Anthony W. Stanson

Penetrating atherosclerotic ulcers of the thoracic aorta: natural history and clinicopathologic correlations

Clinically, penetrating atheromatous ulceration of the aortic wall may be confused with either symptomatic thoracic aneurysm or classic spontaneous aortic dissection. Aortography and computed tomographic (CT) scanning of the thoracic aorta provide specific diagnostic information which permits one to distinguish this lesion from atherosclerotic aneurysm and classic dissection. Hallmarks of findings on aortography and CT scan include the presence of the ulcer and an intramural hematoma. Since the findings may be disarmingly subtle, the potentially progressive and serious nature of this condition may remain unappreciated. Recognition of the penetrating atheromatous ulcer and distinguishing it from aortic dissection arising just distal to the origin of the left subclavian artery is mandatory. Resection of only a conservative segment of the proximal descending aorta suffices for classic dissection in the upper descending thoracic aorta, but the penetrating aortic ulcer requires graft replacement in the area of the ulcer and intramural hematoma.

Clinical Features and Differential Diagnosis of Aortic Dissection: Experience With 236 Cases (1980 Through 1990)

Acute aortic dissection is the most common fatal condition that involves the aorta; nevertheless, despite major advances in noninvasive diagnosis, the correct antemortem diagnosis is made in less than half the cases. To promote continued improvement in the prompt recognition of aortic dissection, we present a review of the Mayo Clinic experience with 235 patients who had 236 substantiated aortic dissections. At the time of initial assessment, 158 patients (67%) had acute and 78 patients (33%) had chronic aortic dissection. Hypertension was the most common predisposing factor (78% of patients overall). The acute onset of severe chest pain was the most common initial complaint (74%), but 33 patients (15%) had painless aortic dissection and abnormal chest roentgenographic findings. Less common manifestations included congestive heart failure, syncope, cerebrovascular accident, shock, paraplegia, and lower extremity ischemia. The initial clinical impression was aortic dissection in 62% of patients overall. In 17 patients (28%), the correct diagnosis was not made before postmortem examination. Although the clinical features of aortic dissection have gained wider appreciation, the diagnosis still remains unsuspected in a substantial number of patients. In a patient who has a catastrophic illness and unexplained symptoms that could be of vascular origin, especially in the presence of chest pain, aortic dissection should always be included in the differential diagnosis.

Klippel-Trénaunay syndrome : Spectrum and management

OBJECTIVE To describe a series of 252 patients with Klippel-Trénaunay syndrome (KTS), a rare congenital malformation characterized by the triad of capillary malformations, atypical varicosities or venous malformations, and bony or soft tissue hypertrophy usually affecting one extremity. MATERIAL AND METHODS We reviewed the clinical characteristics and findings in 136 female and 116 male patients with KTS who underwent assessment at Mayo Clinic Rochester between January 1956 and January 1995. In addition, management options are discussed. RESULTS Capillary malformations (port-wine stains) were found in 246 patients (98%), varicosities or venous malformations in 182 (72%), and limb hypertrophy in 170 (67%). All three features of KTS were present in 159 patients (63%), and 93 (37%) had two of the three features. Atypical veins, including lateral veins and persistent sciatic vein, occurred in 182 patients (72%). Operations performed in 145 patients with KTS included epiphysiodesis, stripping of varicose veins or venous malformations, excision of vascular malformations, amputations, and debulking procedures. CONCLUSION Most patients with KTS should be managed conservatively. The clearest indication for operation is a leg length discrepancy projected to exceed 2.0 cm at skeletal maturity, which can be treated with epiphysiodesis in the growing child. If a functioning deep vein system is present, removal of symptomatic varicosities or localized superficial venous malformations in selected patients can yield good results.

Arterial Fibromuscular Dysplasia

Fibromuscular dysplasia is a nonatherosclerotic, noninflammatory vascular disease that involves primarily the renal and internal carotid arteries and less often the vertebral, iliac, subclavian, and visceral arteries. Although its pathogenesis is not completely understood, humoral, mechanical, and genetic factors as well as mural ischemia may play a role. The natural history is relatively benign, with progression occurring in only a minority of the patients. Typical clinical manifestations are renovascular hypertension, stroke, subarachnoid hemorrhage, abdominal angina, or claudication of the legs or arms. In patients with symptoms, percutaneous transluminal angioplasty has emerged as the treatment of choice in most involved vascular beds.

