Large-vessel Vasculitis Affecting the Aorta and its Branches in Relapsing Polychondritis: Case Series and Systematic Review of the Literature

ObjectiveTo describe the features of large-vessel vasculitis (LVV) as it affects the aorta and its branches in patients with relapsing polychondritis (RP).MethodsRetrospective data and systematic literature review.ResultsTwenty-one patients were identified. LVV diagnosis was subsequent to RP and associated with extrachondral involvement in the majority of patients. Supraaortic vessels were more frequently involved (82%). Fourteen patients (67%) were treated with a conventional synthetic disease-modifying antirheumatic drug (csDMARD) and 7 (33%) with a biological DMARD (bDMARD). Vascular interventional procedures were performed in 10 patients (48%). Premature death due to cardiovascular complications was reported in 3 cases (14%).ConclusionExtraaortic LVV is a serious and overlooked RP manifestation. All patients with RP should be investigated for LVV.

Hybrid treatment of an aortic arch aneurysm in a patient with a patent right and left internal mammary graft

The aim of this report is to describe a known hybrid surgical and endovascular technique for aortic arch aneurysm repair in a special setting of a patient with previous coronary mammary bypass. We present a case of aortic arch aneurysm in a 66-year-old man with previous coronary bypass. He underwent a hybrid endovascular and surgical repair. All supraaortic vessels were debranched, keeping a continuous antegrade blood flow through the left and right internal mammary arteries to avoid myocardial ischemia. Endovascular treatment of aortic arch aneurysms is associated with less mortality and morbidity as compared with open surgery. The hybrid procedure with surgical debranching of the supraaortic vessels is the most commonly used method. In cases of previous coronary bypass with mammary arteries, care must be taken to maintain constant flow through the grafts during debranching.

Time-resolved, contrast-enhanced 3D MR angiography in the subclavian steal effect

The subclavian steal effect indicates atherosclerotic disease of the supraaortic vessels but rarely causes cerebrovascular events in itself. Noninvasive imaging providing detailed anatomic as well as hemodynamic information would therefore be desirable. From a group of 25 consecutive patients referred for MR angiography, four with absent or highly attenuated signal in one of the vertebral arteries on 3D multislab time-of-flight MR angiography were selected to undergo 3D time-resolved contrast-enhanced MR angiography. The time-resolved 3D contrast series (source images and MIPs) were evaluated visually and by graphic analysis of time-intensity curves derived from the respective V1 and V3 segments of both vertebral arteries on the source images. In two cases with high-grade proximal left subclavian stenosis, time-resolved 3D ce-MRA was able to visualise retrograde contrast filling of the left VA. There was a marked delay in time-to-peak between the left and right V1 segments in one case and a shallower slope of enhancement in another. In the other two cases, there was complete or collateralised segmental occlusion of the VAs.