“Treat-Repair-Treat”:Management of Left Main Coronary Compression by a Pulmonary Artery Aneurysm in a Patient with Atrial Septal Defect and Significant Pulmonary Hypertension

Left main coronary compression syndrome(LMCS)may complicate pulmonary artery aneurysms(PAA),usually developed in the context of pulmonary arterial hypertension(PAH).We report the case of a 51-year-old female patient with an atrial septal defect(unsuitable for device closure)complicated by a PAA generating a 90%left main stenosis.The significant PAH held us back from immediate surgery.After specific dual PAH-targeted therapy(sildenafil and bosentan),the atrial septal defect could be closed with a unidirectional valved patch;the PAAinduced LMCS was treated by reductive arterioplasty.The postoperative course was uneventful.Follow-up showed clinical improvement,but PAH treatment was still needed.After three months,coronary angiography showed only an insignificant residual left main stenosis,proving that reductive pulmonary arterioplasty was effective in treating LMCS.Any PAA requires further evaluation for LMCS,a dangerous but treatable complication.The“treat-repair-treat”strategy and shunt-closure with a unidirectional valved patch can both improve surgical prospects of LMCS with shunt-related PAH.

Left Pulmonary Artery Sling Associated with Patent Ductus Arteriosus and Atrial Septal Defect: Evaluation with Multidetector CT

We report a case of left pulmonary artery sling associated with patent ductus arteriosus and atrial septal defect in a 21-month-old child. 256-slice MDCT provides valuable information, such as abnormal origin of the left pulmonary artery, the relationship between pulmonary artery and airway, the diameter of the patent ductus artery and atrial septal defect. The information is helpful in diagnosis, pre-operative evaluation and post-operative follow-up of LPS.

Compression of Left Main Coronary Artery in Patients with Pulmonary Artery Aneurysm and Pulmonary Hypertension

Background: Pulmonary artery aneurysm (PAA) is an unusual finding and its association with left main coronary (LMCA) compression is even more infrequent. Cardiac CT evaluates of presence and size of PAA and the degree of LMCA compression. The aim of this study is to describe two cases of adults with compression of LMCA with PAA associated with PDA and pulmonary hypertension. Case presentation: The first case is a 27-year-old man with PAA (78 mm diameter) and LMCA compression of 70% between the aortic sinus and the PAA. He presented angina as a manifestation of the LMCA compression. During follow-up the patient died. The second case is a 28-year-old man with PAA (110 mm diameter) that compresses LMCA in 55%, he rejected surgical treatment, but he is in close follow-up with medical treatment. Conclusion: Cardiac computed tomography played an important role both in the diagnosis and identification of high-risk PAA patients.

Anomalous origin of left pulmonary artery branch from the aorta with Fallot's tetralogy:one case report

我们报导经常与呼吸窘迫介绍了的一个十岁的男孩的案例后来出生。胸 X 光检查显示出一个扩大右室。Echocardiography 证明 Fallot 的四联剧和左肺的动脉的典型特征不能被设想。然而，心导管插入术透露左肺的动脉在上升主动脉有异常起源。病人经历了缺点的全部的外科的修正。九天以后，他被解除。我们在场发生的文学评论的结果， physiopathological，这稀罕疾病的临床、诊断、外科的特征。

Significance of the left ventricular ejection/filling parameters in patients with pulmonary arterial hypertension:a magnetic resonance imaging based study

Background The goal of this study was to explore the significance of left ventricular(LV)ejection/filling parameters in assessing severity and prognosis of pulmonary arterial hypertension(PAH).Methods Newly diagnosed PAH patients were recruited between July 2011 and December 2013 prospectively.Cardiopulmonary hemodynamics derived from right heart catheterization,and LV ejection/filling parameters derived from CMR were collected.Follow-up data were collected periodically using a specifically-designed network database until April 30,2016.

