Pulmonary arteriovenous malformations associated with migraine with aura.

Migraine with aura (MA) is associated with cardiac right-to-left shunt. We prospectively studied the association between pulmonary arteriovenous malformations (PAVMs) and MA in hereditary haemorrhagic telangiectasia (HHT). All 220 consecutive HHT patients who underwent high-resolution chest computed tomography for PAVM screening were included prospectively. Prior to screening, a structured validated headache questionnaire was completed by 196 patients (57% female; mean+/-sd age 44.6+/-15.2 yrs). Two neurologists diagnosed migraine according to the International Headache Society Criteria. A PAVM was present in 70 (36%) patients. The prevalence of MA was 24% in the presence of a PAVM compared with 6% in the absence of a PAVM (OR 4.6, 95% CI 1.84-11.2; p = 0.001), and MA was an independent predictor for the presence of PAVM using multivariate analysis (OR 3.6, 95% CI 1.21-10.5; p = 0.02). A PAVM was present in 68% of the patients with MA compared with 32% in the non-migraine controls (OR 4.6, 95% CI 1.84-11.2; p = 0.001), and a PAVM was an independent predictor for MA using multivariate analysis (OR 3.0, 95% CI 1.00-9.20; p = 0.05). In conclusion, PAVMs are associated with MA in HHT patients.

Screening for pulmonary arteriovenous malformations using transthoracic contrast echocardiography: a prospective study.

Pulmonary arteriovenous malformations (PAVMs) are associated with severe neurological complications in patients with hereditary haemorrhagic telangiectasia (HHT). The objective of the present study was to prospectively establish the diagnostic value of transthoracic contrast echocardiography (TTCE) as a screening technique for PAVM using chest high-resolution computed tomography (HRCT) as the gold standard for PAVMs. All consecutive adult patients referred for HHT screening underwent a chest HRCT (n = 299), TTCE (n = 281), arterial blood gas analysis (n = 291), shunt fraction measurement (n = 111) and chest radiography (n = 296). TTCE was positive in 87 (58.8%), 12 (16.7%) and four (6.7%) patients, and chest HRCT was positive in 54 (36.5%), three (4.2%) and zero (0%) patients with a definite, possible and negative clinical diagnosis of HHT, respectively. Two patients with a negative TTCE were diagnosed with PAVMs after computed tomography; in both cases the PAVMs were too small to be treated by embolotherapy. The sensitivity of TTCE was 97% (95% confidence interval (CI) 93.6-98.3) and negative predictive value 99% (95% CI 96.9-99.8). The other diagnostic tests showed a considerable lower diagnostic value. The present prospective study shows that transthoracic contrast echocardiography has an excellent diagnostic value and can be used as an initial screening procedure for pulmonary arteriovenous malformations. The high false-positive rate of transthoracic contrast echocardiography possibly represents microscopic pulmonary arteriovenous malformations.

Use of a balloon-expandable, radially reinforced ePTFE endograft after remote SFA endarterectomy: a single-center experience.

PURPOSE: To report our experience with endovascular femoropopliteal bypass grafting using a distensible, radially reinforced polytetrafluoroethylene endograft combined with remote endarterectomy. METHODS: Forty-one patients (33 men; mean age 70 years, range 45-79) with symptomatic femoropopliteal occlusive disease underwent remote endarterectomy of the superficial femoral artery (SFA) followed by implantation of a balloon-expandable Enduring endovascular graft. All patients entered an extensive surveillance program, including angiography and duplex scanning at regular intervals. RESULTS: Endarterectomy and endograft implantation were ultimately successful in all patients; 5 (12%) technical difficulties occurred intraoperatively and were treated with additional endovascular techniques. Control angiography at 1 week postoperatively demonstrated a patent endograft in 39 (95%) patients. Mean ankle-brachial index increased significantly from 0.57 to 0.91 (p < 0.001). Including the 2 early failures, 18 occlusions were documented over a median 15-month follow-up (range 3-24), due mainly to significant stenosis at the proximal and distal anastomoses. In 8 of 10 successfully reopened and revised endografts, reocclusion occurred after a median interval of only 1.8 months. Life-table analysis revealed cumulative primary and secondary patency rates of 42% and 56%, respectively, at 18 months. In the last 12 cases, the proximal end of the graft was sutured end-to-end to the transected SFA, which improved the short-term secondary patency rate to 83%. CONCLUSIONS: Insertion of the Enduring endovascular graft following remote endarterectomy effectively results in a less invasive treatment for femoropopliteal occlusive disease. Additional technical refinements of the procedure may be required to avoid early procedure- and graft-related failures.

Endovascular venous stenting in May-Thurner syndrome.

