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GIRL WITH TAKAYASU ARTERITIS -PROGRESSIVE COURSE OF THE DISEASE AND MULTIPLE SURGICAL INTERVENTIONS

IVAN MALČIĆ ; University Hospital Centre Zagreb, Department of Pediatric Cardiology, University of Zagreb School of Medicine, Zagreb, Croatia
AGNEZA MARIJA PASINI ; Children’s Hospital Zagreb, Department of Pulmology, Allergology, Immunology and Rheumatology, Zagreb, Croatia
MAJA HRABAK PAAR ; Clinical Hospital Centre, Department of Diagnostic and Interventional Radiology, Zagreb, Croatia
HANNES MUELLER ; Klinik für Herz-, Thorax- und Gefäßchirurgie, Kepler Universitätsklinikum Linz Med Campus III., Linz, Austria
RUDOLF MAIR ; Klinik für Herz-, Thorax- und Gefäßchirurgie, Kepler Universitätsklinikum Linz Med Campus III., Linz, Austria
DRAŽEN PERKOV ; Clinical Hospital Centre, Department of Diagnostic and Interventional Radiology, Zagreb, Croatia
KRISTINA MARIĆ ; Clinical Hospital Centre Zagreb, Department of Cardiovascular Medicine, Zagreb, Croatia
MARIJA JELUŠIĆ ; Department of Pediatric Rheumatology and Immunology, University Hospital Centre Zagreb, University of Zagreb School of Medicine, Zagreb, Croatia

Abstract

Takayasu arteritis is a rare large-vessel vasculitis but it can be associated with high mortality rates in childhood. Granulomatous vasculitis usually affects the aorta and/or main branches but also coronary and pulmonary arteries. The course of the disease is unpredictable and management is based on controlling infl ammation and preventing end-organ damage. In this case we describe a patient with progression of vasculitis but so far successful prevention of possible ischemic consequences using immunosupressive and biologic therapy and multiple surgical interventions over the course of the disease. A 14-year-old girl presented with precordial pain and numbness of the left arm. Physical examination revealed the absence of the radial pulse in the left arm. Computed tomography angiography showed subtotal occlusion of the left main coronary artery, subtotal occlusionof the left common carotid artery, subtotal occlusion of the left subclavian artery and stenosis of thoracic aorta below isthmus of aortae. Despite aggresive conservative therapy and cardiosurgical treatment the course of the disease was complicated with restenoses which were resolved with subsequent revascularization procedures. Here we present an adolescent girl with progressive vasculitis and with multiple surgical interventions. Carefully monitoring of the patient and good collaboration between pediatric cardiologist and rheumatologist with radiologists and cardiac surgeons improved life-quality of the patient which now studies at the University and has good physical and mental status.

Keywords

childhood Takayasu arteritis; progressive course; surgical interventions; revascularization

Hrčak ID:

244549

URI

https://hrcak.srce.hr/244549

Publication date:

6.10.2020.

Article data in other languages: croatian

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