Complications and clinical outcome of hepatic artery embolisation in patients with hereditary haemorrhagic telangiectasia.

BACKGROUND: Hepatic 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. METHODS: Staged 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). RESULTS: Two 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. CONCLUSION: The 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.

Systemic therapy with bevacizumab in patients with hereditary hemorrhagic telangiectasia (HHT).

BACKGROUND: Frequent epistaxis, the hallmark of hereditary hemorrhagic telangiectasia (HHT) significantly affects quality of life. Hepatic involvement may be associated with capsular pain, abdominal angina, high-output cardiac failure and portal hypertension with ascites and variceal bleeding. Liver transplantation as well as hepatic artery embolization as invasive treatment options for hepatic involvement are both associated with a certain morbidity and mortality. PATIENTS AND METHODS: Three patients with HHT and symptomatic hepatic involvement prospectively underwent off-label systemic treatment with the vascular endothelial growth factor (VEGF)-inhibitor bevacizumab in 6 cycles. Clinical symptoms and cardiac output were assessed before as well as 3 months after therapy. RESULTS: At 3 months follow-up, capsular pain and abdominal angina were significantly reduced in two of the three patients (Numerical Rating Scale (NRS) grade 4 and 5 changed to grade 2). Cardiac output, which was initially raised in both patients, normalized and was associated with an improvement in the signs and symptoms of cardiac insufficiency by one stage according to the NYHA classification in both patients. Symptoms however returned back gradually to pre-therapeutic levels after 9 months in one of these two patients. In the third patient with a normal cardiac status prior to therapy, a marked subjective improvement in the performance status, fatigability and quality of life was noted. Grade 2 nasal bleeding reduced to grade 0 - 1 in all three patients; this clinical improvement was persistent at 3, 7 and 9 months follow-up. No significant lasting complications were observed. CONCLUSIONS: 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.

Percutaneous interventions for treating ischemic complications of aortic dissection.

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.