Pulmonary arteriovenous malformations in Rendu-Osler-Weber syndrome.

Rendu-Osler-Weber syndrome, also known as hereditary hemorrhagic telangiectasia, is an autosomal dominant hereditary disorder. It is characterized by presence of multiple arteriovenous malformations (AVMs) and telangiectasias. This article reports two cases of patients with Rendu-Osler-Weber syndrome who had pulmonary AVMs and underwent successful endovascular treatment. A brief review of the literature shows that up to 50% of patients with the syndrome have pulmonary AVMs and there is usually a positive family history in these patients. These pulmonary AVMs are multiple in 30% of cases and are associated with the most severe disease complications. Most patients are asymptomatic, even in the presence of AVMs with right-left shunts. When these shunts exceed 25% of the total blood volume, dyspnea, cyanosis, digital clubbing, and extracardiac murmurs may occur. Endovascular treatment is safe and offers control of complications from hereditary hemorrhagic telangiectasia and is currently the treatment of choice for these lesions.

Right iliac arteriovenous fistula due to aneurysm rupture and left iliac artery aneurysm: hybrid treatment.

An arteriovenous fistula (AVF) is an uncommon sequela of spontaneous arterial aneurysm rupture into the adjacent venous system. We describe the case of a 74-year-old patient who underwent endovascular treatment of a right iliac AVF caused by a ruptured common iliac artery (CIA) aneurysm and a distal left CIA aneurysm. Surgery preserved the lumbar and inferior mesenteric arteries because of the need to simultaneously exclude the hypogastric arteries. Dynamic fluid balance phenomena provoked by closure of the AVF are described. The patient had a benign postoperative course with normalization of the severe hemodynamic changes presented prior to the intervention and resolution of respiratory symptoms attributed to pulmonary arterial hypertension.

Radiofrequency Ablation for Axial Reflux Associated with Foam Sclerotherapy for Varicosities in One-Step Approach: A Prospective Cohort Study Comprising Large Diameters Saphenous Veins.

OBJECTIVE: This study assessed the outcomes and impact on the quality of life following one-step outpatient radiofrequency ablation (RFA) and ultrasound guided foam sclerotherapy (USGFS) for large reflux with varicosities in the great saphenous vein (GSV). DESIGN: Prospective, single-centre, analytical cohort. MATERIALS AND METHODS: Thirty symptomatic patients having reflux in the GSV and varicosities (CEAP C3 to C6) were treated with RFA and USGFS simultaneously, in a single-step procedure, from March 2016 to December 2016. They were followed up at 1 week, 6 months, 1 and 3 years. Clinical outcomes, changes in the Quality of Life (QOL) questionnaires SF-36™, VCSS and AVVQ, evolutive vein occlusion rates were assessed by duplex ultrasound, and ulcer closure was checked. RESULTS: The sample was divided into two groups: (Group 1) GSV diameter ≥13.0 mm (median 19.0 [14-24]), 17 subjects, and (Group 2) GSV diameter ≤12.9 mm (median 10.3 [10-12]), 16 subjects. No major adverse event was observed, and the postoperative minor adverse event rates were similar between the two groups. A significant improvement was observed in VCSS and AVVQ from the preoperative levels to the sixth month and the third-year follow-up. Twelve of 13 ulcers had healed at 1 year and remained closed until 3 years. The entire sample had a significant increase in all short form 36 domains, except for mental health in the Group 2 (GSV ≥ 13.0 mm). Overall first week occlusion rate for the whole sample was 90.9% and 69.7% at the 3-year follow-up. No difference in occlusion rate was observed between the two groups at any time. CONCLUSION: Exclusively outpatient combined techniques were safe and feasible in this study with no major adverse events, despite the large diameters of the GSV or ulcer presence. Within 3 years, both diameter groups showed equivalent improvement in all QOL parameters, satisfactory axial occlusion, and maintained ulcer closure.

Malformações arteriovenosas pulmonares na síndrome de Rendu-Osler-Weber / Pulmonary arteriovenous malformations in Rendu-Osler-Weber syndrome

Resumo A síndrome de Rendu-Osler-Weber, também conhecida como telangiectasia hemorrágica hereditária, é uma doença hereditária autossômica dominante. Ela é caracterizada pela presença de múltiplas malformações arteriovenosas e telangiectasias. Este artigo relata dois casos de pacientes com síndrome de Rendu-Osler-Weber que apresentaram malformações arteriovenosas pulmonares e foram submetidos a tratamento endovascular com sucesso. Uma breve revisão da literatura mostra que até 50% dos pacientes com a síndrome têm malformações arteriovenosas pulmonares e geralmente há um histórico familiar positivo nesses pacientes. Em 30% dos casos, elas são múltiplas e estão associadas a complicações mais graves da doença. A maioria dos pacientes é assintomática, mesmo na presença de malformações arteriovenosas com shunt direito-esquerdo. Quando esses shunts excedem 25% do volume total de sangue, podem surgir dispneia, cianose, baqueteamento digital e sopros extracardíacos. O tratamento endovascular oferece segurança e controle das complicações da telangiectasia hemorrágica hereditária, sendo atualmente o tratamento de escolha para essas lesões.

