SEGMENTAL AORTITIS AND AORTIC INCOMPETENCE

An unusual case of a middle-aged male with segmental aortitis and aortic incompetence is described. To our knowledge, only 13 cases have been previously described in the literature, but only two have had anatomopathological confirmation. Aortic incompetence is a rare manifestation of segmental aortitis. Although intrinsic abnormalities of the aortic valve have been proposed, the finding of a normal aortic valve at postmortem examination in 2 of the 3 cases in which the aortic valve was examined, supports the premise that the incompetence is probably secondary to disproportionate dilatation of the aortic root.     

Idiopathic aortitis: an underrecognized vasculitis

Aortitis is a general term denoting inflammation of the aortic wall. Various infectious and non-infectious diseases can be complicated by aortitis; in addition, isolated idiopathic aortitis has also been described. In a 12-year nationwide Danish population-based study, the prevalence of aortitis among 1,210 resected thoracic aorta samples was 6.1%, with nearly three-quarters of cases being idiopathic. Identified risk factors for aortitis included advanced age, a history of connective tissue disease, diabetes mellitus, and heart valve pathology. As in virtually all pathological studies, this study has a bias toward reporting the most severe cases of aortitis requiring surgical repair.     

Isolated bilateral ostial coronary stenosis with proximal right coronary artery occlusion

The incidence of coronary ostial stenosis in patients undergoing coronary arteriography has been found to range between 0.07 and 0.25%. A slightly higher incidence has been observed in patients with angiographically confirmed coronary artery disease: between 0.13 and 2.7%. Bilateral ostial stenosis is even less common. Although cardiovascular syphilis is no longer a prominent condition, it must be considered in the differential diagnosis since it carries a very high risk (50%) of cardiovascular complications if left untreated. Ostial coronary stenosis occurs in 26% of patients with syphilitic aortitis. This paper reports on a 41-year-old Wasserman (WR)-positive woman with progressive angina caused by bilateral ostial coronary stenosis.     

THE DIFFICULT PROXIMAL CORONARY ANASTOMOSIS

Two patients are presented in whom severe calcific aortitis made performance of satisfactory proximal coronary anastomosis impossible. Endarterectomy of the ascending aorta after aortic valve replacement, combined with aortocoronary bypass prior to performance of the proximal anastomoses, was successfully employed in one patient. The second patient underwent resection of an anterior segment of aorta, which was replaced by a woven Dacron patch into which the vein grafts were sewn.     

The Clinical Significance of the Biological False Positive Serologic Reactor: A Study of 113 Cases

Biological false positive serologic reactors were studied during a long-term follow-up of asymptomatic patients with chronic false positive serology for syphilitic reagin. This was done with a view to facilitating the early diagnosis of systemic disease, particularly collagen disorders, which are frequently associated with this finding in women. One hundred and thirteen cases were studied. Thirty-eight were “acute”, i.e. positive for less than six months, 58 were “chronic”, i.e. positive for more than six months, and the remainder still positive but followed for less than six months. Of 39 female chronic reactors, 10 were diagnosed as having collagen disease, and in six the BFP reaction preceded clinical diagnosis of the disease. Five had no apparent disease. In 19 male chronic reactors, there was no evidence of collagen disorders and five were free of any recognizable pathology. The remainder in both sexes were found to have a wide variety of systemic illnesses.     

Rupture of Descemet's membrane secondary to presumed forceps trauma

Ruptures in Descemet's membrane can occur secondary to forceps injury at birth, prolonged labor, blunt trauma, keratoconus, and congenital glaucoma as well as other less frequent causes. The rupture initially causes acute comeal hydrops which resolves within weeks to leave permanent linear thickening of Descemet's membrane. It is important to differentiate Descemet's rupture from other entities like syphilitic interstitial keratitis, posterior polymorphous dystrophy and congenital hereditary endothelial dystrophy which may have a similar clinical presentation. Although treatment is rarely required, proper diagnosis is important to prevent unnecessary referrals and provide appropriate counseling. This case report examines a 66-year-old female with Descemet's rupture secondary to presumed forceps trauma at birth. Discussion of clinical presentation, differential diagnosis and clinical management are presented.     

