AIH患者可能呈阴性反应,导致漏诊。AIH患者死亡率和临床并发症发生率与AAD患者相似,故临床上应予以高度重视。     

Angiodysplasias in the digestive tract and associated cardiovascular diseases

To clarify the possible relationship between angiodysplasia of the gastrointestinal tract and cardiovascular disease, we performed a retrospective study of 58 consecutive patients. In all patients angiodysplasias were identified endoscopically. The median age was 72 years. Fifty-seven per cent of the patients had some clinically obvious cardiovascular disorder. Mitral valve insufficiency was found more often (21%) than aortic valve stenosis (10%). A high incidence of coronary artery disease (45%) and peripheral artery disease (10%) was noted. These findings do not support the concept that angiodysplasia occurs principally in combination with aortic stenosis. The aetiology of these vascular lesions is presumably multifactorial. The vast majority of the lesions seems to be acquired and at least a subgroup is probably an expression of degenerative processes of aging.     

经导管主动脉瓣置换术对主动脉瓣狭窄患者肺动脉压力及左心收缩功能的影响

目的研究经导管主动脉瓣置换术对主动脉瓣狭窄患者肺动脉压力及左心收缩功能的影响。方法选取2017年6月至2018年6月我院收治的76例主动脉瓣狭窄患者,均行经导管主动脉瓣置换术治疗,记录患者手术相关情况,对比分析患者手术前后肺动脉压力及左心收缩功能情况。结果手术即刻成功率为100%,所有患者均未发生严重并发症及心脏不良事件。与术前比较,术后1周患者的肺动脉压力及左心收缩功能均明显改善(P<0.05)。结论经导管主动脉瓣置换术对主动脉瓣狭窄患者肺动脉压力及左心收缩功能具有积极影响,利于其预后改善。     

De BakeyⅠ型主动脉夹层的围术期监护

目的:探讨外科手术治疗主动脉夹层的围手术期临床观察重点和护理体会。方法:34例De BakeyⅠ型主动脉夹层患者围手术期采取严密的监护措施。结果:34例患者均康复出院,临床症状消失,恢复正常生活,无任何并发症发生。结论:De BakeyⅠ型主动脉夹层手术治疗效果满意,早期诊断和及时恰当的治疗、围手术期严密监护和精心护理是降低病死率的关键。     

Influence of age at operation on the results of aortic coarctation surgery

The aim of our study is to determine the ideal operative age of aortic coarctation. 30 patients (20 male and 10 female) have had surgery for coarctation of the aorta between 1990 and 1998. Mean operative age is 4.5 years (range 2 months and 13 years). Crafoord technique was performed in 23 cases, modified Crafoord technique in 3 cases, Waldhausen operation in 3 cases and coarctectomie with insertion of a prosthetic material in one case. A post-coarctectomy syndrome was noted in one case. There were no early deaths. The mean follow-up period is 4.5 years. 8 patients were noted to have late systemic hypertension (26.5%). It is more frequent in patients that have been operated after the age of 5 years (43%). One patient has had a recoarctation (3%). No late death was noted. 4 patients had to undergo a reoperation for cardiac associated anomalies.     

Case studies in acute aortic dissection: Strategies to avoid a catastrophic outcome

Aortic dissection is a catastrophic illness that is a significant source of liability for hospitals if diagnosis and treatment are not done promptly. The diagnosis is often difficult to make because not all dissections have the typical presentation of sudden severe chest pain radiating to the back. Symptoms often include abdominal pain, flu‐like complaints, vomiting and diarrhea, low back pain, stroke syndromes and syncope. Patients at risk include those with Marfan syndrome and other connective tissue diseases, familial aortic disease, age and hypertension. Aortic dissection is a different clinical entity than abdominal aortic aneurysm. Strategies to reduce risk and improve outcome include staff education on various presentations and risk factors, rapid availability of diagnostic testing modalities such as chest CT scan or transesophageal echocardiogram, and protocols to ensure prompt transfer for cardiothoracic surgery.     

