Ascending aortic dissection complicating syphilitic aortitis, late after aortic valve replacement

A 54-year-old man underwent aortic valve replacement for syphilitic aortic regurgitation. Eight years later, he was admitted with sudden precordial discomfort and symptoms of superior vena caval obstruction. Ascending aortic dissection was diagnosed by echocardiography and computed tomography of the thorax. The patient succumbed rapidly after admission. Postmortem findings and histological features were compatible with syphilitic aortitis, without significant atherosclerosis. This case report adds to the sparse literature on aortic dissection complicating syphilitic aortitis, and illustrates that, at certain stages of the disease process, syphilitic aortitis can lead to such a degree of mechanical instability of the aortic wall as to predispose to dissection.     

Aortic valve perforation in the setting of Cogan's syndrome

Cogan's syndrome is a rare disorder characterized by the coexistence of ocular and audio‐vestibular manifestations. Systemic manifestations are quite unusual with pan‐vasculitis and cardiac involvement reported in the form of aortitis with aortic aneurysm, dissection, or extremely rare aortic valve perforation. Hereby, we report a case of a 56‐year‐old woman presented with ocular, audio‐vestibular, and systemic manifestations with medium‐sized vasculitis in the form of multiple splenic artery aneurysms, superior mesenteric artery thrombosis, and cardiovascular involvement in the form of aortic regurgitation due to noncoronary cusp perforation. To the best of our knowledge, this is the second case to report aortic perforation in the setting of Cogan's syndrome.     

An orange-shaped aortic root aneurysm in aortitis syndrome with severe aortic regurgitation.

A 57-year-old man, who had undergone aorto-coronary bypass surgery 4 years before when the shape of the ascending aorta had been normal, had a unique orange-shaped aortic root aneurysm associated with severe aortic regurgitation and congestive heart failure. Replacement of the aneurysm and the aortic valve was successfully carried out, and histopathological examination revealed that the aneurysm was caused by aortitis syndrome.     

Bilateral ostial coronary stenosis and rheumatic aortic valve stenosis

A 49-year-old patient presented with angina pectoris and clinical findings of aortic valve stenosis and regurgitation. Rheumatic aortic valve stenosis and regurgitation was diagnosed on echocardiography. Coronary angiography findings showed severe calcification in the aorta root with right coronary ostial occlusion, and were suggestive of left main ostial stenosis and proximal main stem stenosis, which was confirmed on CT angiography. Curvilinear calcification of the aorta was present on CT angiography. The findings suggested syphilitic aortitis. Syphilis serology was positive (RPR titre 1/16). The angina was caused by severe coronary ostial disease likely due to syphilitic aortitis and exacerbated by the rheumatic aortic valve stenosis and regurgitation.     

Bilateral ostial coronary stenosis and rheumatic aortic valve stenosis

A 49‐year‐old patient presented with angina pectoris and clinical findings of aortic valve stenosis and regurgitation. Rheumatic aortic valve stenosis and regurgitation was diagnosed on echocardiography. Coronary angiography findings showed severe calcification in the aorta root with right coronary ostial occlusion, and were suggestive of left main ostial stenosis and proximal main stem stenosis, which was confirmed on CT angiography. Curvilinear calcification of the aorta was present on CT angiography. The findings suggested syphilitic aortitis. Syphilis serology was positive (RPR titre 1/16). The angina was caused by severe coronary ostial disease likely due to syphilitic aortitis and exacerbated by the rheumatic aortic valve stenosis and regurgitation.     

Transcatheter aortic valve implantation for severe pure aortic regurgitation due to active aortitis

Aortitis is an uncommon systemic inflammatory disease affecting the aorta and its main branches. Severe aortic regurgitation (AR) represents a fearsome complication of aortitis and is associated with an increased mortality rate. Surgical aortic valve replacement represents the only treatment choice for these patients. However, it is associated with a higher risk of medium to long-term complications such as prosthetic valve detachment. This is the first reported case where severe AR secondary to aortitis was managed with transcatheter aortic valve implantation (TAVI). TAVI was safe and effective in this clinical setting and may be considered a viable alternative to high-risk surgery in these complex patients.     