Recommended Criteria for Cardiac Catheterization in Patients with Aortic Valve Disease

Criteria for selection of patients with aortic valve disease for cardiac catheterization are described, based on a study of 81 cases. Children with aortic stenosis warrant catheterization at the time when the clinical diagnosis is made, but in adults this examination may be deferred until symptoms appear or left ventricular hypertrophy is recognized. In patients with pure aortic insufficiency catheterization may be deferred until symptoms appear. When severe stenosis and insufficiency co-exist, the valve is usually heavily calcified. Thirty-seven per cent of patients with aortic valve disease have co-existing mitral lesions and these patients are usually women, are fibrillating and, as a rule, have atrial enlargement in contrast to those with aortic valve disease only. On rare occasions, patients with mitral valve disease have clinically silent but angiographically demonstrable aortic insufficiency; therefore, aortography should precede open-heart correction of a mitral lesion so as to detect minor degrees of aortic insufficiency.     

Kallman's syndrome combined with aortic valve anomaly and epilepsy]

Kallman's syndrome is defined clinically as hypogonadotropic hypogonadism associated with anosmia and other congenital abnormalities. There is no report on Kallman's syndrome coexisting with aortic valve anomaly and epilepsy in the available literature. Therefore, the authors considered worthy presenting a case of a 36-years male [correction of female] patient with Kallmann's syndrome combined with aortic valve anomaly, epilepsy and hyperostosis frontalis. Disease history data, clinical examination, the results of hormonal tests and USG helped to establish the diagnosis. Diagnostic and therapeutical problems in such cases have also been discussed.     

Clinical course after corticosteroid therapy in IgG4-related aortitis/periaortitis and periarteritis: a retrospective multicenter study

Introduction

Immunoglobulin G4 (IgG4)–related aortitis/periaortitis and periarteritis are vascular manifestations of IgG4-related disease. In this disease, the affected aneurysmal lesion has been suspected to be at risk of rupture. In this study, we aimed to clarify the clinical course after corticosteroid therapy in IgG4-related aortitis/periaortitis and periarteritis.

Methods

We retrospectively evaluated clinical features, including laboratory data, imaging findings and the course after corticosteroid therapy, in 40 patients diagnosed with IgG4-related aortitis/periaortitis and periarteritis on the basis of periaortic/periarterial radiological findings, satisfaction of the comprehensive diagnostic criteria or each organ-specific diagnostic criteria, and exclusion of other diseases.

Results

The patients were mainly elderly, with an average age of 66.4 years and with a marked male predominance and extensive other organ involvement. Subjective symptoms were scanty, and only a small proportion had elevated serum C-reactive protein levels. The affected aorta/artery were the abdominal aortas or the iliac arteries in most cases. Thirty-six patients were treated with prednisolone, and the periaortic/periarterial lesions improved in most of them during the follow-up period. Two (50.0%) of four patients with luminal dilatation of the affected lesions before corticosteroid therapy had exacerbations of luminal dilatation after therapy, whereas none of the twenty-six patients without it had a new appearance of luminal dilatation after therapy.

Conclusions

The results of this retrospective multicenter study highlight three important points: (1) the possibility of latent existence and progression of periaortic/periarterial lesions, (2) the efficacy of corticosteroid therapy in preventing new aneurysm formation in patients without luminal dilatation of periaortic/periarterial lesions and (3) the possibility that a small proportion of patients may actually develop luminal dilatation of periaortic/periarterial lesions in IgG4-related aortitis/periaortitis and periarteritis. A larger-scale prospective study is required to confirm the efficacy and safety of corticosteroid therapy in patients with versus those without luminal dilatation and to devise a more useful and safe treatment strategy, including administration of other immunosuppressants.     

X-ray and computed tomographic diagnosis of aneurysm of the thoracic aorta

The paper is concerned with the results of x-ray and CT investigations conducted in 62 patients with suspected aneurysm of the thoracic aorta. The diagnosis was confirmed in 56. Of them 38 patients were operated on, 2 died without surgical intervention. X-ray investigations permitted differential diagnosis of mediastinal formations in 91.5% of cases. However in 2 cases when the combined method of intravenous administration of a contrast medium by means of a syringe was not used, aneurysmal aortic dilatation was not detected by CT. This method was found to be effective for diagnosis of complications of aneurysms of the thoracic aorta (calcinosis, aortic wall stratification and thrombosis of aneurysmal cavity).     

