Abstract:atrial ablation purposes. 3 However, it is a known fact that patients with long-standing AF, giant atria, or both have a much smaller chance to revert back to sinus rhythm after these ablation procedures. 4 Our findings, herein reported, might provide an explanation to understand this fact. As we have described, the morphologic consequences of chronic AF on the normal SN can be synthesized in 3 main features: (1) progressive tissue fibrosis, (2) progressive myocardial cell loss (including P cells), and (3) … Show more
“…Cardiovascular manifestations have been reported in 11% to 56% of these patients and include vasculitis of both large- and medium-sized arteries, aortic regurgitation, and pericarditis ( 2 ). Although aortic insufficiency is the most common cardiovascular complication observed in 4% to 9% of these patients ( 3 ), the combination of aortic insufficiency from aortitis with ostial coronary lesion is exceptionally rare: 7 cases have previously been described in the literature. Of those cases, only 1 patient successfully underwent coronary artery bypass grafting with aortic valve replacement.…”
A 32-year-old man with a history of relapsing polychondritis presented with acute coronary syndrome due to aortitis with ostial coronary artery involvement from his underlying autoimmune condition. Concomitant aortic insufficiency with ostial coronary lesions is a rare complication of relapsing polychondritis, requiring a multidisciplinary team approach for management. (
Level of Difficulty: Advanced.
)
“…Cardiovascular manifestations have been reported in 11% to 56% of these patients and include vasculitis of both large- and medium-sized arteries, aortic regurgitation, and pericarditis ( 2 ). Although aortic insufficiency is the most common cardiovascular complication observed in 4% to 9% of these patients ( 3 ), the combination of aortic insufficiency from aortitis with ostial coronary lesion is exceptionally rare: 7 cases have previously been described in the literature. Of those cases, only 1 patient successfully underwent coronary artery bypass grafting with aortic valve replacement.…”
A 32-year-old man with a history of relapsing polychondritis presented with acute coronary syndrome due to aortitis with ostial coronary artery involvement from his underlying autoimmune condition. Concomitant aortic insufficiency with ostial coronary lesions is a rare complication of relapsing polychondritis, requiring a multidisciplinary team approach for management. (
Level of Difficulty: Advanced.
)
“…Treatment of cardiac-related disease in RP can be challenging, as disease may be asymptomatic and progressive despite therapy [3,10]. Combinations of corticosteroids, methotrexate, azathioprine, infliximab, and cyclophosphamide have been utilized with mixed success, while a recent publication describes successful use of tocilizumab for corticosteroid-resistant RP aortitis [8,[10][11][12][13]. When aortic insufficiency and coronary stenosis are severe, surgical treatment with aortic valve repair and coronary artery bypass grafting are indicated, respectively [4].…”
Section: Discussionmentioning
confidence: 99%
“…It is typically associated with an underlying autoimmune or inflammatory condition such as RP, Takayasu arteritis, Kawasaki disease, Behçet's disease, IgG4-related periarteritis, eosinophilic granulomatosis with polyangiitis, granulomatosis with polyangiitis, and polyarteritis nodosa [14]. To date, there are only five case reports describing coronary vasculitis in RP (Table 1) [8,11,12,15,16]. Notably, cardiac disease was diagnosed during active RP in all four males but only during remission in the female.…”
Relapsing polychondritis (RP) is a systemic autoimmune disease characterized by relapsing and remitting inflammation of the cartilaginous structures of the ears, nose, tracheobronchial tree, and joints. Diagnosis is challenging due to the heterogeneity of clinical manifestations, the relapsing and remitting nature of the disease, the presence of coexistent diseases in at least one-third of patients, and the lack of a diagnostic blood test. Although RP-associated cardiac disease is the second most common cause of death behind tracheobronchial complications, coronary artery vasculitis is rare. This report describes a case of sudden cardiac death due to vasculitis affecting the coronary arteries in a patient with RP. The pathologic findings included obliterative coronary arteritis with plasma cells and storiform fibrosis, features suggesting that IgG4-related disease (IgG4-RD) may have contributed to the patient’s cardiac disease. The literature on vasculitis and cardiac disease in RP and the possible role of IgG4-RD in this setting is also reviewed. The primary take-home message from this case report is the importance of frequent screening for cardiac disease, regardless of symptoms, in patients with RP. In addition, considering the diagnosis of IgG4-RD in some cases thought to be RP may also be warranted.
“…Angiography revealed severe right and left coronary ostial stenosis, the patient expired while awaiting surgery. Steroids were not started postoperatively in this patient as the ESR was normal 2 .…”
We read with interest the article on non infectious aortitis by Chau et al (2006;14(3):175) 1 . The article has several important messages that will be of immense benefi t to surgeons practicing in Asia where the incidence of non infectious aortitis is relatively high.We have encountered two such patients recently. The fi rst patient was a 26 year old lady a known case of relapsing polychondritis, she underwent a aortic valve replacement for severe aortic regurgitation. At surgery she had normal coronary ostia, at follow up she developed severe chest pain and was diagnosed to have anterior and inferior myocardial infarction. Angiography revealed severe right and left coronary ostial stenosis, the patient expired while awaiting surgery. Steroids were not started postoperatively in this patient as the ESR was normal 2 .The second patient was a seventeen year old girl with marfanoid habitus. She was diagnosed to have type A dissection with severe aortic regurgitation on the basis of CT scan and transesophageal echocardiography. At surgery we found that she had a thickened and infl amed aorta but no dissection fl ap was found either in the ascending or arch of aorta. The left coronary ostium was found to be pinpoint, left internal thoracic artery was taken down and anastomosed to the left anterior descending artery.The patient made an uneventful recovery; postoperative 64 slice CT scan revealed a 50% ostial stenosis and a functioning internal thoracic artery graft.Takayasu's arteritis and other infl ammatory aortitis are known to affect the coronary arteries, especially the left and right coronary ostia 3 . Proximal vein anastomosis to the aorta after CABG is prone for stenosis as pointed out by the authors 3 . It has been shown previously that internal thoracic artery grafts can be effectively used when there is no lesion in the subclavian 4 . We would like to reiterate the importance of identifying ostial stenosis during surgery in this group of patients, it is also important to start the patients on steroids to prevent postoperative infl ammatory coronary ostial stenosis.
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