Simultaneous aortic and pulmonary artery aneurysms due to giant cell arteritis.

Abstract

Giant cell arteritis may occasionally affect the aorta and larger pulmonary arteries. This can be part of a systemic disease such as necrotising sarcoid granulomatosis or systemic lupus erythematosus, but may also be due to specific conditions, particularly disseminated temporal arteritis (giant cell arteritis). When the pulmonary arteries alone are affected they are usually medium sized and affected as part of a generalised connective tissue disorder such as rheumatoid arthritis. Takayashu's disease may also affect the larger elastic pulmonary arteries or the main pulmonary trunk. This paper describes a previously unreported condition of aneurysmal dilatation of the ascending aorta and main pulmonary trunk with no concomitant generalised connective tissue disorder. Case reportA 55 year old woman was admitted to hospital after six months of increasing fatigue, three months of shortness of breath, and two recent episodes of paroxysmal nocturnal dyspnoea, although she had no chest pain. She gave no history of rheumatic or scarlet fever.The jugular venous pressure was normal, the pulse regular (82 beats/minute) but collapsing in nature, and the blood pressure 150/50 mm Hg. All peripheral pulses were present. There was moderate left ventricular enlargement.Both heart sounds were present and in the aortic area there was an ejection systolic murmur and a loud diastolic murmur.The results of laboratory tests for rheumatoid arthritis, systemic lupus erythematosus, and syphilis were negative. pulmonary trunk were found. An exudate over the aorta suggested recent active inflammation. The pulmonary artery was extremely thin and densely adherent to the aorta, so that an adequate plane of cleavage could not be found. Dilatation of the aorta continued up to the beginning of the arch and similar pulmonary dilatation was present to the bifurcation of the artery, with considerable thinning of the wall. The right pulmonary artery appeared normal. Frozen section biopsy specimens from both arteries showed active giant cell arteritis. The aortic valve cusps were thickened, allowing considerable regurgitation. The aortic valve was resected and replaced with a 23 mm Bjork-Shiley valve. The aorta was divided longitudinally and replaced with a 32 mm preclotted Dacron graft from the level of the innominate artery to just above the valve. Similarly the pulmonary artery was divided longitudinally (revealing multiple areas of dissection), and replaced from the bifurcation to just above the valve cusps. Subsequent histological examination confirmed the findings from the frozen sections. The patient made a satisfactory postoperative recovery and remained well for three years, with no evidence of recurrent aneurysm formation or valvular incompetence.Histological examination showed the pulmonary artery to be thinned, with a considerable quantity of metachromatic material in the wall. One part of the arterial wall showed early dissection. In the adventitia there was a striking degree of granulomatous arteritis in the form of appreciable adventit...

The main pulmonary artery in adults: a controlled multicenter study with assessment of echocardiographic reference values, and the frequency of dilatation and aneurysm in Marfan syndrome

“…Some MPA aneurysms arise in the absence of PH, and they may have a more benign prognosis than high pressure MPA aneurysms [ 30 , 32 ]. These low pressure MPA aneurysms may arise from pulmonary valve stenosis [ 20 , 34 ], vasculitis such as Behcet’s disease [ 35 ] or giant cell arteritis [ 36 ], infections such as tuberculosis [ 37 ], syphilis [ 38 , 39 ] and septic emboli [ 22 ], extravascular [ 40 ] or endovascular trauma [ 31 , 41 ], or degenerative diseases including atherosclerosis [ 42 ] and media degeneration [ 43 ]. Some “idiopathic” MPA aneurysms may form without any of these causes [ 20 , 23 , 44 ].…”

Multiple pulmonary infarctions caused by giant cell arteritis

“…Prednisolone was increased to 40 mg/day and she was initially anticoagulated. Dyspnoea improved over the following 4 weeks and she remained asymptomatic with ESR of 11 mm in 1 hour 4 months after her discharge from the hospital.…”

Pulmonary Artery Aneurysms: Four Case Reports and Literature Review

“…Behçet disease and giant cell arteritis are the leading vasculitic variants causing PAA. 18,19 It has been noted that aneurysms due to Behçet disease can spontaneously regress following medical treatment of the disease. Surgery is discouraged in Behçet disease due to recurrent false aneurysm formation at the anastomotic sites.…”