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1 二尖瓣脫垂與自律神經疾病二尖 ( 僧帽 ) 瓣脫垂台中榮民總醫院心血管中心何鴻鋆醫師解剖位置僧帽瓣 ( 又稱二尖瓣 ) 位於心臟左心房及左心室間, 心舒張期時, 血液由左心房流向左心室, 心收縮期時, 僧帽瓣則關閉僧帽瓣脫垂僧帽瓣瓣膜或相關器官中組織發生病變, 致使瓣膜鬆大, 鍵索變長等變化原因 : 多為原發的, 即先天造成由於上述之病理變化, 致使僧帽瓣在心收縮期時, 突向左心房側正常僧帽瓣 ( 圖 1) 僧帽瓣脫垂 ( 圖 2) 1

帽瓣脫垂僧帽瓣瓣膜或相關器官中組織發生病變, 致使瓣膜鬆大, 鍵索變長等變化原因 : 多為原發的, 即先天

2 病理解剖僧帽瓣黏液瘤性 (myxomatous) 變性造成過多黏多醣囤積, 進而使瓣膜與肌腱索拉長. 在左心室正常大小時即可能發生可能在健康女性脫水時產生, 水分補足後消失在懷孕或體重增加後可能消失. 可能與 fibrillin 基因異常有關. 引發症狀相關異常 : 對腎上腺刺激敏感度增加增加 catecholamines 異常乙型接受器增加 atrial natriuretic factor, renin-aldosterone 調節異常減少血管內容量鎂離子缺乏發生率目前僧帽瓣脫垂已是最常見的瓣膜性心臟病發生率約為 2-5% 男女之比為 1:2~3 尤好發於身材瘦長或胸廓畸型者開始症狀年齡 : 歲. 很少在青春期以前發生症狀大部份是没有症狀的, 通常是在醫師為病患聽診時意外發現而診斷少部份有症狀, 臨床表現則相當多樣, 包括了呼吸不順暢非典型心絞痛心悸不整脈緊張焦慮疲倦自律神經失調昏厥等少數病患併發嚴重閉鎖不全及心臟功能變化時, 也會產生心臟衰竭的相關症狀十分罕見的病患, 會以猝死或暫時性腦部缺氧來表現身體檢查發現瘦細體型骨骼異常 ( 如漏斗胸背直脊柱後側凸 ) 休息時心緩脈與姿勢性低血壓心臟聽診收縮期 clicks 心尖聽到 l mid-to-late systolic murmur. Click 與心雜音會隨姿勢改變. 僧帽瓣閉鎖不全自律神經失調神經內分泌功能異常 Ehlers-Danlos 症候群表現 ( 如 : 關節活動異常, 異常 abnormal striae, 淤青, 皮膚炎展能力 ) 骨發生不全 Osteogenesis imperfecta 徵象 ( 如 : 藍色鞏膜 ) Marfan 症候群徵象 : 脊柱側凸背直漏斗胸蜘蛛狀指, 手張開比身高高 Stickler 症候群徵象 : ( 如 : 駝背脊柱側凸 mandibular 發育不全視網膜剝離 ) 死亡率罹病率一般而言是良性疾病主要獨發性僧帽瓣閉鎖不全原因 (MR) 90% chordae tendineae 斷裂原因 40% 年輕型中風 10-15% 心內膜炎 (endocarditis) 有結構異常患者 ( 如僧帽瓣增厚變形過長 ) 較易有併發症 ( 如僧帽瓣閉鎖不全惡化心內膜炎猝死 ). 合併有心雜音一般死亡率增加 15-20%. 檢查診斷方法除了聽診之外, 心臟超音波為診斷僧帽瓣脫垂症候群最有力及準確的工具 2

引發症狀相關異常 : 對腎上腺刺激敏感度增加增加 catecholamines 異常乙型接受器增加 atrial natriuretic factor, renin-aldosterone 調節異常減少血管內容量鎂離子缺乏發生率目前僧帽瓣脫垂已是最常見的瓣膜性心臟病發