Transesophageal echocardiographic description of the mechanisms of aortic regurgitation in acute type A aortic dissection: implications for aortic valve repair

OBJECTIVES: The purpose of this study was to use transesophageal echocardiography (TEE) to define the mechanisms of aortic regurgitation (AR) in acute type A aortic dissection so as to assist the surgeon in identifying patients with mechanisms of AR suitable for valve preservation. BACKGROUND: Significant AR frequently complicates acute type A aortic dissection necessitating either aortic valve repair or replacement at the time of aortic surgery. Although direct surgical inspection can identify intrinsically normal leaflets suitable for repair, it is preferable for the surgeon to correlate aortic valve function with the anatomy prior to thoracotomy. METHODS: We studied 50 consecutive patients with acute type A aortic dissection in whom preoperative TEE findings were considered by the surgeons in planning aortic valve surgery. Six patients did not undergo surgery (noncandidacy or refusal) and one patient had had a prior aortic valve replacement and therefore was excluded from the analysis. RESULTS: Twenty-seven patients had no or minimal AR and 22 had moderate or severe AR. In all, there were 16 with intrinsically normal leaflets who had AR due to one or more correctable aortic valve lesion: incomplete leaflet closure due to leaflet tethering in a dilated aortic root in 7; leaflet prolapse due to disrupted leaflet attachments in 8; and dissection flap prolapse through the aortic valve orifice in 5. Of these 16 patients, 15 had successful aortic valve repair whereas just 1 underwent aortic valve replacement after a complicated intraoperative course (unrelated to the aortic valve). Nine patients underwent aortic valve replacement for nonrepairable abnormalities, including Marfan's syndrome in four, bicuspid aortic valve in four, and aortitis in one. In patients undergoing aortic valve repair, follow-up transthoracic echocardiography at a median of three months revealed no or minimal residual AR, and clinical follow-up at a median of 23 months showed that none required aortic valve replacement. CONCLUSIONS: When significant AR complicates acute type A aortic dissection, TEE can define the severity and mechanisms of AR and can assist the surgeon in identifying patients in whom valve repair is likely to be successful.     

Severe aortic regurgitation secondary to idiopathic aortitis

Clinical and morphologic features are described in two relatively young adults with aortic regurgitation secondary to chronic aortitis. The regurgitation in each was severe enough to require aortic valve replacement. Both patients had normochromic, normocytic anemia, considerable weight loss despite congestive cardiac failure, and negative serologic tests for syphilis. These systemic manifestations in association with the aortitis suggest that both had Takayasu's arteritis. In addition, one patient had total occlusion at the origin of one subclavian artery (classic pulseless disease). Takayasu's arteritis must be added to the list of causes of severe aortic regurgitation.     

Flail aortic valve and acute aortic regurgitation due to spontaneous localized intimal tear of ascending aorta

This report describes the transesophageal echocardiographic findings of acute severe aortic regurgitation resulting from localized transverse intimal tear of ascending thoracic aorta which could not be suspected as a cause of flail aortic valve preoperatively. In patients with chest pain and acute aortic regurgitation associated with flail aortic valve, localized intimal tear of aorta should be considered as a possible cause of flail aortic valve.     

Acute aortic regurgitation due to spontaneous rupture of a bicuspid aortic valve: detection by echocardiography

A 42-year-old man was admitted with acute severe aortic regurgitation. There were no signs of a systemic infection. M-mode and two-dimensional echocardiography revealed bicuspid aortic valve and echocardiographic features consistent with aortic leaflet rupture. The diagnosis was confirmed at surgery. This report illustrates that spontaneous rupture of a bicuspid aortic valve should be considered in acute aortic regurgitation without infective endocarditis.     

A case of microscopic polyangiitis associated with aortic valve insufficiency

Microscopic polyangiitis (MPA) is an anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis characterized by inflammation of small-sized vessels. Although there have been some reports of ANCA-associated vasculitis presenting as aortitis syndrome, MPA rarely involves large-sized vessels such as the aorta. We report an unusual case of MPA combined with severe acute aortic valve insufficiency in a 56-year-old man. He initially presented with prolonged fever, skin rash, and rapidly progressive glomerulonephritis. P-ANCA and anti-myeloperoxidase (MPO) antibodies were positive, but the c-ANCA and anti-proteinase-3 antibodies were negative. Skin biopsy of the lower leg showed necrotizing arteritis. Kidney biopsy was also performed, which revealed diffuse necrotizing and crescentic glomerulonephritis (GN) consistent with pauci-immune ANCA-associated GN. Serial echocardiographic evaluations revealed aortic valve changes and worsening acute aortic valve insufficiency over a two-month period. Despite intensive treatment, our patient developed sudden cardiac arrest and died. Our patient demonstrated typical clinical features and histopathologic findings for systemic vasculitis and had a positive anti-MPO antibody, all of which were consistent with the diagnosis of MPA. Thus, MPA may have been the cause of acute aortic valve insufficiency in this case.     

