Giant Thrombosis at Left Anterior Descending Artery Aneurysm in a 10-Year Old Boy with Granulomatosis with Polyangiitis

Abstract

Granulomatosis with polyangiitis (GPA), necrotizing vasculitis of small and medium-sized vessels, is traditionally believed to mainly affect respiratory tract with additional focal kidney involvements as its primary manifestations with a relatively rare annual incidence rate of 20-50 cases per million. Six percent of the affected cases have cardiac involvements; among which, aneurysms comprise the lowest penetrance. By this paper, we aim to cast light on clinical diagnostic and treatment methods of a rare case presentation, a 10-year-old male GPA patient, diagnosed with massive thrombosis at his coronary artery aneurysm. GPA should be considered as differential diagnosis of prolong fever and coronary aneurysms in adolescents.

Figure 1

Short axis view (SAX) of transthoracic echocardiography (TTE) in our patient shows ectasia at left anterior descending (LAD) artery and huge aneurysm with large circular nonorganized thrombosis occupying the majority of the aneurysm, narrowing the internal lumen of the artery.

Figure 2

Saddle nose in our patient with granulomatosis with polyangiitis.

Figure 3

(a) High-resolution, ECG-synchronized computed tomography of the heart with attention to the coronary arteries showed left main coronary ectasia (4.2 mm), fusiform aneurysmal dilation measured 19 mm length, and14 mm transverse diameter with partial thrombosis in the lumen measured 17 × 10 mm in proximal segment of left anterior descending artery (LAD) and aneurysmal dilatation in proximalpart of left circumflex (LCX) in 5 mm diameter. (b) A cavity formation in the left lung apex measure 36 × 27 × 21 mm and some fibrotic and atelectatic bands in bases of both lung.