某部队干部体检异常结果分析

目的 通过分析某部35岁以上干部体检资料,了解35岁以上干部近4年主要体检异常及其年龄分布特征,为该人群的预防保健工作提供依据. 方法 收集某部2012-2015年35岁以上干部体检资料,按照2015年体检阳性率由高到低列出前10位体检异常,采用χ2检验分析异常结果及在35岁以上干部人群中4年间的阳性率差异,通过年龄分层分析男性受检者前10位重要体检异常的年龄分布特征. 结果 2015年男性受检者体检异常阳性率由高到低分别为:主动脉硬化(64.89%)、甲状腺结节(50.64%)、超重(30.89%)、脂肪肝(29.30%)、甘油三酯高(23.57%)等;2012-2015年间主动脉硬化与甲状腺结节阳性率呈上升趋势.女性受检者体检异常阳性率由高到低分别为:甲状腺结节(57.45%)、主动脉硬化(48.94%)、血清总胆固醇高(30.85%)、低密度脂蛋白高(25.53%)、肝囊肿(25.00%)等.2015年男性受检者中器官性异常(主动脉硬化、甲状腺结节、肝囊肿)的阳性率随着年龄增长而升高;代谢性异常(超重、脂肪肝、甘油三酯高)的阳性率随着年龄增长而降低.结论 某部35岁以上干部中甲状腺结节、主动脉硬化等器官性异常阳性率居高,其罹患的风险随年龄增长而增高;高血脂及脂肪肝等代谢性异常阳性率较高,次二类异常为慢性病预防的重点.     

The Novel Bone Alkaline Phosphatase Isoform B1x Is Associated with Improved 5-Year Survival in Chronic Kidney Disease

Circulating alkaline phosphatase (ALP) is an independent cardiovascular risk marker. Serum bone ALP (BALP) isoforms indicate bone turnover and comprise approximately 50% of total circulating ALP. In chronic kidney disease (CKD), mortality is highest in patients with increased ALP and BALP and low bone turnover. However, not all low bone turnover states are associated with increased mortality. Chronic inflammation and oxidative stress, features of protein energy wasting syndrome, induce cardiovascular BALP activity and fibro-calcification, while bone turnover is suppressed. Circulating BALP isoform B1x is associated with low ALP and low bone turnover and has been exclusively detected in CKD. We investigated the association of serum B1x with survival, abdominal aortic calcification (AAC) score, and aortic pulse wave velocity (PWV) in CKD. Serum ALP, BALP isoforms, parathyroid hormone (PTH), PWV, and AAC were measured repeatedly over 2 years in 68 prevalent dialysis patients. Mortality was assessed after 5 years. B1x was detected in 53 patients. A competing risk analysis revealed an association of B1x with improved 5-year survival; whereas, baseline PWV, but not AAC score, predicted mortality. However, PWV improved in 26 patients (53%), and B1x was associated with variation of PWV over time (p = 0.03). Patients with B1x had lower PTH and total ALP, suggesting an association with lower bone turnover. In conclusion, B1x is associated with time-varying PWV, lower circulating ALP, and improved survival in CKD, and thus may be an indicator of a reduced cardiovascular risk profile among patients with low bone turnover.     

Turner综合征患儿合并脏器异常的检测及意义

【目的】 探讨Turner综合征患儿心血管、肾脏、甲状腺异常情况,为Turner综合征患儿诊断后监测提供依据。 【方法】 重庆医科大学附属儿童医院门诊25名诊断Turner综合征患儿,彩色多普勒超声扫描其心血管(24例)、肾脏(25例)、甲状腺(24例)。 【结果】 超声扫描发现,主动脉弓缩窄1例,心血管异常率为4.17%(1/24);肾脏解剖结构异常3例,异常率12%(3/25);7例甲状腺肿大,异常率为29.17%(7/24)。 【结论】 Turner综合征患儿心脏,肾脏,甲状腺异常较常见。心血管、肾脏、甲状腺的检查应该作为Turner综合征患儿监测常规。     