Abstract Rendu-Osler-Weber syndrome, also known as hereditary hemorrhagic telangiectasia, is an autosomal dominant hereditary disorder. It is characterized by presence of multiple arteriovenous malformations (AVMs) and telangiectasias. This article reports two cases of patients with Rendu-Osler-Weber syndrome who had pulmonary AVMs and underwent successful endovascular treatment. A brief review of the literature shows that up to 50% of patients with the syndrome have pulmonary AVMs and there is usually a positive family history in these patients. These pulmonary AVMs are multiple in 30% of cases and are associated with the most severe disease complications. Most patients are asymptomatic, even in the presence of AVMs with right-left shunts. When these shunts exceed 25% of the total blood volume, dyspnea, cyanosis, digital clubbing, and extracardiac murmurs may occur. Endovascular treatment is safe and offers control of complications from hereditary hemorrhagic telangiectasia and is currently the treatment of choice for these lesions.

Fístula arteriovenosa ilíaca à direita por rotura de aneurisma e aneurisma de artéria ilíaca esquerda: tratamento híbrido / Right iliac arteriovenous fistula due to aneurysm rupture and left iliac artery aneurysm: hybrid treatment

Resumo A fístula arteriovenosa (FAV) é uma sequela incomum de rotura espontânea de aneurisma arterial dentro do sistema venoso adjacente. Descrevemos, em um paciente de 74 anos, o tratamento endovascular de FAV ilíaca à direita por aneurisma de artéria ilíaca comum (AIC) roto associado a aneurisma de AIC distal à esquerda, em cuja cirurgia foram preservadas as artérias lombares e mesentérica inferior por necessidade de excluir simultaneamente as artérias hipogástricas. Foram demonstrados os fenômenos na dinâmica do balanço hídrico ocorridos em decorrência da interrupção da FAV. A evolução do paciente foi benigna, com normalização das graves alterações hemodinâmicas que apresentava e com desaparecimento dos sintomas respiratórios atribuídos à hipertensão arterial pulmonar.

Abstract An arteriovenous fistula (AVF) is an uncommon sequela of spontaneous arterial aneurysm rupture into the adjacent venous system. We describe the case of a 74-year-old patient who underwent endovascular treatment of a right iliac AVF caused by a ruptured common iliac artery (CIA) aneurysm and a distal left CIA aneurysm. Surgery preserved the lumbar and inferior mesenteric arteries because of the need to simultaneously exclude the hypogastric arteries. Dynamic fluid balance phenomena provoked by closure of the AVF are described. The patient had a benign postoperative course with normalization of the severe hemodynamic changes presented prior to the intervention and resolution of respiratory symptoms attributed to pulmonary arterial hypertension.

Embolização da reperfusão do aneurisma da artéria poplítea com Onyx / Treatment of recurrent popliteal artery aneurysm with Onyx embolization

O aneurisma da artéria poplítea é o mais frequente dos aneurismas periféricos verdadeiros. Embora seja assintomático, apresenta complicações isquêmicas por embolia ou trombose. A correção deste aneurisma pode ser cirúrgica convencional, com técnica e prognóstico bem estabelecidos, ou endovascular, técnica inovadora e menos invasiva. A reperfusão do saco aneurismático excluído, apesar de rara, está relacionada à intensa circulação colateral. Relatamos um caso de aneurisma de poplítea, que após dois anos de exclusão apresentou fluxo no saco aneurismático com sintomatologia, sendo tratado por técnica endovacular alternativa.

The popliteal artery aneurysm is the most frequent of the peripheral aneurysms. Although asymptomatic, it can complicate with severe limb ischemia due to embolization or thrombosis. The surgical correction presents well-established techniques and results, while the less invasive endovascular therapy is still evolving. The recurrence is very rare and can be related to collateral artery refilling. We report a recurrent popliteal artery aneurysm after two years of conventional ligation and bypass surgery, in which an alternative endovascular strategy was successfully applied.