Treponemal serologic tests; experiences of the Bacteriology Laboratory,California State Department of Public Health

In a study of the relationship of clinical impression regarding syphilis and age, sex and pregnancy status to treponemal serologic test reactivity, it was noted that in diagnostic "problem cases" the standard lipid serologic test titers did not differentiate between syphilitic and biologic false positive reactors. Preliminary data indicated that heroin addiction may be a source of biologic false reactions and that pregnant women with standard serologic test reactivity have a lower treponemal reactivity rate than other women with lipid serologic reactivity.     

36 例急性Stanford A 型主动脉夹层动脉瘤的外科治疗体会

目的:总结急性Stanford A型主动脉夹层动脉瘤的外科治疗体会。方法:选择36例急性Stanford A型主动脉夹层动脉瘤患者,根据病变夹层破口的位置、累及范围、有无合并主动瓣关闭不全选择相应的手术方式,观察患者治疗后的临床疗效并随访治疗6个月后的临床预后。结果:3例患者术中死亡,术中死亡率为8.3%,平均手术时间156.7±56.7min,平均阻断时间98.5±32.7min,平均选择性脑灌注时间56.1±20.7min,术后平均ICU住院时间6.2±3.8d,术后平均总计住院时间25.4±7.3d;术后6个月后随访,3例患者死亡,其余30例患者恢复满意,生活质量与治疗前相比,均得到显著提高。结论:急性Stanford A型主动脉夹层动脉瘤患者应积极施行早期外科治疗,术前快速准确诊断,根据患者病情制定相应的手术方案,术后采取措施避免术后并发症。     

Evidence for prehistoric cardiovascular disease of syphilitic origin on the Northern Plains

The identification of the treponemas among prehistoric Amerindian populations is problematic. This paper presents the evidence for the presence of cardiovascular disease of syphilitic origin on the Plains of North America during prehistoric times. Comparative data from modern populations is used to arrive at a diagnosis.     

36例急性StanfordA型主动脉夹层动脉瘤的外科治疗体会

目的：总结急性StanfordA型主动脉夹层动脉瘤的外科治疗体会。方法：选择36例急性StanfordA型主动脉夹层动脉瘤患者,根据病变夹层破口的位置、累及范围、有无合并主动瓣关闭不全选择相应的手术方式,观察患者治疗后的临床疗效并随访治疗6个月后的临床预后。结果：3例患者术中死亡,术中死亡率为8．3％,平均手术时间156．7±56．7min,平均阻断时间98．5±32．7min,平均选择性脑灌注时1．756．1±20．7min,术后平均ICU住院时间6．2±3．8d,术后平均总计住院时间25．4±7．3d;术后6个月后随访．3例患者死亡,其余30例患者恢复满意,生活质量与治疗前相比,均得到显著提高。结论：急性Stanford A型主动脉夹层动脉瘤患者应积极施行早期外科治疗,术前快速准确诊断,根据患者病情制定相应的手术方案,术后采取措施避免术后并发症。     

Bronchiectasis and hoarseness of voice in takayasu arteritis: a rare presentation

ABSTRACT: BACKGROUND: Takayasu arteritis is a large vessel vasculitis occurring in young females. We report a rare presentation of Takayasu arteritis in a Sri Lankan woman. She presented with bronchiectasis and left recurrent laryngeal nerve palsy prior to the onset of vascular symptoms. This case illustrates an atypical presentation of this disease and the diagnostic dilemma that the physician may be faced with. CASE PRESENTATION: A 39-year-old woman presented with chronic cough, haemoptysis and hoarseness of voice. She had left recurrent laryngeal nerve palsy and high inflammatory markers on investigation. CT thorax revealed aortic wall thickening and traction bronchiectasis. 2 D echocardiogram revealed grade 1 aortic regurgitation compatible with aortitis. She did not have weak peripheral pulses or a blood pressure discrepancy and did not meet American College of Rheumatology (ACR) criteria for diagnosis of Takayasu arteritis at this stage. Tuberculosis, syphilis and sarcoidosis was excluded. While awaiting angiography, she developed left arm claudication and a pericardial effusion. Angiography revealed evidence of Takayasu arteritis and absence of flow in the left subclavian artery. Takayasu arteritis was diagnosed at this stage after a period of eight months from the onset of initial symptoms. She is currently on prednisolone, azathioprine and aspirin. CONCLUSION: Bronchiectasis and recurrent laryngeal nerve palsy is a rare presentation of Takayasu arteritis. Atypical presentations can occur in Takayasu arteritis prior to the onset of vascular symptoms. Elevation of inflammatory markers are an early finding. A high degree of suspicion is needed to identify these patients in the early course of the disease.     