D-二聚体在高危胸痛病因诊断中的价值

目的分析D-二聚体在高危性胸痛病因诊断中的应用价值。 方法回顾性分析2018年1月至2020年5月在扬州大学医学院附属泰兴市人民医院急诊科并明确诊断为高危性胸痛患者120例的临床资料，其中男性75例，女性45例；年龄44～78岁，平均(62.43±5.28)岁；体重指数(18.26～24.37)kg/m²，平均(21.34±0.98)kg/m²。统计高危性胸痛中急性心肌梗死、主动脉夹层、肺栓塞的发生占比情况，测定急性心肌梗死、主动脉夹层、肺栓塞患者的D-二聚体水平，并进行比较。 结果120例高危性胸痛患者中，急性心肌梗死共53例(44.17%)，主动脉夹层共38例(31.67%)，肺栓塞共29例(24.16%)。肺栓塞患者血清D-二聚体水平高于主动脉夹层与急性心肌梗死，且主动脉夹层患者血清D-二聚体水平高于急性心肌梗死，差异有统计学意义(P<0.05)。 结论血清D-二聚体在不同病因所致的高危性胸痛中的水平表达存在明显差异，其在急性心肌梗死患者中表达较低，呈轻度升高，在肺栓塞患者中升高趋势最为显著，故临床上可通过检测高危性胸痛患者的D-二聚体水平，来鉴别诊断急性心肌梗死、主动脉夹层、肺栓塞。     

Perioperative outcomes in octogenarians undergoing cardiac surgery in Puerto Rico

With the advances in surgical techniques and the advent of an aging population, the application of cardiac surgeries has broadened to include those patients over the age of 80. In order to characterize the in-hospital morbidity and mortality of elderly patients undergoing cardiac surgery at our cardiovascular center, a retrospective review of all medical records of consecutive octogenarian patients was made. Between January 1998 and April 1999, 76 patients (mean age (SD), 82.5 (2.6)) underwent cardiac surgery, of which, 61.8% were males. Surgical procedures consisted of isolated coronary artery bypass grafting (CABG) in 64 patients, isolated aortic valve replacement (AVR) in 4 patients, combined CABG and left carotid endarterectomy in 4 patients, combined CABG and AVR in 2 patients, combined CABG and aortic aneurysm repair in 1 patient and combined AVR and aortic dissection repair in 1 patient. Surgery was elective in 51.3% of patients and seventy-one percent (54) experienced at least one postoperative complication. The most common complication was atrial arrhythmia (28) followed by low cardiac output (23) and pneumonia (16). Hospital mortality occurred in 11 (14.7%) patients, an estimate in agreement with previous published studies on octogenarians. Since the number of patients studied was small, these results require confirmation by other cardiovascular centers in the island. Further research to determine independent predictors of postoperative morbidity and mortality, to assess survival and quality of life after cardiac surgery and to evaluate cost-effectiveness considering new trends in managed health care is warranted.     

Cardiac and vascular homograft valves transplant

The advances in the manipulation of human tissues, the development of cryobiology, paediatric cardiac surgery, the impossibility of obtaining an ideal prosthetic cardiac valve and the surgical treatment of cardiovascular infections have revived interest in the use of homografts. The donors of these homografts can be: a) Live donors: aortic and pulmonary valve of the recipient of a heart transplant; b) Multiorgan donors with a diagnosis of death according to neurological criteria, whose heart is rejected for heart transplant; c) Cadaver donors with asystolia of less than 8 hours. Homograft cardiac valves are the substitute of choice in aortic valve endocarditis, patients with counter-indications for anticoagulation, reconstruction of the outflow tract of the right ventricle, aortic valve replacement in children and young adults through the Ross operation, and an optional indication is the aortic valve and/or rising aorta replacement in patients over 60 years of age. Although there are not sufficiently broad series of homogratfs with arterial substitutes, with respect to the number of patients and time of evolution, the results suggest that this can benefit patients with vascular infection, immunodepressed patients or complex patients whose technique during the operation might require a homograft.     

Health-Related Quality of Life After Transcatheter Aortic Valve Implantation in Elderly Patients With Severe Aortic Stenosis

