scholarly journals Applicability of Transthoracic Contrast Echocardiography for the Diagnosis and Treatment of Idiopathic Pulmonary Arteriovenous Malformations

2021 ◽  
Vol 8 ◽  
Author(s):  
Yujiao Deng ◽  
Xin Huang ◽  
Guangyi Wang ◽  
Jian Cao ◽  
Shengshu Wang ◽  
...  
Keyword(s):  
Blood Test ◽  
Arteriovenous Malformations ◽  
Contrast Echocardiography ◽  
Pulmonary Angiography ◽  
Observer Agreement ◽  
Pulmonary Arteriovenous Malformations ◽  
Digital Subtraction ◽  
Pulmonary Shunt ◽  
Grade 3 ◽  
Transthoracic Contrast Echocardiography

Objective: To explore the preferred test to screen for pulmonary arteriovenous malformations (PAVMs) and to predict the probability of interventional embolization.Methods: We performed a retrospective observational study evaluating patients with idiopathic PAVMs from 2009 to 2019. After clinical evaluation, a total of 105 patients were studied, including 71 patients with positive digital subtraction pulmonary angiography (DSPA) findings and 34 with negative DSPA findings. The following patient data were assessed: blood test, chest radiograph, transthoracic contrast echocardiography (TTCE), and DSPA findings.Results: The majority of patients with idiopathic PAVMs were female (66.2% with positive DSPA findings). We found a good κ-coefficient of 0.77 with strong consistency for inter observer agreement concerning the pulmonary right-to-left shunt (RLS) grade on TTCE, which was superior to conventional chest radiographs. The positive predictive value (PPV) of the radiographic features for PAVMs on DSPA was 0.83 (95% CI 0.64–1.0) and 0.44 for the possibility of embolization (95% CI 0.19–0.70). The PPV of the shunt grade of PAVMs on DSPA was 0.14 (95% CI 0.01–0.29) for grade 1, 0.74 (95% CI 0.60–0.88) for grade 2, and 0.97 (95% CI 0.92–1.0) for grade 3. The PPVs of pulmonary shunt grades 2 and 3 on TTCE for the possibility of embolization for PAVMs were 0.21 (95% CI, 0.05–0.36) and 0.87 (95% CI, 0.79–0.99), respectively.Conclusion: TTCE is the preferred screening test for PAVMs. The pulmonary RLS grade on TTCE not only identifies the likelihood of PAVMs but also predicts the probability for embolization.

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.

Bubble contrast echocardiography in detecting pulmonary arteriovenous malformations after modified Fontan operations

2001 ◽  
Vol 11 (5) ◽  
pp. 505-511 ◽  
Author(s):  
Eva Strömvall Larsson ◽  
Laszlo Solymar ◽  
Bengt O. Eriksson ◽  
Anne de Wahl Granelli ◽  
Mats Mellander
Keyword(s):  
Arteriovenous Malformations ◽  
Contrast Echocardiography ◽  
Arterial Oxygen Saturation ◽  
Pulmonary Arteries ◽  
Positive Contrast ◽  
Pulmonary Angiography ◽  
Arterial Oxygen ◽  
Colloid Solution ◽  
Pulmonary Arteriovenous Malformations ◽  
The Right

The development of pulmonary arteriovenous malformations is a well-known complication after Fontan operations, and may result in significant morbidity due to increasing arterial desaturation. We compared the use of bubble contrast echocardiography and pulmonary angiography in detecting such malformations. We also examined which anatomical and haemodynamic variables were associated with their development. Our study includes 20 patients who had undergone modified Fontan procedures, 10 with atriopulmonary and 10 with total cavopulmonary connections, in Gothenburg between 1980 and 1991. All patients underwent cardiac catheterisation and pulmonary angiography. Bubble contrast echocardiography was performed at the same time, with injection of agitated polygelin colloid solution (Haemaccel, Hoechst) into the right and left pulmonary arteries, respectively. Transoesophageal echocardiography was used to detect the appearance of bubble contrast in the pulmonary venous atrium. The aim was also to evaluate the role of hepatic venous blood. Of the 20 patients, 9 (45%) had a positive contrast echocardiography study, compared with only 2 (10%) detected by pulmonary angiography. Patients with positive contrast echocardiography had a significantly lower arterial oxygen saturation than those with negative studies, both at rest (88% vs 95%, p < 0.01) and during exercise testing (78% vs 89%, p = 0.01). Bubble contrast echocardiography is much more sensitive in detecting pulmonary arteriovenous malformations than pulmonary angiography. By injecting echo contrast into the right and left pulmonary arteries, the method can be made highly selective. Pulmonary arteriovenous malformations develop much more frequently in patients with the Fontan circulation than previously reported.

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.

scholarly journals Comparison of Contrast Enhanced Magnetic Resonance Angiography to Computed Tomography in Detecting Pulmonary Arteriovenous Malformations

2020 ◽  
Vol 9 (11) ◽  
pp. 3662
Author(s):  
Daniel A.F. Van den Heuvel ◽  
Marco C. Post ◽  
Ward Koot ◽  
Johannes C. Kelder ◽  
Hendrik W. Van Es ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Magnetic Resonance ◽  
Arteriovenous Malformations ◽  
Imaging Modality ◽  
Contrast Echocardiography ◽  
Pulmonary Arteriovenous Malformations ◽  
Reference Standard ◽  
Contrast Enhanced ◽  
Transthoracic Contrast Echocardiography ◽  
Sensitivity Specificity

