scholarly journals Approach to Pulmonary Arteriovenous Malformations: A Comprehensive Update

2020 ◽  
Vol 9 (6) ◽  
pp. 1927 ◽  
Author(s):  
Shamaita Majumdar ◽  
Justin P. McWilliams

Pulmonary arteriovenous malformations (PAVMs) are abnormal direct vascular communications between pulmonary arteries and veins which create high-flow right-to-left shunts. They are most frequently congenital, usually in the setting of hereditary hemorrhagic telangiectasia (HHT). PAVMs may be asymptomatic or present with a wide variety of clinical manifestations such as dyspnea, hypoxemia, or chest pain. Even when asymptomatic, presence of PAVMs increases patients’ risk of serious, potentially preventable complications including stroke or brain abscess. Transcatheter embolotherapy is considered the gold standard for treatment of PAVMs. Though previous guidelines have been published regarding the management of PAVMs, several aspects of PAVM screening and management remain debated among the experts, suggesting the need for thorough reexamination of the current literature. The authors of this review present an updated approach to the diagnostic workup and management of PAVMs, with an emphasis on areas of controversy, based on the latest literature and our institutional experience.

2019 ◽  
Vol 13 (4) ◽  
pp. 244-246
Author(s):  
Lucio Brugioni ◽  
Chiara Catena ◽  
Eugenio Ferraro ◽  
Serena Scarabottini ◽  
Francesca Mori ◽  
...  

Pulmonary arteriovenous malformations (PAVMs) are abnormal communications between pulmonary arteries and veins. The clinical features suggestive of PAVMs are stigmata of right-to-left shunting (dyspnea, hypoxemia, cyanosis, cerebral embolism, brain abscess), unexplained hemoptysis, or hemothorax.We present a case of young man presented to Emergency Department complaining dyspnea, polycythemia and persistent hypoxemia. Angio-CT scan of the chest detected multiple PAVMs.PAVMs are uncommon in the general population, but they are an important consideration in the differential diagnosis of common pulmonary problems, including hypoxemia, pulmonary nodules, and hemoptysis


2020 ◽  
Vol 15 (1) ◽  
Author(s):  
Peng Teng ◽  
Weidong Li ◽  
Yiming Ni

Abstract Background Pulmonary arteriovenous malformations are uncommon conditions of abnormal communications between pulmonary arteries and veins, which are most commonly congenital in nature. Although such condition is not extremely rare, it is a challenge to the differential diagnosis of pulmonary problems such as hypoxemia and pulmonary lesions. Case presentation We report a meaningful case of a 23-year-old male presented with elevated hemoglobin (23.0 g/dl) on admission. Physical examination revealed cyanosis, digital clubbing and low oxygen saturation on room air. The patient was initially diagnosed as polycythemia vera while the subsequent result of bone marrow aspiration was negative. During further assessment, pulmonary arteriovenous malformations were detected by CT pulmonary angiography. Lobectomy was successfully performed with significant increase in oxygen saturation from 86 to 98%. The hemoglobin decreased to almost normal level of 14.9 g/dl 3 months after surgery and the patient had been followed up for nearly 5 years. Conclusions Pulmonary arteriovenous malformations should be suspected in patients with central cyanosis, digital clubbing, polycythemia, pulmonary lesion and without cardiac malformations. Embolization or surgery is strongly recommended to reduce the risks caused by pulmonary arteriovenous malformations.


2021 ◽  
Vol 8 ◽  
pp. 2333794X2110511
Author(s):  
Daoud Ali Mohamed ◽  
Amarkak Waiss ◽  
Behyamet Onka ◽  
Walid Mohamed ◽  
Nazik Allali ◽  
...  

Pulmonary Arteriovenous Malformations (PAVMs) are structurally abnormal vascular communications between pulmonary arteries and pulmonary veins, which bypass the normal capillary bed and cause a low resistance right-to-left shunt with refractory hypoxemia. Generally, PAVMs were congenital, most commonly associated with (Hereditary hemorrhagic teleangiectasia (HHT). The age of diagnosis is very variable, range neonatal to adulthood, mostly diagnosed in the first 3 decades of life and clinical manifestations occur later in life generally. Here, we report PAVMs discovered incidentally in a 10-years-old child without any known risk factor.


2020 ◽  
Author(s):  
Peng Teng ◽  
Weidong Li ◽  
Yiming Ni

Abstract Background: Pulmonary arteriovenous malformations are uncommon conditions of abnormal communications between pulmonary arteries and veins, which are most commonly congenital in nature. Although such condition is not extremely rare, it is a challenge to the differential diagnosis of pulmonary problems such as hypoxemia and pulmonary lesions.Case presentation: We report a meaningful case of a 23-year-old male presented with elevated hemoglobin (23.0 g/dl) on admission. Physical examination revealed cyanosis, digital clubbing and low oxygen saturation on room air. The patient was initially diagnosed as polycythemia vera while the subsequent result of bone marrow aspiration was negative. During further assessment, pulmonary arteriovenous malformations were detected by CT pulmonary angiography. Lobectomy was successfully performed with significant increase in oxygen saturation from 86% to 98%. The hemoglobin decreased to almost normal level of 14.9 g/dl three months after surgery and the patient had been followed up for nearly 5 years.Conclusions: Pulmonary arteriovenous malformations should be suspected in patients with central cyanosis, digital clubbing, polycythemia, pulmonary lesion and without cardiac malformations. Embolization or surgery is strongly recommended to reduce the risks caused by pulmonary arteriovenous malformations.