Aortic valve replacement for aortic regurgitation caused by aortitis

Between January 1984 and December 1998, 19 patients (16 with Takayasu's arteritis, 3 with non-Takayasu's aortitis) underwent surgical treatment for aortic regurgitation resulting from the aortitis. Of the 19 patients, 14 had aortic valve replacement (AVR) and 5 had aortic root replacement. One patient (5.3%) died of graft infection during the hospital stay. During the follow-up period, 1 (5.6%) of the 18 postoperative patients died of paravalvular leakage due to valve detachment, which also required redo-operations in 2 patients with non-Takayasu's aortitis. Both patients were operated on during the active phase of the inflammation without perioperative steroid therapy. Although transmural pledgeted sutures were used for replacement of the detached prosthetic valve in 1 of these 2 patients, disruption of the aortic wall resulted in recurrence of valve detachment. In the other patient, aortic root replacement was successfully performed with the Cabrol technique in the second operation. Perioperaitve steroid therapy plays an important role in preventing complications after AVR when the valve replacement is carried out during the active phase of the inflammation, and for patients with non-Takayasu's aortitis, aortic root replacement should be considered to reduce the tension on the suture line and the native aortic valve annulus.     

Takayasu's arteritis revealed by aortic regurgitation with ascending aortic aneurysm (About 2 cases)

Aortic regurgitation caused by non-specific aortitis is relatively rare, and is now considered as an important risk factor related to mortality. Aortic valve replacement surgery is the only curative treatment. Aneurismal dilatation of the ascending thoracic aorta associated with aortic regurgitation is a rare involvement in Takayasu, there are many difficult problems in surgical treatment of this lesion, because of its inflammatory nature, so steroid therapy before and after surgery is therefore vital. We report the cases of tow young Moroccans women (32-35 years-old) with an ascending aortic aneurism associated to aortic insufficiency. The subsequent evaluation of the entire aorta, demonstrated the presence of multiple steno-occlusive lesions. Aortic valve replacement was performed associated with graft replacement of the ascending aorta without coronary artery reimplantation. Histopathological examination of the ascending aorta and aortic valve, showed findings in favour Takayasu's arteritis.     

一种慢性主动脉瓣关闭不全动物模型建立的实验研究

目的:探讨通过介入方法破坏主动脉瓣膜,建立一种慢性主动脉瓣膜关闭不全犬模型的可行性。方法: 选择体质量为(18.3±4.6) kg的健康犬10只。经皮送入加硬导丝,在超声引导下穿破主动脉瓣叶,同时进行球囊扩张瓣叶穿孔处。主动脉瓣上造影检查瓣膜破裂口有无明显的反流,心脏超声检查评估主动脉瓣膜的反流量以及左室射血分数。术后将实验犬饲养两个月,再次复查心脏超声评估主动脉瓣膜的反流量及左心室的功能。结果: 10只实验犬中,除两只在建立急性主动脉瓣膜破裂伤时,导丝穿入心肌导致心包填塞,而放弃继续操作外,其余8只犬手术成功,主动脉瓣膜的瞬时反流量达到(5.2±0.8) ml/s。术后第20天、35天,各有1只实验犬死于心力衰竭,其余6只犬存活到两个月。所有存活实验犬均出现活动耐量的减退。复查心脏超声提示,主动脉瓣瞬时反流量为(8.4±1.2) ml/s,比术后即刻有明显增加;左室射血分数为(42.5±1.1)%,比术后即刻明显降低;左心室容积为(69.5±6.8) ml,左心室壁厚度为(8.6±1.7) mm,比术后即刻有明显的增加,以上各项指标差异均有统计学意义（P<0.05）。结论: 通过经皮穿刺主动脉瓣叶加球囊扩张的方法,建立慢性主动脉瓣关闭不全的犬模型可行,效果理想。     

Anterograde balloon valvuloplasty for the treatment of neonatal critical valvar aortic stenosis.

We report our experience with anterograde balloon valvuloplasty in 17 neonates treated between November 1996 and June 2001 for critical aortic stenosis. Patients with hypoplastic left heart syndrome were excluded. Anterograde balloon valvoplasty of the aortic valve was possible in all 17 patients. The mean peak systolic gradient prior to cardiac catheterization was 73 mm Hg (range, 30-117 mm Hg) and decreased to 37 mm Hg (range, 21-60 mm Hg) after the dilation. Aortic regurgitation after balloon valvoplasty was absent or mild in 14/17 patients, moderate in 2 patients, and severe in 1 patient. There was no mortality or echocardiographic evidence for aortic cusp perforation or mitral regurgitation associated with the procedure. Redilation was necessary in 3/17 patients. Two patients are awaiting elective Ross operation. One patient with endocardial fibroelastosis died at 11 months of age. Anterograde balloon valvoplasty can be safely and effectively performed to palliate neonates with critical aortic valve stenosis.     

