A case of pulmonary arteriovenous malformation in the setting of Rendu Osler Weber syndrome

Nikolaou, Irini; Rafailidis, Vasileios; Κάρτας, Αναστάσιος; Κούσκουρας, Κωνσταντίνος; Γιαννακούλας, Γεώργιος

doi:10.1016/j.radcr.2020.12.024

Cited by 3 publications

(3 citation statements)

References 14 publications

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“…The results of routine blood tests were normal. A genetic study could not be performed to exclude Osler-Weber-Rendu syndrome, which is also known as hereditary hemorrhagic telangiectasia[ 4 , 5 ].…”

Section: Case Presentationmentioning

confidence: 99%

“…The first case of pulmonary artery-to-left atrial fistula was reported in 1989 as an unusual cause of cyanosis in a newborn[ 2 ]. A significant right-to-left shunt is clinically marked by effort intolerance, cyanosis, and polycythemia, as well as sometimes bleeding issues related to associated hemangioma[ 3 , 4 ]. Pulmonary artery-to-left atrial fistula is a variant of pulmonary arteriovenous malformation.…”

Section: Introductionmentioning

confidence: 99%

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Device closure of fistula from lower left pulmonary artery to left atrium using a vascular plug: A case report

Mahapatra

Mahanta

Singh

et al. 2021

WJC

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BACKGROUND Pulmonary artery-to-left atrial fistula is a variant of pulmonary arteriovenous fistula and is a developmental anomaly. Delayed presentation, cyanosis and effort intolerance are some of the important features. The diagnosis is confirmed by computed tomography or pulmonary artery angiography. Catheter-based closure is preferred to surgery. CASE SUMMARY Left pulmonary artery-to-left atrial fistula is rare. A 40-year-old male presented with effort intolerance, central cyanosis, and recurrent seizures. He had a large and highly tortuous left pulmonary artery-to-left atrial fistula associated with a large aneurysmal sac in the course. Catheter-based closure was performed using a vascular plug. CONCLUSION Left pulmonary artery-to-left atrial fistula is relatively uncommon compared to right pulmonary artery-to-left atrial fistula. Percutaneous closure by either a transeptal technique or guide wire insertion into the pulmonary vein through the pulmonary artery is preferred. The need for an arteriovenous loop depends on the tortuosity of the course of the fistula and the size of the device to be implanted because a larger device needs a larger sheath, necessitating firm guide wire support to facilitate negotiation of the stiff combination of the delivery sheath and dilator.

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Section: Case Presentationmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Device closure of fistula from lower left pulmonary artery to left atrium using a vascular plug: A case report

Mahapatra

Mahanta

Singh

et al. 2021

WJC

View full text Add to dashboard Cite

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“…It is characterized by recurrent epistaxis, telangiectasias, and multiple arteriovenous malformations (AVMs) involving different body systems. [ 1 ] These vascular malformations are genetic angiodysplasia that is fragile and prone to thrombosis and bleeding. Because of the frequent epistaxis and nostril manipulation, these patients are prone to catching COVID-19 infection.…”

Section: Introductionmentioning

confidence: 99%

A case report on uneventful anticoagulation and persistence of Type 1 Respiratory Failure post severe COVID-19 infection in a patient of Osler–Weber–Rendu syndrome

Daga

Gautam

Mawari

et al. 2022

Journal of Family Medicine and Primary Care

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A BSTRACT The SARS-CoV-2 pandemic has led researchers worldwide to study the patterns of association of SARS-CoV-2 with different diseases, which have been a prime focus of medical literature. Osler–Weber–Rendu syndrome, also known as hereditary hemorrhagic telangiectasia (HHT), is a rare genetic disorder in which patients present with recurrent epistaxis, nostril manipulations, and multiple arteriovenous malformations (AVMs) along with telangiectasias involving internal organs and mucocutaneous areas. In addition, these AVMs are prone to bleeding or act as a nidus for thrombus formation, apart from other serious complications, such as chronic hypoxemia, anemia, pulmonary artery hypertension, heart failure, and cerebrovascular accidents. Here, we present a case report of a patient who presented with acute onset respiratory complaints, had multiple episodes of epistaxis in the past, and was later diagnosed with HHT as per Curaçao criteria in our hospital. Doppler ultrasound over the left calf region showed an AVM. Contrast-enhanced computed tomography (CECT) angiography of the chest and abdomen revealed multiple pulmonary and hepatic AVMs along with splenic and uterine telangiectasias and malformations, who on acquiring severe COVID-19 infection developed complications such as anemia, pulmonary artery hypertension, sepsis, acute kidney injury, and post-COVID-19 persistence of Type 1 respiratory failure. Furthermore, the risk-benefit ratio of anticoagulation therapy in such patients with COVID-19 infection is tricky and challenging. However, our patient was prophylactically anti-coagulated with enoxaparin for 12 days with an uneventful outcome.

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Pulmonary arteriovenous malformation in a case of hereditary haemorrhagic telangiectasia

Reddy,

Nethaji,

Kumar

et al. 2023

Journal of Clinical and Scientific Research

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Pulmonary arteriovenous malformations (PAVMs) are one of the most common serious complications of hereditary haemorrhagic telangiectasia (HHT). We present the case of a 63-year-old woman who presented with respiratory distress, pedal oedema and palpitations. She had a history of recurrent epistaxis and frequent blood transfusions. Family history of telangiectasias over oral and nasal mucosa is present. At the time of presentation, the patient had hypoxia, maintaining oxygen saturation 88% on pulse oximetry while breathing ambient air. Examination revealed decreased breath sounds in the right lung fields. Computed tomography pulmonary angiography showed a ruptured PAVM with haemothorax. The present case highlights the occurrence of a PAVM in a case of HHT.

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A case of pulmonary arteriovenous malformation in the setting of Rendu Osler Weber syndrome

Cited by 3 publications

References 14 publications

Device closure of fistula from lower left pulmonary artery to left atrium using a vascular plug: A case report

Device closure of fistula from lower left pulmonary artery to left atrium using a vascular plug: A case report

A case report on uneventful anticoagulation and persistence of Type 1 Respiratory Failure post severe COVID-19 infection in a patient of Osler–Weber–Rendu syndrome

Pulmonary arteriovenous malformation in a case of hereditary haemorrhagic telangiectasia

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