Deep venous thrombosis and pulmonary embolism. Risk of subsequent malignant neoplasms.

We conducted a noncurrent prospective study of all Olmsted County, Minnesota, residents who had had a lower-extremity venogram, pulmonary angiogram, or lung scan performed because of suspicion of deep venous thrombosis or pulmonary emboli. One hundred thirteen cancer-free patients were followed for 386 person-years from the date of procedure. Nine subsequent cancers were observed compared with 4.5 expected (relative risk, 2.0; 95% confidence interval, 0.9 to 3.8), using total cancer incidence rates for the Rochester, Minn, population. Five hundred seventeen cancer-free controls were followed for 2072 person-years. Twenty subsequent cancers were observed compared with 11.6 expected, yielding a relative risk of 1.7 (95% confidence interval, 1.1 to 2.7). When cases and controls were compared directly, no statistically significant difference in cancer-free survival was found.

Takayasu arteritis. A study of 32 North American patients.

Thirty-two patients (26 female, 6 male) with angiographically diagnosed Takayasu arteritis were seen at the Mayo Clinic between 1971 and 1982. Racial composition of this group was 23 North American Caucasians, 4 Mexicans, 3 Orientals, 1 Native American, and 1 patient of Middle Eastern origin. Incidence of the disease in Olmsted County, Minnesota, was 2.6/million/year. Diagnosis was often delayed for long periods of time, with a median delay of 18 months. Patients had both non-vascular symptoms (arthralgias in 56%, fever in 44%, weight loss in 38%) and symptoms of vascular stenosis such as arm claudication (47%) and hypertension due to renal artery stenosis (41%). All patients had either multiple vascular bruits (94%) or absent pulses (50%). Laboratory findings included anemia (44%) and elevations of erythrocyte sedimentation rate (78%). Almost all patients had multiple sites of arterial involvement documented by angiogram with various combinations of stenosis, luminal irregularity and aneurysm formation. Response to corticosteroid treatment was usually very good, with dramatic improvement in non-vascular symptoms and return of pulses in 8 of the 16 patients with absent pulses prior to treatment. Five-year survival rate from time of diagnosis was 94%. Twelve patients underwent surgical procedures involving the carotid arteries (5 cases), subclavian artery (4 cases) and renal arteries (3 cases). Three aneurysms were resected, one had aortic valve replacement for severe aortic regurgitation, and two patients underwent transluminal angioplasty. Pathologic changes were restricted to the media and adventitial layers of the vessel wall and were indistinguishable from those of giant-cell or temporal arteritis. Takayasu arteritis is more common than previously suspected in North America, is not restricted to any one racial group, and is readily treatable with corticosteroids and surgical vascular reconstruction.

Budd-Chiari syndrome: early intervention with angioplasty and thrombolytic therapy.

We present a complex case of the Budd-Chiari syndrome due to thrombosis of the hepatic veins in the presence of stenosis of the left hepatic vein and membranous obstruction of the inferior vena cava. The acute thrombosis occurred after laparoscopic surgical removal of the gallbladder. Because we strongly suspected the Budd-Chiari syndrome, hepatic venography was performed. The hepatic venous outflow obstruction was relieved by angioplasty and thrombolytic therapy with use of local infusions of urokinase into the clot. We propose that angiography be performed in patients in whom the Budd-Chiari syndrome is suspected and that angioplasty and thrombolytic therapy be initiated early.

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.

Constrictive pericarditis: assessment of current diagnostic procedures.

Constrictive pericarditis frequently poses a diagnostic challenge because of its varied manifestations. Accurate diagnosis is essential, however, because surgical decortication may yield excellent clinical results. Although new diagnostic procedures have helped the clinician to diagnose constrictive pericarditis, the initial clinical suspicion of this diagnosis must be high for appropriate interpretation of these tests. Echocardiography is useful, primarily for distinguishing various other cardiac abnormalities that may simulate constrictive pericarditis. Computed tomography is a valuable procedure for assessment of pericardial thickening. In addition, evaluation of early diastolic filling by computerized digitization in conjunction with echocardiography, angiography, and invasive hemodynamics shows promise as a diagnostic tool. Even with these new diagnostic aids, distinguishing constrictive pericarditis from restrictive cardiomyopathy may be difficult and, in some cases, may necessitate an exploratory operative procedure.

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.

Snare removal of a telectronics accufix atrial J retention wire.

A voluntary recall of the Telectronics Accufix models 330-801 and 329-701 "J"-shaped atrial pacemaker leads has been issued because of fracture and extrusion of the J-shaped retention wire. Such extrusion can result in laceration of the atrium or surrounding vascular structures. Herein we describe a patient with a fractured and extruded retention wire that was snared and removed percutaneously; the atrial lead was left intact, and pacing function was satisfactory. This unique procedure can be used to avoid the morbidity associated with percutaneous lead extraction or thoracotomy and to prevent potential dislodgment and embolization of the retention wire during lead extraction.

Isolated renal artery dissection, presentation, evaluation, management, and pathology.

