The heart in takayasu arteritis

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Synonyms an related keywords: Pulseless diseases, non-specific aorto-arteritis, reverse coarctation, aortic arch syndrome, aortitis syndrome.

Overview

Cardiac repurcussions of takayasu arteritis may include aortic regurgitation and congestive heart failure as a result of myocarditis or increased afterload. Initially, a diagnosis of TA can be missed for months or years because its symptoms can be nonspecific (fever, lethargy, etc.). When a widened mediastinum is appreciated on chest roentgenography and a cancer is suspected, a CT scan will instead show a widened aortic arch, which is often how TA gets diagnosed.[1]

Takayasu arteritis is sometimes called pulseless disease, though a missing peripheral pulse usually occurs late in TA's course. More often individuals with TA present with an asymmetric pulse.[1]

Nine in ten individuals with TA will survive five years, but TA has high residual morbidity.[1]

Types

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. [2]

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. [3]


References

  1. 1.0 1.1 1.2 "Takayasu Arteritis: eMedicine Pediatrics: General Medicine".
  2. Yamada I, Nakagawa T, Himeno Y: Takayasu arteritis: diagnosis with breath-hold contrast-enhanced three- dimensional MR angiography. J Magn Reson Imaging 2000; 11: 481
  3. www.fda.gov

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