BackgroundSevere aortic stenosis may affect both normal daily living and survival. Transcatheter aortic valve implantation (TAVI) has become an appropriate alternative to surgery for elderly patients with high surgical risk, and it results in improved survival in this population.ObjectiveTo describe health-related quality of life (QoL) before, and at 1 and 6 months after TAVI.SettingCardiovascular Department, Toulouse University Hospital, France.MethodsOne hundred sixty-four consecutive patients with at least 6 months follow-up who underwent TAVI in our institution between February 2009 and June 2011 were enrolled in the study. Of this population, 73 (mean age: 82.3 ± 7.3 years, 56% men) completed QoL assessment using the EuroQOL Five Dimensions (EQ-5D) questionnaire and a visual analogue scale at baseline, and at 1 and 6 months. At each visit, medical data and New York Heart Association functional class were collected.ResultsThe median preprocedure EQ-5D index value (interquartile range) was 0.66 (range, 0.36–0.78) showing severely impaired QoL in almost all patients. Significant improvement occurred from baseline to 1 month of follow-up in EQ-5D index value [0.73 (range, 0.59–0.79)] and this was maintained at 0.73 (range, 0.62–0.81) at 6 months. The improvement was significant for 3 dimensions of the EQ-5D score at the 6-month follow-up: usual activities in 43.8% of patients (P < .001); anxiety/depression in 37% (P < .001) and pain/discomfort in 28.8% (P < .05). Mean New York Heart Association class improved significantly from 3.24 at baseline to 2.04 at 1 month and 1.89 at 6 months.ConclusionsIn high-risk patients with severe aortic stenosis, QoL and health status improved substantially at 1 month and improvement persisted 6 months after TAVI.     

Complete atrioventricular septal defect in children with Down's syndrome: good results of surgical correction at younger and younger ages

OBJECTIVE: To evaluate the results of cardiosurgical treatment of children with Down's syndrome and a complete atrioventricular septal defect (cAVSD). DESIGN: Retrospective. METHOD: Data were collected from the records of all patients with Down's syndrome who had been subjected to primary corrective surgery for cAVSD in the period 1980-2003 in Leiden, The Netherlands. Exclusion criteria were: concomitant tetralogy of Fallot or interruption of the aortic arch and palliative banding of the pulmonary artery. Children with an abnormal shape of the ventricle making it impossible to correct biventricularly were not considered in the study. Data on mortality >30 days after the operation and on repeated surgery were included in the analysis if the duration of follow-up was at least 5 years. RESULTS: The group comprised 148 children: 75 girls and 73 boys. The median age at time of surgery was 20 weeks (range: 6 weeks to 3.7 years) and showed a statistically significant decrease during the period under investigation. Of 4 children lost to clinical follow-up actual data could be obtained. The median duration offollow-up was 6 years and 7 months (range: 38 days to 23 years and 11 months). 28 children (19%) died. The mortality within 30 days after the operation decreased from 0-38% in the period 1980-1989 via 0-30% in the period 1990-1999 to 0% in 2000-2003. The percentage ofreoperations related to the correction ofcAVSD (14%; 14/98) did not seem to increase. Of the correction-related reoperations, 14% (2/14) were followed by a second reoperation. Conclusion. In the period 1980-2003, children with Down's syndrome and a cAVSD were corrected surgically at a younger and younger age. The early mortality decreased to 0% in the years 2000-2003. Echocardiography in the first weeks of life for all children with Down's syndrome makes it possible to diagnose those with a cAVSD early enough for surgical repair.     

Nutrition Therapy for the Critically Ill Surgical Patient With Aortic Aneurysmal Rupture

Background: Our goal is to define nutrition therapy in critically ill patients after surgical repair of acute ruptured or dissecting aortic aneurysm to identify opportunities for quality improvement. Methods: International, prospective studies in 2007–2009 and 2011 were combined. Sites provided institutional and patient characteristics including from intensive care units (ICUs) admission to ICU discharge for a maximum of 12 days. We selected patients with aortic aneurysmal rupture or acute dissection staying in the ICU for ≥ 3 days. Results: There were 104 eligible patients from 72 distinct ICUs analyzed. Overall, 86.5% received artificial nutrition. There were 50.0% patients who received enteral nutrition (EN) only, 29.8% patients received a combination of EN and parenteral nutrition (PN), 6.7% patients received PN only, and 13.5% did not receive any nutrition. The mean time from admission to initiation of EN was 3.0 days (SD ± 2.4 days). The adequacy of calories from nutrition support was 46.8% (range 0%‐111%) with a mean of 10.0 kcal/kg/day. Of the total of 83 patients who received EN, 53 patients (63.8%) had interruption of EN. The reasons included fasting, intolerance, patients deemed too sick for enteral feeding, and loss of enteral feeding route. For patients with gastrointestinal intolerance, 3/30 patients (10%) received small bowel feeding and 23/30 patients (76.7%) of patients received motility agents. Conclusion: Postoperative critically ill patients with aortic aneurysmal rupture or acute dissection are at high risk for inadequate nutrition therapy, and there may be inadequate utilization of strategies to improve nutrition uptake.     

