The heart in takayasu arteritis
| Cardiology Network |
 Discuss The heart in takayasu arteritis further in the WikiDoc Cardiology Network |
| Adult Congenital |
|---|
| Biomarkers |
| Cardiac Rehabilitation |
| Congestive Heart Failure |
| CT Angiography |
| Echocardiography |
| Electrophysiology |
| Cardiology General |
| Genetics |
| Health Economics |
| Hypertension |
| Interventional Cardiology |
| MRI |
| Nuclear Cardiology |
| Peripheral Arterial Disease |
| Prevention |
| Public Policy |
| Pulmonary Embolism |
| Stable Angina |
| Valvular Heart Disease |
| Vascular Medicine |
Template:WikiDoc Cardiology News Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Associate Editor: Cafer Zorkun, M.D., Ph.D. [2]
Please Join in Editing This Page and Apply to be an Editor-In-Chief for this topic: There can be one or more than one Editor-In-Chief. You may also apply to be an Associate Editor-In-Chief of one of the subtopics below. Please mail us [3] to indicate your interest in serving either as an Editor-In-Chief of the entire topic or as an Associate Editor-In-Chief for a subtopic. Please be sure to attach your CV and or biographical sketch.
Synonyms an related keywords: Pulseless diseases, non-specific aorto-arteritis, reverse coarctation, aortic arch syndrome, aortitis syndrome.
Overview
Four types of late-phase Takayasu arteritis are described on the basis of the sites of involvement as follows:
- Type I: Classic pulseless type that involves the brachiocephalic trunk, carotid arteries, and subclavian arteries
- Type II: Combination of type I and III
- Type III: Atypical coarctation type that involves the thoracic and abdominal aortas distal to the arch and its major branches
- Type IV: Dilated type that involves extensive dilatation of the length of the aorta and its major branches
The most common type is type III, which is found in as many as 65% of patients. The most commonly involved vessels include the left subclavian artery (50%), left common carotid artery (20%), brachiocephalic trunk, renal arteries, celiac trunk, superior mesenteric artery, and pulmonary arteries (50%). Infrequently, the axillary, brachial, vertebral, coronary, and iliac arteries are involved.
Diagnostic evaluation
Computed Tomography and MRA are the best examination methods for detailed anatomic evaluation possibilities. All diagnostic modalities as follow;
- Chest radiography: Chest radiographs may reveal widening of the ascending aorta, irregular descending aorta, aortic calcifications, and rib notching (late findings).
- Duplex Doppler may be used to evaluate and monitor disease in the common carotids and subclavian arteries; however, this imaging study is not useful in evaluating the aorta.
- Carotid evaluation reveals a homogenous circumferential thickening of the vessel wall that is distinguishable from atherosclerotic thickening.
- High-resolution ultrasonography
- Gallium-67 radionuclide scan: This scan may demonstrate increased uptake in the aorta and branches.
- Computed Tomography and MRA (magnetic resonance angiography) may demonstrate mural thickening of the aorta and luminal narrowing. Use of contrast may reveal inflammatory lesions prior to the development of stenoses; these lesions may be missed by angiography. Aortic lesions including stenosis, dilatation, wall thickening, and mural thrombi are well visualized on MRI, which is less adequate in visualizing distal lesions of the subclavian vessels and common carotids. [1]
One disadvantage of MRA is that pressure differentials cannot be measured across lesions in which imaging findings regarding their hemodynamic significance are inconclusive.
- Vessel edema: Non-contrast T2-weighted STIR images may be used to monitor edema in the aortic wall, which may be a surrogate for inflammation; edema may present more than 94% of patients with clinically active disease.
Important Note: Gadolinium-based contrast agents [gadopentetate dimeglumine (Magnevist), gadobenate dimeglumine (MultiHance), gadodiamide (Omniscan), gadoversetamide (OptiMARK), gadoteridol (ProHance)] have recently been linked to the development of nephrogenic systemic fibrosis (NSF) or nephrogenic fibrosing dermopathy (NFD). The disease has occurred in patients with moderate to end-stage renal disease after being given a gadolinium-based contrast agent to enhance MRI or MRA scans.
In December 2006, the FDA had received reports of 90 such cases. Worldwide, over 200 cases have been reported, according to the FDA. NSF/NFD is a debilitating and sometimes fatal disease. Characteristics include red or dark patches on the skin; burning, itching, swelling, hardening, and tightening of the skin; yellow spots on the whites of the eyes; joint stiffness with trouble moving or straightening the arms, hands, legs, or feet; pain deep in the hip bones or ribs; and muscle weakness. [2]
-
This MR angiogram demonstrates findings typical of Takayasu arteritis. A 32 years old Asian female has CRF and weak functioning iatrogenic fistula in left upper limb. The cause of malfunction can be explained by narrowing and occlusion of upper limb arteries. Notice also narrowed abdominal aorta. (Image courtesy of Dr Ahmed Haroun) -
MRA: A 15 year-old girl with known Takayasu arteritis presented for MRI to investigate back pain. The axial T1-weighted post-gadolinium MRI above shows thickened, enhancing aortic wall, consistent with large vessel vasculitis. (Image courtesy of Dr Laughlin Dawes)