Anomalous left coronary artery from pulmonary artery: Case series and brief review

Anomalous origin of left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary anomaly. In this study, we present all the ALCAPA patients which were admitted at our institution during April 2007-December 2010. Retrospective review of these patients regarding their clinical presentation and the use of diagnostic modalities will be presented in this series. There were total of five patients, three male and 2 female, with age range of 2 - 12 months. The most common symptoms at presentation were tachypnea (4/5) and poor feeding with irritability (3/5). Electrocardiogram was abnormal in 2/5 cases and chest X ray revealed cardiome-galy with pulmonary congestion in 4/5 patients. Echocardiogram showed mitral valve regurgitation in 5/5 cases (3 with moderate and 2 with mild to moderate), Left ventricular dilatation/dysfunction in 4/5 patients, echogenic left ventricular papillary muscles in 4/5 patients and prominent right coronary with strong suspecision of ALCAPA in 4/5 patients. Coronary angiography was performed in 4/5 cases to confirm the diagnosis. We conclude that by thorough clinical assessment along with ECG and CXR, the diagnosis of ALCAPA can be strongly suspected. Echocardiogram can almost always make the diagnosis of ALCAPA and coronary angiography can confirm the diagnosis in rare atypical cases.

Double Compression of Left Atrium and Pulmonary Artery by a Huge Descending Aortic Aneurysm with Intramural Hematoma Mimicking Pulmonary Embolism: A Case Report

Hemodynamic instability secondary to left atrial (LA) compression by an aortic aneurysm is a rare entity. We report?the case of a 43-year old woman with no previous diagnosis of congestive heart failure who was admitted for an initial diagnosis of pulmonary embolism (PE) based on shortness of breath, hypotension and D-Dimers?elevation. The electrocardiogram and blood counts were within normal limits. The chest X-ray revealed widening of the mediastinum. Transthoracic echocardiography demonstrated LA compression by a large descending thoracic aortic aneurysm. Left and right ventricle systolic functions were preserved. Chest angiography showed LA and left pulmonary artery (LPA) compression by a descending aortic aneurysm and an intramural hematoma with no evidence of PE evidence. Emergency surgery could not be done because of her financial status. She was treated medically and was discharged 1?week later with significant improvement. However she remained hypotensive.

Device closure of fistula from left lower pulmonary artery to left atrium using a vascular plug:A case report

BACKGROUND Pulmonary artery-to-left atrial fistula is a variant of pulmonary arteriovenous fistula and is a developmental anomaly.Delayed presentation,cyanosis and effort intolerance are some of the important features.The diagnosis is confirmed by computed tomography or pulmonary artery angiography.Catheter-based closure is preferred to surgery.CASE SUMMARY Left pulmonary artery-to-left atrial fistula is rare.A 40-year-old male presented with effort intolerance,central cyanosis,and recurrent seizures.He had a large and highly tortuous left pulmonary artery-to-left atrial fistula associated with a large aneurysmal sac in the course.Catheter-based closure was performed using a vascular plug.CONCLUSION Left pulmonary artery-to-left atrial fistula is relatively uncommon compared to right pulmonary artery-to-left atrial fistula.Percutaneous closure by either a transeptal technique or guide wire insertion into the pulmonary vein through the pulmonary artery is preferred.The need for an arteriovenous loop depends on the tortuosity of the course of the fistula and the size of the device to be implanted because a larger device needs a larger sheath,necessitating firm guide wire support to facilitate negotiation of the stiff combination of the delivery sheath and dilator.

A Case of Pediatric Heart Failure Caused by Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery: Case Report and Literature Review

A female patient aged 3 months and 10 days was admitted to the cardiology department because of symptoms of heart failure.According to the echocardiography results,the patient received a diagnosis of primary endocardial fi broelastosis and was treated withγ-globulin,prednisone,digoxin,and diuretics.Coronary computed tomographic angiography and coronary angiography were performed as there was no improvement after 2 months of treatment.Finally,the patient received a diagnosis of anomalous origin of the left coronary artery from the pulmonary artery(ALCAPA).ALCAPA is a rare congenital heart defect that can cause severe heart failure during infancy,and is easily misdiagnosed clinically.In this report,we show the process of misdiagnosis of the case and consult the relevant literature,hoping to improve the understanding and early diagnosis of ALCAPA.