Pulmonary Arteriovenous Fistulas: Mayo Clinic Experience, 1982-1997

OBJECTIVE To describe the results of analysis of clinical, physiologic, diagnostic, and therapeutic aspects and complications in patients with pulmonary arteriovenous fistulas (PAVFs). PATIENTS AND METHODS Retrospective review of medical records of all patients with the diagnosis of PAVF evaluated at Mayo Clinic Rochester from 1982 through 1997. Demographic characteristics, presence or absence of hereditary hemorrhagic telangiectasia, clinical features, and results of imaging studies and blood gas analyses, treatments, and complications related to PAVFs were reviewed. RESULTS Among the 93 patients, 44 were male and 49 female. The mean age at the time of evaluation was 40 years (range, 5-83 years). Fifteen patients (16%) were asymptomatic. History of hereditary hemorrhagic telangiectasia was present in 52 patients (56%). Notable clinical findings included epistaxis in 46 (49%), hemoptysis in 14 (15%), cyanosis in 27 (29%), clubbing in 18 (19%), dyspnea in 53 (57%), and pulmonary bruits/murmurs in 32 (34%). Chest x-ray films with or without tomograms showed abnormal findings in 87 (94%), of which 68 (73%) suggested PAVF. Polycythemia was detected in 12 (13%). Pretherapy arterial PO2 measured on room air averaged 56 mm Hg (range, 32-95 mm Hg), and the posttherapy PO2 averaged 77 mm Hg (range, 46-110 mm Hg). Echocardiography with indocyanine green dye was diagnostic of extracardiac right-to-left shunt in 26 (90%) of 29 patients tested. Diagnostic studies revealed single lesions in 32 patients (34%) and multiple lesions in 61 (66%). The most prominent complications of the disease were neurologic events in 34 patients (37%). These complications included transient ischemic attacks, hemiplegia, brain abscesses, and seizures. Surgical resection alone was carried out in 18 patients (19%), embolization therapy alone in 41 (44%), and both therapies in 7 (8%). The 48 patients treated with embolization required 78 embolization sessions with more than 200 lesions occluded. Complications of treatment included postembolization hemothorax in 1 patient and right-sided hemiparesis in another patient. Follow-up disclosed that 1 patient died from PAVF-related complications. CONCLUSIONS Among our patients with PAVFs, hereditary hemorrhagic telangiectasia was observed in more than half and neurologic complications in more than one third. Because of the considerable risk of neurologic and other complications, definitive treatment should be considered in patients with PAVFs. Embolization is currently the preferred treatment in most patients. Frequent follow-up of treated patients is necessary because PAVFs tend to increase both in number and in size over time.

Splanchnic Artery Aneurysms

Autopsy studies suggest that splanchnic artery aneurysms may be more frequent than abdominal aortic aneurysms. These aneurysms are important to recognize because up to 25% may be complicated by rupture, and the mortality rate after rupture is between 25% and 70%. However, little is known about the natural history and clinical presentation of splanchnic artery aneurysms. Splenic artery aneurysms are the most common of the splanchnic artery aneurysms; multiple aneurysms are present in approximately one third of patients. Hepatic artery pseudoaneurysms are more common than true aneurysms because of increasing numbers of hepatobiliary interventional procedures. The diagnosis of splanchnic artery aneurysm should be considered in any patient with abdominal pain, a pulsatile mass, or an abdominal bruit with or without associated bleeding. However, most aneurysms are asymptomatic and are detected incidentally on imaging studies. Treatment, which can be either surgical or interventional radiology-based, should be considered in all patients with symptoms related to the aneurysms, if the aneurysm is more than 2 cm in diameter, if the patient is pregnant, or if there is demonstrated growth of the aneurysm.

Cogan's syndrome: 18 cases and a review of the literature

In this article, we review 78 cases of Cogans syndrome--18 from our institution and 60 from the English medical literature--and describe the clinical, laboratory, radiographic, and pathologic findings associated with this disorder. Patients with Cogans syndrome should be examined by an ophthalmologist, otolaryngologist, and internist because, in addition to the eye and audiovestibular involvement, systemic manifestations are common. An elevated erythrocyte sedimentation rate, anemia, leukocytosis, and thrombocytosis are common but nonspecific laboratory abnormalities. Serious outcomes include deafness and, less frequently, vasculitis, aortic insufficiency, blindness, and death. Glucocorticoids seem to be effective therapy for active Cogans syndrome. Aortic valve replacement and vascular bypass grafting are indicated in selected cases. The roles of cytotoxic-immunosuppressive agents and cochlear implantation remain to be clarified.

