Two-dimensional contrast echocardiography in the detection and follow-up of congenital pulmonary arteriovenous malformations

Barzilai, Benico; Waggoner, Alan D.; Spessert, Cheryl; Picus, Daniel; Goodenberger, Daniel M.

doi:10.1016/0002-9149(91)90287-u

Cited by 170 publications

(109 citation statements)

References 7 publications

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“…It is also useful to detect unusual venous connections, such as a vena cava to the left atrium, 313,314 diagnose the presence of a persistent left superior vena cava, 313 establish shunt level (eg, atrial versus ventricular), identify coronary sinus ASDs, 315 and establish whether late-onset cyanosis in the adult is attributable to a cardiac or intrapulmonary shunt. 316 In Glenn shunt and Fontan patients with cyanosis, saline contrast can identify the presence of acquired intrapulmonary shunts caused by venous collaterals or AV malformations, the latter often associated with lack of hepatic blood flow to 1 lung.…”

Section: Strain Imagingmentioning

confidence: 99%

Congenital Heart Disease in the Older Adult

Bhatt¹,

Foster²,

Kuehl³

et al. 2015

Circulation

205

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Section: Strain Imagingmentioning

confidence: 99%

Congenital Heart Disease in the Older Adult

Bhatt¹,

Foster²,

Kuehl³

et al. 2015

Circulation

205

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“…Although there might be concern about complications resulting from paradoxical cerebral air embolism, side-effects of TTCE appear to be rare [20]. Its sensitivity for detecting PAVMs has proven excellent in mainly retrospective studies [4,[21][22][23][24]. The aim of the present prospective study is to establish the role of TTCE in screening for PAVM compared with chest CT as gold standard.…”

Section: Screening For Pavmsmentioning

confidence: 95%

Screening for pulmonary arteriovenous malformations using transthoracic contrast echocardiography: a prospective study

Gent

Post²,

Luermans³

et al. 2008

Eur Respir J

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Pulmonary arteriovenous malformations (PAVMs) are associated with severe neurological complications in patients with hereditary haemorrhagic telangiectasia (HHT). The objective of the present study was to prospectively establish the diagnostic value of transthoracic contrast echocardiography (TTCE) as a screening technique for PAVM using chest highresolution computed tomography (HRCT) as the gold standard for PAVMs.All consecutive adult patients referred for HHT screening underwent a chest HRCT (n5299), TTCE (n5281), arterial blood gas analysis (n5291), shunt fraction measurement (n5111) and chest radiography (n5296).TTCE was positive in 87 (58.8%), 12 (16.7%) and four (6.7%) patients, and chest HRCT was positive in 54 (36.5%), three (4.2%) and zero (0%) patients with a definite, possible and negative clinical diagnosis of HHT, respectively. Two patients with a negative TTCE were diagnosed with PAVMs after computed tomography; in both cases the PAVMs were too small to be treated by embolotherapy. The sensitivity of TTCE was 97% (95% confidence interval (CI) 93.6-98.3) and negative predictive value 99% (95% CI 96.9-99.8). The other diagnostic tests showed a considerable lower diagnostic value.The present prospective study shows that transthoracic contrast echocardiography has an excellent diagnostic value and can be used as an initial screening procedure for pulmonary arteriovenous malformations. The high false-positive rate of transthoracic contrast echocardiography possibly represents microscopic pulmonary arteriovenous malformations.KEYWORDS: Contrast echocardiography, hereditary haemorrhagic telangiectasia, pulmonary arteriovenous malformations A pulmonary arteriovenous malformation (PAVM) is a direct communication between a pulmonary artery and pulmonary vein. A PAVM results in a right-to-left shunt (RLS) causing hypoxaemia and risk of paradoxal embolism through bypassing the filtering capillary network. Complications occurring in patients with PAVM and hereditary haemorrhagic telangiectasia (HHT) are stroke (10-19%), transient ischaemic attacks (6-37%), cerebral abscess (5-9%), migraine headaches, seizures, massive haemoptysis and (spontaneous) haemothorax [1][2][3]. Antibiotic prophylaxis prior to procedures carrying the risk of bacteraemia is recommended [3][4][5]. At least 80% of PAVMs are associated with HHT [3]. HHT is an autosomal dominant disorder characterised by vascular abnormalities varying from small telangiectases to large arteriovenous malformations. The clinical diagnosis is based on the Curaçao criteria [6]. Based on genetic analysis, HHT is divided into types 1 and 2, corresponding with mutations in the genes ENG and ACVRL1, coding for endoglin and activin A receptor type-like kinase 1, respectively [7,8]. The prevalence of PAVM, as documented by chest high-resolution computed tomography (HRCT), in all HHT patients is 20-45% [4,9]. The PAVM prevalence differs between these two subtypes, in HHT type 1 the prevalence is 48%, whereas the prevalence in HHT type 2 is 5% [10]. PAVMs can...

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“…Contrast echocardiography requires an injection of agitated saline into a peripheral vein. In healthy individuals, the created bubbles are trapped in the lungs-whereas in patients with PAVFs, the bubbles are immediately seen in the left atrium via transthoracic echocardiography [14]. Radionuclide imaging can also be employed to measure the shunt fraction.…”

Section: Discussionmentioning

confidence: 99%

Multiple Pulmonary Arteriovenous Fistula in a Mother and His Son: A Case Report

Firouzi¹,

Behrooj²,

Pirborj³

et al. 2017

J Cardiovasc Dis Diagn

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Multiple Pulmonary Arteriovenous Fistulas (PAVFs) are rare vascular abnormalities in the general population, with a report on 15000 autopsies revealing only 3 cases of multiple PAVFs. However, these abnormal communications between pulmonary arteries and veins are commonly seen in patients with Hereditary Hemorrhagic Telangiectasia (HHT). Several conditions such as cirrhosis, chest trauma, metastatic carcinoma, schistosomiasis, and systemic amyloidosis are accountable for acquired form PAVF. We describe 2 patients (mother and son) with multiple PAVFs and no apparent signs or symptoms suggestive of HHT. A pulmonary artery injection wes performed for Case 1 (mother) and showed bilateral PAVFs with aneurysmal dilation. Given the diagnosis of PAVFs in the mother, we considered the same pathology for the child's symptoms. Therefore, the boy also underwent pulmonary angiography, which revealed multiple bilateral PAVFs. Both patients underwent interventional angiography and the PAVFs were occulted with stainless coil. In conclusion, the presence of multiple PAVFs should be considered in the differential diagnosis of patients with unexplained exertional dyspnea and cyanosis, even in the absence of diagnostic features suggestive of HHT.

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Two-dimensional contrast echocardiography in the detection and follow-up of congenital pulmonary arteriovenous malformations

Cited by 170 publications

References 7 publications

Congenital Heart Disease in the Older Adult

Congenital Heart Disease in the Older Adult

Screening for pulmonary arteriovenous malformations using transthoracic contrast echocardiography: a prospective study

Multiple Pulmonary Arteriovenous Fistula in a Mother and His Son: A Case Report

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