Prognostic value of the echocardiographic right/left ventricular end-diastolic diameter ratio in patients with idiopathic pulmonary arterial hypertension

Background Previous studies have shown that an echocardiographic right/left ventricular end-diastolic diameter ratio(RV/LV ratio)≥0.9 is an independent predictor of poor prognosis in patients with acute pulmonary embolism. The prognostic value of the RV/LV ratio in patients with idiopathic pulmonary arterial hypertension(IPAH) is still unknown. Methods We retrospectively enrolled 95 consecutive patients with newly diagnosed IPAH and 16 of them were reevaluated by echocardiography at 3-12 months following targeted therapy.Follow-up data were obtained by telephone interviews and review of the patients’ records.Results The RV/LV ratio was in parallel with the severity of World Health Orgnization(WHO) functional class and mean right atrial pressure.The RV/LV ratio was positively correlated with total pulmonary resistance(P P P 2 saturation(P P = 0.001),weight and absence of targeted therapy were independent predictors of death.No significant changes in the RV/LV ratio before and after targeted therapy were observed. A baseline RV/LV ratio≥0.84 or a further increase in the RV/LV ratio during targeted therapy indicated a poor prognosis. Conclusions The RV/LV ratio helps to assess the severity of IPAH and serves as an independent predictor of prognosis in patients with IPAH.

Treatment with neurohormonal inhibitors and prognostic outcome in pulmonary arterial hypertension with risk factors for left heart disease

BACKGROUND Despite major advances in pharmacologic treatment,patients with pulmonary arterial hypertension(PAH)still have a considerably reduced life expectancy.In this context,chronic hyperactivity of the neurohormonal axis has been shown to be detrimental in PAH,thus providing novel insights on the role of neurohormonal blockade as a potential therapeutic target.AIM To evaluate the application and prognostic effect of neurohormonal inhibitors(NEUi)in a single-center sample of patients with idiopathic PAH and risk factors for left heart disease.METHODS We analyzed data retrospectively collected from our register of right heart catheterizations performed consecutively from January 1,2005 to October 31,2018.Patients on beta-blocker,angiotensin-converting enzyme inhibitor,angiotensin receptor blocker or mineralocorticoid receptor antagonist at the time of right heart catheterization were classified as NEUi users and compared to NEUi nonrecipients.RESULTS Complete data were available for 57 PAH subjects:27 of those(47.4%)were taking at least one NEUi at the time of right heart catheterization and were compared with the remaining 36 NEUi non-recipients.NEUi users were older and had a higher cardiovascular risk profile compared to non-recipients.Additionally,NEUi non-users had a higher probability of dying during the course of follow-up than NEUi recipients(56.7%vs 25.9%,log-rank P=0.020).CONCLUSION The above data highlighted a subgroup of patients with PAH and comorbidities for left heart disease in which NEUi use has shown to be associated with improved survival.Future prospective studies are needed to identify the most appropriate therapeutic strategies in this subset population.

Ambulatory pulmonary artery pressure monitoring in advanced heart failure patients

Heart failure(HF) is an emerging epidemic associate with significant morbidity,mortality,and health care expenditure. Although there were major advances in pharmacologic and device based therapies for the management of HF,mortality of this condition remains high. Accurate monitoring of HF patients for exacerbations is very important to reduce recurrent hospitalizations and its associated complications. With the failure of clinical signs,tele-monitoring,and laboratory bio-markers to function as early markers of HF exacerbations,more sophisticated techniques were sought to accurately predict the circulatory status in HF patients in order to execute timely pharmacological intervention to reduce frequent hospitalizations. Cardio MEMSTM(St. Jude Medical,Inc.,Saint Paul,Minnesota) is an implantable,wireless pulmonary arterial pressure(PAP) monitoring system which transmits the patient's continuous PAPs to the treating health care provider in the ambulatory setting. PAP-guided medical therapy modification has been shown to significantly reduce HF-related hospitalization and overall mortality. In advanced stages of HF,wireless access to hemodynamic information correlated with earlier left ventricular assist device implantation and shorter time to heart transplantation.