Isolated nontraumatic renal artery dissection is rare. In this communication, 35 cases are presented; 24 cases (group 1) (22 male, 2 female) were diagnosed angiographically, and 11 (group 2) (10 male, 1 female) were observed at autopsy. In group 1, 23 of the patients were hypertensive when they were first seen, and in 17 of them the hypertension was of recent onset. Additional presenting signs and symptoms included flank pain (10 patients), gross hematuria (5), and headaches (6). Renal function was satisfactory. Renal vein renin levels could be lateralized in 8 of 16 patients. Isotope renograms performed in 18 patients, showed unilateral abnormalities in 7, bilateral abnormalities in 6, and normal results in 5. Angiograms showed that the dissection was unilateral in 18 cases and bilateral in 6 cases. Fibromuscular dysplasia was observed radiographically in 22 cases and was bilateral in 12. In group 1, 13 patients were treated with antihypertensive medication only, and 11 underwent operation. At follow-up (mean 52.0 months), the mean blood pressure were 128/88 mm Hg and 139/89 mm Hg for the medical and surgical groups, respectively. Eleven medical and nine surgical patients continued to require antihypertensive drugs at follow-up. Among the 11 patients in group 2, only 4 were hypertensive. In only one case the dissection may have contributed significantly to the patient's death. These studies indicate that isolated nontraumatic renal artery dissection most commonly occurs in young men with coexistent fibromuscular dysplasia. Hypertension is commonly present and therapy should be directed toward its control. In this study, blood pressure control was effectively accomplished with medical therapy.

Surgical treatment of congenital pelvic arteriovenous malformation.

Congenital arteriovenous fistulas often pose difficult diagnostic and management problems. Surgical therapy has been associated with a propensity for incomplete obliteration and recurrence. A case of a large congenital pelvic arteriovenous malformation, initially misdiagnosed as an atrial septal defect, was defined by selective roentgenographic and contrast echographic techniques and successfully treated by total surgical excision.

Abdominal angina and neurofibromatosis.

A young man with severe abdominal pain and weight loss due to intestinal ischemia was examined. At operation, the origins of the celiac and superior mesenteric arteries were found to be compressed by plexiform neurofibromatosis. Symptoms were relieved by reconstructive arterial surgery.

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.

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

In this article, we review 78 cases of Cogan's 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 Cogan's 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 Cogan's 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.

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.

Percutaneous transluminal angioplasty in the lower extremities: a 5-year experience.

From January 1979 to March 1984, percutaneous transluminal angioplasty (PTA) was used to treat 148 limbs of 135 Mayo Clinic patients with occlusive arterial disease of the lower extremities. The procedure was technically successful in more than 95% of the attempts. The outcome was clinical improvement in 89 limbs and no improvement in 40 limbs; in 19 limbs, PTA was technically successful but the patient was dismissed from the hospital and lost to follow-up before the extent of improvement could be determined. Mean ankle/brachial pressure indices increased after PTA in those with clinical improvement but not in those without improvement. Clinical improvement was less likely to follow PTA in patients with advanced age, diabetes, severe initial symptoms, low ankle/brachial indices, or distal occlusive disease. In patients with improvement after PTA, the mean follow-up period was 33 months; during that time, failure (defined as recurrence of the original symptoms or the need for repeat PTA or operation) occurred at a rate of 6.4% per year. Serious complications occurred after three procedures (2.0%). We conclude that PTA is technically feasible and generally safe for many patients with occlusive arterial disease of the lower limbs.

Percutaneous placement of the Greenfield vena caval filter.

During the period from August 1986 to August 1987, 50 patients underwent percutaneous placement of a Greenfield vena caval filter from the right femoral vein, left femoral vein, or right internal jugular vein at our institution. All 50 patients had a contraindication to anticoagulation therapy or had complications of anticoagulation for deep venous thrombosis or pulmonary emboli. The percutaneous placement was accomplished in the angiographic suite with use of local anesthesia and was well tolerated by all patients. Only three complications related to the percutaneous approach occurred during the short-term follow-up (3 months to 1 year). These complications were deep venous thrombosis of the leg in two patients and misplacement of the filter in one patient. The three patients tolerated these complications well. We conclude that placement of Greenfield vena caval filters can be readily accomplished by means of percutaneous entry. Our experience demonstrated minimal associated morbidity and no mortality.

Microvascular pedal bypass for salvage of the severely ischemic limb.

Bypass to the pedal arteries was performed with use of the operating microscope and standard microsurgical technique in 37 patients with severe, chronic ischemia of a lower extremity. Twenty-one patients (57%) had three or more cardiovascular risk factors, and 22 (59%) had diabetes. Preoperative arteriography identified a pedal artery suitable for bypass in all but one patient. The greater or lesser saphenous vein was used in all patients, most frequently as a nonreversed, translocated vein graft. An arm vein was used as part of a composite graft in only one patient. No early deaths occurred, and only one patient had a perioperative myocardial infarction. Although five grafts occluded within 30 days, four were successfully revised, and 36 patients had a patent graft at the time of dismissal from the hospital. At 1 year, the primary graft patency rate (patency without revision) was 60.8%, and the secondary patency rate was 68.8%. One early and six late amputations were performed; the cumulative 1-year limb salvage rate was 82.4%. Grafts with an intraoperative flow rate of 50 ml/min or more had a better patency rate than those with a lower flow rate. The presence of diabetes did not adversely affect long-term patency. Of the 34 patients who were alive at the time of this report, 27 (79%) had a functional foot that allowed ambulation, had no rest pain, and had no substantial loss of tissue. Pedal bypass should be considered for critical, chronic ischemia, even if the patient has an increased surgical risk and advanced distal atherosclerotic disease.