Detection of allergy to antibiotics in dermatology--venereology practice

A total of 205 stationary patients with and without drug disease were examined with the purpose of diagnosing allergy to the widely used antibiotics. The reaction of leucocytosis was used in the studies and the antibiotic concentration was increased up to 1 gamma/ml. Sufficiently high specificity of the reaction for diagnosis of the medicamentous allergy was determined. Increased sensitivity was most frequent to penicillins. Participation of biologically active substances, such as heparin and histamine in exacerbation reaction in syphilitic patients treated with penicillin was found. The most pronounced increase in the levels of these substances in the blood of the patients was observed 6 hours after the beginning of the treatment without any connection without any connection with the clinical signs of the exacerbation reaction.     

老年Stanford A 型主动脉夹层外科治疗*

目的:总结老年Stanford A型主动脉夹层外科治疗经验,探讨手术方式的选择,以提高手术疗效。方法:2008年9月至2011年5月对31例老年Stanford A型主动脉夹层行手术治疗,根据夹层破口位置、累及范围、主动脉根部病变情况采取相应术式,W-heat手术2例,David+全弓置换+支架象鼻术3例,Bentall+全弓置换+支架象鼻术9例,改良Wheat+全弓置换+支架象鼻术1例,升主动脉+全弓置换+支架象鼻术16例。同时行冠状动脉旁路移植术4例,心包剥脱术1例。结果:全组体外循环(221±43)min,平均心肌阻断(132±41)min,深低温停循环(47±12)min。术后并发症12例(38.7%),其中2例死亡,8例治愈(66.7%),2例术后出现肾功能衰竭家属放弃治疗。全组病人出院前复查主动脉CTA,见升主动脉、弓部人工血管血流通畅,支架位置正常,无明显移位。支架远端降主动脉假腔闭合率87.1%。随访2~35个月,术后近期死亡1例(3.2%),无再次手术者。结论:对老年StanfordA型主动脉夹层这一高危人群,术中根据其病变部位施行最佳的外科手术方式,可明显降低死亡率,改善患者预后。     

THE VALUE OF ECHOCARDIOGRAPHY IN THE MANAGEMENT OF ACUTE AORTIC INSUFFICIENCY

Three cases of acute rupture of the aortic cusp complicating bacterial endocarditis are described. Special emphasis is placed on the value of serial echocardiography as a means of identifying progressive changes in aortic valve anatomy during the destructive process of the disease. Prior to the results of blood cultures, an echocardiogram confirmed a diagnosis of vegetations on the valve cusps. It also demonstrated flailing of the aortic cusp, which was confirmed at operation. Echocardiographic findings of flailing aortic valves in these patients coincided with the onset of acute aortic insufficiency and contributed to the timing of surgical intervention for replacement of the affected valves.     

Factitious Aortic Valve Insufficiency

One hundred consecutive aortograms, performed with careful attention to recommended technical details, were reviewed to identify cases of “factitious” aortic valve insufficiency, viz. aortic regurgitation seen during aortography for which there is no clinical evidence. Five patients with this condition were identified. Two of these subsequently underwent mitral valve replacement under cardiopulmonary by-pass. Aortic insufficiency was not detected during this procedure and the aortic valve appeared to be anatomically normal at postmortem examination. That factitious aortic insufficiency may exist should be remembered when aortography is used to differentiate aortic from pulmonary valve insufficiency.