BACKGROUND: Chronic pulsatile compression of the left common iliac vein between the crossing right common iliac artery and the lowest lumbar vertebral body may induce focal intimal proliferation of the vein (May-Thurner syndrome), resulting in impaired venous return and left iliofemoral thrombosis. Corrective surgical treatment requires extensive dissection. In this report, we describe our experience with endovascular venous stenting in May-Thurner syndrome. METHODS: Six patients with symptomatic May-Thurner syndrome were treated with percutaneous transluminal angioplasty and implantation of self-expanding stents. RESULTS Postprocedure phlebography revealed patent iliofemoral veins with unimpeded venous outflow and disappearance of collaterals in all patients. No procedure-related complications occurred. At follow-up (median, 12 months), 5 of 6 patients were free of symptoms. In one patient lower extremity edema was aggravated despite a patent stented segment of the left iliac vein. The patient continues to wear support stockings to compensate for continuing venous insufficiency. Color coded duplex scanning revealed patency at regular intervals in 5 patients. In one patient, occlusion of the stented venous segment with return of symptoms was detected at one month. Patency could not be restored despite catheter-directed thrombolytic therapy. After angioplasty, however, adequate collateral circulation was restored and symptoms resolved completely. CONCLUSIONS: Endovascular venous stenting in May-Thurner syndrome is technically feasible, and leads to reduction of symptoms in the majority of patients with high patency rates in the medium-term. This approach may prove to be a percutaneous alternative to surgical treatment.

A narrow-waisted abdominal aortic aneurysm complicating endovascular repair.

PURPOSE: To present a case in which a narrow waist in an abdominal aortic aneurysm (AAA) complicated endovascular repair using a modular bifurcated stent-graft. METHODS AND RESULTS: A 68-year-old man underwent endovascular repair of a 5.9-cm asymptomatic AAA with a self-expanding modular bifurcated stent-graft. After insertion and deployment of the stent-graft, the intraoperative completion angiogram disclosed unexpected incomplete deployment of the contralateral iliac limb due to a narrow waist in the aortic aneurysm. Subsequent angioplasty did not increase iliac stent-graft diameter. At follow-up, a tapered course of the contralateral iliac leg persisted, without hemodynamic significance. CONCLUSIONS: A narrow waist in an AAA may be considered an additional important anatomical characteristic in assessing suitability for endovascular repair.

Bleeding risk of cerebrovascular malformations in hereditary hemorrhagic telangiectasia.

OBJECT: Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal-dominant vascular dysplasia with a high prevalence of cerebrovascular malformations (CVMs), mostly manifested as arteriovenous malformations (AVMs). The natural history and bleeding risk of these CVMs is unknown. The authors investigated the risk of bleeding in conjunction with clinical and radiological features in patients with HHT and proven CVMs. METHODS: Intravenous digital subtraction (DS) angiography was used to screen 196 patients with HHT for the presence of CVMs. Patients with abnormal results on DS angiography were asked to undergo a conventional cerebral angiographic study. All patients with a proven CVM were assessed by a neurologist. The bleeding risk was retrospectively and prospectively calculated for patients with AVMs only, as well as for the whole cohort of patients with CVMs. Twenty-four patients (12.2%; 16 female and eight male), aged 14 to 66 years (mean 35.4 years) with one or more CVMs were identified. Fifteen patients (62.5%) had a CVM and a pulmonary AVM. Eleven patients (45.8%) exhibited no neurological signs of their CVM; six (25%) had headache or migraine; four (16.7%) had seizures; and three (12.5%) had an intracranial hemorrhage. Twenty-two patients had at least one AVM (with a total of 28 AVMs), whereas two patients only had telangiectases. Twenty-seven AVMs were small (96%), 36% were located in eloquent areas of the brain, and 82% had superficial venous drainage. One third of the patients had multiple CVMs. The bleeding risk for patients with at least one AVM ranged from 0.41 to 0.72% per year, and for the whole cohort the range was 0.38 to 0.69% per year. Calculation of the bleeding risk as determined by lesion-years ranged from 0.36 to 0.56% per year for patients with AVMs and from 0.27 to 0.46% per year for all patients with CVMs. CONCLUSIONS: Patients with HHT have a high risk of harboring a CVM, especially in the presence of a pulmonary AVM. These CVMs are mostly low-grade AVMs (Spetzler-Martin Grade I or II), are frequently multiple, and have a lower risk of bleeding than that associated with sporadic AVMs. Female patients are more often affected than male patients. The inherent low sensitivity of DS angiography screening for CVMs may yield false negative results.

Endovascular femoropopliteal bypass combined with remote endarterectomy in SFA occlusive disease: initial experience.