3 處理治療原則為針對症狀處理, 無法根治無症狀者, 僧帽瓣脫垂症候群患者應避免熬夜吸菸喝酒及咖啡等較刺激性的飲食一般日常生活及運動没有特別限制對於心悸胸痛不適焦慮交感神經系統亢進或疲倦感較明顯的患者, 使用乙型交感神經阻斷劑及鎮定劑的治療效果不錯少數臨床上懷疑有心律不整的患者可以接受 24 小時心電圖檢查, 如有適應症, 之後亦可安排心臟電氣生理檢查如有心房纖維顫動暫時性腦部缺氧或中風時, 可以使用阿斯匹靈或抗凝血劑來預防栓塞症的再次發生當極少數病患會產生重度僧帽瓣閉鎖不全, 心內膜炎, 複雜性心室心律不整及暫時性腦缺血, 則應由心臟專科醫師做特別處理危險分級臨床表徵處置原則低度危險 : 無收縮期心雜音心臟超音波無僧帽瓣逆流女性 <45 歲對病人說明病情, 無需使用預防性抗生素心超音波每五年追蹤一次中度危險 : 有收縮期心雜音心臟超音波有輕度僧帽瓣逆流需使用預防性抗生素心臟超音波每二至三年追蹤一次高度危險 : 心臟超音波有中重度僧帽瓣逆流需使用預防性抗生素每年追蹤一次心臟超音波如有心衰竭症狀, 考慮手術治療自律神經系統 Autonomic Nervous System 自律神經疾病自律神經系統 (ANS) 以協調方式控制體內環境利用反射機制 (reflex action),ans 控制心跳血壓消化呼吸血中酸鹼平衡等等身體功能 In the ANS there are 2 nerves between the central nervous system (CNS) and the organ. The nerve cell bodies for the second nerve are organized into ganglia: CNS -> Preganglionic nerve -> Ganglion -> Postganglionic nerve -> Organ At each junction neurotransmitters are released and carry the signal to the next nerve or organ. 3

防栓塞症的再次發生當極少數病患會產生重度僧帽瓣閉鎖不全, 心內膜炎, 複雜性心室心律不整及暫時性腦缺血, 則應由心臟專科醫師做特別處理危險分級臨床表徵處置原則低度危險 : 無收縮期心雜音心臟超音波無僧帽瓣逆流女性

4 The ANS has 2 Divisions, Sympathetic and Parasympathetic, Which Differ in Anatomy and Function The sympathetic nerves come from the thoracic and lumbar regions of the spinal cord. The preganglionic nerves are short and synapse in paired ganglia adjacent to the spinal cord The adrenal medulla, technically an endocrine gland, is functionally a part of the sympathetic nervous system Adrenal medulla is like a modified autonomic ganglion + postsynaptic nerve (see diagram below) Parasympathetic nerves come from the cranial and sacral regions of the CNS. They have long preganglionic nerves which synapse at ganglia near or on the organ innervated The 2 branches use the same transmitter, acetylcholine (ACh), in the ganglia. At the organ quite different transmitters are used: Parasympathetic = ACh Sympathetic = norepinephrine (NE). Note: norepinephrine is the same thing as noradrenaline. Note: there are a few sympathetic nerves where the transmitter at the organ is ACh Sympathetic fibers releasing ACh: sweat glands, piloerector muscles, some blood vessels Comparison of the 2 systems The Sympathetic is the "Fight or Flight" Branch of the ANS Emergency situations, where the body needs a sudden burst of energy, are handled by the sympathetic system The sympathetic system increases cardiac output and pulmonary ventilation, routes blood to the muscles, raises blood glucose and slows down digestion, kidney filtration and other functions not needed during emergencies Whole sympathetic system tends to "go off" together In a controlled environment the sympathetic system is not required for life, but it is essential for any stressful situation The Parasympathetic is the "Rest and Digest" Branch of the ANS The parasympathetic system promotes normal maintenance of the body- acquiring building blocks and energy from food and getting rid of the wastes It promotes secretions and mobility of different parts of the digestive tract. Also involved in urination, defecation. Does not "go off" together; activities initiated when appropriate The vagus nerve (cranial number 10) is the chief parasympathetic nerve Other cranial parasympathetic nerves are: III (oculomotor), VII (facial) and IX (glossopharyngeal) The Hypothalamus Has Central Control of the ANS The hypothalamus is involved in the coordination of ANS responses, One section of the hypothalamus seems to control many of the "fight or flight" responses; another section favors "rest and digest" activities 4

system Adrenal medulla is like a modified autonomic ganglion + postsynaptic nerve (see diagram below) Parasympathetic nerves come from the cranial and sacral regions of the CNS.