Congenital heart disease and pregnancy

During pregnancy cardiac output rises by 40-50%. At first stroke volume in particular increases, with the heart rate rising mainly in the second part of the pregnancy. More and more women with congenital heart disease are reaching adulthood and want to become pregnant. The haemodynamic changes during pregnancy are an additional burden for women with heart disease. Risk factors for occurrence of important cardiac problems during pregnancy are: previous cardiac events, lower functional class, cyanosis, left-sided heart obstruction or a diminished systolic function of the systemic ventricle. Pregnancy is contraindicated in pulmonary hypertension, in Marfan syndrome with an ascending aorta diameter of > 50 mm and in severe mitral or aortic valve stenosis. The recurrence risk for children varies between 3 and 50%, depending on the nature of the congenital heart disease. Foetal outcome is depending on the haemodynamic situation and medication use of the mother. ACE inhibitors are contraindicated during pregnancy.     

先天性主动脉瓣下狭窄的超声诊断分析

目的 探讨主动脉瓣下狭窄(SAS)的超声心动图诊断价值.方法 回顾性分析30例SAS的超声心动图特征及手术所见.结果 单纯SAS 9例,其中隔膜型3例,肌肥厚性5例,二尖瓣畸形导致的1例.21例合并有其他心血管畸形,其中室间隔缺损(VSD)6例,动脉导管未闭(PDA)7例,主动脉瓣狭窄(AA)5例、主动脉缩窄(COA)3例.根据三尖瓣反流、心内或大血管水平分流压差,估测肺动脉收缩压52～75mm Hg.2例患者漏诊弥漫型SAS;1例被误诊为肥厚型心肌病(HCM).结论 SAS合并其他心血管畸形在解剖、血流动力学上有许多特异之处,把握其特殊表现,有助于超声心动图做出准确诊断,从而为手术治疗提供有价值的参考.     

Cardiac involvement in spondylarthropathies

Cardiac involvement is a real manifestation of spondylarthropathies and include specially aortic regurgitation and conduction troubles. We present a prospective open study of fifty patients with spondylarthropathy (responded to Amor criteria) in order to evaluate the frequency of cardiac involvement, to see its type and if we can evaluate a group of patients able to this complication. We have included forty-four men and six women with a mean age of 38 years. The disease evolve for 8.8 years in mean. Ag HLA B27 was present in 70% of the cases. Thirty patients have ankylosing spondylitis, although twenty have a secondary spondylarthropathy: psoriasic rheumatism (12 cases), inflammatory bowel disease: Crohn's disease (4 cases), RCH (three cases) and Fiessenger le Roy-Reiter syndrome in one case. All the patients have had a cardiac check up with research of clinical cardiac manifestation, thoracic chest, trans-thoracic echographi, Halter rhythmic done in five cases only. Cardiac involvement is found in five cases (10%): aortic regurgitation in 3 cases (6%) and mitral regurgitation in 2 cases (4%). These valvular disease are well tolerated.     

维持性血液透析患者中MIA综合征的临床分析

目的探讨血液透析患者MIA综合征的发生率、临床表现及与透析的充分性之间的相关性。方法对142例血透患者进行了营养指标、慢性炎症状况、心血管疾病的横断面调查。结果142例维持性血透患者普遍存在着营养不良，其发生率为轻中度42．3％（60／142），重度7．0％（10／142），总发生率为49．3％（70／142）。142例血CRP为0．33—44（5．55±10．22）mg／L，慢性炎症35例（24．6％）超过正常值（8mg／L）。142例有心血管疾病表现的总发生率为52．1％（74／142）。提示维持性血透患者存在营养不良、炎症、心血管疾病是较常见的。而兼有营养不良、炎症、心血管疾病即MIA综合征者为19．0％（27／142），但MIA综合征的发生与透析充分性无明显关系。结论血液透析患者中存在MIA综合征，且营养不良、心血管疾病及炎症之间可能是相互关联的。