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Penyakit Takayashu Penyakit Takayashu merupakan bentuk penyakit panarteritis tidak spesifik , yang melibatkan aorta torakalis dan

aorta abdominalis dgn oklusi segmental cabang-cabang besar brakhiosefalika dan visceral. Umumnya yang terkena penyakit ini adalah perempuan umur 20-40 tahun. Etiologi penyakit ini tidak diketahui. Kaichiro Ishikawa (1976) menyebutkan nama lain yaitu , Oclussive Thromboaortopathy (Otap) dan membeaginya dalam 3 klasifikasi, yaitu : I. II. Otap tanpa komplikasi, dgn atau tanpa terkena a.pulmonalis Otap dgn mono komplikasi , yg dibagi 2 yaitu : a. IIa : monokomplikasi tidk parah (bentuk ringan dan sedang) b. IIb : mono komplikasi parah Otap dgn multi komplikasi

III.

Riwayat alamiah penyakit ini pada stadium I umumnya baik dimana dapat terjadi penyembuhan spontan bila diagnosis dan terapi dilakukan secara dini. Pada stadium IIa dan IIb perjalanan penyakit cukup progresifdimana aktifitas menurun dgn sekuele sirkulasi. Prognosis pada stadium III dalam banyak hal buruk dan perjalanan penyakitnya progresif. Selain itu juga terjadi penurunan aktifitas dengan sekuele sirkulasi.
Takayasu arteritis is a chronic vasculitis of unknown etiology that targets the aorta and its major branches. The estimated annual incidence is 1.2 to 2.6 cases per million. Between 80% and 90% of affected persons are women, with onset typically between the ages of 10 and 40. Most reported cases have been from Asia and Africa, but it is a worldwide disease. Patients typically present with systemic complaints suchas malaise and fever; focal symptoms are related to inflammation of the affected vessel and include cerebrovascular ischemia (brachiocephalic or carotid artery involvement), myocardial ischemia (coronary artery), arm claudication (brachiocephalic or subclavian artery), or hypertension (renal artery). The carotid and limb pulses are diminished or absent in nearly 85% of patients at the time of diagnosis; hence, this condition is often termed “pulseless” disease. Takayasu arteritis is also an uncommon cause of aortic aneurysm or aortic dissection. Histologic examination of affected vessels reveals continuous or patchy granulomatous infl ammation with lymphocytes, histiocytes, and multinucleated giant cells, resulting in intimal proliferation, disruption of the elastic lamina, and fi brosis. Antiendothelial antibodies may also play a role in the disease. Steroid and cytotoxic drugs may reduce vascular infl ammation and alleviate symptoms of Takayasu arteritis. Surgical bypass of obstructed vessels may be helpful in severe cases. The 5-year survival rate is 80% to 90%.

Aneurisma adalah pelebarah dinding arteri dapat sendiri-sendiri atau multiple. Dasarnya adalah akibat lemahnya dinding pembuluh arteri secara congenital ataupun didapat. Penyebab utama yg didapat adalah arteriosklerosis , dimana akibat trauma atau peradangan menimbulkan kerusakan dinding pembuluh arteri

15. is a contained rupture of the vessel wall that develops when blood leaks out of the vessel lumen through a hole in the intimal and medial layers and is contained by a layer of adventitia or perivascular organized thrombus (see Fig. depending on the extent of the vessel’s circumference within the aneurysm (see Fig.Aortic Aneurysms An aneurysm is an abnormal localized dilatation of an artery. Pseudoaneurysms develop at sites of vessel injury caused by infection or trauma. The term aneurysm is applied when the diameter of a portion of the aorta has increased by at least 50% compared with normal. or involve both locations. the more common type. or false aneurysm. Ascending thoracic aortic aneurysms typically are characterized by cystic medial degeneration (also termed cystic medial necrosis).1). is characterized by symmetrical dilation of the entire circumference of a segment of the aorta. creating a large bulge of the vessel wall. with subsequent accumulation of collagenous and mucoid material within the medial layer.1). A saccular aneurysm is a localized outpouching involving only a portion of the circumference. which is a generalized yet lesser increase of the aortic diameter. A true aneurysm represents a dilatation of all three layers of the aorta. True aneurysms are characterized as either fusiform or saccular. aneurysms are distinguished from diffuse ectasia. Etiology and Pathogenesis of True Aortic Aneurysms The etiology of aortic aneurysm formation varies depending on the location of the lesion. 15. A fusiform aneurysm. In the aorta. such as puncture of the vessel during surgery or percutaneous catheterization. . Aneurysms may be confi ned to the abdominal aorta (most common). They are very unstable and are prone to rupture completely. smooth muscle cells decrease in number. the thoracic aorta. a pseudoaneurysm. They may also appear in peripheral and cerebral arteries. a condition of degeneration and fragmentation of elastic fibers. and acid mucopolysaccharide ground substance accumulates within the vessel wall. In contrast. Ectasia develops in older patients as elastic fiber fragments.

