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OBJECTIVE: To present a case series of pulmonary arteriovenous malformations (PAVMs), describing the main clinical findings, the number/location of pulmonary vascular abnormalities, the clinical complications, and the treatment administered. METHODS: This was a retrospective observational study evaluating patients with PAVM divided into two groups: hereditary hemorrhagic telangiectasia (HHT); and idiopathic PAVM (iPAVM). RESULTS: A total of 41 patients were selected for inclusion, but only 33 had PAVMs. After clinical evaluation, 27 and 6 were diagnosed with HHT and iPAVM, respectively. In the HHT group, the mean age was 49.6 years and 88.9% were female. In that group, 4 patients had an SpO2 of < 90% and the most common clinical finding was epistaxis. In the iPAVM group, the mean age was 48.1 years and 83.3% were female. In that group, 3 patients had an SpO2 of < 90%. Computed tomographic pulmonary angiography showed that most of the PAVMs were in the lower lobes: 56.4% in the HHT group and 85.7% in the iPAVM group. Embolization was performed in 23 patients (in both groups). At this writing, 10 patients are scheduled to undergo the procedure. One of the patients who underwent embolization was subsequently referred for pulmonary resection. CONCLUSIONS: In both of the PAVM groups, there was a predominance of women and of fistulas located in the lower lobes. Few of the patients had respiratory symptoms, and most had an SpO2 > 90%. The treatment chosen for all patients was percutaneous transcatheter embolization.

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ABSTRACT Objective: To present a case series of pulmonary arteriovenous malformations (PAVMs), describing the main clinical findings, the number/location of pulmonary vascular abnormalities, the clinical complications, and the treatment administered. Methods: This was a retrospective observational study evaluating patients with PAVM divided into two groups: hereditary hemorrhagic telangiectasia (HHT); and idiopathic PAVM (iPAVM). Results: A total of 41 patients were selected for inclusion, but only 33 had PAVMs. After clinical evaluation, 27 and 6 were diagnosed with HHT and iPAVM, respectively. In the HHT group, the mean age was 49.6 years and 88.9% were female. In that group, 4 patients had an SpO2 of < 90% and the most common clinical finding was epistaxis. In the iPAVM group, the mean age was 48.1 years and 83.3% were female. In that group, 3 patients had an SpO2 of < 90%. Computed tomographic pulmonary angiography showed that most of the PAVMs were in the lower lobes: 56.4% in the HHT group and 85.7% in the iPAVM group. Embolization was performed in 23 patients (in both groups). At this writing, 10 patients are scheduled to undergo the procedure. One of the patients who underwent embolization was subsequently referred for pulmonary resection. Conclusions: In both of the PAVM groups, there was a predominance of women and of fistulas located in the lower lobes. Few of the patients had respiratory symptoms, and most had an SpO2 > 90%. The treatment chosen for all patients was percutaneous transcatheter embolization.

RESUMO Objetivo: Apresentar uma série de casos de malformações arteriovenosas pulmonares (MAVP) e descrever os principais achados clínicos, a quantidade e localização das MAVP, as complicações clínicas e os tratamentos realizados. Métodos: Estudo retrospectivo observacional que avaliou pacientes com MAVP divididos em dois grupos: telangiectasia hemorrágica hereditária (THH) e MAVP idiopática (MAVPi). Resultados: Foram avaliados 41 pacientes, sendo 33 pacientes portadores de MAVP. Após a avaliação clínica, 27 e 6 foram diagnosticados com THH e MAVPi, respectivamente. No grupo THH a média de idade foi de 49,6 anos e 88,9% eram do sexo feminino. Desses pacientes, 4 tinham SpO2 < 90% e o achado clínico mais frequente era epistaxe. No grupo MAVPi a média de idade foi de 48,1 anos, sendo que 83,3% eram do sexo feminino. Desses, 3 tinham SpO2 < 90%. Após a realização de angiotomografia de tórax observou-se que a maior parte das MAVP se situava nos lobos inferiores, totalizando 56,4% e 85,7% nos grupos THH e MAVPi, respectivamente. O tratamento por embolização foi realizado em 23 pacientes nos dois grupos, enquanto 10 aguardavam o procedimento até o momento da escrita deste estudo. Um paciente submetido à embolização foi encaminhado para ressecção pulmonar. Conclusões: Em ambos os grupos de pacientes com MAVP observou-se uma predominância de mulheres e de fístulas localizadas nos lobos inferiores. A maioria era assintomático respiratório com SpO2 > 90%. O tratamento de escolha para todos foi a embolização percutânea por cateter.

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Hereditary hemorrhagic telangiectasia (HHT) usually presents in association with pulmonary arteriovenous malformations (PAVMs). In addition, the incidence of venous thromboembolism tends to be increased in these patients. A 74-year-old female with HHT presented with cyanosis and hypoxemia. Contrast-enhanced multislice computed tomography (MSCT) revealed two left PAVMs and one in the right upper lobe. Both left PAVMs were treated with embolotherapy. Follow-up MSCT revealed an incidental pulmonary embolism in the right pulmonary branches. Deep venous thrombosis was confirmed and anticoagulation was initiated. Follow-up MSCT revealed the resolution of thromboembolism. Finally, embolotherapy was performed. This case illustrates the chronic adaptation to hypoxemia and adds further evidence to the relative safety of anticoagulation treatment in these patients.