OBJECTIVES: to evaluate the feasibility of endovascular femoropopliteal bypass in combination with remote endarterectomy of the superficial femoral artery (SFA). DESIGN: prospective, open study. MATERIALS: thirteen patients with chronic lower-leg ischaemia due to femoropopliteal occlusive disease underwent 14 SFA remote endarterectomy procedures followed by endovascular ePTFE femoropopliteal bypass. Primary endografting was performed in seven cases. The indication for endograft insertion was vessel-wall perforation during endarterectomy in the remaining seven cases. METHODS: pre- and postoperative clinicl and haemodynamic data were collected and compared. Technical problems and procedure-related complications were noted. RESULTS: initial technical success was achieved in all 14 limbs. However, four early reocclusions occurred after 1, 4, 6 and 10 weeks postoperatively. Two late reocclusions were detected after 16 and 22 months without any preceding symptoms or haemodynamic changes. Primary and secondary patency rates were 61% and 70% at two years, probably due to graft-related factors, such as lack of radial force, graft folding or kinking, and possibly altered mechanical or thrombogenetic properties after dilatation of the ePTFE graft. CONCLUSIONS: endovascular femoropopliteal endo-bypass after SFA remote endarterectomy is a feasible procedure. Further technical improvements are necessary to avoid procedure- and graft-related early failures.

Percutaneous transluminal angioplasty of the infrarenal aorta: initial outcome and long-term clinical and angiographic results.

OBJECTIVE: Report of 1-8 year follow-up of patients treated by percutaneous transluminal angioplasty (PTA) for obstructive atherosclerosis of the infrarenal aorta. DESIGN: Cohort study. MATERIALS: Thirty-eight patients aged 26-81 years (mean 50 years) were submitted to undergo percutaneous transluminal angioplasty of the infrarenal aorta. All patients had symptomatic isolated stenotic lesions of the aorta located below the renal arteries and above the bifurcation. Stenoses at bifurcation-level and the iliac arteries were excluded. METHODS: PTA of the aorta was performed under local anaethesia in the radiological department. Clinical symptoms and ankle/brachial indices were registered before, directly after the procedure and at follow-up. Angiography was performed in all patients pre- and post-procedure, and at follow-up. All patients received angiography in March 1995. Clinical data were analysed based on intention-to-treat. RESULTS: Initial clinical and angiographic success was achieved in 36 patients (94%). Mean follow-up was 34 months (range 1-92). Recurrent stenosis was seen in seven patients (19%) at follow-up. Only five (13%) of these had recurrent symptoms and were treated successfully with a second PTA. No major complications were seen. CONCLUSION: These results show PTA to be a safe, minimally-invasive and effective alternative to surgery in case of symptomatic stenosis of the infrarenal abdominal aorta.

Endovascular remote endarterectomy in femoropopliteal occlusive disease: one-year clinical experience with the ring strip cutter device.

OBJECTIVES: One-year clinical outcome of a new endovascular treatment for long segmental arterial occlusive disease using a ring strip cutter (RSC) to minimise surgical exposure. DESIGN: Prospective, open study. MATERIALS: Thirty-eight consecutive RSC procedures in 36 consecutive patients with lengthy occlusive (34) or multiple stenotic (4) femoropopliteal lesions were performed. Indications for operation were disabling claudication in 25 (66%), rest pain in 3 (8%), and gangrene in 10 (26%) patients. METHODS: A newly developed endovascular ring strip cutter device was used to perform a remote endarterectomy through a single groin incision. Clinical data were analysed based on intention-to-treat. RESULTS: Initial angiographic, clinical and haemodynamic success was achieved in all 38 (100%) limbs. Mean ankle-brachial index increased significantly from 0.62 +/- 0.14 to 1.02 +/- 0.14 postoperatively (p = 0.01). Four failures have occurred during follow-up. After one-year experience the cumulative (assisted) primary and secondary patency rates are 80% and 85% respectively. Duplex surveillance has detected progressive recurrent stenoses in 10 cases. CONCLUSIONS: Remote endarterectomy of long segmental femoropopliteal occlusive disease through a single groin incision with the Ring Strip Cutter device is a safe and effective procedure. The early patency rates are good. Further long-term results are needed to evaluate this technique.

Case report: bronchial to coronary artery anastomosis--a potential hazard in bronchial artery embolization.

Bronchial artery embolization is a well-established treatment for patients with haemoptysis. Communications between coronary and bronchial vessels have been seen on coronary angiography. This report describes a case of a bronchial to coronary artery anastomosis diagnosed prior to embolization in a patient with haemoptysis.

Unusual complications after embolization of a pulmonary arteriovenous malformation.

