Secondary hypertension due to isolated interrupted aortic arch in a 45-year-old person: A case report.

RATIONALE: Though it is rare, isolated interrupted aortic arch (IAA) could lead to hypertension. Surgical repair is the only effective curative method to treat IAA conditions and patients with IAA can hardly survive to adulthood with medication alone. We report an IAA case that of a 45-year-old male patient who survived for 45 years without surgical treatment. PATIENT CONCERNS: A 45-year-old man was referred to the hospital presenting with abnormal blood pressure level. Both computed tomography angiogram (CTA) and angiography revealed IAA. DIAGNOSES: The patient was diagnosed as IAA based on computed tomography angiogram (CTA) and angiography. INTERVENTIONS: The patient's blood pressure was severely high and refractory. He refused surgical treatment and accepted antihypertensive medication for 10 days. OUTCOMES: The patient's office blood pressure level was abnormal, fluctuating between 140/90 and 160/100 mm Hg, but 24-hour ambulatory blood pressure monitoring showed normal level. LESSONS: Hypertension due to IAA could be controlled with medications, even surgery is not performed. The discrepancy between ambulatory and office blood pressure levels may be due to the white coat effect.

MRI and FDG-PET in the assessment of inflammatory aortic arch syndrome in complicated courses of giant cell arteritis.

OBJECTIVES: To evaluate the use of MRI and FDG-PET for the diagnosis and measurement of disease activity of inflammatory aortic arch syndrome in patients with complicated giant cell arteritis. METHODS: MRI and FDG-PET were performed for 25 patients with giant cell arteritis who presented with a complicated disease course despite immunosuppressive therapy. Disease activity of the thoracic aorta and the supra-aortic arteries as assessed by both modalities was compared with serological (C-reactive protein (CRP), erythrocyte sedimentation rate (ESR)) and clinical findings (Birmingham vasculitis activity score (BVAS.2)). Additionally, the usefulness of MRI for assessment of vessel wall thickening, aneurysms and stenoses was evaluated. RESULTS: In 17/25 patients, MRI disclosed structural vessel lesions suspicious for vasculitis. Active disease was detected by MRI, thoracic PET, and whole body PET in 22, 14 and 20 patients, respectively. While serological and clinical findings correlated significantly with each other, there was no concordance with MRI and only low, non-significant correlation of PET with CRP (r(s) = -0.158, 0.136), ESR (r(s) = -0.232, 0.320) and BVAS.2 (r(s) = -0.064, 0.221) for disease activity. CONCLUSIONS: MRI and PET are unreliable for assessing large-vessel inflammation in patients with giant cell arteritis and pre-existing immunosuppressive therapy. MRI is valuable for its ability to detect morphological vessel lesions, such as aneurysms and stenoses.

[Coronary artery bypass graftingin a patient with aortitis syndrome; report of a case].

We present a case of surgical treatment for a coronary lesion due to aortitis syndrome. A 41-year-old woman, suffering from aortitis syndrome and under prednisolone (PSL) therapy, underwent coronary artery bypass grafting (CABG). Surgical treatment was performed according to the aortic no-touch technique, but the patient showed a poor cardiac performance 5 days after the operation. This accident was resolved by increasing the PSL dose. Aortitis syndrome treated with PSL needs careful perioperative management besides an operative procedure.

[Modern principles of the infusion therapy in operative interventions on aorta and its branches]. / Suchasni pryntsypy infuziinoï terapiï pry operatyvnykh vtruchanniakh na aorti ta ïï hilkakh.

The experience of anesthesiological support of operative interventions on aorta and its branches was summarized. Original method of the small-volume infusional therapy during anesthesy was elaborated and introduced. Statistical indexes, witnessing the advantages of the method proposed, were adduced.

Bilateral anterior ischemic optic neuropathy as a presentation of Takayasu's disease.

Takayasu's disease is an arteritis with a predilection for the proximal branches of the aortic arch. Chronic retinal hypoxic changes and ischemic ocular inflammation, resulting from cervical vascular occlusion, have been described extensively. Anterior ischemic optic neuropathy has rarely been reported in this condition, and only in conjunction with other ophthalmic findings. Here we present the case of a patient who developed bilateral anterior ischemic optic neuropathy in the absence of other eye findings, and was subsequently found to have an aortic arch syndrome compatible with Takayasu's disease.

[Open heart surgery in patients with systemic diseases requiring steroid treatment].