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It is unclear whether pulmonary arteriovenous malformations (PAVMs) in hereditary hemorrhagic telangiectasia develop later in life or are preformed in childhood. We show that the prevalence of PAVMs in children is similar to that in their parents, providing evidence against de novo PAVM formation in hereditary hemorrhagic telangiectasia.

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OBJECTIVE: To evaluate the clinical features in a large cohort of pediatric patients with genetically confirmed hereditary hemorrhagic telangiectasia (HHT) and to identify possible predictors of arteriovenous malformation (AVM) onset or clinical significance. STUDY DESIGN: Prospective cross-sectional survey of all children subjected to screening for AVMs in the multidisciplinary HHT center. All patients proved to be carriers of endoglin mutations or activin A receptor type-II-like kinase 1 mutations, defined as HHT1 and HHT2, respectively. A full clinical-radiological protocol for AVM detection was adopted, independent from presence or absence of AVM-related symptoms. RESULTS: Forty-four children (mean age, 10.3 years; range, 1-18) were subjected to a comprehensive clinical-radiologic evaluation. This investigation disclosed cerebrovascular malformations in 7 of 44 cases, pulmonary AVMs in 20 of 44 cases, and liver AVMs in 23 of 44 cases. Large visceral AVMs were found in 12 of 44 children and were significantly more frequent in patients with HHT1. Only large AVMs were associated with symptoms and complications. CONCLUSIONS: Children with HHT have a high prevalence of AVMs; therefore, an appropriate clinical and radiological screening protocol is advisable. Large AVMs can be associated with complications in childhood, whereas small AVMs probably have no clinical risk.

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Las malformaciones arteriovenosas pulmonares (MAVP), son verdaderas fístulas vasculares de alto flujo y baja presión carentes de filtro capilar pulmonar. Consisten en la unión de una arteria a una vena mediante unsaco aneurismático. Se trata de una patología muy poco frecuente y con una alta asociación con la enfermedad telangiectasica hereditaria. A continuación se realiza un análisis de tres casos con diferentes formas clínicas de presentación.Se destaca la gran importancia de la angio tomografía como métodode diagnóstico así como la alta eficacia de la arteriografía con embolizacion para su resolución.

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Las malformaciones arteriovenosas pulmonares (MAVP), son verdaderas fístulas vasculares de alto flujo y baja presión carentes de filtro capilar pulmonar. Consisten en la unión de una arteria a una vena mediante unsaco aneurismático. Se trata de una patología muy poco frecuente y con una alta asociación con la enfermedad telangiectasica hereditaria. A continuación se realiza un análisis de tres casos con diferentes formas clínicas de presentación.Se destaca la gran importancia de la angio tomografía como métodode diagnóstico así como la alta eficacia de la arteriografía con embolizacion para su resolución.

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El síndrome de Rendu-Osler-Weber es un trastorno hereditario de los vasos sanguíneos, con gran variedad de manifestaciones clínicas que predisponen a fenómenos hemorrágicos (epistaxis, hemorragia digestiva) asociados a telangiectasias en piel y mucosas, acompañados generalmente de anemia ferropriva secundaria. Dichas manifestaciones generalmente subestiman la presencia de otras malformaciones arteriovenosas en órganos como pulmón y cerebro, cuyas complicaciones pueden determinar un riesgo vital para el paciente. La sospecha dada por alguno de los eventos descritos, sumado a la presencia de antecedentes familiares con la misma patología, obligan a realizar la búsqueda activa de malformaciones arteriovenosas en órganos potencialmente afectados para evaluar la posibilidad de un eventual tratamiento, el cual ante cualquier tipo de manifestación, y según avala la literatura, sigue siendo sintomático.

Osler-Weber-Rendu syndrome is an inherited disorder of the vessels, with a great variety of clinical manifestations that predispose to hemorrhage (epistaxis, gastrointestinal bleeding) associated to telangiectasis in skin and mucoses, usually with secondary ferropenic anemia. Those manifestations generally underestimate the presence of other arteriovenous malformations in organs like lungs and brain, where complications are of vital risk for the patient. The suspect given by any of the events described previously, added to the presence of family medical history for the same disorder, make necessary an active search of arteriovenous malformations in potentially affected organs to evaluate the possibility of an eventual treatment, which is for any type of manifestation and according to the literature, still symptomatic.

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Multiple arteriovenous fistulas (AVF) is an infrequent pathology, in which lung transplant is controversial. We report a patient with hereditary hemorrhagic telangiectasia who underwent a single lung transplant with excellent evolution, and three years later (that is, at the time of this paper), she is still alive.

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