1、自動(dòng)脈弓離斷合并自動(dòng)脈弓離斷合并IIIIII型自型自動(dòng)脈夾層動(dòng)脈夾層1 1例例吉林市中心醫(yī)院 黃侖 陳丹 周菲 男性患者，男性患者，4545歲。因突發(fā)胸歲。因突發(fā)胸骨后撕裂樣劇痛伴大汗骨后撕裂樣劇痛伴大汗9 9小時(shí)小時(shí) 于于20212021年年4 4月月5 5日日2323時(shí)急診時(shí)急診入院。入院。 發(fā)現(xiàn)血壓增高發(fā)現(xiàn)血壓增高5 5年，血壓最高達(dá)年，血壓最高達(dá)“180/110mmHg180/110mmHg 。 1010年前診斷為年前診斷為“胃潰瘍，經(jīng)治療好轉(zhuǎn)，胃潰瘍，經(jīng)治療好轉(zhuǎn)， 現(xiàn)無明顯胃部不適?，F(xiàn)無明顯胃部不適。 否認(rèn)糖尿病、高脂血癥病史。否認(rèn)糖尿病、高脂血癥病史。 吸煙史吸煙史1010年，每天

2、年，每天2020支。支。 重要體征：血壓180/100mmHg，急性病容，口唇無發(fā)紺，頸靜脈無怒張，雙肺呼吸音清，未聞及干濕羅音。心界不大，心率75次/分，節(jié)律規(guī)整，L3-L4聞及4/6全收縮期吹風(fēng)樣粗糙雜音，掩蓋第一心音，無心包摩擦音。腹軟，肝脾肋下未觸及，雙下肢無水腫。 橈動(dòng)脈搏動(dòng)良好，雙側(cè)股、腘、脛后及足背橈動(dòng)脈搏動(dòng)良好，雙側(cè)股、腘、脛后及足背動(dòng)脈搏動(dòng)減弱，四肢末梢暖。雙側(cè)各大動(dòng)脈動(dòng)脈搏動(dòng)減弱，四肢末梢暖。雙側(cè)各大動(dòng)脈區(qū)頸、腋、腎、腹自動(dòng)脈區(qū)頸、腋、腎、腹自動(dòng)脈 無血管雜音，無血管雜音，雙側(cè)股動(dòng)脈區(qū)可聞及雙側(cè)股動(dòng)脈區(qū)可聞及2/62/6級(jí)級(jí)SMSM血管雜音，血管雜音，雙上肢肱動(dòng)脈血壓雙上肢

3、肱動(dòng)脈血壓180/100mmHg180/100mmHg，雙下，雙下肢腘動(dòng)脈血壓肢腘動(dòng)脈血壓110/70mmHg 110/70mmHg ，無周圍血，無周圍血管征。管征。 。 冠狀動(dòng)脈粥樣硬化性心臟病 急性前間壁心肌梗死？ Killip分級(jí)級(jí) 自動(dòng)脈夾層？ 高血壓3級(jí)極高危組 給予止痛、鎮(zhèn)靜對(duì)癥處置給予止痛、鎮(zhèn)靜對(duì)癥處置 進(jìn)一步提檢心肌酶、肌鈣蛋白進(jìn)一步提檢心肌酶、肌鈣蛋白 、血常規(guī)、凝血常規(guī)、血?dú)夥治鲅Ｒ?guī)、凝血常規(guī)、血?dú)夥治?胸胸片片 、心臟彩色、心臟彩色Dopple Dopple 、自動(dòng)脈、自動(dòng)脈CTACTA 型自動(dòng)脈夾層動(dòng)脈瘤型自動(dòng)脈夾層動(dòng)脈瘤 De BaKey De BaKey 分型分型

4、 自動(dòng)脈弓離斷自動(dòng)脈弓離斷A A型型 高血壓高血壓3 3級(jí)極高危組級(jí)極高危組 討論討論1 1、自動(dòng)脈弓離斷的診斷能否確立？、自動(dòng)脈弓離斷的診斷能否確立？2 2、自動(dòng)脈弓離斷與胸降自動(dòng)脈夾層能否有、自動(dòng)脈弓離斷與胸降自動(dòng)脈夾層能否有因果關(guān)系？因果關(guān)系？3 3、能否同時(shí)合并、能否同時(shí)合并VSDVSD胸骨左緣雜音出現(xiàn)的胸骨左緣雜音出現(xiàn)的緣由？緣由？ 1 1、自動(dòng)脈弓離斷的診斷、自動(dòng)脈弓離斷的診斷 能否確立？能否確立？Prevalence 1% of all congenital heart disease 1.3% of critical congenital heart disease 0.003