A pulmonary arteriovenous malformation was embolized in a patient with hereditary hemorrhagic telangiectasia. Several unusual complications, including early deflation of a detachable balloon, migration of a coil, and development of severe pulmonary hypertension, occurred. Pulmonary hypertension was attributed to a coexistent left-to-right shunt caused by a large hepatic arteriovenous malformation.

Iliac artery stenoses after percutaneous transluminal angioplasty: follow-up with duplex ultrasonography.

PURPOSE: To assess iliac artery stenosis before and up to 1 year after percutaneous transluminal angioplasty (PTA) with duplex ultrasound (DUS) to determine the incidence of residual and recurrent stenoses and correlate these findings to clinical outcome. PATIENTS AND METHODS: Sixty-one patients with 70 iliac artery segments treated with PTA were examined. The peak systolic velocity (PSV) ratio (PSV ratio = PSV in stenosis divided by PSV proximal or distal to stenosis) was determined by DUS before PTA and 1 day, 3 months and 1 year after PTA. Three categories of results were identified by using PSV ratios at the site of the treated stenosis 1 day and 1 year after PTA (good result, residual stenosis, and recurrent stenosis). The DUS-determined anatomic result was correlated with the clinical outcome at 1 year. Clinical outcome was classified according to Society for Vascular Surgery/International Society for Cardiovascular Surgery (SVS/ISCVS) criteria. RESULTS: Good results with DUS (PSV ratio 1 day and 1 year after PTA > or = to 2.5) were found in 45 of 70 segments (64.3%), residual stenoses (PSV ratio > or .5 1 day after PTA) in 15 of 70 segments (21.4%), and recurrent stenosis (PSV ratio 1 day after PTA < 2.5 and 1 year after PTA > or = 2.5) in 10 of 70 segments (14.3%). PSV ratios of residual stenoses decreased significantly between 1 day and 1 year after PTA because some residual stenoses improved hemodynamically in time. Clinical results were significantly better in patients with a good result compared with other patients. However, the clinical outcome of patients with residual stenoses was not significantly different from the patients with good DUS results. CONCLUSION: Some residual stenoses improved sonographically after PTA. Clinical results at 1 year are highly variable within different groups. Clinical outcome of patients with residual stenoses did not differ from patients with good DUS results, whereas clinical outcome in patients with recurrent stenoses was worse than in the other groups.

Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease): new insights in pathogenesis, complications, and treatment.

Hereditary hemorrhagic telangiectasia (HHT), also known as Osler-Weber-Rendu disease, is a hereditary disorder leading to easily bleeding telangiectases on skin and mucosal surfaces, and it is associated with the presence of arteriovenous malformations (AVMs) in multiple organ systems. These AVMs may cause serious complications when they are located in the lungs, liver, or brain. The prevalence of AVMs in patients with HHT might be higher than previously estimated. Nowadays, treatment is often possible. In some families, mutations have been shown in the gene encoding for a transforming growth factor receptor, endoglin. Genetic heterogeneity has been demonstrated, suggesting involvement of other transforming growth factor receptors. This might explain the variable clinical expression of the disease. In view of the high prevalence of pulmonary and cerebral AVMs, all patients with HHT should be screened for their presence, and relatives of patients with HHT should be investigated for presence of the disease.

Screening family members of patients with hereditary hemorrhagic telangiectasia.

PURPOSE: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant inherited disorder which may give rise to arteriovenous malformations in lungs and brain. When left untreated, these may cause serious complications. We screened family members of HHT patients for presence of the disease and associated pulmonary or cerebral arteriovenous malformations. PATIENTS AND METHODS: We investigated 98 family members of HHT patients on an outpatient basis. A stepped screening protocol was used based on prevalence of different manifestations of HHT. RESULTS: Thirty-six cases of HHT were found in the 98 persons screened. Pulmonary arteriovenous malformations were found in 12 of the 36 patients (33%), and cerebral arteriovenous malformations in 4 (11%). Therapy was recommended in 9 patients with pulmonary arteriovenous malformations and in 2 with cerebral arteriovenous malformations. CONCLUSIONS: Family members of known HHT patients should be encouraged to engage in a screening program, since the prevalence of potentially serious localizations is higher than previously thought.

Aortobronchial fistula after correction of congenital cardiovascular abnormalities.

Aortobronchial fistula (ABF) is a rare but highly lethal condition. Four patients with ABF, 10-25 years after surgical repair of a congenital cardiovascular abnormality are reported. All patients presented with haemoptysis. Computed tomographic (CT) scan and aortography were inconclusive in two, diagnostic in one and not performed in another. Three patients underwent operation: all survived and are free of symptoms with a follow-up of 2-8 yrs. The fourth patient died before operation due to massive haemorrhage into the lung. In all patients, the fistula was secondary to aortic interposition of patch grafts.