Transcatheter repair of aortic leaflet perforation in a patient with prior surgical bicuspid aortic valve repair

A 47‐year‐old male underwent surgery for functional bicuspid aortic valve severe regurgitation with a fused right and left coronary cusp. The patient presents nine years after surgical bicuspid aortic valve repair with symptomatic severe aortic regurgitation, diagnosed by TEE and MRI, caused primarily from a perforation located at the base of the surgically fused coronary cusps. The patient had a minimally dilated aortic root that did not yet necessitate surgical intervention. We present a novel percutaneous bicuspid aortic valve perforation repair that potentially decreases the number of surgical operations the patient must undergo during his lifetime. © 2015 Wiley Periodicals, Inc.     

Mitral regurgitation associated with aortitis syndrome

Three patients with mitral regurgitation (MR) associated with aortitis syndrome are presented. All had multiple lesions of the large sized arteries, calcification of the aorta, mild inflammatory findings, a chronic course, and congestive heart failure. MR was observed by ventriculography in all 3 patients. Case 1 had mitral valve prolapse and secondary systemic hypertension. Case 2 showed mildly thickened mitral valve leaflets and had moderate aortic regurgitation (AR). Case 3 had massive AR. The grade of MR was moderate in Cases 1 and 2, and massive in Case 3. The left ventricle was moderately dilated in Cases 1 and 2 but contracted sufficiently and symmetrically in all 3 patients. Other than the prolapse, no significant mitral valve deformity or left ventricular asynergy was evident by ventriculography. The incidence of MR was 3.1% of 128 patients with aortitis syndrome observed in our clinic. MR may be found in the late stage of aortitis syndrome. It may be caused by a mild valvular lesion related to aortitis syndrome and be exacerbated by increased hemodynamic loads such as those which occur in secondary hypertension and AR.     

升主动脉瘤的外科治疗

目的总结升主动脉瘤的临床诊断和外科治疗经验。方法自2003年3月至2008年11月,20例升主动脉瘤患者中马凡综合征11例、升主动脉瘤伴主动脉瓣关闭不全3例、单纯升主动脉瘤3例、急性夹层动脉瘤伴主动脉瓣关闭不全2例、升主动脉瘤伴主动脉瓣关闭不全并感染性心内膜炎1例。行单纯Bentall手术10例、Bentall手术加右半弓人工血管置换2例、Wheat手术4例、Cabrol手术1例、David手术1例、主动脉瓣置换加主动脉弓置换加象鼻手术1例、主动脉瓣置换术加升主动脉成形术1例。结果全组无手术死亡和术后严重并发症发生。结论早期诊断、精细的手术操作技巧、据病情选择合适的手术方式,是改善升主动脉根部瘤患者预后的关键。     

An autopsy case of aortitis resulting in a tear of the aortic valve due to a rupture of the aneurysm of Valsalva's sinus]

A 43-year-old man was admitted to a hospital because of acute dyspnea and nocturnal orthopnea. Echocardiogram and chest CT showed the dilation of thoracic aorta from the root to ascending portion. On the third hospital day, he died suddenly. At autopsy, the cause of death was indicated to be a tear of an aortic valve due to a rupture of the aneurysm of Valsalva's sinus, followed by acute aortic regurgitation and acute cardiac insufficiency. Histopathological findings of thoracic aorta revealed mesoaortitis, characterized by patchy destruction of the media with a moth-eaten appearance of the medial elastic laminae and a microgranuloma formation, a perivascular mononuclear cell infiltration of the vasa vasorum, and a fibrous thickening of the intima and adventitia. However, there were no abnormalities in main branches of aorta and abdominal aorta, and no systemic vasculitis. This case is a rare one in the clinical course, and may be important to be differentiated from other cases with aortitis, especially Takayasu arteritis and syphilitic aortitis.     

Non-infectious aortitis: an important cause of severe aortic regurgitation

From 1995 to 2004, 13 patients with severe aortic regurgitation due to non-infectious aortitis underwent aortic valve surgery at our center. Twenty-eight operations (18 aortic valve replacements and 10 Bentall procedures including 1 aortic root replacement with a homograft) were performed due to a high incidence of prosthetic valve or valved conduit dehiscence (54%). Steroid therapy reduced the incidence of re-operation for anastomotic dehiscence within one year from 90% to 18%. Strategies to prevent dehiscence of the prosthetic aortic valve, which include institution of immunosuppression, replacement of the diseased aorta, and monitoring inflammatory indices, are discussed.