Our surgical experiences in 9 patients who required steroid treatment for systemic diseases before, during and after the open heart surgery were reviewed. Subjects included 3 patients with systemic lupus erythematodes, 3 with aortitis syndrome, 1 with Behçet disease and 1 with rheumatoid arthritis. Cardiovascular lesion was aortic valve regurgitation in 2, Stanford A aortic dissection in 1 and ischemic heart disease in 3 patients. Duration of morbidity for systemic diseases before the surgery ranged between 0 nd 102 months, with a mean of 36 months. Steroid treatment was continued for 4 to 216 months (mean 70+/-76 months) before the surgery at a dose of 5-40 mg per day for conversion into prednisolone. In principal, methylprednisolone was given during the surgery, and the prednisolone was given at a dose of 20-140 mg per day on the day of operation or on the first postoperative day. Surgical procedures included a aortic valve prosthesis with Dacron cloth skirt implantation in 1 patient, surgical angioplasty of the left main coronary ostium in 1 and internal thoracic arteries grafting in 2 patients. Hospital mortality was 1 patient due to low cardiac output syndrome. Acute renal failure occurred in 2 patients with systemic lupus erythematodes, and wound complication was observed in 2 patients. In our experience, appropriate treatment for systemic diseases, timing of surgery and continuation of steroid treatment at an appropriate dose during and after the surgery seemed very important such as surgical procedure in order to prevent postoperative complications such as periprosthetic leakage and failure of anastomosis.

[A case of surgery for annuloaortic ectasia and aortic regurgitation complicated by ulcerative colitis and aortitis syndrome].

We performed an operation for AAE and AR complicated by ulcerative colitis and aortitis syndrome. The patient was a 20-year-old male who had been treated for ulcerative colitis in our hospital since 1983, when he was 18 years old. In 1985, he was admitted to our hospital for treatment and evaluation of left heart failure. He was diagnosed as having AAE and AR due to aortitis syndrome, and steroid therapy was started. He developed heart failure, and surgery was indicated. At operation, before clamping the aorta we made a composite graft. The ascending aorta and aortic valve were replaced by the composite graft, and button-shaped coronary ostia were sutured directly into the graft. His postoperative course was uneventful and he was discharged. He is now maintained on steroid therapy for his aortitis syndrome.

Regression of carotid stenoses after corticosteroid therapy in occlusive thromboaortopathy (Takayasu's disease).

A 34-year-old, single Japanese woman with active Takayasu's disease presented with stenosed bilateral common carotid arteries, subtotal occlusion of the left subclavian artery, and complete occlusion of the ipsilateral proximal vertebral artery, as demonstrated by angiography. A right subclavian-left vertebral bypass using an autologous saphenous vein graft was successfully performed after 36 days of corticosteroid therapy. Subsequently, warfarin was administered in addition to prednisolone. After 32 months of treatment with a gradual reduction from 50 to 3.75 mg of prednisolone daily, angiography revealed a functioning bypass graft and regression of bilateral common carotid stenoses.

Aortic arch arteritis in the elderly. An important manifestation of giant cell arteritis.

Five elderly patients with aortic arch syndrome secondary to large-vessel vasculitis all presented with upper-extremity claudication and absence of blood pressure and pulses in the affected extremity. Diagnosis was suspected by a markedly elevated erythrocyte sedimentation rate associated with constitutional symptoms and angiograms showing changes consistent with arteritis of the aortic arch vessels. Treatment with high-dose corticosteroids resulted in rapid resolution of constitutional symptoms and improved functional use of the upper extremity. Recognition of this steroid-responsive disease is essential to avoid unnecessary reconstructive surgery and to prevent catastrophic events such as aortic dissection or visual loss, which may result from the underlying disease process.

Takayasu's disease. A report of 3 cases.

Three patients with Takayasu's disease are described. In 2 cases aortography demonstrated an occlusion of the great vessels at the origin of the arch of the aorta. In 1 patient there was concomitant aneurysmal dilatation of the brachiocephalic trunk. Common and internal carotid artery stenosis occurred in 1 patient and was associated with hemiplegia and blindness. All 3 patients had constitutional symptoms and signs of the disease as well as markedly elevated erythrocyte sedimentation rates; 2 patients had moderate clinical responses to steroid administration in the short term and 1 developed bilateral calf vein thrombosis, which responded satisfactorily to conservative management.