7.0 T MRI评估青藏高原环境下亚硒酸钠改善大鼠肺动脉高压后左心室功能的初步研究

目的利用心脏磁共振(cardiac magnetic resonance,CMR)组织追踪技术评估高原低氧环境下亚硒酸钠(sodium selenite,SE)对肺动脉高压(pulmonary arterial hypertension,PAH)后左心室功能的改善作用并初步探索SE提升PAH后左心室功能的潜在机制。材料与方法46只雄性SD大鼠于购置第二日从成都(海拔500 m)陆运至青海省玉树藏族自治州高原动物实验室(海拔4250 m),随机分为对照组(n=10)、模型组[野百合碱(monocrotaline,MCT)组,n=20]和治疗组(SE组,n=16)。高原低氧环境下饲养28周后,MCT组和SE组大鼠均接受一次性腹腔注射60 mg/kg的MCT以建立PAH模型,而对照组则接受等量的生理盐水注射。一周后,SE组大鼠通过灌胃方式给予0.7 mg/kg的SE持续治疗一个月,对照组和MCT组进行常规饲养。干预完成后将大鼠运回成都。随机从三组中各选取8只大鼠进行CMR成像,以评估左心室功能、应变和T2弛豫时间。CMR扫描结束后取材大鼠心脏组织和血液分别进行病理、血生化检测。结果相较于对照组,MCT组的左心室射血分数(left ventricular ejection fraction,LVEF)(61.36%±4.50%)和左心室整体周向应变(left ventricular global circumferential strain,LVGCS)(−19.81%±0.84%)显著降低(P值均<0.05)。然而,与MCT组相比,SE组的LVEF(75.29%±5.67%)、左心室整体径向应变(left ventricular global radial strain,LVGRS)(42.90%±5.94%)和LVGCS(−21.43%±1.33%)明显提高(P值均<0.05),表明SE治疗提高了PAH后左心室功能。对照组和MCT组血清中谷胱甘肽过氧化物酶(glutathione peroxidase,GSH-Px)的含量差异具有统计学意义[对照组vs.MCT组:(16544.38±3734.02)U/mL vs.(9974.00±900.80)U/mL,P<0.05],MCT组血清中丙二醛(malondialdehyde,MDA)相较于对照组有所增加[MCT组vs.对照组:9.00(7.60,13.20)μmol/L vs.3.86(3.60,6.20)μmol/L,P<0.01],提示MCT组大鼠抗氧化能力下降。经SE干预后,SE组的大鼠表现出血清中超氧化物歧化酶(superoxide dismutase,SOD)(292.60±44.38)U/mL和GSH-Px(17843.26±3585.44)U/mL水平的升高以及MDA[5.37(5.10,6.20)μmol/L]水平的降低(P值均<0.05)。凋亡染色表明,与对照组相比,MCT组左心室的相对荧光强度显著增高(P<0.001),而治疗后SE组的相对荧光强度较MCT组明显降低(P<0.001)。结论CMR组织追踪技术能够定量评估肺动脉高压后左心室功能的异常;SE在高原低氧环境下能改善PAH后左心室功能,其机制可能与其提高大鼠抗氧化能力、减轻心肌细胞凋亡有关。

Correlation analysis between lung ultrasound scores and pulmonary arterial systolic pressure in patients with acute heart failure admitted to the emergency intensive care unit

Background:No convenient,inexpensive,and non-invasive screening tools exist to identify pulmonary hypertension(PH)-left heart disease(LHD)patients during the early stages of the disease course.This study investigated whether different methods of lung ultrasound(LUS)could be used for the initial investigation of PH-LHD.Methods:This was a single-center prospective observational study which was performed in the Zigong Fourth People’s Hospital.We consecutively enrolled patients with heart failure(HF)admitted to the emergency intensive care unit from January 2018 to May 2020.Transthoracic echocardiography and LUS were performed within 24 h before discharge.We used the Spearman coefficient for correlation analysis between ultrasound scores and pulmonary arterial systolic pressure(PASP).Bland-Altman plots were generated to inspect possible bias,and receiver operating characteristic(ROC)curves were calculated to assess the relationship between ultrasound scores and an intermediate and high echocardiographic probability of PH-LHD.Results:Seventy-one patients were enrolled in this study,with an overall median age of 79(interquartile range:71.5–84.0)years.Among the 71 patients,36(50.7%)cases were male,and 26(36.6%)had an intermediate and high echocardiographic probability of PH.All four LUS scores in patients with an intermediate and high probability of PH were significantly higher than in patients with a low probability of PH(P<0.05).The correlation coefficient(r)between different LUS scoring methods and PASP was moderate for the 6-zone(r=0.455,P<0.001),8-zone(r=0.385,P=0.001),12-zone(r=0.587,P<0.001),and 28-zone(r=0.535,P<0.001)methods.In Bland-Altman plots,each of the four LUS scoring methods had a good agreement with PASP(P<0.001).The 8-zone and 12-zone methods showed moderately accurate discriminative values in differentiating patients with an intermediate and high echocardiographic probability of PH(P<0.05).