Background: Computed tomography (CT) is considered the imaging modality of choice to diagnose pulmonary arteriovenous malformations PAVMs. The drawback of this technique is that it requires ionizing radiation. Magnetic resonance (MR) imaging does not have the limitation, but little is known about the performance of MR compared to CT for the detection of PAVMs. The aim of this study is to investigate the sensitivity of contrast-enhanced MR angiography (CE-MRA) in the detection of PAVMs with feeding artery diameters (FAD) > 2 mm. Methods: Patients with a grade 2 or 3 shunt on screening transthoracic contrast echocardiography (TTCE) were asked to participate. Included patients underwent chest CT and CE-MRA. CT was considered the reference standard. CT and CE-MRA scans were anonymized and assessed for the presence of PAVMs with FAD > 2 mm by one and two readers respectively. Data analysis was performed on per patient and per PAVM basis. Results: Fifty-three patients were included. 105 PAVMs were detected on CT, 45 with a FAD ≥ 2 mm. In per patient analysis, sensitivity and specificity of CE-MRA were 92% and 97% respectively for reader 1 and 92% and 62% for reader 2. Negative and positive predictive value (NPV/PPV) were 93% and 96% for R1 and 90% and 67% for R2. In per PAVM analysis, sensitivity, specificity, NPV and PPV were 96%, 99%, 100% and 86% for R1 and 93%, 96%, 100% and 56% for R2, respectively. Conclusions: CE-MRA has excellent sensitivity and NPV for detection of PAVMs with FAD ≥ 2 mm and can therefore be used to detect these PAVMs. We are hopeful that future advancements in CE-MRA technology will reduce false positive rates and allow for more broad use of CE-MRA in PAVM diagnosis and management.

scholarly journals Follow-up of pulmonary right-to-left shunt in hereditary haemorrhagic telangiectasia

2016 ◽  
Vol 47 (6) ◽  
pp. 1750-1757 ◽  
Author(s):  
Veronique M.M. Vorselaars ◽  
Sebastiaan Velthuis ◽  
Repke J. Snijder ◽  
Cornelis J.J. Westermann ◽  
Jan A. Vos ◽  
...  
Keyword(s):  
Arteriovenous Malformations ◽  
Contrast Echocardiography ◽  
Neurological Complications ◽  
Hereditary Haemorrhagic Telangiectasia ◽  
Pulmonary Arteriovenous Malformations ◽  
Initial Screening ◽  
History Of ◽  
Transthoracic Contrast Echocardiography

Pulmonary arteriovenous malformations (PAVMs) are associated with severe neurological complications in hereditary haemorrhagic telangiectasia (HHT). Transthoracic contrast echocardiography (TTCE) is recommended for screening of pulmonary right-to-left shunts (RLS). Although growth of PAVMs is shown in two small studies, no studies on follow-up with TTCE exist.All HHT patients underwent a second TTCE 5 years after initial screening. Patients with a history of PAVM embolisation were excluded. Pulmonary RLS grade on TTCE after 5 years was compared to the grade at screening.200 patients (53.5% female, mean±sd age at screening 44.7±14.1 years) were included. Increase in RLS grade occurred in 36 (18%) patients, of whom six (17%) underwent embolisation. The change in grade between screening and follow-up was not more than one grade. Of patients with nontreatable pulmonary RLS at screening (n=113), 14 (12.4%) underwent embolisation. In patients without pulmonary RLS at initial screening (n=87), no treatable PAVMs developed during follow-up.Within 5 years, no treatable PAVMs developed in HHT patients without pulmonary RLS at initial screening. Increase in pulmonary RLS grade occurred in 18% of patients, and never increased by more than one grade. Of patients with nontreatable pulmonary RLS at initial screening, 12% underwent embolisation.

Rat model of pulmonary arteriovenous malformations after right superior cavopulmonary anastomosis

2002 ◽  
Vol 283 (5) ◽  
pp. H2151-H2156 ◽  
Author(s):  
Sandra L. Starnes ◽  
Brian W. Duncan ◽  
Charles H. Fraga ◽  
Shailesh Y. Desai ◽  
Thomas K. Jones ◽  
...  
Keyword(s):  
Von Willebrand Factor ◽  
Rat Model ◽  
Microvessel Density ◽  
Arteriovenous Malformations ◽  
Pulmonary Angiography ◽  
Time Dependent ◽  
Pulmonary Arteriovenous Malformations ◽  
Cavopulmonary Anastomosis ◽  
Von Willebrand ◽  
Willebrand Factor

We developed a rat model of pulmonary arteriovenous malformations after cavopulmonary anastomosis. We sought to determine whether this model reproduces the angiographic and histologic features seen in the human condition. Eight Sprague-Dawley rats underwent a right superior cavopulmonary anastomosis with the use of microsurgical techniques. Between 2 and 13 mo, pulmonary angiography was performed, the animals were euthanized, and the lungs were removed. Microscopic sections of the lung were stained with an endothelial-specific antibody (von Willebrand factor). Microvessel density was determined by counting vessels staining positively for von Willebrand factor, and the shunted and nonshunted (control) lungs were compared for each animal. Pulmonary angiography revealed time-dependent development of arteriovenous malformations. Microvessel density demonstrated a time-dependent increase in the shunted lung compared with the control lung (simple linear regression of the ratio of the microvessel density of the shunted lung divided by the microvessel density of the control lung on time; R 2 = 0.79, P = 0.003). This animal model reproduces the same angiographic and microscopic features of pulmonary arteriovenous malformations that develop in humans after cavopulmonary anastomosis. This appears to be a valid model that may be used to further study etiologic mechanisms for this phenomenon.

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