5 The Adrenal Medulla is an Extension of the Sympathetic Nervous System The adrenal medulla behaves like a combined autonomic ganglion and postsynaptic sympathetic nerve (see diagram above) Releases both norepinephrine and epinephrine in emergency situations Releases a mixture of epinephrine (E = 80%) and norepinephrine (NE = 20%) Epinephrine = adrenaline This action is under control of the hypothalamus Comparison of the Actions if the Sympathetic & Parasympathetic Systems Usually (but not always) both sympathetic and parasympathetic nerves go to an organ and have opposite effects You can predict about 90% of the sympathetic and parasympathetic responses using the 2 phrases: "Fight or Flight" and "Rest and Digest". Special cases: Occasionally the 2 systems work together: in sexual intercourse the parasympathetic promotes erection and the sympathetic produces ejaculation Eye: the sympathetic response is dilation and relaxation of the ciliary muscle for far vision (parasympathetic does the opposite) Urination: the parasympathetic system relaxes the sphincter muscle and promotes contraction of muscles of the bladder wall -> urination (sympathetic blocks urination) Defecation: the parasympathetic system causes relaxation of the anal sphincter and stimulates colon and rectum to contract -> defecation (sympathetic blocks defecation Autonomic nerve symptoms A D G H N V Autonomic neuropathy... autonomic nervous system Dysautonomia... autonomic nervous system Gastroparesis... autonomic nervous system Horner's syndrome... sympathetic nervous system Neuropathy... autonomic nervous system Vascular neuropathy... autonomic nervous system Autonomic neuropathy Disease of the autonomic (involuntary) nervous system. Causes of autonomic neuropathy Diabetes Hereditary sensory and autonomic neuropathy type 2 Hereditary sensory and autonomic neuropathy type 5 Amyloid polyneuropathy (Portuguese) Fabry's disease Achalasia-addisonian syndrome Amyloidosis Bradbury-Eggleston syndrome Paraneoplastic limbic encephalitis Dysautonomia failure of the sympathetic or parasympathetic components of the ANS, but dysautonomia involving excessive ANS activities also can occur. Causes: Guillain-Barre Syndrome Diabetes Alcoholism Multiple system atrophy Familial dysautonomia Autonomic neuropathy 5

hypothalamus Comparison of the Actions if the Sympathetic & Parasympathetic Systems Usually (but not always) both sympathetic and parasympathetic nerves go to an organ and have opposite effects You

6 Gastroparesis Gastroparesis -- causes Slow stomach emptying from stomach nerve damage a disorder in which the stomach takes too long to empty its contents. most often a complication of type 1 diabetes. At least 20% of people with type 1 diabetes develop gastroparesis. It also occurs in people with type 2 diabetes, although less often. Gastroparesis happens when nerves to the stomach are damaged or stop working. If the vagus nerve is damaged, the muscles of the stomach and intestines do not work normally, and the movement of food is slowed or stopped. Functional gastroparesis Type 1 diabetes Type 2 diabetes Gallbladder disease Pancreatitis Postviral syndromes Anorexia nervosa Stomach damage Stomach surgery Vagus nerve surgery Gastroesophageal reflux disease Stomach cancer Smooth muscle disorders Amyloidosis Scleroderma Abdominal migraine Parkinson's disease Metabolic disorders Hypothyroidism Horner's syndrome Horner's syndrome A syndrome associated with defective sympathetic innervation to one side of the face, including the eye. Clinical features include miosis; mild blepharoptosis; and hemifacial anhidrosis (decreased sweating). lesions of the brain stem; cervical spinal cord; first thoracic nerve root; apex of the lung; carotid artery; carvernous sinus; and apex of the orbit may cause this condition. From Miller et al., Clinical Neuro-Ophthalmology, 4th ed, pp A syndrome associated with defective sympathetic innervation to one side of the face, including the eye. Clinical features include miosis; mild blepharoptosis; and hemifacial anhidrosis (decreased sweating). lesions of the brain stem; cervical spinal cord; first thoracic nerve root; apex of the lung; carotid artery; carvernous sinus; and apex of the orbit may cause this condition. From Miller et al., Clinical Neuro-Ophthalmology, 4th ed, pp Horner's syndrome -- causes Neuropathy: Aortic aneurysm, thoracic Neurofibromatosis type 1 Goitre Dissecting aortic aneurysm Thyroid carcinoma Bronchogenic carcinoma Multiple sclerosis Cluster headache Carotid artery dissection Klumpke paralysis Cavernous sinus thrombosis Sympathectomy Posterior inferior cerebellar artery syndrome Syringomyelia Any pathology of the peripheral nerves Causes: Diabetic peripheral neuropathy Diabetes Type 1 diabetes Type 2 diabetes Charcot-Marie-Tooth syndrome 6

Gastroparesis happens when nerves to the stomach are damaged or stop working.

7 Vascular neuropathy Vascular neuropathy is Neuropathy of the autonomic nerves that control the blood vessels, leading them not to properly constrict and expand to control BP. Causes: Diabetic neuropathy Type 1 diabetes Type 2 diabetes Thanks for your attention 7

to properly constrict and expand to control BP.

第一章緒論

第一章緒論第五章實證結果第一節敘述統計表 11 表 12 分別為男女癌症, 實驗組與控制組樣本之基本特性此為罹癌前一年度樣本特性由於我們以罹癌前一年有在就業, 即投保類別符合全民健康保險法中所規定之第一類被保險人, 且年齡介於 35 至