超声心动图评价动脉性肺动脉高压患者左室功能的研究进展

动脉性肺动脉高压(PAH)是由肺血管重塑导致肺动脉压力升高的进展性疾病,属于第一类肺动脉高压。其主要表现为肺血管阻力进行性增高进而出现右心衰竭,且因双心室依赖性和血流动力学等因素,左心功能亦会受到影响。近年来,超声心动图凭借实时性、无辐射和可重复性等优点,已成为临床上检查心脏结构与功能异常的重要检查。随着斑点追踪成像、压力应变环和三维超声心动图等新技术的日渐成熟,超声心动图对心肌功能的分析更加便捷、准确。本文就超声心动图新技术在评价PAH患者左室功能中的研究进展进行综述。

重度肺动脉高压患者的左心室射血分数及NT-ProBNP与预后相关

目的探讨重度肺动脉高压(PAH)患者超声心动图测量左心功能相关参数与预后的关系。方法回顾性分析2018年10月~2021年12月于中国人民解放军北部战区总医院先心病内科住院治疗的55例第一大类PAH患者(不包括艾森曼格综合征)及慢性血栓栓塞性肺动脉高压。并依据随访中是否出现临床恶化事件分为临床恶化组(n=20)与无临床恶化组(n=35),采用单因素和多因素COX比例风险回归模型、Kaplan-Meier生存曲线分析重度PAH患者超声心动图测量左心功能参数与预后的关系。结果55例患者中有20例出现临床恶化事件,其中2例(4%)发生全因死亡,10例(18%)因PAH恶化再住院,8例(14%)治疗升级。与无临床恶化组相比,临床恶化组的右心室/左心室内径比值、右心室内径与LVEF更大(均P<0.01),每搏量指数与左心室舒张末期内径更小(均P<0.05),血清总胆红素(P<0.05)与NT-ProBNP更高(均P<0.01)。多因素Cox风险回归分析表明:LVEF[HR 1.66(1.29~2.14);P<0.01]及NT-ProBNP[HR 1.001(1.000~1.002);P<0.05]是重度PAH患者预后的独立预测因素。受试者工作特征曲线表明LVEF 71%和NT-ProBNP 724.5 pg/ml是最佳截点值,灵敏度及特异度均较高。Kaplan-Meier生存分析显示LVEF>71%者及NT-ProBNP>724.5 pg/ml的临床恶化发生率均显著升高(均P<0.01)。结论超声心动图测量LVEF及NT-ProBNP可很好预测重度PAH患者的临床恶化事件发生率。

高原地区婴幼儿完全型房室间隔缺损的外科处理策略及随访结果

目的 评估高原地区婴幼儿合并肺动脉高压(PAH)完全型房室间隔缺损(cAVSD)患儿的外科处理策略及随访结果。方法 选择2009年12月~2021年12月期间28例2岁以下cAVSD合并PAH的患儿进行一期矫治,其中男15例,女13例,年龄(2.5~21.0)月,体质量(2.5~11.0)kg,其中合并中度及以上肺动脉高压患者15例,设为A组。合并轻度及正常肺动脉压患者13例,设为B组。对两组患者的ICU住院时间、呼吸机带管时间、主动脉阻闭时间、整体住院时间、死亡率等指标进行对比,并进行随访。术中采用改良单片法3例,双片法25例,术中直接缝合左侧房室瓣瓣裂孔17例,裂孔补片加宽及腱索移植1例,行左房室瓣后瓣瓣环成形3例。结果 两组患儿的年龄、体质量、Rastelli分型、唐氏综合征、共同瓣返流和室间隔缺损大小数据值均无统计学差异。与B组比较,A组患儿呼吸机带管时间明显变长(P<0.05),其他指标如ICU住院时间、整体住院时间、主动脉阻闭时间和病死率均无明显差异。与A组比较,B组轻度左侧房室瓣返流比例较低(P<0.05),其余项目室间隔残余分流、中重度左侧房室瓣返流、右侧房室瓣返流、完全性房室传导阻滞与术后早期死亡均无明显差异。术后随访所有患儿的随访时间为(8±3)年,心脏超声心动图随访统计结果为:二尖瓣关闭不全轻度18例、中度5例、重度2例,三尖瓣轻度关闭不全19例、中度4例、重度2例,2例重度二尖瓣关闭不全患者均接受再次瓣膜修复/置换术,全组存活病例均无左室流出道狭窄,三尖瓣重度反流1例、中度反流2例。A组的三例死亡病例根据患者在监护室最后一次超声评估结果为二尖瓣重度反流1例、中度反流1例、轻度反流1例。结论 高原地区的婴幼儿完全性房室间隔缺损患儿,其肺血管病变早、肺动脉高压进展快,应尽早进行手术治疗,手术成功的关键是避免三度房室传导阻滞及瓣膜成型的确切效果,左侧房室瓣关闭不全的手术修复效果是导致术后死亡及中远期再次手术干预的重要原因。