Outcomes of Atherosclerotic Renal Artery Stenosis Managed Without Revascularization

OBJECTIVE To determine how often patients with renal artery stenosis (RAS) managed without revascularization progress to accelerated hypertension and/or renal failure. PATIENTS AND METHODS We examined the outcomes of 68 patients (mean +/- SEM age, 71.8 +/- 0.9 years) with high-grade (>70%) RAS identified between 1989 and 1993 who were treated without renal revascularization for at least 6 months after angiography. The time to last follow-up averaged 38.9 +/- 2.8 months. Other vascular beds were affected in 66 of the 68 patients. End points were revascularization, nephrectomy, dialysis, or death. RESULTS The mean +/- SEM serum creatinine level rose from 1.4 +/- 0.1 to 2.0 +/- 0.2 mg/dL (P<.001). Mean +/- SEM blood pressure did not change (157 +/- 3/83+/-2 vs 155 +/- 3/79 +/- 2 mm Hg), but the need (mean +/- SEM) for medication increased from 1.6+/-0.1 to 1.9+/-0.1 drugs (P=.02). Four patients (5.8%) eventually underwent renal revascularization for refractory hypertension (1 patient), for progressive stenosis (1 patient), and during aortic reconstruction (2 patients). One additional patient underwent nephrectomy to improve blood pressure control. Five others (7.4%) developed end-stage renal disease (ESRD) for reasons other than progressive vascular disease, namely, diabetes (3 patients), atheroemboli (1 patient), and contrast toxicity without RAS progression (1 patient). In 1 further case, the reason for ESRD was unknown, and it may have been caused by vascular occlusion. During follow-up, 19 patients died of unrelated causes, including myocardial infarction and stroke. CONCLUSIONS These data indicate that antihypertensive medication requirements increased and renal function deteriorated modestly in a subset of patients with atherosclerotic RAS managed initially without vascular intervention. Many achieved stable blood pressure for many years. Deterioration of renal function and mortality risk were greatest in patients with bilateral stenosis or stenosis to a solitary functioning kidney. These results reinforce the need for meticulous follow-up for disease progression but underscore the role of competing risks and high mortality from other cardiovascular diseases, which primarily determine the outcomes in patients with RAS and widespread atherosclerotic disease.

Reconstruction of large veins for nonmalignant venous occlusive disease

To evaluate the effectiveness of venous grafting, we reviewed the management and clinical course of 28 patients (21 males and seven females) who underwent 29 reconstructions of large veins for benign disease. There were 12 patients with superior vena cava (SVC) syndrome, two with subclavian vein thrombosis, and 15 with occlusion of the inferior vena cava (IVC) or iliac veins. One of these patients underwent both IVC and SVC reconstructions. Reconstruction of the SVC was performed with spiral saphenous vein graft (SSVG) in nine patients and expanded polytetrafluoroethylene (ePTFE) in three. All seven straight SSVGs had documented patency at a median of 7 months (2 weeks to 5 years) after reconstruction. Six patients had complete relief of symptoms. Two patients with bifurcated SSVG had early occlusion of one graft limb. Two of the three ePTFE grafts needed early thrombectomy. One graft reoccluded at 6 months and two were patent at 2 and 5 years. The two subclavian vein reconstructions with axillary-jugular ePTFE grafts with an arteriovenous fistula had documented early patency. Both patients had rapid resolution of symptoms. The IVC or iliac vein was reconstructed with ePTFE graft in 11 patients, SSVG in three, and Dacron in one. A femorofemoral arteriovenous fistula was added in eight patients with ePTFE grafts. Seven of the 11 ePTFE grafts had documented patency at the last follow-up (median 9 months; range 2 weeks to 5 years). None of the three SSVGs had documented long-term patency. The one Dacron cavoatrial graft occluded at 3 years. A straight SSVG continues to be our first choice for SVC replacement. Short, large-diameter ePTFE grafts perform the best in the abdomen. Femorocaval or long iliocaval grafts need an arteriovenous fistula to maintain patency. Long-term patency after closure of the fistula is still unknown. Femorocaval grafts with poor venous inflow have limited chance of success. Failed or failing grafts may be salvaged by early thrombectomy. Venous reconstruction to treat selected patients with symptoms with large vein occlusion continues to be a viable option.

What are the keys to successful adrenal venous sampling (AVS) in patients with primary aldosteronism

Adrenal venous sampling (AVS) is the criterion standard to distinguish between unilateral and bilateral adrenal disease in patients with primary aldosteronism. The keys to successful AVS include appropriate patient selection, careful patient preparation, focused technical expertise, defined protocol, and accurate data interpretation. The use of AVS should be based on patient preferences, patient age, clinical comorbidities, and the clinical probability of finding an aldosterone‐producing adenoma. AVS is optimally performed in the fasting state in the morning. AVS is an intricate procedure because the right adrenal vein is small and may be difficult to locate – the success rate depends on the proficiency of the angiographer. The key factors that determine the successful catheterization of both adrenal veins are experience, dedication and repetition. With experience, and focusing the expertise to 1 or 2 radiologists at a referral centre, the AVS success rate can be as high as 96%. A centre‐specific, written protocol is mandatory. The protocol should be developed by an interested group of endocrinologists, radiologists and laboratory personnel. Safeguards should be in place to prevent mislabelling of the blood tubes in the radiology suite and to prevent sample mix‐up in the laboratory.