Reliability of Contrast Echocardiography to Rule Out Pulmonary Arteriovenous Malformations and Avoid CT Irradiation in Pediatric Patients with Hereditary Hemorrhagic Telangiectasia

2014 ◽  
Vol 32 (1) ◽  
pp. 42-48 ◽  
Author(s):  
Carma Karam ◽  
Jacques Sellier ◽  
Nicolas Mansencal ◽  
Carole Fagnou ◽  
Sandra Blivet ◽  
...  
Keyword(s):  
Hereditary Hemorrhagic Telangiectasia ◽  
Arteriovenous Malformations ◽  
Pediatric Patients ◽  
Contrast Echocardiography ◽  
Pulmonary Arteriovenous Malformations ◽  
Rule Out

scholarly journals Pulmonary arteriovenous malformations in children with hereditary hemorrhagic telangiectasia: a longitudinal study

2018 ◽  
Vol 8 (3) ◽  
pp. 204589401878669 ◽  
Author(s):  
Katie L. Mowers ◽  
Lynn Sekarski ◽  
Andrew J. White ◽  
R. Mark Grady
Keyword(s):  
Longitudinal Study ◽  
Hereditary Hemorrhagic Telangiectasia ◽  
Arteriovenous Malformations ◽  
Clinical Symptoms ◽  
Contrast Echocardiography ◽  
Genetic Diagnosis ◽  
Pulmonary Arteriovenous Malformations ◽  
Initial Screening ◽  
And Gender ◽  
Transthoracic Contrast Echocardiography

Pulmonary arteriovenous malformations (PAVMs) often occur in children with hereditary hemorrhagic telangiectasia (HHT). A 14-year longitudinal study of PAVMs in children with HHT was undertaken to assess the prevalence, the clinical impact, and progression of these malformations. This was a retrospective, single-center study from May 2002 to December 2016 of 129 children with HHT diagnosed using Curacao criteria and/or confirmed by genetic testing. Transthoracic contrast echocardiography (TTCE) was the primary screening modality in all patients and PAVMs were diagnosed based on Barzilai criteria. Moderately positive TTCE (Barzilai criteria ≥ 2) was confirmed with subsequent contrast chest CT. New PAVMs were diagnosed with a positive TTCE after an initial negative TTCE. Embolization of PAVMs were performed according to HHT consensus guidelines. Of 129 children with HHT, 76 (59%) were found to have PAVMs. Sixty-seven (88%) were positive for PAVMs on initial screening. Of 63 children without PAVMs on initial screening, 31 were followed for >1 year. Nine of the 31 (29%) developed new PAVMs after initial negative study. Thirty-eight (50%) of the total 76 children with PAVMs had or developed lesions large enough to be treated with embolization. Nine patients with PAVMs initially too small to be treated with embolization, developed progression of disease and ultimately were treated with embolization over time. The majority, 60% (23/38), of the children with large PAVMs had no related clinical symptoms. After embolization, 21% (8/38), of patients underwent repeat interventions. Genetic diagnosis, age, and gender were not associated with risk of having PAVM nor with need for repeat interventions. Nearly 60% of children with HHT develop PAVMs. The risk for new PAVMs to develop, small PAVMs to become large, and previously embolized PAVMs to require further intervention remains throughout childhood. Thus, children with HHT require continued follow-up until adulthood.

scholarly journals Transthoracic contrast echocardiography for detection of pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia: How can the process be improved for patient safety?

2014 ◽  
Vol 1 (2) ◽  
pp. 18
Author(s):  
Xiangning Fan ◽  
Jonathan Choy ◽  
Miriam Shanks ◽  
Dilini P. Vethanayagam
Keyword(s):  
Hereditary Hemorrhagic Telangiectasia ◽  
Arteriovenous Malformations ◽  
Best Practice ◽  
Contrast Echocardiography ◽  
Air Embolism ◽  
Pulmonary Arteriovenous Malformations ◽  
Echocardiography Laboratory ◽  
Systemic Air Embolism ◽  
Ischemic Attack ◽  
Transthoracic Contrast Echocardiography

Introduction: Patients with hereditary hemorrhagic telangiectasia (HHT) are at risk for pulmonary arteriovenous malformations (PAVMs). Extracardiac right-to-left shunts due to PAVMs can lead to systemic emboli; clinical sequelae including strokes and brain abscesses. Regular screening is required, with appropriate medical/interventional management to alleviate morbidity and mortality from PAVMs.  International HHT guidelines recommend standardized transthoracic contrast echocardiogram (TTCE) with agitated saline (in an HHT Foundation-accredited laboratory) as the initial screening test, and, when needed, non-contrast computerized tomography of the chest (CT Chest) to follow up on a positive TTCE. Case presentation: A 40-year-old woman was seen for evaluation of HHT and referred for TTCE as part of her PAVM screen. Following administration of agitated saline, she developed a headache, visual changes in her right eye, and right arm weakness, followed by posturing and stiffness in her right arm. Her symptoms resolved after five minutes, consistent with a transient ischemic attack (TIA). Clinical impact: Protocols at our institution were amended following this index case and echocardiography laboratory protocols were adjusted to use a maximum of 0.1 cc air to 9.9 cc saline for TTCE studies in the HHT population. Only the attending echocardiologist who is familiar with possible systemic air embolism during bubble contrast echocardiography in patients with HHT should perform agitation and infusion of contrast and should directly review the images for appropriate visualization during the exam for this test to be appropriately interpreted. Paradoxical emboli due to right-to-left shunt are important to recognize. Best practice for PAVM screening in HHT patients includes use of standardized protocols for the performance and reporting of TTCE in the evaluation of pulmonary shunts.

Hereditary Hemorrhagic Telangiectasia (HHT)

2010 ◽  
pp. 204-207
Author(s):  
Edward C. Rosenow
Keyword(s):  
Hereditary Hemorrhagic Telangiectasia ◽  
Arteriovenous Malformations ◽  
Pulmonary Arteriovenous Malformations

• 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...

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