Adrenal insufficiency as a manifestation of disseminated non-Hodgkin's lymphoma.

OBJECTIVE: To analyze the clinical characteristics, laboratory features, and outcome in five patients who had biochemically proven adrenal insufficiency attributable to pathologically confirmed non-Hodgkin's lymphoma (NHL). MATERIAL AND METHODS: We retrospectively reviewed the medical records of all patients at Mayo Clinic Rochester during the period from 1976 to 1994 to identify those with both NHL, as listed in the surgical pathology tissue registry, and adrenal insufficiency. Histologically, the patients were classified on the basis of the working formulation and the revised European-American lymphoma classification. RESULTS: Three patients had diffuse large cell NHL, one patient had small noncleaved NHL, and one patient had cutaneous T-cell NHL. All five patients had stage IV disease. Adrenal insufficiency was confirmed by morning and evening determinations of serum cortisol levels and cosyntropin stimulation tests. All patients demonstrated loss of circadian rhythm. The median age of the patients was 77 years (range, 60 to 89). Three of the five patients died without treatment from 5 to 22 days after assessment. One patient died of a cerebrovascular accident. Despite initiation of chemotherapy, two patients died of progressive NHL at 7 weeks and 7 months. CONCLUSION: In our experience, biochemically proven adrenal insufficiency in patients with NHL is a manifestation of clinically advanced disease in elderly patients. A stepwise diagnostic approach is critical for the appropriate management of such patients.

Extraction of the Telectronics Accufix 330-801 atrial lead: the Mayo Clinic experience.

OBJECTIVE: To document a cumulative experience with removal of the Telectronics 330-801 Accufix atrial lead after it was withdrawn from the market. DESIGN: We reviewed our results with 96 attempted lead extraction procedures through Dec. 1, 1995. MATERIAL AND METHODS: All patients underwent preoperative assessment, including fluoroscopic screening of the lead. Patients had blood typing and screening done before the extraction procedure, and extractions were performed with cardiac surgical intervention available. Pacing dependence was assessed to determine the need for temporary pacing. RESULTS: The mean patient age was 65.7 +/- 1.6 years and the mean duration of lead implantation was 31.0 +/- 1.4 months. Of the 96 lead extractions attempted, 94 were successful. Of the 96 cases, the retention wires were normal in 29, fractured without protrusion in 34, and fractured with protrusion in 33. Fifty-three leads were removed with use of simple traction only, and seven leads were removed by using a locking stylet and telescoping sheaths. The Cook workstation was used in the removal of 32 leads through the femoral vein; in 1 of these 32 patients, the retention wire was removed before lead extraction. The retention wire was removed but the lead was left intact in one patient. One lead was removed during an opertive maze procedure. Associated complications were minimal. CONCLUSION: Although lead extraction is a potentially fatal procedure, the Telectronics 330-801 Accufix atrial lead can be extracted safely with minimal morbidity when performed by experienced personnel.

Rheumatoid vasculitis manifesting as intra-abdominal hemorrhage.

Rheumatoid vasculitis, an extra-articular component of rheumatoid arthritis, causes a wide spectrum of manifestations that range from clinically insignificant to life-threatening disease. As a systemic necrotizing arteritis, rheumatoid vasculitis is usually characterized by end-organ ischemia. Herein we describe a patient with abdominal pain and syncope due to intra-abdominal hemorrhage from a ruptured aneurysm of the inferior pancreaticoduodenal artery in the setting of rheumatoid vasculitis. Although the intra-abdominal hemorrhage was the unusual manifestation of rheumatoid vasculitis in this patient, he had a history of prior extra-articular rheumatoid disease, including pulmonary fibrosis and Sjögren's syndrome with associated parotid lymphoproliferative disease. In patients with rheumatoid arthritis who have abdominal pain and an unexplained rapidly decreasing hemoglobin concentration, the diagnosis of intra-abdominal hemorrhage from a ruptured visceral aneurysm due to rheumatoid vasculitis should be considered, even in the absence of other indications of systemic vasculitis.

Transluminal angioplasty for basilar artery stenosis.

This report describes for the first time the successful use of transluminal balloon-catheter dilation (angioplasty) for high-grade basilar artery atherosclerotic stenotic lesions in two patients with frequent, severe, progressing symptoms that were unresponsive to anticoagulant therapy. Excellent angiographic and short-term clinical results have been obtained. These results and the relative frequency of these lesions justify further evaluation of this approach.