2018 ◽  
pp. 315-324
Author(s):  
Jeffrey S. Pollak

Pulmonary arteriovenous malformations (PAVMs) are dilated directly, connecting pulmonary arteries and veins with no capillary bed. They are generally congenital and frequently occur in patients with autosomal-dominant hereditary hemorrhagic telangiectasia (HHT), so they should be screened for PAVM. HHT causes variable-sized arteriovenous malformations (AVMs) from telangiectases to larger connections and affects mucocutaneous surfaces. Epistaxis is the most common symptom, and these vascular lesions can occur in other organs, including the lungs, central nervous system, liver, and gastrointestinal (GI) tract. PAVMs can result in hypoxemia with dyspnea, paradoxical embolization with ischemic stroke and abscess formation, and hemorrhage. Treatment consists of embolization of sufficiently sized lesions; antibiotic prophylaxis before procedures prone to produce bacteremia, such as dental processes that can cause abscesses; and avoidance of air or clots in intravenous (IV) lines. Lifelong follow-up is essential to assess for adequacy of embolization and enlargement of PAVMs that are too small to embolize.


2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Federica Di Guardo ◽  
Viviana Lo Presti ◽  
Giuliana Costanzo ◽  
Elisa Zambrotta ◽  
Luisa Maria Di Gregorio ◽  
...  

Pulmonary arteriovenous malformations (PAVMs) are anatomical abnormalities consisting in a direct connection between pulmonary arteries and veins. Most of PAVMs are related to Hereditary Hemorrhagic Teleangiectasia, whereas only 10 to 20% are isolated sporadic cases. PAVMs tend to increase in size naturally; however, several factors can influence their growth such as pulmonary arterial hypertension, puberty, and pregnancy. Clinical manifestations are related to the right-to-left shunting and include dyspnoea, hypoxia, and pulmonary hypertension. The presence of PAVMs during pregnancy is associated with an increased risk of complications such as their rupture, haemothorax, and hypovolemic shock. The treatment reserved to PAVMs was the surgical resection of the lung lobe involving the malformation. Due to the worldwide acceptance of endovascular technique, the transcatheter embolization (TCE) is today considered as the mainstay of treatment. Recent studies reported the safeness of the TCE during pregnancy if performed by an experienced radiologist, at second or third trimesters when radiation exposure is believed to have minimal effect on the foetus. However, although the TCE during pregnancy represents an option, the treatment prior to pregnancy has to be considered the auspicial solution. Our study reports the case of a dyspnoeic pregnant woman with unknown pAVM causing hemothorax and simultaneously treated for pAVM reparation, left lower lobe resection, and hysterectomy. Postoperative treatment of embolization was performed to definitively close the pAVM.


Author(s):  
Edward C. Rosenow

• Also known as Osler-Weber-Rendu disease • Bruits heard in about half of cases, but they are small or not subpleural • Bruits become louder with inspiration • 80% to 90% of patients with pulmonary arteriovenous malformations (AVMs) have HHT; only 30% of patients with HHT have pulmonary AVM...


Open Medicine ◽  
2015 ◽  
Vol 10 (1) ◽  
Author(s):  
Rossella Masiello ◽  
Carlo Iadevaia ◽  
Edoardo Grella ◽  
Carmelindo Tranfa ◽  
Francesco Cerqua ◽  
...  

Abstract Pulmonary arteriovenous Malformations (PAVMs) are a rare vascular alteration characterized by abnormal communications between the pulmonary arteries and veins resulting in an extracardiac right-to-left (R-L) shunt. The majority of PAVMs are associated with an autosomal dominant vascular disorder also known as Osler-Weber- Rendu Syndrome. PAVMs appearance can be both single and multiple. Clinical manifestations include hypoxemia, dyspnea cyanosis, hemoptysis and cerebrovascular ischemic events or abscesses. We report a case of an 18 year old female with severe respiratory failure caused by a relapse of multiple unilateral pulmonary arterovenous fistula. Symptoms at admission include dyspnea, cyanosis and clubbing. The patient underwent pulmonary angio-TC scan, brain CT and echocardiography. The thoracic angio-CT scan showed the presence of PAVMs of RUL and RLL; a marked increase of right bronchial artery caliber and its branches with an aneurismatic dilatation was also observed. The patient underwent percutaneous transcatheter embolization using Amplatzer Vascular Plug IV; a relevant clinical and functional improvement was subsequently recorded. Embolization is effective in the treatment of relapsing PAVMS.


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