Sytemic bevacizumab therapy could become an important therapy option in the non-invasive medical treatment of patients with HHT. Further studies to document long-term results, to determine the appropriate drug dosage as well as to evaluate the necessity of a maintenance drug regime are warranted.

BackgroundHepatic artery embolisation (HAE) in patients with hereditary haemorrhagic telangiectasia (HHT) is controversial because of the associated complications and unproven long-term benefit. We present our results in 20 such patients over a time span of 17 years.MethodsStaged HAE was performed using polyvinyl alcohol (PVA) particles and coils. Complications, clinical symptoms and cardiac output were assessed before and after therapy as well as at the end of follow-up (median 92 months, range 26–208 months).ResultsTwo patients died within 30 days following HAE (10 %). Four further deaths resulted from causes unrelated to HAE. Ischaemic cholangitis, cholecystitis and focal hepatic necrosis with biliary sepsis necessitated re-intervention in four patients. In all but one patient, clinical symptoms resolved with mean cardiac output falling from 11.84 ± 3.22 l/min pre-treatment to 8.13 ± 2.67 l/min at the end of follow-up (P < 0.001). One patient required liver transplantation for de novo symptoms of portal hypertension 4 years after primary symptoms had been cured by HAE.ConclusionThe 30-day mortality of HAE in patients with HHT is 10 %. The rate of complications requiring re-intervention is 20 %. Clinical response at long-term follow-up is satisfactory.Key Points• Hepatic artery embolisation (HAE) in hereditary haemorrhagic telangiectasia (HHT) provides long-term benefit.• Mortalities of HAE and liver transplantation in HHT patients are comparable.• In HHT, complications of HAE are lower than those of liver transplantation.• Complications of HAE can be further reduced by refinement of technique.• Complications include ischaemic cholangitis, hepatic necrosis, biliary sepsis and death.

The aim of this study was to evaluate the role of percutaneous interventions in treating ischemia complicating aortic dissection. Forty-five patients with ischemia complicating aortic dissection were treated by balloon fenestration, true lumen stenting, angioplasty, or thrombolysis. Clinical and laboratory examinations were performed before and after intervention, and at the end of follow-up (median 37 months). Eighteen dissections were acute, 9 sub-acute, and 18 chronic. Mesenterohepatic ischemia resolved in 16 of 18 patients; lactate and SGOT values fell from 2.89 to 1.23 mmol/L (p=0.006) and from 165.9 to 59.7 U/L (p=0.034), respectively. In patients with renal ischemia, creatinine levels fell from 360.1 to 196.3 micromol/L (p=0.007) accompanied by a significant reduction in blood pressure. Limb-threatening ischemia resolved in three of four patients; in 21 claudicants, the mean walking distance improved from 272 to 1,283 m (p=0.001). Spinal ischemia resolved completely or partially in six of eight patients. Adjunctive surgical measures were necessary in six patients. Overall 30-day mortality in the 45 patients was 6.7%; all three deaths were in patients with acute dissections (mortality in this subgroup 16.7%). Ischemia complicating aortic dissection can be effectively treated by percutaneous interventions resulting in good early and mid-term outcomes.

Endoluminal therapy is indicated in lower extremity ischaemia with Fontaine grades IIb, III and IV. In the presence of significant limitations, interventions are carried out even in grade IIa claudicants. In addition to the TASC A and B lesions, TASC C and D lesions are increasingly being treated endoluminally as well. Presently, technical success rates of revascularization procedures are above 90% in the iliac vessels and between 79% and 95% in the femoro-popliteal segments. Concentric, non-calcified iliac stenoses are primarily treated with balloon angioplasty (PTA) followed by optional stenting when necessary. For occlusions and heavily calcified lesions, primary stenting is recommended. Primary PTA is the mainstay of treatment in femoro-popliteal vessels with stents being used as a "bail-out" option in case of suboptimal PTA. However, initial reports proving the superiority of primary stenting over PTA with optional stenting have already appeared. Results of PTA with drug-coated balloons for prevention of early restenosis are promising. In the near future, primary PTA with optional stenting in the femoro-popliteal segments may give way to drug-coated balloon angioplasty or primary stenting.

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