5、 per 1000 livebirths Aortic arch usually severe clinical symptoms, 80% of the newborns died of heart failure in January, survived to an age of less than 10% Prevalence Associated anomaly PDA and VSD (90%) bicuspid aortic valve ( 60% ) subaortic stenosis (20% ) valvey deformit (10%) deformit truncus

6、arteriosus (10%)Interruption of the ortic ArchClassificationType AType BType CInterruption of the ortic ArchClassificationDiagnosis Clinical features Chest X ray Echocardiography Angiography MR CTscanClinical features 1. Most of interrupted aortic arch symptoms of sick children at the early stage af

7、ter birth, there congestive heart failure, dyspnea, oliguria acidosis.2. Signs purple to the difference. limbs, pulse and blood pressure are not equal. heart murmur. ECG Right ventricular hypertrophy ST-T wave abnormalities QT prolongChest X ray Cardiomegaly increased pulmonary vascular markings, an

8、d pulmonary venous congestion or pulmonary edema Narrow upper mediastinum or increase cardiothymic silhouette: absence of the thymusEchocardiography Diagnostic for IAA. the ascending aorta follows a straight course to its branches without the normal continuous curvature to the descending aorta. The

9、V sign: type B. The W sign: type A.Cardiac catheterization Accurate diagnosis Site of interruption Size and type of VSD Whether the right subclavian artery is aberrantCTscan2 2、胸降自動(dòng)脈夾層的診斷、胸降自動(dòng)脈夾層的診斷 能否確立？能否確立？pathophysiology 出現(xiàn)夾層撕裂影響器官供血的表現(xiàn)。出現(xiàn)夾層撕裂影響器官供血的表現(xiàn)。pathophysiology Smoking Family history Hype

10、rlipidemia Left ventricular hypertrophy Hypertension Cocaine Age 疼痛特點(diǎn)疼痛特點(diǎn)Acute Chest Pain:Acute - sudden or recent onset (usually within minutes to hours), presenting typically 24 hrsChest - thorax midaxillary to midaxillary line, xiphoid to suprasternum notchPain noxious uncomfortable sensationAche

11、 or discomfort TriageChest pain, Quality radiation/migration, severity, onset, duration, frequency, progression and provoking or relieving factors of painSignificant abnormal pulseAbnormal blood pressureDyspnea EvaluationAirwayBreathingCirculationVital SignsFocused examCardiac, pulmonary, vascular C

12、hest wall - Costosternal synd Costochrondritis - Precordial catch synd Slipping Rib Synd Xiphodynia- Radicular Synd Intercostal Nerve Fibromyalgia Pleuritic- Pulmonary Embolism- Pneumonia- Spontaneous pneumo- Pericarditis- Pleurisy Visceral Pain- Typical Exertional Angina Atypical Angina Unstable An

13、gina- Acute Myocardial Infarction (AMI)- Aortic Dissection- Pericarditis- Esophageal Reflux or spasm Esophageal Rupture- Mitral Valve Prolapse疼痛特點(diǎn)疼痛特點(diǎn) 1 1、中年男性，高血壓病史、中年男性，高血壓病史5 5年，血壓最高達(dá)年，血壓最高達(dá)“180/110mmHg“180/110mmHg ， 未系統(tǒng)降壓，平素血壓動(dòng)搖大。吸煙史未系統(tǒng)降壓，平素血壓動(dòng)搖大。吸煙史1010年。年。 2 2、負(fù)重誘發(fā)突發(fā)性胸骨后劇痛。、負(fù)重誘發(fā)突發(fā)性胸骨后劇痛。 3 3、疼痛呈撕裂樣，較為銳利，疼痛開場(chǎng)即到達(dá)頂峰，、疼痛呈撕裂樣，較為銳利，疼痛開場(chǎng)即到達(dá)頂峰， 有向后背穿透感。運(yùn)用有向后背穿透感。運(yùn)用“ “硝酸酯類藥物疼痛不緩解。硝酸酯類藥物疼痛不緩解。 4 4、查體：血壓、查體：血壓180/110mmHg180/110mmHg。 5 5、自動(dòng)脈、自動(dòng)脈CTACTA提示胸自動(dòng)脈起