Some patients may be candidates for minimally invasive repair. and advanced age. In general. Angiotensin II receptor antagonists. surgical treatment is considered for ascending aortic aneurysms _5.0 cm. its dimensions should be closely monitored through repeated imaging every 6 to 12 months. _-Blockers may reduce the expansion rate of thoracic aortic aneurysms in patients with Marfan syndrome. Lilly. in which a transluminally placed endovascular stent graft is positioned across the aneurysm.. especially those with abdominal aortic aneurysms. Patients with aneurysms involving multipleaortic segments have staged repairs.Aneurysms of the descending thoracic and abdominal aorta are usually associated with atherosclerosis and its risk factors. and for smaller aneurysms that enlarge at a rate _1. Ascending aortic aneurysms in patients with Marfan syndrome (in whom the risk of complications is greater) should be considered for surgical repair if the diameter is _5 cm.5 to 6. male gender. Treatment Treatment of an aortic aneurysm is based on its size and the patient’s overall medical condition. Aneurysms of the abdominal aorta or of the large peripheral arteries may also be discovered by careful palpation during physical examination. Once an aneurys m is identifi ed. or magnetic resonance angiography. hypertension. smoking cessation). resulting in cough. The diagnosis is confi rmed by ultrasonography. are undergoing clinical trials in Marfan syndrome. Surgical repair of abdominal aortic aneurysms involves placement of a prosthetic graft. for abdominal aortic aneurysms measuring 5. which also inhibit TGF-_. resulting in aortic regurgitation and symptoms of congestive heart failure. Surgical repair is generally recommended for descending thoracic aortic aneurysms measuring 6. is currently recommended for patients with small aneurysms. Patients are maintained on cardiopulmonary bypass as the aneurysm is resected and replaced with a prosthetic Dacron graft. The operative mortality for such procedures at high-volume institutions is 1% to 2%. Aneurysms of the ascending aorta may dilate the aortic ring. may be aware of a pulsatile mass. The mortality associated with elective surgical repair of thoracic aortic aneurysms is 3% to 5%. Referensi Buku Ajar Kardiologi UI Pathophysiology of Heart Disease FIFTH EDITION.Editor Leonard S. Abdominal aortic aneurysms may cause abdominal or back pain or nonspecifi c gastrointestinal symptoms. and involvement of the recurrent laryngeal nerve may lead to hoarseness. Compression of the esophagus can result in dysphagia. Thoracic aortic aneurysms may compress the trachea or mainstem bronchus.5 to 7. in which one segment is corrected at a time. Clinical Presentation and Diagnosis Most aneurysms are asymptomatic. and results appear to be similar to that of surgical repair. contrast-enhanced computed tomography (CT). .0 cm.g.0 cm_year. dyspnea. including riskfactor reduction (e. including smoking. or pneumonia. Aortic aneurysms are often fi rst suspected when dilatation of the vessel is observed on chest or abdominal radiographs.5 cm or more. Others present with symptoms related to compression of neighboring structures by an expanding aneurysm. particularly if the wall is calcifi ed. it is not clear whether they are effective for other causes or types of aneurysms. A Collaborative Project of Medical Students and Faculty. dyslipidemia. Percutaneous endovascular repair of infrarenal abdominal aortic aneurysms with stent grafts can be performed in selected patients with less acute morbidity. though some patients. Medical management.