体外膜氧合成功救治左冠状动脉异常起源于肺动脉术后低心排血量综合征患儿1例

左冠状动脉异常起源于肺动脉(anomalous left coronary artery origin from pulmonary artery,ALCAPA)是一种发病率低、预后较差的先天性心脏畸形,常在婴儿期需要外科手术矫治。低心排血量综合征是指各种原因导致心排出量降低的一种病理异常表现,是心脏术后导致患者死亡的主要原因之一。体外膜氧合(extracorporeal membrane oxygenation,ECMO)是一种可以临时替代心肺功能的装置,在重症及急救领域应用广泛,但在ALCAPA术中及术后应用相关经验报道较少,本文分享一例ECMO成功救治ALCAPA术后低心排血量综合征的案例。

Diagnosis and treatment of heart failure with preserved left ventricular ejection fraction

Nearly six million people in United States have heart failure.Fifty percent of these people have normal left ventricular(LV)systolic heart function but abnormal diastolic function due to increased LV myocardial stiffness.Most commonly,these patients are elderly women with hypertension,ischemic heart disease,atrial fibrillation,obesity,diabetes mellitus,renal disease,or obstructive lung disease.The annual mortality rate of these patients is 8%-12%per year.The diagnosis is based on the history,physical examination,laboratory data,echocardiography,and,when necessary,by cardiac catheterization.Patients with obesity,hypertension,atrial fibrillation,and volume overload require weight reduction,an exercise program,aggressive control of blood pressure and heart rate,and diuretics.Miniature devices inserted into patients for pulmonary artery pressure monitoring provide early warning of increased pulmonary pressure and congestion.If significant coronary heart disease is present,coronary revascularization should be considered.

左冠状动脉异常起源于肺动脉综合征:临床表现及CTA影像学特征

目的:左冠状动脉异常起源于肺动脉(anomalous left coronary artery from the pulmonary artery,ALCAPA)综合征是一种罕见的先天性冠状动脉畸形,临床漏诊及误诊率高,目前鲜见ALCAPA综合征的临床表现及冠状动脉CT血管成像(computed tomography angiography,CTA)影像特点的相关报道,本研究旨在总结ALCAPA综合征的临床表现及冠状动脉CTA影像特征,提升临床诊疗水平。方法:回顾性收集2013年1月1日至2023年2月1日中南大学湘雅二医院收治的24例ALCAPA综合征患者的临床资料,根据左、右冠状动脉侧支循环情况分为婴儿型(7例)和成人型(17例),比较两型患者的临床表现及CTA影像特点。结果:24例ALCAPA综合征患者的男女比例为1꞉5,婴儿型中位发病年龄为3.22个月,成人型中位发病年龄为22.0岁。婴儿型早期即出现左心功能不全的症状;而成人型临床表现多样,7例患者无症状因体检发现心脏杂音而就诊,6例患者出现慢性心肌缺血症状,2例患者出现恶性心律失常。CTA示11例患者左冠状动脉起源于肺动脉左后窦;7例婴儿型患者均出现左心扩大、左室肥厚、左心功能下降,左、右冠状动脉间缺乏侧支循环,冠状动脉管径正常。17例成人型患者均出现冠状动脉扩张、迂曲及发达的侧支循环;7例成人型患者左心功能保留。结论:两型ALCAPA患者的临床及影像学特点不同,CTA表现具有特征性,可作为ALCAPA综合征明确诊断、分型、手术评估及术后随访的手段。